Pleurisy – inflammation of pleura, usually producing an exudative pleural effusion and stabbing chest pain worsened by respiration and cough.
Pleurisy may result from an underlying lung process (eg, pneumonia, infarction, irritating substance into the pleural space (eg, with a ruptured esophagus, amebic empyema, or pancreatic pleurisy); transport of an infectious or noxious agent or neoplastic cells to the pleura via the bloodstream or lymphatics; parietal pleural injury (eg, trauma, especially rib fracture, or epidemic pleurodynia /due to coxsackievirus B/); asbestos-related pleural disease in which asbestos particles reach the pleura by traversing the conducting airways and respiratory tissues; or, rarely, pleural effusion related to drug ingestion.
The pleura usually first becomes edematous and congested. Cellular infiltration follows, and fibrinous exudate develops on the pleural surface. The exudate may be reabsorbed or organized into fibrous tissue resulting in pleural adhesions. In sme diseases (eg, epidemic pleurodynia), the pleurisy remains dry or fibrinous, with no significant exudaiton of fluid from the inflamed pleura. More often, pleural exudate develops from an outpouring of fluid rich in plasma proteins from damaged capillaries. Occasionally, marked fibrous or even calcific thickening of pleura (eg, asbestos pleural plaques, idiopathic pleural calcification) develops without an antecedent acute pleurisy.
Symptoms and Signs
Sudden pain is the dominant symptom of pleurisy. Typically, pleuritic pain is a stubbing sensation aggravated by breathing and coughing, but it can vary. It may only a vague discomfort, or it may occur only when the patient breathes deeply or coughs. The visceral pleura is insensitive; pain results from inflammation of parietal pleura, which is mainly innervated by intercostal nerves. Pain is usually felt over the pleuritic site but may be referred to distant regions. Irritation of posterior and peripheral portioms of the diaphragmatic pleura, which are supplied by the lower six intercostal nerves, may cause pain referred to the lower chest wall or abdomen and may simulate intra-abdominal disease. Irritation of the central portion of the diaphragmatic pleura, innervated by the phrenic nerves, causes pain referred to the neck and shoulder.
Respiration is usually rapid and shallow. Motion of the affected side may be limited. Breath sounds may be diminished. A pleural friction rub, although infrequent, is the characteristic physical sign.It may not be accompanied by pleuritic pain, but it usually is. The friction rub varies from a few intermittent sounds that may simulate crackles to a fully developed harsh grating, creaking, or leathery sound synchronous with respiration, heard on inspiration and expiration. Friction sounds due to pleuritis adjacent to the heart (pleuropericardial rub) may vary with the heart beat as well.
When pleural effusion develops, pleuritic pain usually subsides. Percussion dullness, absent tactile fremitus, decreased or absent breath sounds, and egophony at the upper border of the fluid are then noticeable. The larger the effusion, the more obvious the above signs. A large effusion may produce or contribute to dyspnea through diminished lung volume, especially if there is underlying pulmonary disease, mediastinal shift to the contralateral side, and diminished function and recruitment of inspiratory muscles due to an expanded thoracic cage.
Pleurisy is readily diagnosed when characteristic pleuritic pain occurs. A pleural friction rub is pathognomonic. Pleurisy that produces referred abdominal pain is usually differentiated from acute inflammatory abdominal disease by x-ray and clinical evidence of a respiratory process; absence of nausea, vomiting, and disturbed bowel function; marked aggravation of pain by deep breathing or coughing; shallow rapid breathing; and a tendency toward relief of pain by pressure on the chest wall or abdomen. Intercostal neuritis may be confused with pleurisy, but the pain is rarely related to respiration and there is no friction rub. With herpetic neuritis, development of the characteristic skin eruption is diagnostic. Miocardial infarction, spontaneous pneumothorax, pericarditis, and chest wall lesions may simulate pleurisy. A plueral friction rub may be confused with a friction rub of pericarditis (pericardial rub), which is heard best over the left border of the sternum in the third and forth interspaces, is characteristically a to-and-fro sound synchronous with the heartbeat, and is not influenced significantly by respiration.
Chest x-rays are of limited value in diagnosing fibrinous pleurisy. The pleural lesion causes no shadow, but an associated pulmonary or chest wall lesion may. The presence of a pleural effusion, generally small, confirms the presence of acute pleurisy
Treatment of the underlying disease is essential.
Chest pain may be relieved by wrapping the entire chest with two or three 6-in-wide nonadhesive elastic bandages, which must be reapplied once or twice daily. Acetaminophen 0.65 g qid or an NSAID is often effective. Oral narcotics may be necessary, but cough suppression may be not desired.
Adequate bronchial drainage must be provided to prevent pneumonia. A patient receiving narcotics should be urged to breathe deeply and cough when pain relief from the drug is maximal. Antibiotics and bronchodilators should be considered for treatment of associated bronchitis.
x-ray and CT Scan findings
Essentials of Diagnosis
• Development of pulmonary symptoms about 1-2 weeks after possible aspiration, bronchial obstruction, or previous pneumonia.
• Septic fever and sweats, and periodic sudden ex pectoration of large amounts of purulent, foul-smelling, or "musty" sputum. Hemoptysis may occur.
• X-ray density with central radiolucency and fluid level.
Lung abscess develops when necrosis and liquefaction occur in an area where necrotizing pneumonia is present (picture 1-3). Symptoms and signs occur 1-2 weeks after the following events: (1) massive-aspira tion of upper respiratory tract secretions and microbial flora, especially during profound suppression of cough reflex (eg, with alcohol, drugs, unconsciousness, anesthesia, brain trauma); (2) bronchial obstruction (eg, by atelectasis, foreign body, neoplasm); (3) pres ence of pneumonias, especially those caused by gram-negative bacteria or staphylococci; or (4) forma tion of septic emboli from other foci of infection, or, during bacteremia, with pulmonary infarcts. Abscess is more commonly in the lower dependent portions of the lung. The main etiologic organisms are related to the underlying condition, but a dense mixed anaerobic flora is often prominent, particularly when aspiration has occurred.
A. Symptoms and Signs: Onset may be abrupt or gradual. Symptoms include septic fever, sweats, cough, and chest pain. Cough is often nonproductive at onset. Expectoration of foul-smelling brown or gray sputum (anaerobic flora) or of purulent sputum without odor (pyogenic organism) may occur abruptly and in large quantity. Blood-streaked sputum is also common.
Pleural pain, especially with coughing, is common because the abscess is often subpleural. Weight loss, anemia, and pulmonary osteoarthropathy may appear when the abscess becomes chronic (8-12 weeks after onset).
Physical findings may be minimal. Consolidation due to pneumonitis surrounding the abscess is the most frequent finding. Rupture into the pleural space produces signs of fluid or pneumothorax.
B. Laboratory Findings: Sputum cultures are usually inadequate in determining the bacterial cause of a lung abscess. Transtracheal aspirates should be obtained with the proper technique employed to cul ture anaerobic organisms in addition to the usual aerobic cultures. Special methods of transporting specimens are required for anaerobic organisms, and appropriate culture media and methods must be employed.
Smear and cultures for the tubercle bacilli are required, especially in lesions of the upper lobe and in chronic abscess.
C. X-Ray Findings: A dense shadow is the initial finding. A central radiolucency, often with a visible fluid level, appears as surrounding densities subside. Computerized tomography can supply the detailed lo calization of the abscess and may also reveal primary lesions (eg, bronchogenic carcinoma) and provide guidance for contemplated surgery. Various x-ray pro cedures also permit localization of pleural involvement to facilitate drainage.
D. Instrumental Examination: Fiberoptic bronchoscopy may help to 'diagnose location and nature of obstructions (foreign body, tumor), obtain specimens for microbiologic and pathologic examina tion, and, occasionally, aid drainage.
Differentiate from other causes of pulmonary cavitation: tuberculosis, bronchogenic carcinoma, mycotic infections, and staphylococcal or gram-nega tive bacterial pneumonia.
Postural drainage and bronchoscopy are impor tant to promote drainage of secretions.
A. Acute Abscess: Intensive antibacterial ther apy is necessary to prevent further destruction of lung tissue. While cultures and sensitivity tests are pending, treatment should be started with penicillin G, 2-6 million units daily. In penicillin hypersensitivity clindamycin and chloramphenicol are alternatives. If the patient improves on antimicrobial drugs (and postural drainage),
the drugs should be continued for 4—8 weeks. If the patient fails to respond significantly to the initial treatment, laboratory results may suggest other antimicrobials, eg, nafcillin for staphylococci, cefotaxime for Klebsiella, cefoxitin or metronidazole for mixed anaerobes. Postural drainage is important adjunctive treatment. Percutaneous catheter drainage has been used successfully in selected cases. Surgical therapy is indicated mainly for severe hemoptysis and for me infrequent abscesses that fail to respond to antimicrobial management. Failure of fever to subside after 2 weeks of therapy, abscess diameter of more than 6 cm, and very thick cavity waits are all factors that lessen the likelihood of success with nonsurgical treatment alone.
B. Chronic Abscess: After acute systemic man ifestations have subsided, the abscess may persist. Although many patients with chronic lung abscess can be cured with long-term treatment with antibacterial agents, surgery may occasionally be required.
Rupture of pus into the pleural space (empyema) causes severe symptoms: increase in fever, marked pleural pain, and sweating; the patient becomes “tox ic" in appearance. Adequate drainage of empyema is mandatory. In chronic abscess, severe and even fatal hemorrhage may occur. Metastatic brain abscess is a well-recognized complication, and the infection may seed other organ sites. Bronchiectasis may occur as a sequela to lung abscess even when the abscess itself is cured.
The prognosis in acute abscess is excellent with prompt and intensive antibiotic therapy. About 80% of patients are healed within 7-8 weeks. The incidenee of chronic abscess is consequently low; Inchronic cases, surgery is curative.
Essentials of Diagnosis:
• Chronic cough with expectoration of large amounts of purulent sputum; hemoptysis.
• Rales and rhonchi over lower lobes.
• X-ray of chest reveals little; bronchograms show characteristic dilatations.
Bronchiectasis is a dilatation of small and medium-sized bronchi resulting from destruction of bronchial elastic and muscular elements. It may be, caused by pulmonary infections (eg, pneumonia, per tussis, tuberculosis) or by a bronchial obstruction (eg, foreign bodies or extrinsic pressure). In many patients, a history of onset following one or more episodes of pulmonary infection, usually in early childhood, is obtained. However,since infection does not regularly produce significant bronchiectasis, unknown intrinsic host factors presumably are present. The incidence of
the disease has been reduced by treating pulmonary infections with antibiotics.
A. Symptoms and Signs: Most patients with bronchiectasis have a history of chronic cough with expectoration of large volumes of sputum, especially upon awakening. The sputum has a characteristic qual ity of "layering out" into 3 layers upon standing, a frothy top layer, a middle clear layer, and a dense particulate bottom layer. It is usually purulent in ap pearance and foul-smelling.
Intermittent hemoptysis, occasionally in dangerous proportions, is often combined with intercurrent respiratory infections. Symptoms occur most often in patients with idiopathic bronchiectasis (ie, childhood respiratory infections). However, patients who have bronchiectasis secondary either to tuberculosis or chronic obstruction may not exhibit characteristic symptoms. Idiopathic bronchiectasis occurs most fre quently in the middle and lower lobes and posttuberculous bronchiectasis in the upper lobes. Hemoptysis is thought to result from erosion of bronchiolar mucosa with resultant destruction of un derlying blood vessels. Pulmonary insufficiency may result from progressive destruction of pulmonary tis sue.
Physical findings consist primarily of rales and rhonchi over the affected segments. If the condition is far-advanced, emaciation, cyanosis, and digital club bing may appear.
B. Laboratory Findings: There are no charac teristic laboratory findings. If hypoxemia is chronic and severe, secondary polycythemia may develop. There may be either restrictive or obstructive pulmo nary function defects associated with bronchiectasis. Hypoxemia and hypocapnia or hypercapnia may also be associated with the disease, depending on the se verity of the underlying condition. .
C.X-Ray Findings (figure 1-6): Plain films of the chest often show increased bronchopulmonary markings in af fected segments; in severe cases there may be areas of radiodensities surrounding portions of radiolucency. Early in the course of bronchiectasis, however, the chest x-ray may be normal.
The differential diagnosis includes other disorders that lead to chronic cough, sputum produc tion, and hemoptysis, ie, chronic bronchitis, tuber culosis, and bronchogenic carcinoma. The diagnosis of bronchiectasis is suggested by the patient's history and can be confirmed only by bronchographic examination or histopathologic examination of surgically removed tissue.
Recurrent infection in poorly drained pulmonary segments leads to chronic suppuration and may cause pulmonary insufficiency. Complications include hemoptysis, respiratory failure, chronic cor pulmonale, and amyloidosis. There is also an increased incidence of brain abscess, which is thought to be secondary to abnormal anastomoses between bron chial (systemic) and pulmonary venous circulation. These anastomoses produce right-to-left shunts and allow for the dissemination of septic emboli.
. A. General Measures and Medical Treatment:
1. Environmental changes- The patient should avoid exposure to all common pulmonary irritants such as smoke, fumes, and dust and should stop smoking cigarettes.
2. Control of bronchial secretions (improved drainage)-
a. Postural drainage often gives effective relief of symptoms and should be utilized in every case. The patient should assume the position that gives maximum drainage, usually lying on a bed in the prone, supine, or right or left lateral decubitus position with the hips elevated on several pillows and no pillow under the head. Any effective position should be main tained for 10 minutes, 2-4 times a day. The first drainage should be done upon awakening and tee last drainage at bedtime. Family members can be trained in the art of chest percussion to facilitate drainage of secretions.
b. Liquefaction of thick sputum may be pro moted by inhaling warm mists and, in some cases, mucolytic agents such as acetylcysteine or 5% sodium bicarbonate given by aerosol may also be helpful.
3. Control of respiratory infection-Exposure to respiratory infections should be minimized and the patient should be vaccinated against influenza and pneumococcal pneumonia. Antibiotic therapy is indicated for acute exacerbations (ie, increased production of purulent sputum, hemoptysis, etc). Long-term or prophylactic antibiotic therapy is controversial, since it has not been conclusively shown to be of lasting benefit. Therefore, it seems rational to treat acute exacerbations in order to control infection but mini mize the emergence of resistant strains. Because the bacteria most commonly involved are H inftuenzae and S pneumoniae, the drug most commonly employed is ampicillin, 250-500 mg orally every 6 hours for 5 days. Alternative therapies for the penicillin-allergic patient are erythromycin, given in the same dosage schedule as ampicillin, or trimethoprim-sulfamethoxazole, 2 double-strength tablets twice a day for 5 days.
B. Surgical Treatment: Surgical treatment is most often employed when hemoptysis with bron-chiectasis is recurrent and severe. Despite antibiotic therapy, localized bronchiectasis (eg, in a lower lobe or segment) with progressive uncontrolled infection and sputum production may be an indication for surgical removal of the affected segments.
Bronchiectasis is also associated with mucoviscidosis. It is thought to be secondary to the thick viscid secretions that cannot be cleared by normal cough mechanisms and that lead to stasis of.sputum and chronic infection. This disorder, usually associated with sinusitis, may be accompanied by other manifes tations of mucoviscidosis. Its most common organisms are S aureus or Pseudomonas aeruginosa.
Bronchiectasis is also associated with certain ab normalities of cellular ciliary function, the mostcommon of which is Kartagener's syndrome, a combina tion of sinusitis, situs in versus, andbronchiectasis. Patients with this disorder show immotile cilia second ary to ultrastructural abnormalities, stasis of sputum, failure to clear secretions, and chronic pulmonary infection that results in bronchiectasis.
Antibiotic treatment of mucoviscidosis and Kartagener's syndrome must be guided by sensitivity studies of organisms cultured from sputum.
Iodized contrast media instilled into the bronchial tree (a bronchogram) demonstrates saccular, cylindric, or fusiform dilatation of small and medium bronchi with consequent loss of the normal branching pattern. Cylindric changes of bronchiectasis that may result from acute pneumonia will revert to normal after 6-8 weeks, but saccular dilatations represent long-standing damage and permanent disease.
Patients with Cor Pulmonale
CHRONIC COR PULMONALE
Background: Cor pulmonale is defined as an alteration in the structure and function of the right ventricle caused by a primary disorder of the respiratory system. Pulmonary hypertension is the common link between lung dysfunction and the heart in cor pulmonale. Right-sided ventricular disease caused by a primary abnormality of the left side of the heart or congenital heart disease is not considered cor pulmonale, but cor pulmonale can develop secondary to a wide variety of cardiopulmonary disease processes. Although cor pulmonale commonly has a chronic and slowly progressive course, acute onset or worsening cor pulmonale with life-threatening complications can occur.
Pathophysiology: Several different pathophysiologic mechanisms can lead to pulmonary hypertension and, subsequently, to cor pulmonale. These pathogenetic mechanisms include (1) pulmonary vasoconstriction due to alveolar hypoxia or blood acidemia; (2) anatomic compromise of the pulmonary vascular bed secondary to lung disorders, eg, emphysema, pulmonary thromboembolism, interstitial lung disease; (3) increased blood viscosity secondary to blood disorders, eg, polycythemia vera, sickle cell disease, macroglobulinemia; and (4) idiopathic primary pulmonary hypertension. The result is increased pulmonary arterial pressure.
The right ventricle (RV) is a thin-walled chamber that is more a volume pump than a pressure pump. It adapts better to changing preloads than afterloads. With an increase in afterload, the RV increases systolic pressure to keep the gradient. At a point, further increase in the degree of pulmonary arterial pressure brings significant RV dilation, an increase in RV end-diastolic pressure, and circulatory collapse. A decrease in RV output with a decrease in diastolic left ventricle (LV) volume results in decreased LV output. Since the right coronary artery, which supplies the RV free wall, originates from the aorta, decreased LV output diminishes blood pressure in the aorta and decreases right coronary blood flow. This is a vicious cycle between decreases in LV and RV output.
Right ventricular overload is associated with septal displacement toward the left ventricle. Septal displacement, which is seen in echocardiography, can be another factor that decreases LV volume and output in the setting of cor pulmonale and right ventricular enlargement. Several pulmonary diseases cause cor pulmonale, which may involve interstitial and alveolar tissues with a secondary effect on pulmonary vasculature or may primarily involve pulmonary vasculature. Chronic obstructive pulmonary disease (COPD) is the most common cause of cor pulmonale in the United States.
Cor pulmonale usually presents chronically, but 2 main conditions can cause acute cor pulmonale: massive pulmonary embolism (more common) and acute respiratory distress syndrome (ARDS). The underlying pathophysiology in massive pulmonary embolism causing cor pulmonale is the sudden increase in pulmonary resistance. In ARDS, 2 factors cause RV overload: the pathologic features of the syndrome itself and mechanical ventilation. Mechanical ventilation, especially higher tidal volume, requires a higher transpulmonary pressure. In chronic cor pulmonale, right ventricular hypertrophy (RVH) generally predominates. In acute cor pulmonale, right ventricular dilatation mainly occurs
right ventricular dilatation
- In the US: Cor pulmonale is estimated to account for 6-7% of all types of adult heart disease in the United States, with chronic obstructive pulmonary disease (COPD) due to chronic bronchitis or emphysema the causative factor in more than 50% of cases. Although the prevalence of COPD in the United States is about 15 million, the exact prevalence of cor pulmonale is difficult to determine because it does not occur in all cases of COPD and the physical examination and routine tests are relatively insensitive for the detection of pulmonary hypertension. In contrast, acute cor pulmonale usually is secondary to massive pulmonary embolism. Acute massive pulmonary thromboembolism is the most common cause of acute life-threatening cor pulmonale in adults. In the United States, 50,000 deaths are estimated to occur per year from pulmonary emboli and about half occur within the first hour due to acute right heart failure.
- Internationally: Incidence of cor pulmonale varies among different countries depending on the prevalence of cigarette smoking, air pollution, and other risk factors for various lung diseases.
Mortality/Morbidity: Development of cor pulmonale as a result of a primary pulmonary disease usually heralds a poorer prognosis. For example, patients with COPD who develop cor pulmonale have a 30% chance of surviving 5 years. However, whether cor pulmonale carries an independent prognostic value or it is simply reflecting the severity of underlying COPD or other pulmonary disease is not clear. Prognosis in the acute setting due to massive pulmonary embolism or ARDS has not been shown to be dependent on presence or absence of cor pulmonale.
History: Clinical manifestations of cor pulmonale generally are nonspecific. The symptoms may be subtle, especially in early stages of the disease, and mistakenly may be attributed to the underlying pulmonary pathology.
- The patient may complain of fatigue, tachypnea, exertional dyspnea, and cough.
- Anginal chest pain also can occur and may be due to right ventricular ischemia (it usually does not respond to nitrates) or pulmonary artery stretching.
- Hemoptysis may occur because of rupture of a dilated or atherosclerotic pulmonary artery. Other conditions, such as tumors, bronchiectasis, and pulmonary infarction, should be excluded before attributing hemoptysis to pulmonary hypertension. Rarely, the patient may complain of hoarseness due to compression of the left recurrent laryngeal nerve by a dilated pulmonary artery.
- Variety of neurologic symptoms may be seen due to decreased cardiac output and hypoxemia.
- In advanced stages, passive hepatic congestion secondary to severe right ventricular failure may lead to anorexia, right upper quadrant abdominal discomfort, and jaundice.
- Syncope with exertion, which may be seen in severe disease, reflects a relative inability to increase cardiac output during exercise with a subsequent drop in the systemic arterial pressure.
- Elevated pulmonary artery pressure can lead to elevated right atrial pressure, peripheral venous pressure, and then capillary pressure and by increasing the hydrostatic gradient, it leads to transudation of fluid, which appears as peripheral edema. Although this is the simplest explanation for peripheral edema in cor pulmonale, other hypotheses explain this symptom, especially in a fraction of patients with COPD who do not show increase in right atrial pressure. A decrease in glomerular filtration rate (GFR) and filtration of sodium and stimulation of arginine vasopressin (which decreases free water excretion) due to hypoxemia play important pathophysiologic roles in this setting and may even have a role for peripheral edema in patients with cor pulmonale who have elevated right atrial pressure.
Physical: Physical findings may reflect the underlying lung disease or pulmonary hypertension, RVH, and RV failure.
- On inspection, an increase in chest diameter, labored respiratory efforts with retractions of chest wall, distended neck veins with prominent a or v waves, and cyanosis may be seen.
- On auscultation of the lungs, wheezes and crackles may be heard as signs of underlying lung disease. Turbulent flow through recanalized vessels in chronic thromboembolic pulmonary hypertension may be heard as systolic bruits in the lungs. Splitting of the second heart sound with accentuation of the pulmonic component can be heard in early stages. A systolic ejection murmur with sharp ejection click over the region of the pulmonary artery may be heard in advanced disease, along with a diastolic pulmonary regurgitation murmur. Other findings upon auscultation of the cardiovascular system may be third and fourth sounds of the heart and systolic murmur of tricuspid regurgitation.
- RVH is characterized by a left parasternal or subxiphoid heave. Hepatojugular reflex and pulsatile liver are signs of RV failure with systemic venous congestion.
- On percussion, hyperresonance of the lungs may be a sign of underlying COPD; ascites can be seen in severe disease.
- Disorders with primary involvement of pulmonary vasculature and circulation
- Repeated pulmonary emboli
- Pulmonary vasculitis
- Pulmonary veno-occlusive disease
- Congenital heart disease with left-to-right shunting
- Sickle cell disease
- High altitude disease with pulmonary vasoconstriction
- Primary pulmonary hypertension
- Disorders with secondary involvement of pulmonary vasculature and circulation
- Parenchymal lung diseases (interstitial lung diseases, chronic obstructive lung diseases)
- Neuromuscular disorders (eg, myasthenia gravis, poliomyelitis, amyotrophic lateral sclerosis)
- Obstructive and central sleep apnea
- Thoracic deformities (eg, kyphoscoliosis)
Other Problems to be Considered:
Congestive (biventricular) heart failure
Primary pulmonic stenosis
Primary pulmonary hypertension
Right-sided heart failure due to congenital heart diseases
Right heart failure due to right ventricular infarction
- A general approach to diagnose cor pulmonale and to investigate its etiology starts with routine laboratory tests, chest radiography, and electrocardiography. Echocardiography gives valuable information about the disease and its etiology. Pulmonary function tests may become necessary to confirm the underlying lung disease. Ventilation/perfusion (V/Q) scan or chest CT scan may be performed if history and physical examination suggest pulmonary thromboembolism as the cause or if other diagnostic tests do not suggest other etiologies. Right heart catheterization is the most accurate but invasive test to confirm the diagnosis of cor pulmonale and gives important information regarding the underlying diseases. Any abnormal result in each of these tests may need further diagnostic evaluation in that specific direction.
- Laboratory investigations are directed toward defining the potential underlying etiologies as well as evaluating complications of cor pulmonale. In specific instances, appropriate lab studies may include the following: hematocrit for polycythemia (which can be a consequence of underlying lung disease but can also increase pulmonary arterial pressure by increasing viscosity), serum alpha1-antitrypsin if deficiency is suspected, and antinuclear antibody level for collagen vascular disease such as scleroderma. Hypercoagulability states can be evaluated by serum levels of proteins S and C, antithrombin III, factor V Leyden, anticardiolipin antibodies, and homocysteine.
- Arterial blood gas tests may provide important information about the level of oxygenation and type of acid-base disorder.
- Elevated brain natriuretic peptide (BNP) level alone is not adequate to establish presence of cor pulmonale, but it helps to diagnose cor pulmonale in conjunction with other noninvasive tests and in appropriate clinical settings. An elevated BNP level may actually be a natural mechanism to compensate for elevated pulmonary hypertension and right heart failure by promoting diuresis and natriuresis, vasodilating systemic and pulmonary vessels, and reducing circulating levels of endothelin and aldosterone.
- Imaging studies may show evidence of underlying cardiopulmonary diseases, pulmonary hypertension, or its consequence, right ventricular enlargement.
- Chest roentgenography: In patients with chronic cor pulmonale, the chest radiograph may show enlargement of the central pulmonary arteries with oligemic peripheral lung fields. Pulmonary hypertension should be suspected when the right descending pulmonary artery is larger than 16 mm in diameter and the left pulmonary artery is larger than 18 mm in diameter. Right ventricular enlargement leads to an increase of the transverse diameter of the heart shadow to the right on the posteroanterior view and filling of the retrosternal air space on the lateral view. These findings have reduced sensitivity in the presence of kyphoscoliosis or hyperinflated lungs.
- Echocardiography (picture 12): Two-dimensional echocardiography usually demonstrates signs of chronic right ventricular pressure overload. As this overload progresses, increased thickness of the right ventricular wall with paradoxical motion of the interventricular septum during systole occurs. At an advanced stage, right ventricular dilatation occurs and the septum shows abnormal diastolic flattening. In extreme cases, the septum may actually bulge into the left ventricular cavity during diastole resulting in decreased diastolic volume of LVand reduction of LV output.
Doppler echocardiography is used now to estimate pulmonary arterial pressure, taking advantage of the functional tricuspid insufficiency that is usually present in pulmonary hypertension. Doppler echocardiography is considered the most reliable noninvasive technique to estimate pulmonary artery pressure. The efficacy of Doppler echocardiography may be limited by the ability to identify an adequate tricuspid regurgitant jet, which may be further enhanced by using saline contrast.
(A) Parasternal long axis view obtained from a patient admitted for septic shock secondary to a severe aortic endocarditis (arrows indicate vegetations) associated with a massive regurgitation and dilated left ventricle. (B) Parasternal short axis view obtained from a patient with an acute respiratory distress syndrome and associated cor pulmonale. The right ventricle was markedly enlarged and the ventricular septum bulged towards the left ventricular cavity at end systole, due to severe pulmonary hypertension (arrow). (C) Apical four-chamber view obtained from a ventilated patient with refractory hypoxemia. The contrast study (intravenous injection of saline microbubbles) revealed a large interatrial right-to-left shunt through a patent foramen ovale, which participated to persistent hypoxemia: left cardiac cavities were filled up by the microbubbles within two cardiac cycles. (D) Subcostal view obtained from a patient presenting with shock and pulsus paradoxus. A mild pericardial effusion responsible for prolonged right atrial collapse during the cardiac cycle (arrow) was consistent with a tamponade, and the patient underwent successful pericardotomy. LV, left ventricle; RV, right ventricle; LA, left atrium; RA, right atrium; Ao, ascending aorta.
- Ventilation/perfusion (V/Q) lung scanning, pulmonary angiography, and chest CT scanning may be indicated to diagnose pulmonary thromboembolism as the underlying etiology of cor pulmonale. These tests may be performed early in the diagnostic workup if any evidence of pulmonary embolism appears in history and physical examination. The test may also be considered later in the workup if other tests are not suggestive of any other etiology. Pulmonary thromboembolism has a wide range of clinical presentations from massive embolism with acute and severe hemodynamic instability to multiple chronic peripheral embolisms that may present with cor pulmonale.
- Ultrafast, ECG-gated CT scanning has been recently evaluated to study RV function. In addition to estimating right ventricular ejection fraction (RVEF), it can estimate RV wall mass. Its use is still experimental, but with further improvement, it may be used to evaluate the progression of cor pulmonale in the near future.
- Magnetic resonance imaging (MRI) of the heart is another modality that can provide valuable information about RV mass.
- Radionuclide ventriculography can determine RVEF noninvasively.
- Electrocardiography (ECG): ECG abnormalities in cor pulmonale reflect the presence of RVH, RV strain, or underlying pulmonary disease. These electrocardiographic changes may include right axis deviation, R/S amplitude ratio in V1 greater than 1 (increase in anteriorly directed forces may be a sign of posterior infarct), R/S amplitude ratio in V6 less than 1, P-pulmonale pattern (an increase in P wave amplitude in leads 2, 3, and aVF), S1Q3T3 pattern and incomplete (or complete) right bundle branch block, especially if pulmonary embolism is the underlying etiology, low-voltage QRS because of underlying COPD with hyperinflation and increased AP diameter of the chest. Severe RVH may reflect as Q waves in the precordial leads that may be interpreted as anterior myocardial infarction by mistake (on the other hand, since electrical activity of the RV is significantly less than the LV, small changes in RV forces may be lost in ECG).
Criteria of Cor Pumonale
I. QRS morphology
- Limb leads
1. Right axis deviation
2. S > R in lead I
- Precordial leads
1. S > R in V5 - V6
2. S > R in V2
3. Lead V1 rs, Rs, or rS
- Orthogonal leads
1. S > R in lead X
2. Tall R wave in lead II
II. T wave variable
III. P wave
- Limb leads
1. Vertical axis
2. P > 3 mm in lead II or III
- Precordial leads
1. Negative P in lead V1
- Additionally, many rhythm disturbances may be present in chronic cor pulmonale; these range from isolated premature atrial depolarizations to various supraventricular tachycardia, including paroxysmal atrial tachycardia, multifocal atrial tachycardia, atrial fibrillation, atrial flutter, and junctional tachycardia. These dysrhythmias may be triggered by processes secondary to the underlying disease, (eg, anxiety, hypoxemia, acid-base imbalance, electrolyte disturbances, excessive use of bronchodilators, heightened sympathetic activity).Life-threatening ventricular tachyarrhythmias are less common.
- In selected cases, pulmonary function testing may be indicated to determine underlying obstructive or interstitial lung disease.
- Cardiac catheterization: Right-heart catheterization is considered the most precise method for diagnosis and quantification of pulmonary hypertension. It is indicated when echocardiography cannot assess the severity of a tricuspid regurgitant jet, thus excluding an assessment of pulmonary hypertension. Right-heart catheterization is occasionally important for differentiating cor pulmonale from occult left ventricular dysfunction, especially when the presentation is confusing. Another indication may be for evaluation of the potential reversibility of pulmonary arterial hypertension with vasodilator therapy or when a left-side catheterization is indicated.
- Lung biopsy occasionally may be indicated to determine underlying etiology.
Medical Care: Medical therapy for chronic cor pulmonale is generally focused on treatment of the underlying pulmonary disease and improving oxygenation and RV function by increasing RV contractility and decreasing pulmonary vasoconstriction. However, the approach might be different to some degree in an acute setting with priority given to stabilizing the patient.
Cardiopulmonary support for patients experiencing acute cor pulmonale with resultant acute RV failure includes fluid loading and vasoconstrictor (eg, epinephrin) administration to maintain adequate blood pressure. Of course, the primary problem should be corrected, if possible. For example, for massive pulmonary embolism, consider administration of anticoagulation, thrombolytic agents or surgical embolectomy, especially if circulatory collapse is impending, consider bronchodilation and infection treatment in patients with COPD and consider steroid and immunosuppressive agents in infiltrative and fibrotic lung diseases.
Oxygen therapy, diuretics, vasodilators, digitalis, theophylline, and anticoagulation therapy are all different modalities used in the long-term management of chronic cor pulmonale.
- Oxygen therapy is of great importance in patients with underlying COPD, particularly when administered on a continuous basis. With cor pulmonale, the partial pressure of oxygen (PO2) is likely to be below 55 mm Hg and decreases further with exercise and during sleep.
Oxygen therapy relieves hypoxemic pulmonary vasoconstriction, which then improves cardiac output, lessens sympathetic vasoconstriction, alleviates tissue hypoxemia, and improves renal perfusion. The Nocturnal Oxygen Therapy Trial (NOTT), a multicenter randomized trial, showed that continuous low-flow oxygen therapy for patients with severe COPD resulted in significant reduction in the mortality rate. In general, in patients with COPD, long-term oxygen therapy is recommended when PaO2 is less than 55 mm Hg or O2 saturation is less than 88%. However, in the presence of cor pulmonale or impaired mental or cognitive function, long-term oxygen therapy can be considered even if PaO2 is greater than 55 mm Hg or O2 saturation is greater than 88%.
Although it is not clear whether oxygen therapy has a mortality rate benefit in patients with cor pulmonale due to pulmonary disorders other than COPD, it may provide some degree of symptomatic relief and improvement in functional status. Therefore, oxygen therapy plays an important role in both the immediate setting and long-term management, especially in patients who are hypoxic and have COPD.
- Diuretics are used in the management of chronic cor pulmonale, particularly when the right ventricular filling volume is markedly elevated and in the management of associated peripheral edema. Diuretics may result in improvement of the function of both the right and left ventricles; however, diuretics may produce hemodynamic adverse effects if they are not used cautiously. Excessive volume depletion can lead to a decline in cardiac output. Another potential complication of diuresis is the production of a hypokalemic metabolic alkalosis, which diminishes the effectiveness of carbon dioxide stimulation on the respiratory centers and lessens ventilatory drive. The adverse electrolyte and acid-base effect of diuretic use can also lead to cardiac arrhythmia, which can diminish cardiac output. Therefore, diuresis, while recommended in the management of chronic cor pulmonale, needs to be used with great caution.
- Vasodilator drugs have been advocated in the long-term management of chronic cor pulmonale with modest results. Calcium channel blockers, particularly oral sustained-release nifedipine and diltiazem, can lower pulmonary pressures, although they appear more effective in primary rather than secondary pulmonary hypertension. Other classes of vasodilators, such as beta agonists, nitrates, and angiotensin-converting enzyme (ACE) inhibitors have been tried but, in general, vasodilators have failed to show sustained benefit in patients with COPD and they are not routinely used. A trial of vasodilator therapy may be considered only in patients with COPD with disproportionately high pulmonary blood pressure.
Beta-selective agonists have an additional advantage of bronchodilator and mucociliary clearance effect. Right heart catheterization has been recommended during initial administration of vasodilators to objectively assess the efficacy and detect the possible adverse hemodynamic consequences of vasodilators. The Food and Drug Administration (FDA) has approved epoprostenol, treprostinil, bosentan, and iloprost for treatment of primary pulmonary hypertension. Epoprostenol, treprostinil, and iloprost are prostacyclin PGI2 analogues and have potent vasodilatory properties. Epoprostenol and treprostinil are administered intravenously and iloprost is an inhaler. Bosentan is a mixed endothelin-A and endothelin-B receptor antagonist indicated for pulmonary arterial hypertension (PAH), including primary pulmonary hypertension (PPH). In clinical trials, it improved exercise capacity, decreased rate of clinical deterioration, and improved hemodynamics. PDE5 inhibitor sildenafil has also been intensively studied and recently approved by the FDA for treatment of pulmonary hypertension based on a large randomized study. Sildenafil promotes selective smooth muscle relaxation in lung vasculature. Not enough data are available regarding the efficacy of these drugs in patients with secondary pulmonary hypertension such as in patients with COPD.
- The use of cardiac glycosides, such as digitalis, in patients with cor pulmonale has been controversial, and the beneficial effect of these drugs is not as obvious as in the setting of left heart failure. Nevertheless, studies have confirmed a modest effect of digitalis on the failing right ventricle in patients with chronic cor pulmonale. It must be used cautiously, however, and should not be used during the acute phases of respiratory insufficiency when large fluctuations in levels of hypoxia and acidosis may occur. Patients with hypoxemia or acidosis are at increased risk of developing arrhythmias due to digitalis through different mechanisms including sympathoadrenal stimulation.
- In addition to bronchodilatory effect, theophylline has been reported to reduce pulmonary vascular resistance and pulmonary arterial pressures acutely in patients with chronic cor pulmonale secondary to COPD. Theophylline has a weak inotropic effect and thus may improve right and left ventricular ejection. As a result, considering the use of theophylline as adjunctive therapy in the management of chronic or decompensated cor pulmonale is reasonable in patients with underlying COPD.
- Anticoagulation with warfarin is recommended in patients at high risk for thromboembolism. The beneficial role of anticoagulation in improving the symptoms and mortality in patients with primary pulmonary arterial hypertension clearly was demonstrated in a variety of clinical trials. The evidence of benefit, however, has not been established in patients with secondary pulmonary arterial hypertension. Therefore, anticoagulation therapy may be used in patients with cor pulmonale secondary to thromboembolic phenomena and with underlying primary pulmonary arterial hypertension.
- Phlebotomy is indicated in patients with chronic cor pulmonale and chronic hypoxia causing severe polycythemia, defined as hematocrit of 65 or more. Phlebotomy results in a decrease in mean pulmonary artery pressure, a decrease in mean pulmonary vascular resistance, and an improvement in exercise performance in such patients. There is, however, no evidence of improvement in survival. Generally, phlebotomy should be reserved as an adjunctive therapy for patients with acute decompensation of cor pulmonale and patients who remain significantly polycythemic despite appropriate long-term oxygen therapy. Replacement of the acute volume loss with a saline infusion may be necessary to avoid important decreases in systemic blood pressure.
- No surgical treatment exists for most diseases that cause chronic cor pulmonale. Pulmonary embolectomy is efficacious for unresolved pulmonary emboli, which contribute to pulmonary hypertension. Uvulopalatopharyngoplasty in selected patients with sleep apnea and hypoventilation may relieve cor pulmonale. Single-lung, double-lung, and heart-lung transplantation are all used to salvage the terminal phases of several diseases (eg, primary pulmonary hypertension, emphysema, idiopathic pulmonary fibrosis, cystic fibrosis) complicated by cor pulmonale. Apparently, lung transplantation will lead to a reversal of right ventricular dysfunction from the chronic stress of pulmonary hypertension. Strict selection criteria for lung transplant recipients must be met, however, because of the limited availability of organ donors.
Diuretics are used to decrease the elevated right ventricular filling volume in patients with chronic cor pulmonale. Calcium channel blockers are pulmonary artery vasodilators that have proven efficacy in the long-term management of chronic cor pulmonale secondary to primary pulmonary arterial hypertension. New FDA-approved prostacyclin analogues and endothelin-receptor antagonists are available for treatment of PPH. The beneficial role of cardiac glycosides, namely digitalis, on the failing right ventricle are somewhat controversial; they can improve right ventricular function but must be used with caution and should be avoided during acute episodes of hypoxia.
In the management of cor pulmonale, the main indication for oral anticoagulants is in the setting of an underlying thromboembolic event or primary pulmonary arterial hypertension. Methylxanthines, like theophylline, can be used as an adjunctive treatment for chronic cor pulmonale secondary to COPD. Besides the moderate bronchodilatory effect of methylxanthine, it improves myocardial contractility, causes mild pulmonary vasodilatory effect, and enhances the diaphragmatic contractility.
Drug Category: Diuretics -- Are used to decrease the elevated right ventricular filling volume in patients with chronic cor pulmonale.
Furosemide (Lasix) -- Example of diuretic agents used in the management of chronic cor pulmonale. Furosemide is a powerful loop diuretic that works on thick ascending limb of Henle loop, causing a reversible block in reabsorption of sodium, potassium, and chloride.
Adult dose- 20-80 mg/d PO/IV/IM; may titrate to maximum dose of 600 mg/d
Pediatric dose- 1-2 mg/kg/dose PO; not to exceed 6 mg/kg/dose; do not administer more frequent than q6h
1 mg/kg IV/IM slowly under close supervision; not to exceed 6 mg/kg
Contraindications- Documented hypersensitivity; hepatic coma; anuria; concurrent severe electrolyte depletion
Interactions- Metformin decreases furosemide concentrations; furosemide interferes with hypoglycemic effect of antidiabetic agents and antagonizes muscle-relaxing effect of tubocurarine; auditory toxicity appears to be increased with coadministration of aminoglycosides and furosemide; hearing loss of varying degrees may occur; anticoagulant activity of warfarin may be enhanced when taken concurrently with this medication; increased plasma lithium levels and toxicity are possible when taken concurrently with this medication
Pregnancy- C - Safety for use during pregnancy has not been established.
Precautions- Perform frequent serum electrolyte, carbon dioxide, glucose, creatinine, uric acid, calcium, and BUN determinations during first few months of therapy and periodically thereafter
Drug Category: Calcium channel blockers -- These agents inhibit movement of calcium ions across the cell membrane, depressing both impulse formation (automaticity) and conduction velocity.
Drug Name- Nifedipine (Procardia) -- Especially in the sustained-release form, nifedipine is a calcium channel blocker that has proven to be fairly effective in the management of chronic cor pulmonale caused by primary pulmonary hypertension. Modifies the entry of calcium into the cells by blocking the slow or voltage-dependent calcium channels, resulting in vasodilation, which improves myocardial oxygen delivery. Sublingual administration generally is safe, despite theoretical concerns.
Adult Dose- 10-30 mg SR cap PO tid; not to exceed 120-180 mg/d
30-60 mg SR tab PO qd; not to exceed 90-120 mg/d
Pediatric Dose- Not recommended
Contraindications- Documented hypersensitivity
Interactions- Monitor oral anticoagulants when used concomitantly; coadministration with any agent that can lower BP, including beta-blockers and opioids, can result in severe hypotension; H2 blockers (cimetidine) may increase toxicity
Pregnancy- C - Safety for use during pregnancy has not been established.
Precautions- Aortic stenosis; angina; congestive heart failure; pregnancy; nursing mothers; may cause lower extremity edema; allergic hepatitis has occurred but is rare
Drug Category: Cardiac glycosides -- These agents decrease AV nodal conduction primarily by increasing vagal tone.
Drug Name- Digoxin (Lanoxin) -- Has a positive inotropic effect on failing myocardium. Effect is achieved via inhibition of the Na+/K+-ATPase pump, leading to increase in intracellular sodium concentration along with concomitant increase in intracellular calcium concentration by means of calcium-sodium exchange mechanism. Net result is augmentation of myocardial contractility.
Adult Dose- 0.125-0.375 mg PO qd; may be administered qod; available in PO/IV/IM preparations
Pediatric Dose- 8-10 mcg/kg/d PO/IV/IM; maximum dose 100-150 mcg/kg/d
Contraindications- Documented hypersensitivity; beriberi heart disease; idiopathic hypertrophic subaortic stenosis; constrictive pericarditis; carotid sinus syndrome
Interactions- Medications that may increase digoxin levels include alprazolam, benzodiazepines, bepridil, captopril, cyclosporine, propafenone, propantheline, quinidine, diltiazem, aminoglycosides, oral amiodarone, anticholinergics, diphenoxylate, erythromycin, felodipine, flecainide, hydroxychloroquine, itraconazole, nifedipine, omeprazole, quinine, ibuprofen, indomethacin, esmolol, tetracycline, tolbutamide, and verapamil; medications that may decrease serum digoxin levels include aminoglutethimide, antihistamines, cholestyramine, neomycin, penicillamine, aminoglycosides, oral colestipol, hydantoins, hypoglycemic agents, antineoplastic treatment combinations (eg, carmustine, bleomycin, methotrexate, cytarabine, doxorubicin, cyclophosphamide, vincristine, procarbazine), aluminum or magnesium antacids, rifampin, sucralfate, sulfasalazine, barbiturates, kaolin/pectin, and aminosalicylic acid
Pregnancy- C - Safety for use during pregnancy has not been established.
Precautions- Hypokalemia may reduce positive inotropic effect of digitalis; IV calcium may produce arrhythmias in digitalized patients; hypercalcemia predisposes patient to digitalis toxicity; hypocalcemia can make digoxin ineffective until serum calcium levels are normal; magnesium replacement therapy must be instituted in patients with hypomagnesemia to prevent digitalis toxicity; patients diagnosed with incomplete AV block may progress to complete block when treated with digoxin; exercise caution in hypothyroidism, hypoxia, and acute myocarditis
Drug Category: Anticoagulants -- These agents may reduce incidence of embolisms when used fast, effectively, and early.
Drug Name- Warfarin (Coumadin) -- Most commonly used oral anticoagulant. Interferes with hepatic synthesis of vitamin K-dependent coagulation factors. Used for prophylaxis and treatment of venous thrombosis, pulmonary embolism, and thromboembolic disorders.
Adult Dose- 2-10 mg/d PO/IV qd; adjust dose to an INR of 1.5:2 or higher depending on the condition requiring anticoagulation
Pediatric Dose- Administer weight-based dose of 0.05-0.34 mg/kg/d PO/IV; adjust dose according to desired INR
Contraindications- Documented hypersensitivity; severe liver or kidney disease; open wounds; GI ulcers
Interactions- Griseofulvin, carbamazepine, glutethimide, estrogens, nafcillin, phenytoin, rifampin, barbiturates, cholestyramine, colestipol, vitamin K, spironolactone, oral contraceptives, and sucralfate may decrease anticoagulant effects; oral antibiotics, phenylbutazone, salicylates, sulfonamides, chloral hydrate, clofibrate, diazoxide, anabolic steroids, ketoconazole, ethacrynic acid, miconazole, nalidixic acid, sulfonylureas, allopurinol, chloramphenicol, cimetidine, disulfiram, metronidazole, phenylbutazone, phenytoin, propoxyphene, sulfonamides, gemfibrozil, acetaminophen, and sulindac may increase anticoagulant effects
Pregnancy- D - Unsafe in pregnancy
Precautions- Dose needs to be adjusted to INR; caution in bleeding tendency and hazardous active hemorrhagic conditions, malignant hypertension, patients at high risk of recurrent trauma, (eg, people with alcoholism or psychosis, unsupervised patients who are senile); warfarin anaphylaxis, hepatic, renal, thyroid, allergic, and hematologic hypocoagulable conditions and disorders; do not switch brands after achieving therapeutic response; caution in active tuberculosis or diabetes; patients with protein C or S deficiency are at risk of developing skin necrosis
Drug Category: Methylxanthines -- Potentiate exogenous catecholamines and stimulate endogenous catecholamine release and diaphragmatic muscular relaxation, which, in turn, stimulates bronchodilation.
Drug Name- Theophylline (Aminophyllin, Theo-24, Theolair, Theo-Dur) -- Mechanism of action is not well defined yet. Was formerly thought that this drug increases intracellular cyclic AMP by causing inhibition of phosphodiesterase; however, current data do not support that.
Adult Dose- Loading dose: 5.6 mg/kg IV over 20 min (based on aminophylline)
Maintenance dose: IV infusion at 0.5-0.7 mg/kg/h; also available in oral preparation
Pediatric Dose- 6 weeks to 6 months: 0.5 mg/kg/h loading dose IV in first 12 h (based on aminophylline), followed by maintenance infusion of 12 mg/kg/d thereafter; may administer continuous infusion by dividing total daily dose by 24 h
6 months to 1 year: 0.6-0.7 mg/kg/h loading dose IV in first 12 h, followed by maintenance infusion of 15 mg/kg/d; may administer as continuous infusion as above
>1 year: Administer as in adults
Contraindications- Documented hypersensitivity; uncontrolled arrhythmias; peptic ulcers; hyperthyroidism; uncontrolled seizure disorders
Interactions- Effects may decrease with aminoglutethimide, barbiturates, carbamazepine, ketoconazole, loop diuretics, charcoal, hydantoins, phenobarbital, phenytoin, rifampin, isoniazid, and sympathomimetics; effects may increase with allopurinol, beta-blockers, ciprofloxacin, corticosteroids, disulfiram, quinolones, thyroid hormones, ephedrine, carbamazepine, cimetidine, erythromycin, macrolides, propranolol, and interferon
Pregnancy- C - Safety for use during pregnancy has not been established.
Precautions- Has low serum therapeutic-to-toxicity ratio, and, therefore, serum level monitoring is important; peptic ulcer; hypertension; tachyarrhythmias; hyperthyroidism; compromised cardiac function; do not inject IV solution faster than 25 mg/min; patients diagnosed with pulmonary edema or liver dysfunction are at greater risk of toxicity because of reduced drug clearance
Drug Category: Endothelin receptor antagonists -- Competitively bind to endothelin-1 (ET-1) receptors ETA and ETB causing reduction in pulmonary artery pressure (PAP), pulmonary vascular resistance (PVR), and mean right atrial pressure (RAP).
Drug Name- Bosentan (Tracleer) -- Endothelin receptor antagonist indicated for the treatment of pulmonary arterial hypertension in patients with WHO Class III or IV symptoms, to improve exercise ability and decrease rate of clinical worsening. Inhibits vessel constriction and elevation of blood pressure by competitively binding to endothelin-1 (ET-1) receptors ETA and ETB in endothelium and vascular smooth muscle. This leads to significant increase in cardiac index (CI) associated with significant reduction in pulmonary artery pressure (PAP), pulmonary vascular resistance (PVR), and mean right atrial pressure (RAP). Due to teratogenic potential, can only be prescribed through the Tracleer Access Program (1-866-228-3546).
Adult Dose- <40 kg: 62.5 mg PO bid; not to exceed 125 mg/d
>40 kg: 62.5 mg PO bid for 4 wk initially, then increase to 125 mg PO bid
Pediatric Dose- Not established; 62.5 mg PO bid recommended if <40 kg, or >12 years; not to exceed 125 mg/d
Contraindications- Documented hypersensitivity; coadministration with cyclosporine A or glyburide
Interactions- Toxicity may increase when administered concomitantly with inhibitors of isoenzymes CYP450 2C9 and CYP450 3A4 (eg, ketoconazole, erythromycin, fluoxetine, sertraline, amiodarone, and cyclosporine A); induces isoenzymes CYP450 2C9 and CYP450 3A4 causing decrease in plasma concentrations of drugs metabolized by these enzymes including glyburide as well as other hypoglycemics, cyclosporine A, hormonal contraceptives, simvastatin, and possibly other statins; hepatotoxicity increases with concomitant administration of glyburide
Pregnancy- X - Contraindicated in pregnancy
Precautions- Causes at least 3-fold elevation of liver aminotransferases (ie, ALT, AST) in about 11% of patients; may elevate bilirubin (serum aminotransferase levels must be measured prior to initiation of treatment and then monthly); caution in patients with mildly impaired liver function (avoid in patients with moderate or severe liver impairment); not recommended while breastfeeding; monitor hemoglobin levels after 1 and 3 mo of treatment and every 3 mo thereafter; exclude pregnancy before initiating treatment and prevent thereafter by use of reliable contraception; headache and nasopharyngitis may occur
References and sources
- Allewelt M, Schuler P, Bolcskei PL. Ampicillin + sulbactam vs clindamycin +/- cephalosporin for the treatment of aspiration pneumonia and primary lung abscess. Clin Microbiol Infect. Feb 2004;10(2):163-70.
- Appelbaum PC, Spangler SK, Jacobs MR. Beta-lactamase production and susceptibilities to amoxicillin, amoxicillin-clavulanate, ticarcillin, ticarcillin-clavulanate, cefoxitin, imipenem, and metronidazole of 320 non-Bacteroides fragilis Bacteroides isolates and 129 fusobacteria from 28 U.S. Antimicrob Agents Chemother. Aug 1990;34(8):1546-50.
- Bandt PD, Blank N, Castellino RA. Needle diagnosis of pneumonitis. Value in high-risk patients. JAMA. Jun 19 1972;220(12):1578-80.
- Bartlett JG, Gorbach SL, Tally FP. Bacteriology and treatment of primary lung abscess. Am Rev Respir Dis. May 1974;109(5):510-8.
- Bartlett JG, Finegold SM. Anaerobic infections of the lung and pleural space. Am Rev Respir Dis. Jul 1974;110(1):56-77.
- Bartlett JG. HIV infection and surgeons. Curr Probl Surg. Apr 1992;29(4):197-280.
- Finegold SM, George WL, Mulligan ME. Anaerobic infections. Part II. Dis Mon. Nov 1985;31(11):1-97.
- Finegold SM, Rolfe RD. Susceptibility testing of anaerobic bacteria. Diagn Microbiol Infect Dis. Mar 1983;1(1):33-40.
- Herth F, Ernst A, Becker HD. Endoscopic drainage of lung abscesses: technique and outcome. Chest. Apr 2005;127(4):1378-81.
- Hirshberg B, Sklair-Levi M, Nir-Paz R. Factors predicting mortality of patients with lung abscess. Chest. Mar 1999;115(3):746-50.
- Howe C, Sampath A, Spotnitz M. The pseudomallei group: a review. J Infect Dis. Dec 1971;124(6):598-606.
- La Scola B, Michel G, Raoult D. Isolation of Legionella pneumophila by centrifugation of shell vial cell cultures from multiple liver and lung abscesses. J Clin Microbiol. Mar 1999;37(3):785-7.
- Mansharamani N, Balachandran D, Delaney D. Lung abscess in adults: clinical comparison of immunocompromised to non-immunocompromised patients. Respir Med. Mar 2002;96(3):178-85.
- Mansharamani NG, Koziel H. Chronic lung sepsis: lung abscess, bronchiectasis, and empyema. Curr Opin Pulm Med. May 2003;9(3):181-5.
- Narushima M, Suzuki H, Kasai T. Pulmonary nocardiosis in a patient treated with corticosteroid therapy. Respirology. Mar 2002;7(1):87-9.
- Perlino CA. Metronidazole vs clindamycin treatment of anaerobic pulmonary infection. Failure of metronidazole therapy. Arch Intern Med. Oct 1981;141(11):1424-7.
- Pohlson EC, McNamara JJ, Char C. Lung abscess: a changing pattern of the disease. Am J Surg. Jul 1985;150(1):97-101.
- Sanders CV, Hanna BJ, Lewis AC. Metronidazole in the treatment of anaerobic infections. Am Rev Respir Dis. Aug 1979;120(2):337-43.
- Light RW. Clinical practice. Pleural effusion. N Engl J Med. Jun 20 2002;346(25):1971-7.
- Light RW, ed. Pleural Diseases. 5th ed. Baltimore, Md: Lippincott Williams & Wilkins; 2007.
- Light RW, Jenkinson SG, Minh VD, George RB. Observations on pleural fluid pressures as fluid is withdrawn during thoracentesis. Am Rev Respir Dis. May 1980;121(5):799-804.
- Marchi E, Teixeira LR, Vargas FS. Management of malignancy-associated pleural effusion: current and future treatment strategies. Am J Respir Med. 2003;2(3):261-73.
- Romero Candeira S, Hernбndez Blasco L, Soler MJ, Muсoz A, Aranda I. Biochemical and cytologic characteristics of pleural effusions secondary to pulmonary embolism. Chest. Feb 2002;121(2):465-9.
- Sahn SA. Management of complicated parapneumonic effusions. Am Rev Respir Dis. Sep 1993;148(3):813-17.
- Sahn SA. State of the art. The pleura. Am Rev Respir Dis. Jul 1988;138(1):184-234.
- Sahn SA, Good JT Jr. Pleural fluid pH in malignant effusions. Diagnostic, prognostic, and therapeutic implications. Ann Intern Med. Mar 1988;108(3):345-9.
- Sallach SM, Sallach JA, Vasquez E, Schultz L, Kvale P. Volume of pleural fluid required for diagnosis of pleural malignancy. Chest. Dec 2002;122(6):1913-7.
- Seibert AF, Haynes J Jr, Middleton R, Bass JB Jr. Tuberculous pleural effusion. Twenty-year experience. Chest. Apr 1991;99(4):883-6.
- Shaw P, Agarwal R. Pleurodesis for malignant pleural effusions. Cochrane Database Syst Rev. 2004;CD002916.
- Stark P. The pleura. In: Taveras JM, Ferrucci JT, eds. Radiology: Diagnosis/Imaging/Intervention. Philadelphia, Pa: Lippincott-Raven; 2000:1-29.
- Walker-Renard PB, Vaughan LM, Sahn SA. Chemical pleurodesis for malignant pleural effusions. Ann Intern Med. Jan 1 1994;120(1):56-64.
- West SD, Davies RJ, Lee YC. Pleurodesis for malignant pleural effusions: current controversies and variations in practices. Curr Opin Pulm Med. Jul 2004;10(4):305-10.
Contributors and acknowledgement
Further Inpatient Care:
- Appropriate treatment is directed both at the underlying etiology and at correction of hypoxia when present.
Further Outpatient Care:
- Patients with cor pulmonale generally require close attention in the outpatient setting.
- Regular assessment of oxygen needs and pulmonary function are appropriate.
- Many patients benefit from a formal program of pulmonary rehabilitation.
- Complications of cor pulmonale include syncope, hypoxia, pedal edema, passive hepatic congestion, and death.
- The prognosis of cor pulmonale is variable depending upon underlying pathology.
- Patients with cor pulmonale due to COPD have a high 2-year mortality.
- Patient education regarding the importance of adherence to medical therapy is vital because appropriate treatment of both hypoxia and underlying medical illness can improve mortality and morbidity.
- Making a diagnosis of cor pulmonale should be followed by further investigation to determine the underlying lung pathology. Sometimes a common lung disease such as COPD is not the only lung pathology as the cause of cor pulmonale; other lung diseases may coexist.
- When diagnosing cor pulmonale, considering the possibility of thromboembolic disease and primary pulmonary hypertension as possible etiologies is important.
- Note the importance of continuous supplemental oxygen therapy in appropriate patients, as well as the dangers of cigarette smoking while using supplemental oxygen. Elevation of carboxyhemoglobin in the blood due to smoking can significantly decrease the effect of O2 on arterial O2 content.
Cor pulmonale is not a disease, per se, but a manifestation common to many disease states. Accordingly, a variety of medical and surgical treatments are available, but therapy must be based on the etiologic and pathophysiologic factors responsible. In addition, therapy must be individualized, taking into account the severity of symptoms and prognosis. Treatment of the underlying disorder, if one can be identified, is the first approach to the treatment of cor pulmonale. Improving airflow, alveolar ventilation, and gas exchange through the use of bronchodilators, corticosteroids, mucolytics, and, occasionally, assisted ventilation often ameliorates the pulmonary hypertensive state in patients with parenchymal disease. Supple-mental oxygen therapy reduces the degree of pulmonary hypertension in hypoxemic cor pulmonale by abolishing hypoxic vasoconstriction; indeed, low-flow continuous On therapy is the only modality that has been proven to prolong life in cor pulmonale due to chronic obstructive lung disease.
Because of the ominous prognosis associated with pulmonary hypertension from any cause, an aggressive approach to treatment is warranted as soon as a clinical diagnosis is made. It should be emphasized that physical findings and noninvasive studies alone are insufficient to confirm the presence and severity of pulmonary hypertension; right-heart catheterization is necessary both to establish the diagnosis and to monitor therapeutic responses.
As stated previously, therapy directed at improving gas exchange and alleviating hypoxic vasoconstriction is the initial step in the treatment for cor pulmonale secondary to parenchymal lung disease, and it should be initiated at the earliest sign of cor pulmonale. Although this approach is often successful, the maximal effects may not be clinically apparent for several months.
Patients with PPH should also be considered candidates for aggressive therapy immediately upon the establishment of a diagnosis. However, patients with severe, overt right-heart failure pose the greatest risk for adverse effects and are less likely to derive benefit from medical therapy.
Mechanisms of Action of Drugs Used
Oxygen. Low-flow supplemental oxygen therapy alleviates hypoxic pulmonary vasoconstriction and may halt the progressive vascular remodeling that is seen inpatients with cor pulmonale due to severe parenchymal lung disease. Whereas it may relieve the subjective sensation of dyspnea in patients with non hypoxemic cor pulmonale, supplemental oxygen does not usually pro-duce hemodynamic improvement in these patients
Methylxanthines. Theophylline, the most widely used methytxanthine derivative, has several potentially beneficial effects in cor pulmonale: Theophylline improves airflow by its bronchodilator effects and by a direct enhancement of mucociliary clearance. In addition, theophylime enhances diaphragmatic contractility, decreasing the work of breathing. It has also been suggested that theophylline improves right ventricular function in patients with chronic obstructive pulmonary disease with cor pulmonale, possibly by a direct vasodilator effect on the pulmonary circulation. Finally, the modest diuretic effects of theophylline may limit fluid retention in patients with right ventricular dysfunction.
Vasodilators. The rationale for the use of vasodilators in pulmonary hypertension is based on the suggestion that pulmonary vasoconstriction is present, to varying degrees, and that systemic vasodilators exert comparable effects on pulmonary vascular smooth muscle. Reduction in vascular smooth muscle tone would reduce right ventricular afterload, thereby improving right ventricular function and oxygen transport to the peripheral tissues. A variety of vasodilators have been shown to reduce pulmonary vasoconstriction in experimental and clinical conditions, including the calcium channel blockers, prostaglandins I and E, nitrates. Other agents, such as the angiotensin converting enzyme inhibitors, appear far less active on the pulmonary vascular bed. It should also be emphasized that the presence or degree of reversible vasoconstriction is variable in cor pulmonale. It may be a predominant factor in some patients, especially those with hypoxemic lung disease and primary pulmonary hypertension, but it is unlikely to contribute substantially to the hypertensive state in patients with chronic thrombotic pulmonary hypertension or cor pulmonale due to connective tissue disease.
Dosage, Routes of, and Practical Considerations in Drug Administration
Oxygen. Patients with hypoxemic cor pulmonale should be treated with low-flow oxygen delivered via nasal cannula and to achieve an arterial Po greater than 60 to 65 torr. Oxygen therapy should be used for at least 18 hours per day, and preferably for 24 hours per day; even intermittent alveolar hypoxia is sufficient to promote ongoing pulmonary vasoconstriction. Some authors have suggested empirically increasing the flow rate by 1 liter per minute during sleep because hypoventilation with resultant hypoxemia is common in patients with cor pulmonale due to obstructive lung disease Although oxygen concentrators are efficient and cost effective devices for the delivery of continuous supplemental oxygen, they are of limited benefit in ambulatory patients because of their size and electrical requirements. Liquid oxygen systems, which are portable albeit more expensive, allow patients to ambulate while still receiving supplemental oxygen.
In some patients with pulmonary hypertension, arterial hypoxemia may be due to right-to-left shunting through a patent foramen ovale. Such patients may not substantially increase arterial Po in response to sup-plemental oxygen, but dyspnea and activity tolerance may nevertheless be improved.
Methylxanthines. I prefer to use theophylline inpatients with cor pulmonale due to chronic obstructive pulmonary disease in doses that achieve low therapeutic levels. Higher serum levels (15 to 20 u,g per milliliter) may be associated with adverse effects, such as tachycardia, arrhythmias, nausea, and tremors. Oral sustained-release preparations, titrated to achieve the desired serum concentration, can be administered twice daily and provide reliable bioavailabity.
Vasodilators. The use of vasodilators in pulmonary hypertension should be considered experimental, and their role in management is unclear. My approach is to withhold their use in patients with cor pulmonale due to lung disease until conventional therapy has proven in adequate. In contrast, patients with primary pulmonary hypertension are viewed as potential candidates for vasodilator therapy as soon as the diagnosis is established because no other modality of treatment has proven any more successful. Prostacyclin (PGI) is well suited for this purpose in that it is a potent, titratabic, short-acting agent. Individuals who manifest reductions in pulmonary artery pressure and pulmonary vascular resistance in response to the acute intravenous infusion of prostacyclin are more likely to respond in a similar fashion to oral or transdermal vasodilators. As of this writing, prostacyclin is an experimental agent that has not been approved by the Food and Drug Administration for general use; commercially available intravenous agents, may be suitable alternatives, although they may be less potent than prostacyclin.
Individuals who manifest beneficial responses to intravenous prostacyclin are treated with oral or topical vasodilators. The calcium channel blockers appear to be the most potent agents, although side effects are common and may preclude their use Nifedipine and diltiazem hydrochloride appear equally effective; verapamil is generally not used because it is less potent in the pulmonary vascular bed and it possesses negative effects. I usually begin therapy with sustained-release nifedipine in doses of 30 mg once daily, increasing the dose as tolerated; sustained-release diltiazem hydrochloride therapy is instituted in doses of 120 mg once daily, increasing as tolerated. Studies have suggested that large doses of these agents may be necessary to produce sustained responses in pulmonary hypertension, although side effects may limit the doses that can be achieved. Survival is improved in patients who are responsive to calcium channel blocking therapy.
If calcium channel blockers cannot be used because of adverse effects, I consider nitrates as a second line of therapy in patients with demonstrated pulmonary vasoreactivity. I prefer to use topical nitroglycerin ointment, every 6 hours,increasing as tolerated.
Patients who are refractory to these approaches may be considered candidates for continuous intravenous infusion of prostacyclin. This approach, which is still experimental, may be particularly useful as a bridge to transplantation in severely impaired individuals. Prostacyclin is delivered intravenously from a portable syringe pump connected to a chronic indwelling central venous catheter.
Anticoagulation. Recent experience suggests that PPH patients who arc treated with anticoagulants tend to live longer. I treat patients with PPH or chronic thrombotic pulmonary vascular disease with warfarin, adjusting the dose to achieve a prothrombin time of approximately 1 5 times control I generally do not treat patients with other causes of cor pulmonale with anticoagulants unless a specific indication exists.
Cardiac Glycosides and Diuretics. Cardiac glycosides appear to be of limited usefulness in cor pulmonale due to parenchymal lung disease unless left vcntricular dysfunction is present. Furthermore, the risk of digitalis toxicity is increased in the setting of chronic lung disease, in part because of the presence of hypoxemia, and diuretic-induced hypokalemia. Accordingly, I use cardiac glycosides in this setting only when supraventricular tachyarrhythmias requiring atrioventricular node blockade are present; verapamil may bean alternative, although its other hemodynamic effects may limit its usefulness
Some authors have advocated combining cardiac glycosides with calcium channel blocker therapy inpatients with primary pulmonary hypertension to counteract the negative inotropic effects of nifedipine or diltiazem hydrochloride.
Diuretics should be used cautiously in patients with cor pulmonale because excessive reduction in right-heart preload may actually compromise right ventricular function. In addition, the hypokalemia and metabolic alkalosis that may result from diuretic use are poorly tolerated by patients with severe chronic lung disease. Finally, many patients with hypoxemic cor pulmonale experience a gradual hemodynamic response to supple-mental oxygen therapy, precluding the need for diuretics.
Despite these caveats, patients with persistent or severe volume overload attributable to right-heart failure should be treated with diuretics. Furoscmide, doses of 40 to 120 mg per day, are usually sufficient. In refractory situations, potent diuretics, such as metolazone in doses of 2.5 to 5 mg, may be added. Monitoring of serum electrolytes is mandatory when these agents are used, and aggressive potassium or magnesium replacement may be necessary.
Side Effects of the Drugs Used
Low-flow supplemental oxygen therapy is generally safe and is well tolerated by most patients with hypoxemic lung disease. Modest increases in PCo can accompany its use in hypercapnic individuals with chronic obstructive pulmonary disease, but overt suppression of respiratory drive is unlikely unless very high flow rates are used or other factors precipitating acute respiratory failure are present. The use of nasal cannulas can produce nasal mucosal drying and irritation, which can be minimized by keeping flow rates lower than 4 to5 liters per minute and applying topical lubricants to the mucosa.
Side effects from bronchodilators are generally minor and include tachycardia, tremor, and nervousness. Selective beta-agonists and cholinergic agonists administered by inhalation are better tolerated than oral beta-agonists. Oral theophylline preparations should be given in doses that achieve scrum levels and that arc not accompanied by side effects.
The major adverse effects of vasodilators are systemic hypotension, deterioration in gas exchange, and depression of cardiac contractility. Because there is noselective pulmonary vasodilator, most patients with cor pulmonale experience some degree of systemic vasodilation in response to the administration of a vasodilator. Patients with "fixed" pulmonary vascular disease are more likely to experience hypotension with vasodilators because cardiac output is unlikely to increase.
Some vasodilators, particularly calcium channel blockers and nitrates, can worsen gas exchange by increasing perfusion to poorly ventilated lung units. The hypoxemia may be poorly tolerated by individuals with underlying parenchymal lung disease and pre-existent impaired gas exchange. Careful monitoring of arterial blood gases or saturation is important in this setting.
The calcium channel blocking agents may also precipitate a deterioration in right-heart function as a result of their negative inotropic properties. The phenomenon is more common with verapamil than with either nifedipine or diltiazem hydrochloride, but it can occur with any of these compounds and at any dose. Differentiating drug-induced heart failure from disease progression or drug-induced fluid retention (which occurs in up to 30 percent of patients taking calcium channel blockers) is often difficult and may require empirically reducing the dose or repeated right-heart catheterization.
Assessment of Therapeutic Responses
Although improvement in symptoms, such as decreased exertional dyspnea, is suggestive of a beneficial therapeutic response, evaluation by invasive or noninvasive techniques is usually required. Echocardiography and radionuclide ventriculography are useful in providing a qualitative assessment of right ventricular function. Right ventncular catheterization, is the preferred approach in monitoring therapy.
The debate regarding the definition of a beneficial response to vasodilator therapy remains unsettled. I treat patients with vasodilators if they experience a sustained reduction in pulmonary vascular resistance greater than 25 to 30 percent, which is produced by a reduction in pulmonary artery pressure, increase in cardiac output, or both. An increased pulmonary artery pressure, decreased cardiac output, symptomatic systemic hypotension, or substantial deterioration in gas exchange (usually due to either right-to-left shunting through a patent foramen ovale or increased V/Q mismatching) constitute contraindications to vasodilator therapy.
Johnk804 on September 06, 2014:
I am just seeming both for blog sites which give independent, nutritious commentary on all difficulties or blogging sites which have a liberal or droppedwing slant. Thank you.. dddegaffcgdg
Department Health & Human Services of Tasmania Royal Hobart Hospital on October 31, 2010:
Cor pulmonale is failure of the right side of the heart caused by prolonged high blood pressure in the pulmonary artery and right ventricle of the heart.
Cor pulmonale is also called right-sided heart failure.
How does the Heart Function?
In a normal heart, the left side produces a higher level of blood pressure in order to pump blood to the body. The right side of the heart pumps blood through the lungs under much lower pressure.
Any condition that leads to prolonged high blood pressure in the arteries or veins of the lungs will be poorly tolerated by the right ventricle of the heart. When the right ventricle of the heart fails or is unable to properly pump against these abnormally high pressures, this is called cor pulmonale.
What Causes Cor Pulmonale?
Almost any chronic lung disease or condition causing prolonged low blood oxygen can lead to cor pulmonale. Some common causes of cor pulmonale are:
Chronic obstructive pulmonary disease (COPD)
Obstructive sleep apnea
Central sleep apnea
Primary pulmonary hypertension
Diffuse interstitial pulmonary fibrosis
Chronic thromboembolic pulmonary disease
Pulmonary vascular disease
Symptoms of Cor Pulmonale
The most common symptoms of cor pulmonale are:
Shortness of breath
Swelling of the feet or ankles
Bluish color to the skin
Distension of the neck veins
Abnormal fluid collection in the abdomen
Enlargement of the liver
Abnormal heart sounds
Can Cor Pulmonale be Treated?
Yes. Treatment is directed at the underlying illness. In some cases, supplemental oxygen may be prescribed to increase the level of oxygen in the blood.
Surgery may be used to reverse heart defects that cause cor pulmonale. Blood thinning medications may also be prescribed.
Complications of Cor Pulmonale
Cor pulmonale may lead to severe fluid retention, life-threatening shortness of breath, shock, and in severe cases, death.
Sisters of Mercy Health System on October 31, 2010:
Further Inpatient Care
Appropriate treatment is directed both at the underlying etiology and at correction of hypoxia when present.
Further Outpatient Care
Patients with cor pulmonale generally require close attention in the outpatient setting.
Regular assessment of oxygen needs and pulmonary function are appropriate.
Many patients benefit from a formal program of pulmonary rehabilitation.
Complications of cor pulmonale include syncope, hypoxia, pedal edema, passive hepatic congestion, and death.
The prognosis of cor pulmonale is variable depending upon underlying pathology.
Patients with cor pulmonale due to COPD have a high 2-year mortality.
Patient education regarding the importance of adherence to medical therapy is vital because appropriate treatment of both hypoxia and underlying medical illness can improve mortality and morbidity.
Making a diagnosis of cor pulmonale should be followed by further investigation to determine the underlying lung pathology. Sometimes a common lung disease such as COPD is not the only lung pathology as the cause of cor pulmonale; other lung diseases may coexist.
When diagnosing cor pulmonale, considering the possibility of thromboembolic disease and primary pulmonary hypertension as possible etiologies is important.
Note the importance of continuous supplemental oxygen therapy in appropriate patients, as well as the dangers of cigarette smoking while using supplemental oxygen. Elevation of carboxyhemoglobin in the blood due to smoking can significantly decrease the effect of O2 on arterial O2 content.
Institut Municipal d'Investigació Médica Hospital del Mar on October 31, 2010:
Medical therapy12 for chronic cor pulmonale is generally focused on treatment of the underlying pulmonary disease and improving oxygenation and RV function by increasing RV contractility and decreasing pulmonary vasoconstriction. However, the approach might be different to some degree in an acute setting with priority given to stabilizing the patient.
Cardiopulmonary support for patients experiencing acute cor pulmonale with resultant acute RV failure includes fluid loading and vasoconstrictor (eg, epinephrine) administration to maintain adequate blood pressure. Of course, the primary problem should be corrected, if possible. For example, for massive pulmonary embolism, consider administration of anticoagulation, thrombolytic agents or surgical embolectomy, especially if circulatory collapse is impending; consider bronchodilation and infection treatment in patients with COPD; and consider steroid and immunosuppressive agents in infiltrative and fibrotic lung diseases.
Oxygen therapy, diuretics, vasodilators, digitalis, theophylline, and anticoagulation therapy are all different modalities used in the long-term management of chronic cor pulmonale.
Oxygen therapy is of great importance in patients with underlying COPD13 , particularly when administered on a continuous basis. With cor pulmonale, the partial pressure of oxygen (PO2) is likely to be below 55 mm Hg and decreases further with exercise and during sleep.
Oxygen therapy relieves hypoxemic pulmonary vasoconstriction, which then improves cardiac output, lessens sympathetic vasoconstriction, alleviates tissue hypoxemia, and improves renal perfusion. The Nocturnal Oxygen Therapy Trial (NOTT), a multicenter randomized trial, showed that continuous low-flow oxygen therapy for patients with severe COPD resulted in significant reduction in the mortality rate.14 In general, in patients with COPD, long-term oxygen therapy is recommended when PaO2 is less than 55 mm Hg or O2 saturation is less than 88%. However, in the presence of cor pulmonale or impaired mental or cognitive function, long-term oxygen therapy can be considered even if PaO2 is greater than 55 mm Hg or O2 saturation is greater than 88%.
Although whether oxygen therapy improves survival in patients with cor pulmonale due to pulmonary disorders other than COPD is not clear, it may provide some degree of symptomatic relief and improvement in functional status. Therefore, oxygen therapy plays an important role in both the immediate setting and long-term management, especially in patients who are hypoxic and have COPD.
Diuretics are used in the management of chronic cor pulmonale, particularly when the right ventricular filling volume is markedly elevated and in the management of associated peripheral edema. Diuretics may result in improvement of the function of both the right and left ventricles; however, diuretics may produce hemodynamic adverse effects if they are not used cautiously. Excessive volume depletion can lead to a decline in cardiac output. Another potential complication of diuresis is the production of a hypokalemic metabolic alkalosis, which diminishes the effectiveness of carbon dioxide stimulation on the respiratory centers and lessens ventilatory drive. The adverse electrolyte and acid-base effect of diuretic use can also lead to cardiac arrhythmia, which can diminish cardiac output. Therefore, diuresis, while recommended in the management of chronic cor pulmonale, needs to be used with great caution.
Vasodilator drugs have been advocated in the long-term management of chronic cor pulmonale with modest results. Calcium channel blockers, particularly oral sustained-release nifedipine15 and diltiazem, can lower pulmonary pressures, although they appear more effective in primary rather than secondary pulmonary hypertension.16 Other classes of vasodilators, such as beta agonists, nitrates, and angiotensin-converting enzyme (ACE) inhibitors have been tried but, in general, vasodilators have failed to show sustained benefit in patients with COPD and they are not routinely used. A trial of vasodilator therapy may be considered only in patients with COPD with disproportionately high pulmonary blood pressure.
Beta-selective agonists have an additional advantage of bronchodilator and mucociliary clearance effect. Right heart catheterization has been recommended during initial administration of vasodilators to objectively assess the efficacy and detect the possible adverse hemodynamic consequences of vasodilators.
The Food and Drug Administration (FDA) has approved epoprostenol, treprostinil, bosentan, and iloprost for treatment of primary pulmonary hypertension. Epoprostenol, treprostinil, and iloprost are prostacyclin (PGI2) analogues and have potent vasodilatory properties.17 Epoprostenol and treprostinil are administered intravenously and iloprost is an inhaler. Bosentan is a mixed endothelin-A and endothelin-B receptor antagonist indicated for pulmonary arterial hypertension (PAH), including primary pulmonary hypertension (PPH). In clinical trials, it improved exercise capacity, decreased rate of clinical deterioration, and improved hemodynamics.17
The PDE5 inhibitor sildenafil has been intensively studied18,19,20 and approved by the FDA for treatment of pulmonary hypertension. Sildenafil promotes selective smooth muscle relaxation in lung vasculature.21 Tadalafil, another PDE5 inhibitor, was also recently approved by the FDA for the treatment of PAH to improve exercise ability.22
Not enough data are available regarding the efficacy of these drugs in patients with secondary pulmonary hypertension such as in patients with COPD.
The use of cardiac glycosides, such as digitalis, in patients with cor pulmonale has been controversial, and the beneficial effect of these drugs is not as obvious as in the setting of left heart failure. Nevertheless, studies have confirmed a modest effect of digitalis on the failing right ventricle in patients with chronic cor pulmonale.23 It must be used cautiously, however, and should not be used during the acute phases of respiratory insufficiency when large fluctuations in levels of hypoxia and acidosis may occur. Patients with hypoxemia or acidosis are at increased risk of developing arrhythmias due to digitalis through different mechanisms including sympathoadrenal stimulation.
In addition to bronchodilatory effect, theophylline has been reported to reduce pulmonary vascular resistance and pulmonary arterial pressures acutely in patients with chronic cor pulmonale secondary to COPD.24 Theophylline has a weak inotropic effect and thus may improve right and left ventricular ejection. Low doses of theophylline have also been suggested to have anti-inflammatory effects that help to control underlying lung diseases such as COPD.25 As a result, considering the use of theophylline as adjunctive therapy in the management of chronic or decompensated cor pulmonale is reasonable in patients with underlying COPD.
Anticoagulation with warfarin is recommended in patients at high risk for thromboembolism. The beneficial role of anticoagulation in improving the symptoms and mortality in patients with primary pulmonary arterial hypertension has been demonstrated in several studies.26,27,28,29 The evidence of benefit, however, has not been established in patients with secondary pulmonary arterial hypertension. Therefore, anticoagulation therapy may be used in patients with cor pulmonale secondary to thromboembolic phenomena and with underlying primary pulmonary arterial hypertension.
Phlebotomy is indicated in patients with chronic cor pulmonale and chronic hypoxia causing severe polycythemia, defined as hematocrit of 65 or more. Phlebotomy results in a decrease in mean pulmonary artery pressure, a decrease in mean pulmonary vascular resistance,30 and an improvement in exercise performance in such patients. However, no evidence suggests improvement in survival. Generally, phlebotomy should be reserved as an adjunctive therapy for patients with acute decompens
Hospital Italiano de Buenos Aires on October 31, 2010:
Other Problems to Be Considered
Congestive (biventricular) heart failure
Primary pulmonic stenosis
Primary pulmonary hypertension
Right-sided heart failure due to congenital heart diseases
Right heart failure due to right ventricular infarction
Ventricular septal defect
High-output heart failure
Laboratory investigations are directed toward defining the potential underlying etiologies as well as evaluating complications of cor pulmonale. In specific instances, appropriate lab studies may include the following: hematocrit for polycythemia (which can be a consequence of underlying lung disease but can also increase pulmonary arterial pressure by increasing viscosity), serum alpha1-antitrypsin if deficiency is suspected, and antinuclear antibody level for collagen vascular disease such as scleroderma. Hypercoagulability states can be evaluated by serum levels of proteins S and C, antithrombin III, factor V Leyden, anticardiolipin antibodies, and homocysteine.
Arterial blood gas tests may provide important information about the level of oxygenation and type of acid-base disorder.
Elevated brain natriuretic peptide (BNP) level alone is not adequate to establish presence of cor pulmonale, but it helps to diagnose cor pulmonale in conjunction with other noninvasive tests and in appropriate clinical settings. An elevated BNP level may actually be a natural mechanism to compensate for elevated pulmonary hypertension and right heart failure by promoting diuresis and natriuresis, vasodilating systemic and pulmonary vessels, and reducing circulating levels of endothelin and aldosterone.
A general approach to diagnose cor pulmonale and to investigate its etiology starts with routine laboratory tests, chest radiography, and electrocardiography. Echocardiography gives valuable information about the disease and its etiology. Pulmonary function tests may become necessary to confirm the underlying lung disease. Ventilation/perfusion (V/Q) scan or chest CT scan may be performed if history and physical examination suggest pulmonary thromboembolism as the cause or if other diagnostic tests do not suggest other etiologies. Right heart catheterization is the most accurate but invasive test to confirm the diagnosis of cor pulmonale and gives important information regarding the underlying diseases. Any abnormal result in each of these tests may need further diagnostic evaluation in that specific direction.
Imaging studies may show evidence of underlying cardiopulmonary diseases, pulmonary hypertension, or its consequence, right ventricular enlargement.
Chest roentgenography: In patients with chronic cor pulmonale, the chest radiograph may show enlargement of the central pulmonary arteries with oligemic peripheral lung fields. Pulmonary hypertension should be suspected when the right descending pulmonary artery is larger than 16 mm in diameter and the left pulmonary artery is larger than 18 mm in diameter. Right ventricular enlargement leads to an increase of the transverse diameter of the heart shadow to the right on the posteroanterior view and filling of the retrosternal air space on the lateral view. These findings have reduced sensitivity in the presence of kyphoscoliosis or hyperinflated lungs.
Echocardiography: Two-dimensional echocardiography usually demonstrates signs of chronic RV pressure overload. As this overload progresses, increased thickness of the RV wall with paradoxical motion of the interventricular septum during systole occurs. At an advanced stage, RV dilatation occurs and the septum shows abnormal diastolic flattening. In extreme cases, the septum may actually bulge into the left ventricular cavity during diastole resulting in decreased diastolic volume of LV and reduction of LV output.
Doppler echocardiography is now used to estimate pulmonary arterial pressure, taking advantage of the functional tricuspid insufficiency that is usually present in pulmonary hypertension. Doppler echocardiography is considered the most reliable noninvasive technique to estimate pulmonary artery pressure. The efficacy of Doppler echocardiography may be limited by the ability to identify an adequate tricuspid regurgitant jet, which may be further enhanced by using saline contrast.
Ventilation/perfusion (V/Q) lung scanning, pulmonary angiography, and chest CT scanning may be indicated to diagnose pulmonary thromboembolism as the underlying etiology of cor pulmonale. These tests may be performed early in the diagnostic workup if any evidence of pulmonary embolism appears in history and physical examination. The test may also be considered later in the workup if other tests are not suggestive of any other etiology. Pulmonary thromboembolism has a wide range of clinical presentations from massive embolism with acute and severe hemodynamic instability to multiple chronic peripheral embolisms that may present with cor pulmonale.
Ultrafast, ECG-gated CT scanning has been evaluated to study RV function. In addition to estimating right ventricular ejection fraction (RVEF), it can estimate RV wall mass. Its use is still experimental, but with further improvement, it may be used to evaluate the progression of cor pulmonale in the near future.
Magnetic resonance imaging (MRI) of the heart is another modality that can provide valuable information about RV mass, septal flattening, and ventricular function.9
Radionuclide ventriculography can determine RVEF noninvasively. Myocardial perfusion may also show a permanent increase in brightness of the right ventricle.10
Electrocardiography (ECG) abnormalities in cor pulmonale reflect the presence of RVH, RV strain, or underlying pulmonary disease. These electrocardiographic changes may include the following:
Right axis deviation
R/S amplitude ratio in V1 greater than 1 (increase in anteriorly directed forces may be a sign of posterior infarct)
R/S amplitude ratio in V6 less than 1
P-pulmonale pattern (an increase in P wave amplitude in leads 2, 3, and aVF)
S1 Q3 T3 pattern and incomplete (or complete) right bundle branch block, especially if pulmonary embolism is the underlying etiology
Low-voltage QRS because of underlying COPD with hyperinflation
This ECG shows some typical abnormalities that may be seen in cor pulmonale and other chronic pulmonary diseases: (1) R/S ratio >1 in V1 and
Advocate Health Care on October 31, 2010:
Etiologies of cor pulmonale can mechanistically be categorized in 5 groups.
Pulmonary vasoconstriction due to alveolar hypoxia or blood academia: This can result in pulmonary hypertension and if the hypertension is severe enough, it causes cor pulmonale.
Various lung disorders: Parenchymal or alveolar lung disorders may anatomically compromise the vasculature beds and cause elevated pulmonary blood pressure. Chronic obstructive pulmonary disorder is the most common cause of cor pulmonale. Other lung disorders that may cause cor pulmonale are pulmonary thromboembolism, interstitial lung disease, and adult respiratory distress syndrome. Also, some connective tissue disorders with pulmonary involvement may result in pulmonary hypertension and cor pulmonale.
Blood disorders that are associated with increased blood viscosity such as polycythemia vera, sickle cell disease, macroglobulinemia
Increased blood flow in the pulmonary vasculature
Idiopathic primary pulmonary hypertension
Klinikum und Fachbereich Medizin der Johann Wolfgang Goethe-Universität Frankfurt am Main on October 31, 2010:
Clinical manifestations of cor pulmonale generally are nonspecific. The symptoms may be subtle, especially in early stages of the disease, and mistakenly may be attributed to the underlying pulmonary pathology.
The patient may complain of fatigue, tachypnea, exertional dyspnea, and cough.
Anginal chest pain also can occur and may be due to right ventricular ischemia (it usually does not respond to nitrates) or pulmonary artery stretching.
Hemoptysis may occur because of rupture of a dilated or atherosclerotic pulmonary artery. Other conditions, such as tumors, bronchiectasis, and pulmonary infarction, should be excluded before attributing hemoptysis to pulmonary hypertension. Rarely, the patient may complain of hoarseness due to compression of the left recurrent laryngeal nerve by a dilated pulmonary artery.
A variety of neurologic symptoms may be seen due to decreased cardiac output and hypoxemia.
In advanced stages, passive hepatic congestion secondary to severe right ventricular failure may lead to anorexia, right upper quadrant abdominal discomfort, and jaundice.
Syncope with exertion, which may be seen in severe disease, reflects a relative inability to increase cardiac output during exercise with a subsequent drop in the systemic arterial pressure.
Elevated pulmonary artery pressure can lead to elevated right atrial, peripheral venous, and capillary pressure. By increasing the hydrostatic gradient, it leads to transudation of fluid and accumulation of peripheral edema. Although this is the simplest explanation for peripheral edema in cor pulmonale, other hypotheses explain this symptom, especially in a fraction of patients with COPD who do not show increase in right atrial pressure. A decrease in glomerular filtration rate (GFR) and filtration of sodium and stimulation of arginine vasopressin (which decreases free water excretion) due to hypoxemia play important pathophysiologic roles in this setting and may even have a role for peripheral edema in patients with cor pulmonale who have elevated right atrial pressure.6
Physical findings may reflect the underlying lung disease or pulmonary hypertension, RVH, and RV failure.
On inspection, an increase in chest diameter, labored respiratory efforts with retractions of the chest wall, distended neck veins with prominent a or v waves, and cyanosis may be seen.
On auscultation of the lungs, wheezes and crackles may be heard as signs of underlying lung disease. Turbulent flow through recanalized vessels in chronic thromboembolic pulmonary hypertension7 may be heard as systolic bruits in the lungs. Splitting of the second heart sound with accentuation of the pulmonic component can be heard in early stages. A systolic ejection murmur with sharp ejection click over the region of the pulmonary artery may be heard in advanced disease, along with a diastolic pulmonary regurgitation murmur. Other findings upon auscultation of the cardiovascular system may be third and fourth sounds of the heart and systolic murmur of tricuspid regurgitation.
RVH is characterized by a left parasternal or subxiphoid heave. Hepatojugular reflux and pulsatile liver are signs of RV failure with systemic venous congestion.
On percussion, hyperresonance of the lungs may be a sign of underlying COPD; ascites can be seen in severe disease.
Examination of the lower extremities reveals evidence of pitting edema. Edema in cor pulmonale is strongly associated with hypercapnia
Sanford Health Sioux Falls, SD on October 31, 2010:
Cor pulmonale is estimated to account for 6-7% of all types of adult heart disease in the United States, with chronic obstructive pulmonary disease (COPD) due to chronic bronchitis or emphysema the causative factor in more than 50% of cases.
At present, cor pulmonale accounts for 10-30% of decompensated heart failure related admissions in the United States.4
Although the prevalence of COPD in the United States is about 15 million, the exact prevalence of cor pulmonale is difficult to determine because it does not occur in all cases of COPD, and the physical examination and routine tests are relatively insensitive for the detection of pulmonary hypertension.
In contrast, acute cor pulmonale is usually secondary to massive pulmonary embolism.
Acute massive pulmonary thromboembolism is the most common cause of acute life-threatening cor pulmonale in adults.
In the United States, 50,000 deaths are estimated to occur per year from pulmonary emboli and about half occur within the first hour due to acute right heart failure.
The incidence of cor pulmonale varies among different countries depending on the prevalence of cigarette smoking, air pollution, and other risk factors for various lung diseases.
Development of cor pulmonale as a result of a primary pulmonary disease usually heralds a poorer prognosis. For example, patients with COPD who develop cor pulmonale have a 30% chance of surviving 5 years. However, whether cor pulmonale carries an independent prognostic value or is simply reflecting the severity of underlying COPD or other pulmonary disease is not clear.
Prognosis in the acute setting due to massive pulmonary embolism or ARDS has not previously been shown to be dependent on the presence or absence of cor pulmonale. However, a prospective, multicenter cohort study by Volschan et al indicates that in cases of pulmonary embolism, cor pulmonale may be a predictor of inhospital mortality.5 The authors collected demographic, comorbidity, and clinical manifestation data on 582 patients admitted to emergency or intensive care units and diagnosed with pulmonary embolism. Assessing the information using logistic regression analysis, the investigators built a prediction model. Their results indicated that in hemodynamically stable patients with pulmonary embolism, the following factors may be independent predictors of inhospital mortality:
Age over 65 years
Bed rest for longer than 72 hours
Chronic cor pulmonale
Centre Hospitalier Universitaire de Toulouse on October 31, 2010:
Cor pulmonale is defined as an alteration in the structure and function of the right ventricle caused by a primary disorder of the respiratory system. Pulmonary hypertension is the common link between lung dysfunction and the heart in cor pulmonale. Right-sided ventricular disease caused by a primary abnormality of the left side of the heart or congenital heart disease is not considered cor pulmonale, but cor pulmonale can develop secondary to a wide variety of cardiopulmonary disease processes. Although cor pulmonale commonly has a chronic and slowly progressive course, acute onset or worsening cor pulmonale with life-threatening complications can occur.1
Several different pathophysiologic mechanisms can lead to pulmonary hypertension and, subsequently, to cor pulmonale. These pathogenetic mechanisms include (1) pulmonary vasoconstriction due to alveolar hypoxia or blood acidemia, (2) anatomic compromise of the pulmonary vascular bed secondary to lung disorders (eg, emphysema, pulmonary thromboembolism, interstitial lung disease, adult respiratory distress syndrome, and rheumatoid disorders), (3) increased blood viscosity secondary to blood disorders (eg, polycythemia vera, sickle cell disease, macroglobulinemia), (4) increased blood flow in pulmonary vasculature, and (5) idiopathic primary pulmonary hypertension. The result is increased pulmonary arterial pressure.
The right ventricle (RV) is a thin-walled chamber that is more a volume pump than a pressure pump. It adapts better to changing preloads than afterloads. With an increase in afterload, the RV increases systolic pressure to keep the gradient. At a point, a further increase in the degree of pulmonary arterial pressure produces significant RV dilation, an increase in RV end-diastolic pressure, and RV circulatory collapse. A decrease in RV output with a decrease in diastolic left ventricle (LV) volume results in decreased LV output. Since the right coronary artery, which supplies the RV free wall, originates from the aorta, decreased LV output diminishes blood pressure in the aorta and decreases right coronary blood flow. What ensues is a vicious cycle between decreases in LV and RV output.
Genetic investigations have confirmed that morphogenesis of the right and left ventricle originated from different sets of progenitor cells and sites. This polymorphism could explain the differing rates of hypertrophy of the right and left ventricles.2
Right ventricular overload is associated with septal displacement toward the left ventricle. Septal displacement, which is seen on echocardiography, can be another factor that decreases LV volume and output in the setting of cor pulmonale and right ventricular enlargement. Several pulmonary diseases cause cor pulmonale, which may involve interstitial and alveolar tissues with a secondary effect on pulmonary vasculature or may primarily involve pulmonary vasculature. Chronic obstructive pulmonary disease (COPD) is the most common cause of cor pulmonale in the United States.
Cor pulmonale usually presents chronically, but 2 main conditions can cause acute cor pulmonale: massive pulmonary embolism (more common) and acute respiratory distress syndrome (ARDS). The underlying pathophysiology in massive pulmonary embolism causing cor pulmonale is the sudden increase in pulmonary resistance. In ARDS, 2 factors cause RV overload: the pathologic features of the syndrome itself and mechanical ventilation. Mechanical ventilation, especially higher tidal volume, requires a higher transpulmonary pressure. In chronic cor pulmonale, right ventricular hypertrophy (RVH) generally predominates. In acute cor pulmonale, right ventricular dilatation mainly occurs. In the case of ARDS, cor pulmonale is associated with increased possibility of right to left shunt through patent foramen ovale and caries poorer prognosis.
Klinikum Rechts der Isar on October 31, 2010:
Cor pulmonale is failure of the right side of the heart brought on by long-term high blood pressure in the pulmonary arteries and right ventricle of the heart.
Normally, the left side of the heart produces a higher blood pressure in order to pump blood to the body. The right side of the heart pumps blood through the lungs under much lower pressure.
Any condition that leads to prolonged high blood pressure in the arteries of the lungs (called pulmonary hypertension) puts a strain on the right side of the heart. When the right ventricle is unable to properly pump against these abnormally high pressures, it is called cor pulmonale.
Almost any chronic lung disease or condition causing prolonged low blood oxygen levels can lead to cor pulmonale. A few of these causes include:
Central sleep apnea
Chronic obstructive pulmonary disease (COPD)
Chronic thromboembolic pulmonary disease
Interstitial lung disease
Obstructive sleep apnea
Primary pulmonary hypertension
Pulmonary vascular disease
Secondary pulmonary hypertension
Chest discomfort, usually in the front of the chest
Shortness of breath
Swelling of the feet or ankles
Symptoms of underlying disorders (wheezing, coughing)
Exams and Tests
Abnormal fluid collection in the abdomen
Abnormal heart sounds
Bluish color to the skin (cyanosis)
Enlargement of the liver
Swelling (distension) of the neck veins, indicating high right-heart pressures
Swelling of the ankles
The following tests may help diagnose cor pulmonale:
Blood antibody tests
Blood test for brain natriuretic peptide (BNP)
CT scan of the chest
Lung biopsy (rarely performed)
Measurement of blood oxygen by arterial blood gas (ABG)
Pulmonary function tests
Right heart catheterization
Ventilation and perfusion scan of the lungs (V/Q scan)
Treatment is directed at the illness that is causing cor pulmonale. Supplemental oxygen may be prescribed to increase the level of oxygen in the blood.
There are many medicines available to treat cor pulmonale.
Bosentan or sildenafil may be given by mouth
Calcium channel blockers are often used to treat early cases
Prostacyclin may be given through injection or breathing in (inhalation)
Blood thinning (anticoagulant) medications may also be prescribed. Surgery may be needed to reverse heart defects that cause the condition. In very advanced cases, a heart and lung transplant may be advised.
The outcome depends on the cause of the condition. Giving oxygen often improves symptoms, stamina, and survival.
Treating primary pulmonary hypertension often leads to greater stamina and a longer life. In some cases, a lung transplant or heart-lung transplant can extend survival.
Progressive pulmonary hypertension and cor pulmonale may lead to:
Life-threatening shortness of breath
Severe fluid retention
When to Contact a Medical Professional
Call your health care provider if you experience shortness of breath or chest pain.
Avoiding behaviors that lead to chronic lung disease (especially cigarette smoking) may prevent the eventual development of cor pulmonale. Careful evaluation of childhood heart murmurs may prevent cor pulmonale caused by certain heart defects.
Right-sided heart failure
Institut de Recherches Cliniques de Montreal on October 31, 2010:
The symptoms of pulmonary heart disease depend on the stage of the disorder. In the early stages, one may have no symptoms but as pulmonary heart disease progresses, most individuals will develop the symptoms like:
Shortness of breath which occurs on exertion but when severe can occur at rest
Chronic wet cough
Swelling of the abdomen with fluid (ascites)
Swelling of the ankles and feet (pedal edema)
Enlargement or prominent neck and facial veins
Enlargement of the liver
Bluish discoloration of face
Presence of abnormal heart sounds
possible bi-phasic atrial response shown on an EKG due to hypertrophy
In many cases, the diagnosis of pulmonary heart disease is not easy as both the lung and heart disease can produce similar symptoms. Most patients undergo an ECG, chest X ray, echocardiogram, CT scan of the chest and a cardiac catheterization. During a cardiac catheterization, a small flexible tube is inserted from the groin and under x ray guidance images of the heart are obtained. Moreover the technique allows measurement of pressures in the lung and heart which provide a clue to the diagnosis.
Each year there are about 20,000 deaths and close to 280,000 hospital admissions among individuals who have pulmonary heart disease. The majority of individuals affected by pulmonary heart disease are women less than 65 years of age. Infants who are born with congenital heart disorders (esp. holes in the heart like a VSD) are prone to pulmonary artery disease. In infants the earlier the heart disease is treated, the better the prognosis. While pulmonary heart disease is serious, it is much less common than coronary artery disease.
Blood backs up into the systemic venous system, including the hepatic vein. Chronic congestion in the centrilobular region of the liver leads to hypoxia and fatty changes of more peripheral hepatocytes, leading to what is known as nutmeg liver.
Chest X-Ray - Right ventricular hypertrophy, right atrial dilatation, prominent pulmonary artery, peripheral lung fields show reduced vascular markings
ECG - Right ventricular hypertrophy - right axis deviation, prominent R wave in lead V1 & inverted T waves in right precordial leads
Echocardiogram - Right ventricular dilatation and tricuspid regurgitation is likely
Elimination of the cause is the most important intervention. Diuretics for RVF, In pulmonary embolism, thrombolysis (enzymatic dissolution of the blood clot) is advocated by some authorities if there is dysfunction of the right ventricle, and is otherwise treated with anticoagulants. In COPD, long-term oxygen therapy may improve cor pulmonale.
Cor pulmonale may lead to congestive heart failure (CHF), with worsening of respiration due to pulmonary edema, swelling of the legs due to peripheral edema and painful congestive hepatomegaly (enlargement of the liver due to tissue damage as explained in the Complications section. This situation requires diuretics (to decrease strain on the heart), sometimes nitrates (to improve blood flow), phosphodiesterase inhibitors such as sildenafil or tadalafil and occasionally inotropes (to improve heart contractility). CHF is a negative prognostic indicator in cor pulmonale.
Oxygen is often required to resolve the shortness of breath. Plus, oxygen to the lungs also helps relax the blood vessels and eases right heart failure. When wheezing is present, majority of the patients require bronchodilators. A variety of drugs have been developed to relax the blood vessels in the lung. Calcium channel blockers are used but only work in a few cases. Other novel medications that need to be inhaled or given intravenously include prostacyclin derivatives.
All patients with pulmonary heart disease are maintained on blood thinning medications to prevent formation of blood clots.
When medical therapy fails, one may require a transplant. However, since the lungs are damaged, both the heart and lungs needs to be transplanted. With a shortage of donors this therapy is only done 10-15 times a year in North America.
While not all lung diseases can be prevented one can reduce the risk of lung disease. This means avoiding or discontinuing smoking. Patients with end stage emphysema or chronic obstructive lung disease always end up with right heart failure. When working in environments where there are chemicals, wear masks to prevent inhalation of dust particles
Nara Prefectural University Hospital on October 31, 2010:
Cor pulmonale (Latin cor, heart + New Latin pulm?n?le, of the lungs) or pulmonary heart disease is enlargement of the right ventricle of the heart as a response to increased resistance or high blood pressure in the lungs.
Chronic cor pulmonale usually results in right ventricular hypertrophy (RVH), whereas acute cor pulmonale usually results in dilatation.
Hypertrophy is an adaptive response to a long-term increase in pressure. Individual muscle cells grow larger(in thickness) and change to drive the increased contractile force required to move the blood against greater resistance.
Dilatation is a stretching(in length) of the ventricle in response to acute increased pressure.
To be classified as cor pulmonale, the cause must originate in the pulmonary circulation system. Two major causes are vascular changes as a result of tissue damage (e.g. disease, hypoxic injury, chemical agents, etc.), and chronic hypoxic pulmonary vasoconstriction. RVH due to a systemic defect is not classified as cor pulmonale.
When untreated, cor pulmonale can lead to right-heart failure and death.
The heart and lung are intricately related. Whenever the heart is affected by disease, the lungs will follow and vice versa. Pulmonary heart disease is by definition a condition when the lungs cause the heart to fail.
The heart has two pumping chambers. The left ventricle pumps blood throughout the body. The right ventricle pumps blood to the lungs where it is oxygenated and returned to the left heart for distribution. In normal circumstances, the right heart pumps blood into the lungs without any resistance. The lungs usually have minimal pressure and the right heart easily pumps blood through.
However when there is lung disease present, like emphysema, chronic obstructive lung disease (COPD) or pulmonary hypertension- the small blood vessels become very stiff and rigid. The right ventricle is no longer able to push blood into the lungs and eventually fails. This is known as pulmonary heart disease. Pulmonary heart disease is also known as right heart failure or cor pulmonale. The chief cause of right heart failure is the increase in blood pressure in the lungs (pulmonary artery).
There are several mechanisms leading to pulmonary hypertension and cor pulmonale:
Anatomic changes in vascularization
Increased blood viscosity
Idiopathic or primary pulmonary hypertension
Massive pulmonary embolization
Exacerbation of chronic cor pulmonale
Increased pulmonary bloodpressure due to left ventricle insufficiency (Backward failure)
Loss of lung tissue following trauma or surgery
Pierre Robin sequence
End stage Pneumoconiosis
T1-4 Vertebral subluxation
Obstructive sleep apnea
Sickle cell anemia
Bronchopulmonary dysplasia (in infants)
Emory Healthcare on October 31, 2010:
Bronchiectasis is an irreversible widening (dilation) of portions of the breathing tubes or airways (bronchi) resulting from damage to the airway wall.
The most common cause is severe or repeated respiratory infections.
Most people develop a chronic cough, and some also cough up blood and have chest pain and recurrent episodes of pneumonia.
Chest x-rays are usually done to determine the extent and severity of the disorder.
People usually take antibiotics and drugs to suppress the build-up of mucus.
Bronchiectasis can result when conditions directly injure the bronchial wall or indirectly lead to injury by interfering with normal airway defenses. Airway defenses include tiny projections (cilia) on the cells that line the airways. These cilia beat back and forth, moving the thin liquid layer of mucus that normally coats the airways. Harmful particles and bacteria trapped in this mucus layer are moved up to the throat and coughed out or swallowed.
Whether airway injury is direct or indirect, areas of the bronchial wall are damaged and become chronically inflamed. The inflamed bronchial wall becomes less elastic, resulting in the affected airways becoming wider and flabby and developing small outpouchings or sacs that resemble tiny balloons. Inflammation also increases secretions (mucus). Because cells with cilia are damaged or destroyed, these secretions accumulate in the widened airways and serve as a breeding ground for bacteria. The bacteria further damage the bronchial wall, leading to a vicious circle of infection and airway damage.
In bronchiectasis, mucus production increases, the cilia are destroyed or damaged, and areas of the bronchial wall become chronically inflamed and are destroyed.
Bronchiectasis may affect many areas of the lung (diffuse bronchiectasis), or it may appear in only one or two areas (focal bronchiectasis). Typically, bronchiectasis causes widening of medium-sized airways, but often smaller airways become scarred and destroyed.
Complications: The inflammation and infection can extend to the small air sacs of the lungs (alveoli) and cause pneumonia, scarring, and a loss of functioning lung tissue. Severe scarring and loss of lung tissue can ultimately strain the right side of the heart as the heart tries to pump blood through the altered tissue. The right-sided heart strain can lead to a form of heart failure called cor pulmonale (see Pulmonary Hypertension:Pulmonary Hypertension).
Very severe cases of bronchiectasis, which occur more commonly in underdeveloped countries and in people who have advanced cystic fibrosis, may impair breathing enough to cause abnormally low levels of oxygen and high levels of carbon dioxide in the blood, a condition called respiratory failure (see Respiratory Failure and Acute Respiratory Distress Syndrome: Respiratory Failure).
The most common cause is severe or repeated respiratory infections. Other causes include
Immune deficiency disorders
Hereditary disorders, such as primary ciliary dyskinesia or cystic fibrosis, in which the ability to clear the airway of organisms that cause infection is impaired (see Cystic Fibrosis (CF): Cystic Fibrosis.)
Mechanical factors, such as airway obstruction caused by an inhaled object or a lung tumor
Inhaling toxic substances that injure the airways, such as noxious fumes, gases, smoke (including tobacco smoke), and injurious dust (for example, silica and coal dust)
Occasionally, a condition that affects larger airways, called allergic bronchopulmonary aspergillosis, occurs in people with asthma. Allergic bronchopulmonary aspergillosis is an allergic reaction to the Aspergillus species, which is a fungal organism. It can cause mucous plugs that obstruct the airways and lead to bronchiectasis (see Allergic and Autoimmune Diseases of the Lungs: Allergic Bronchopulmonary Aspergillosis).
Bronchiectasis can develop at any age, but the process often begins in early childhood. However, symptoms may not appear until much later. In most people, symptoms begin gradually, usually after a respiratory infection, and tend to worsen over the years. Most people develop a chronic cough that produces sputum. The amount and type of sputum depend on the extent of the disease and whether there is a complicating infection. Often, people have coughing spells only early in the morning and late in the day. Coughing up of blood (hemoptysis) is common because the damaged airway walls are fragile and have increased numbers of blood vessels. Hemoptysis may be the first or only symptom.
Recurrent fever or chest pain, with or without frequent bouts of pneumonia, may also occur. People with widespread bronchiectasis may develop wheezing or shortness of breath. People whose bronchiectasis progresses to cor pulmonale or respiratory failure also have fatigue, lethargy, and worsening shortness of breath, particularly with exertion.
Some Causes of Bronchiectasis
Bacterial infections, such as whooping cough or Staphylococcus
Fungal infections, such as aspergillosis
Mycobacterial infections, such as tuberculosis
Viral infections, such as influenza, adenoviral infection, respiratory syncytial virus infection, or measles
Enlarged lymph glands
Injury from noxious fumes, gases, or particles
Inhalation of stomach acid and food particles
Primary ciliary dyskinesia, including Kartagener's syndrome
Immunoglobulin deficiency syndromes
White blood cell dysfunction
Certain autoimmune or hyperimmune disorders, such as rheumatoid arthritis and ulcerative colitis
Drug abuse, such as heroin abuse
Human immunodeficiency virus (HIV) infection
Young's syndrome (obstructive azoospermia)
Yellow nail syndrome (with lymphedema)
Doctors may suspect bronchiectasis because of a person's symptoms or the presence (currently or in the past) of a condition thought to cause bronchiectasis. Tests are done to confirm the diagnosis and assess the extent and location of the disease. Chest x-rays can often detect the lung changes caused by bronchiectasis. However, occasionally, x-ray results are normal. Computed tomography (CT) is the most sensitive test to identify and confirm the diagnosis and to determine the extent and severity of the disease.
After bronchiectasis is diagnosed, tests are often done to check for disorders that may be causing or contributing to it. Such tests may include the following:
Measuring certain proteins in blood
Testing for HIV infection and other immune system disorders
Measuring the salt level in sweat (which is abnormal in people with cystic fibrosis)
Examining nasal, bronchial, or sperm specimens with a special microscope
Other tests to determine if the cilia are structurally or functionally defective
When bronchiectasis is limited to one area—for example, a lung lobe or segment—doctors may do a bronchoscopy (see Symptoms and Diagnosis of Lung Disorders: Bronchoscopy) to determine whether an inhaled foreign object or lung tumor is the cause. Other tests may be done to identify underlying disorders, such as allergic bronchopulmonary aspergillosis or tuberculosis.
Genetic testing for cystic fibrosis may be needed when there is a family history, repeated respiratory infections, or other unusual findings in a child or young adult, even when other typical features of cystic fibrosis are absent.
Early identification and treatment of conditions that tend to cause bronchiectasis may prevent its development or reduce its severity. More than half the cases of bronchiectasis in children can be accurately diagnosed and promptly treated.
Childhood immunizations against measles and whooping cough, improved living conditions, and good nutrition have markedly reduced the number of people who develop bronchiectasis. Annual influenza vaccines, pneumococcal vaccine, and use of appropriate antibiotics early in the course of lung infections help to prevent bronchiecta
City of Hope, Duarte California on October 31, 2010:
Bronchiectasis (pronounced bron-kee-ek'-tas-is) is a condition of the airways in the lungs. These airways are tube-like structures that branch from the trachea into the right and left lungs. When a person has bronchiectasis the airways are permanently and abnormally widened (dilated) and/or thickened. Mucus can collect in the abnormal airways. The mucus is difficult to clear because of the damage to the normal ways the airways clear the mucus.
Bronchiectasis is caused by one or more infectious insults to the lungs. This can lead to episodes of infection and then, worsening bronchiectasis. These insults are more likely to occur if there is impaired drainage of your airways or a defect in your immune defenses.
Early diagnosis and treatment of bronchiectasis is very important.
There are many causes of bronchiectasis - some are acquired and others you may be born with (congenital). The following is a list of the most important and/or common ones.
Infections can be a cause of bronchiectasis. They may also be a consequence which in turn can lead to worsening bronchiectasis. Examples include:
Viral infections (measles, adenovirus, influenza),
Bacterial infections (Pseudomonas aeruginosa, Staphylococcus aureus, Klebsiella),
Mycobacterial infections (tuberculosis, Mycobacterium avium complex) and
Fungal infections (histoplasmosis).
Immune diseases include:
Antibody deficiencies (e.g., common variable immunodeficiency) and
White blood cell dysfunctions (e.g., chronic granulomatous disease).
Chronic pulmonary aspiration is caused by the recurrent inhalation of oral or stomach material into your lungs. This can lead to post-inflammatory pnemonitis. The aspiration can occur from:
Impaired ability to swallow (oropharyngeal dysphagia) which may cause saliva or food to enter the lung.
Gastroesophageal reflux disease (GERD) which occurs when the valve of smooth muscle between the esophagus and the stomach does not function properly. This muscle band is called the lower esophageal sphincter.
Normally the lower esophageal sphincter acts as a guard to prevent stomach contents (acid and non-acid) from backing up into the esophagus. An increase of the pressure in the stomach and/or relaxation of the muscle tone of the valve may cause reflux to occur. The stomach contents may enter your lungs and irritate the airways. Some signs and symptoms of GERD include: heartburn, sour taste in mouth, or swallowing problems, but many (possibly up to 30%) of people with GERD (some with consequent bronchiectasis) may have no symptoms ("silent" GERD).
Rheumatoid arthritis, Sjogren's syndrome and Wegener's granulomatosis are examples of rheumatologic, autoimmune or connective tissue diseases that can cause bronchiectasis.
Impaired Drainage of the Airways
Impaired drainage of secretions can be due to cystic fibrosis (both classical and non-classical/atypical adult variants).
Impaired drainage can also be caused by diseases that affect the function of the cilia. The cilia are small hairs that line the airways and move together to clear secretions from the airways. Diseases such as primary ciliary dyskinesia, Kartagener's syndrome and Young's syndrome all have altered cilia function.
Obstruction of the Airways
Obstruction of the airways can be caused from a growth or tumor.
Chronic obstructive pulmonary disease (COPD) and allergic bronchopulmonary aspirgillosis are diseases that can cause obstruction of the airways.
Alpha-1 Antitrypsin Deficiency or Anomaly
Symptoms of bronchiectasis include a cough. The cough may be productive of mucus. With infections the mucus may be discolored, foul-smelling and may contain blood (hemoptysis). Shortness of breath, wheezing, weight loss, and fatigue can also occur. Some people with bronchiectasis also have chronic sinusitis. This requires further evaluation since bronchiectasis and sinusitis may be due to the same underlying disease.
If left untreated, symptoms of bronchiectasis may progress. Further symptoms may include increasing shortness of breath, worsening quality of life and even heart failure.
A multiple step process usually leads to the diagnosis of bronchiectasis. Many factors are considered and different tests are conducted. The evaluation for bronchiectasis often includes:
A complete medical history and physical examination by a healthcare provider.
A chest CT scan (a specialized X-ray which produces detailed slice-like pictures) of the lungs.
Breathing tests, called pulmonary function tests. These determine the presence and severity of abnormal airflow out of the lungs.
Specific screening or diagnostic tests for some of the possible underlying diseases that may cause bronchiectasis, based on the history and physical exam.
Treatment of any identified specific causes, including those listed under "Causes of Bronchiectasis" is important. Examples include:
Treatment of chronic infections such as non-tuberculous mycobacteria,
Treatment of immune diseases with immune globulin if appropriate,
Treatment of swallowing disorders and GERD leading to chronic pulmonary aspiration,
Prompt treatment or removal of any foreign object, growth or tumor causing obstruction of the airways, and
Treatment of other chronic lung diseases.
Treatment of Co-Morbid Conditions
Gastroesphageal Reflux Disease - In some people the muscle between the esophagus and stomach may not work well. This can allow some backflow of stomach contents (acid and non-acid) into the esophagus. This reflux response may lead to inflammation and then scarring with bronchiectasis. The Med Facts, Gastroesophageal Reflux Disease, discusses this topic in more detail.
Chronic Sinusitis - Sinusitis is an inflammation (swelling) of the mucous membranes that line the sinus cavities. This can interfere with normal sinus drainage and cause increased mucus production. Sinusitis and bronchiectasis are often associated with each other, and exacerbation of one can lead to a flare of the other. The Med Facts, Sinusitis, discusses this topic in more detail.
The management of bronchiectasis is long-term and is directed at:
Improving the clearance of sputum, also called bronchopulmonary hygiene,
Controlling infection (both prevention and treatment),
Identifying and treating co-morbid conditions often seen with bronchiectasis (such as GERD and chronic sinusitis),
Improving muscle strength and endurance through pulmonary rehabilitation and
Identifying the need for surgical resection of affected segments or lobes of the lung.
Your healthcare provider will evaluate your history and recommend the best management plan for you.
Bronchopulmonary Hygiene Therapy
Improved clearance of mucus is the cornerstone of the management of bronchiectasis and includes several components. They include:
Inhaled medication (bronchodilator and/or inhaled steroid) and
Airway clearance measures (oscillating positive expiratory pressure device, high-frequency chest wall oscillation vest).
Your healthcare provider may recommend one or more of them depending on your individual needs.
Inhaled Bronchodilators - An inhaled bronchodilator medication opens the airways by relaxing the smooth muscles around the airways. This type of medication is available in a number of inhaled forms. Commonly used inhaled short-acting bronchodilators include:
ProAir®, Proventil® HFA, Ventolin® HFA (albuterol)
Inhaled long-acting bronchodilators may also be used. They include:
Inhaled Steroids - Inhaled steroids reduce and prevent swelling inside the airways. Common inhaled steroids include:
Inhaled Steroid and Long-Acting Bronchodilator Combinations
Common combinations of inhaled steroid and long-acting bronchodilator include:
Advair® (Flovent® and Serevent®)
Symbicort® (Pulmicort® and Foradil®)
Airway Clearance Measures
Ochsner Health System on October 31, 2010:
Bronchiectasis is destruction and widening of the large airways.
If the condition is present at birth, it is called congenital bronchiectasis.
If it develops later in life, it is called acquired bronchiectasis.
Bronchiectasis is often caused by recurrent inflammation or infection of the airways. It most often begins in childhood as a complication from infection or inhaling a foreign object.
Cystic fibrosis causes about half of all bronchiectasis in the United States. Recurrent, severe lung infections (pneumonia, tuberculosis, fungal infections), abnormal lung defenses, and obstruction of the airways by a foreign body or tumor are some of the risk factors.
The condition can also be caused by routinely breathing in food particles while eating.
Symptoms often develop gradually, and may occur months or years after the event that causes the bronchiectasis.
They may include:
Bluish skin color
Chronic cough with large amounts of foul-smelling sputum
Clubbing of fingers
Coughing up blood
Cough that gets worse when lying on one side
Shortness of breath that gets worse with exercise
Exams and Tests
When listening to the chest with a stethoscope, the doctor may hear small clicking, bubbling, wheezing, rattling, or other sounds, usually in the lower lobes of the lungs.
Tests may include:
Aspergillosis precipitin test (to check for signs of the aspergillosis fungus)
Complete blood count (CBC)
PPD skin test to check for a prior tuberculosis infection
Serum immunoglobulin electrophoresis
Sweat test or other cystic fibrosis testing
Treatment is aimed at controlling infections and bronchial secretions, relieving airway obstruction, and preventing complications.
Regular, daily drainage to remove bronchial secretions is a routine part of treatment. A respiratory therapist can show the patient coughing exercises that will help.
Antibiotics, bronchodilators, and expectorants are often prescribed for infections.
Surgery to resect the lung may be needed if medicine does not work or if the patient has massive bleeding.
With treatment, most people can lead normal lives without major disability.
Coughing up blood
Low oxygen levels (in severe cases)
When to Contact a Medical Professional
Call your health care provider if:
Chest pain or shortness of breath gets worse
There is a change in color or amount of the phlegm you cough up, or if it is bloody
Other symptoms get worse or do not improve with treatment
The risk may be reduced if lung infections are promptly treated.
Childhood vaccinations and a yearly flu vaccine help reduce the chance of some infections. Avoiding upper respiratory infections, smoking, and pollution may also reduce your risk of infection.
Acquired bronchiectasis; Congenital bronchiectasis
Montreal Neurological Institute and Hospital on October 31, 2010:
Bronchiectasis may be the sequela of a variety of necrotizing infections that are either poorly treated or not treated at all and are not occurring in the setting of another associated condition. This was particularly common in developed countries prior to the widespread use of antibiotics26 and today remains an important cause of bronchiectasis in developing countries, where antibiotics are used inconsistently.14,15
Typical offending organisms that have been known to cause bronchiectasis include Klebsiella species, Staphylococcus aureus, Mycobacterium tuberculosis, Mycoplasma pneumoniae, nontuberculous mycobacteria, measles virus, pertussis virus, influenza virus, herpes simplex virus, and certain types of adenovirus.7,26
Infection with respiratory syncytial virus in childhood may also result in bronchiectasis.
MAC infection deserves special mention for its propensity to occur in the setting of human immunodeficiency virus (HIV) and in hosts who are immunocompetent.32 MAC infection has been observed especially in women who are nonsmokers; are older than 60 years; and have a consistent history, positive acid-fast bacilli on sputum smear, and a CT scan with small regular nodules and findings of bronchiectasis.8,11,23
Once a patient develops bronchiectasis, many of these same organisms colonize the damaged bronchi and may result in ongoing damage and episodic infectious exacerbations. The organisms found most typically include Haemophilus species (47-55% of patients) and Pseudomonas species (18-26% of patients).33,34
Although not a primary cause of bronchiectasis, patients with non-CF bronchiectasis often develop chronic bronchial infection with Pseudomonas aeruginosa via a mechanism involving biofilm formation and the release of virulence factors. This suggests that Pseudomonas species may promote disease progression and may be related to worsening lung function and increased morbidity and mortality.35
Focal postobstructive bronchiectasis may occur in a number of clinical settings (eg, endobronchial tumors, broncholithiasis, bronchial stenosis from infections, encroachment of hilar lymph nodes, foreign body aspiration).
Right-middle lobe syndrome is a specific type of bronchial obstruction that may result in bronchiectasis. It results from an abnormal angulation of the lobar bronchus at its origin, predisposing it to obstruction, subsequent infection, and development of bronchiectasis.
In adults, foreign body aspiration often takes place in the setting of altered mental status and involves unchewed food. Patients may also aspirate chewed materials from the stomach, including food, acid, and microorganisms.
After aspiration, a postobstructive pneumonia may occur, with subsequent development of focal bronchiectasis. Bronchiectasis may also develop in the setting of chronic aspiration. Further recognized is that a history of gastroesophageal reflux is a risk factor for aspiration and that the organism Helicobacter pylori may be playing a role in the development of bronchiectasis in this group of patients.36,37,38
CF and its variants are likely the most common cause of bronchiectasis in the United States and other industrialized nations. CF is an autosomal recessive disease affecting approximately 1 in 2,500 whites and 1 in 17,000 blacks in the United States.39 Estimates indicate 10,000 adults in the United States in 2005 would have CF, and this would comprise 40% of the total CF population.40
CF is a multisystem disorder that affects the chloride transport system in exocrine tissues, primarily secondary to a defect in the CF transmembrane regulator (CFTR) protein. Multiple genetic variants exist, and the importance of patients that have genetic heterozygous mutations remains to be elucidated. However, a reasonable assumption is that CF can be divided into 2 groups of patients: (1) those with classic disease that is readily diagnosed based on clinical and laboratory data and (2) those with less severe disease that manifests later in life and who have ambiguous genetic testing results.41,42,43
The major pulmonary finding in CF is bronchiectasis, which is an almost universal feature of this disease. It may be the sole feature of CF in adults or those with genetic variations of the disease.
Bronchiectasis associated with CF is believed to occur secondary to mucous plugging of proximal airways and chronic pulmonary infection, especially with mucoid P aeruginosa.44
Young syndrome is clinically similar to CF and may represent a genetic variant of the disease. It is most commonly seen in North American males and is a leading cause of male infertility.
Patients have bronchiectasis (often predominant in the lower lobes), sinusitis, and obstructive azoospermia, but they are not affected with the other findings of CF.
It is most often observed in middle-aged men. The pathogenesis of bronchiectasis is believed to be similar to that of CF. The criterion standard for diagnosis is electron microscopic analysis of the structure of the cilia.
Primary ciliary dyskinesia
Primary ciliary dyskinesia is a group of inherited disorders that may affect 1 in 15,000-30,000 persons. It is manifested by immotile or dyskinetic cilia and/or sperm. This may lead to poor mucociliary clearance, recurrent pulmonary infections, and, ultimately, bronchiectasis.46,47
A variant of this condition, initially described by Kartagener, encompassed the clinical triad of situs inversus, nasal polyps or sinusitis, and bronchiectasis in the setting of immotile cilia of the respiratory tract.48
Allergic bronchopulmonary aspergillosis49
Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity reaction to inhaled Aspergillus antigen that is characterized by bronchospasm, bronchiectasis, and immunologic evidence of a reaction to Aspergillus species.
ABPA should be suspected in patients with a productive cough who also have a long history of asthma-type symptoms that do not respond to conventional therapy.
Bronchiectasis is believed to be secondary to airway plugging by viscid secretions containing hyphae of Aspergillus species. The resulting bronchiectasis is thin-walled and affects the central and medium-sized airways.
CT scanning of the chest exhibits central airway bronchiectasis, differentiating this condition from other causes of bronchiectasis.
Other features of ABPA include eosinophilia, elevated immunoglobulin E (IgE) levels, and dramatic responses to corticosteroids.
Immunodeficiency states may occur in the setting of congenital and acquired immunodeficiency. The most common congenital conditions (albeit rare) involve B-lymphocyte functions, specifically hypogammaglobulinemia. The latter may involve an immunoglobulin G (IgG) subclass deficiency; X-linked agammaglobulinemia; or selective immunoglobulin A (IgA), immunoglobulin M (IgM), or IgE deficiency.50,51,52,53
Patients with hypogammaglobulinemia usually present in childhood with repeated sinus or pulmonary infections, although it has been diagnosed in adults who did not have a history of repeated infections. Establishing the diagnosis is important because gammaglobulin replacement may reduce the number of infections and resultant lung injury.
HIV disease, with resultant acquired immunodeficiency syndrome (AIDS), has been implicated in the development of bronchiectasis and demonstrates the accelerated bronchial damage that may occur from repeated infections in patients who are immunosuppressed. Bronchiectasis in HIV infection has occurred with and without obvious preceding pulmonary infection and may occur secondary to immunologic dysfunction from the HIV disease itself.32,54,55
Congenital anatomic defects and connective-tissue disorders
Bronchopulmonary sequestration is a congenital abnormality classified as either intralobar or extralobar and results in chronic lower respiratory tact infections that lead to bronchiectasis.
Williams-Campbell syndrome (congenital cartilage deficiency) is the absence of cartilage from lobar to first- to second-generation segmental airwa
Maine Medical Center on October 31, 2010:
Findings are nonspecific and may be attributed to other conditions. Most commonly, crackles, rhonchi, wheezing, and inspiratory squeaks may be heard upon auscultation. General findings may include digital clubbing, cyanosis, plethora, wasting, and weight loss. Nasal polyps and signs of chronic sinusitis may also be present. In advanced disease, the physical stigmata of cor pulmonale may be observed. Note the following:
Crackles and rhonchi are often observed in association with active infections and acute exacerbations.
Crackles are nonspecific and may occur in as many as 73% of patients.28
Scattered wheezing may be heard in approximately one third of patients. Wheezing may be due to airflow obstruction from secretions, destruction of the bronchial tree leading to airway collapsibility, or concomitant conditions.7,28
Digital clubbing is an inconsistent finding in approximately 2-3% of patients.28 It is more frequent in patients with moderate-to-severe bronchiectasis.
Cyanosis and plethora are rare findings secondary to polycythemia from chronic hypoxia.
Wasting and weight loss are suggestive of advanced disease but are not diagnostic of bronchiectasis.
In severe cases, findings are consistent with cor pulmonale. Right-sided heart failure may be observed, including peripheral edema, hepatomegaly, and hypoxia. This can ultimately lead to progressive respiratory failure.
Asklepios Klinikum Bad Abbach on October 31, 2010:
In clinical practice, the classic manifestations of bronchiectasis are cough and daily mucopurulent sputum production, often lasting months to years. Blood-streaked sputum or hemoptysis may result from airway damage associated with acute infection.
A rare variant known as dry bronchiectasis manifests by episodic hemoptysis with little-to-no sputum production. Dry bronchiectasis is usually a sequela of tuberculosis and is found in the upper lobes.
Although patients often report repetitive pulmonary infections that require antibiotics over several years, a single episode of a severe infection may result in bronchiectasis, often occurring in childhood.26 These include tuberculosis, pertussis, or severe bacterial pneumonia. Today, CF is the most common cause of bronchiectasis in children and young adults.7
Less specific symptoms include dyspnea, pleuritic chest pain, wheezing, fever, weakness, and weight loss.
Patients may relate multiple episodes of bronchitis or pulmonary infections, which are exacerbations of bronchiectasis and often require antibiotics. These acute bacterial infections are often heralded by the onset of increased sputum production over baseline, increased viscidity of sputum, and, occasionally, a foul odor of the sputum. Rarely, low-grade fever may occur.
Patients may experience an increase in generalized constitutional symptoms, such as fatigue and malaise, as well as increased dyspnea, shortness of breath, wheezing, or pleuritic pain.
In bronchiectasis, secondary infection, or poorly treated pneumonia, the discrete pathogens are often unknown, but most patients relate a history of childhood infections that may include tuberculosis, pertussis, or Mycoplasma species infection.26
Most individuals have never smoked (55%) or have smoked too little to account for their degree of cough, findings of obstruction on spirometry testing, and daily sputum production.
Bronchiectasis is a morphologic diagnosis and may exist with relatively few symptoms.
Chronic productive cough is prominent,27 occurring in up to 98% of patients. Sputum is typically produced on a daily basis in greater than 70% of patients, with one study reporting production in 96% of patients.28 Some patients only produce sputum with acute upper respiratory tract infections, but otherwise they have quiescent disease. Sputum is typically mucoid and without a rancid odor; however, during infectious exacerbations, sputum becomes purulent and may develop an offensive odor. In the past, total daily sputum amount has been used to characterize the severity of bronchiectasis, with less than 10 mL defined as mild bronchiectasis, 10-150 mL defined as moderate bronchiectasis, and greater than 150 mL defined as severe bronchiectasis. Today, bronchiectasis is most often classified by radiographic findings. In patients with CF, the volume of sputum produced is generally much greater than that associated with other etiologies of bronchiectasis.
Hemoptysis occurs in 56-92% of patients with bronchiectasis. Hemoptysis may be massive and life threatening secondary to bronchial artery bleeding.7,28,29 Hemoptysis is more commonly observed in dry bronchiectasis, although this presentation of bronchiectasis is rare. Hemoptysis is generally mild and manifested by blood flecks in the patient's usual purulent sputum. This is often the factor that leads patients to consult a physician. Bleeding usually originates from dilated bronchial arteries, which contain blood at systemic (rather than pulmonary) pressures. Therefore, massive hemoptysis may occur but is rarely a cause of death.
Dyspnea may occur in as many as 72% of patients but is not a universal finding. A 2006 review reported a rate of 62%.28 Dyspnea typically occurs in patients with extensive bronchiectasis observed on chest radiographs. Marked dyspnea is more likely to be secondary to a concomitant illness, such as chronic bronchitis or emphysema.
Wheezing is commonly reported and may be due to airflow obstruction following destruction of the bronchial tree. Similar to dyspnea, it may also be secondary to concomitant conditions such as asthma.
Pleuritic chest pain is an intermittent finding, occurring in 19-46% of patients.28 It is most commonly secondary to chronic coughing but also occurs in the setting of acute exacerbation.
Fatigue is commonly reported (73% of patients).28
Weight loss often occurs in patients with severe bronchiectasis. This is believed to be secondary to increased caloric requirements associated with the increased work of coughing and clearing secretions. Weight loss suggests advanced disease but is not diagnostic of bronchiectasis.
Fever may occur in the setting of acute infectious exacerbations.
Also of interest is that urinary incontinence occurs more frequently in women with bronchiectasis versus age-matched controls (47% vs 12%).30 The etiology of this is unclear.
King Chulalongkorn Memorial Hospital, Faculty of Medicine Chulalongkorn University on October 31, 2010:
In 1950, Reid characterized bronchiectasis as cylindrical, cystic, or varicose in nature.6
Cylindrical bronchiectasis involves diffuse mucosal edema, with resultant bronchi that are dilated minimally but have straight, regular outlines that end squarely and abruptly
Cystic or saccular bronchiectasis has ulceration with bronchial neovascularization and a resultant ballooned appearance that may have air-fluid levels
Varicose bronchiectasis has a bulbous appearance with a dilated bronchus and interspersed sites of relative constriction and, potentially, obstructive scarring. The latter may subsequently result in postobstructive pneumonitis and additional parenchymal damage
Currently no systematic data are available to detail the incidence or prevalence of bronchiectasis. A general theory is that the emergence of vaccines and antibiotics in the 20th century has resulted in a decline in the rate of bronchiectasis.7
The best data available suggest that the prevalence of bronchiectasis mirrors the socioeconomic conditions of the population under study, being significantly less prevalent in areas where immunizations and antibiotics are readily available. Bronchiectasis is relatively uncommon in the United States, with a prevalence of approximately 100,000 cases based on data from the 1980s. That said, the number of bronchiectasis cases in the United States that are associated with atypical mycobacteria or other environmental factors reportedly has increased.8,9,10,11
Bronchiectasis may be underdiagnosed because it is no longer included in survey data and often goes unreported. The exception is bronchiectasis associated with CF; the latter occurs with a prevalence of 1 in 2500 white births. CF is the largest single cause of chronic lung infections and bronchiectasis in industrialized nations.12 Native Americans in Alaska comprise a subgroup with higher than expected prevalence, with a 4-fold higher rate of bronchiectasis than the general population.13 Overall, identifying the true incidence remains a challenge, given the lack of specific symptoms and lack of readily available noninvasive screening tests for population studies.
Bronchiectasis remains a major cause of morbidity in less-developed countries, especially in countries with limited access to medical care and antibiotic therapy.14,15
Mortality is difficult to estimate given the difficulty in identifying prevalence and the lack of definitive studies. A study of 400 patients in 1940, prior to widespread antibiotic use, revealed a mortality rate of greater than 30%, with most patients dying within 2 years and at an age of younger than 40 years.16 A retrospective study in 1981, after the widespread use of antibiotics, reported a mortality rate of 13%.17
A more recent study from Finland identified 842 patients aged 35-74 years with bronchiectasis and followed them for 8-13 years. These patients were also compared with asthma and chronic obstructive pulmonary disease (COPD) controls. The mortality rate was not found to be significantly different among the 3 groups (bronchiectasis, asthma, COPD), with mortality rates of 28%, 20%, and 38% respectively.18,19
Currently, mortality is more often related to progressive respiratory failure and cor pulmonale than to uncontrolled infection. Life-threatening hemoptysis may also occur but is uncommon.
Additional complications include chronic bronchial infection, recurrent pneumonia, empyema, pneumothorax, and lung abscess. Amyloidosis and metastatic abscesses occurred in the preantibiotic era but are rarely observed today.
A more recent study looked at the risk factors on mortality for adults with non-CF bronchiectasis and reported that preventative care (ie, vaccinations), regular physician visits, and higher body mass index at baseline were associated with reduced mortality. A higher mortality was associated with advanced age, poor functional status, more severe disease based on radiographic findings, and evidence of hypoxemia or hypercapnia.20
No racial predilection exists other than those that may be associated with socioeconomic status.
Evidence suggests that non – CF-related bronchiectasis is more common and more virulent in women, particularly slender white women older than 60 years. In these patients, bronchiectasis is often caused by primary Mycobacterium avium complex (MAC) infection and has been called the Lady Windermere syndrome, named after a character in a novel by Oscar Wilde.21,22,23
In the preantibiotic era and in today's less-developed countries, symptoms usually began in the first decade of life. Today, the age of onset, except for those with CF, has moved into adulthood.24
Although limited, epidemiologic studies suggest that persons aged 60-80 years have the highest frequency of bronchiectasis—again likely from the rise in atypical mycobacterial infections. The differences in prevalence between age groups are a direct reflection of the differences in prevalence of the underlying causes of bronchiectasis, lung disease, and/or chronic infections.25
The eMedicine Pediatrics article Bronchiectasis may be of interest.
Roswell Park Cancer Institute on October 31, 2010:
Bronchiectasis is an uncommon disease that results in the abnormal and permanent distortion of one or more of the conducting bronchi or airways, most often secondary to an infectious process. First described by Laennec in 1819, later detailed by Sir William Osler in the late 1800s, and further defined by Reid in the 1950s, bronchiectasis has undergone significant changes in regard to its prevalence, etiology, presentation, and treatment.1
Bronchiectasis can be categorized as a chronic obstructive pulmonary lung disease manifested by airways that are inflamed and easily collapsible, resulting in air flow obstruction with shortness of breath, impaired clearance of secretions often with disabling cough, and occasionally hemoptysis. Severe cases can result in progressive impairment with respiratory failure.2,3
Bronchiectasis most often presents as (1) a focal process involving a lobe, segment, or subsegment of the lung or (2) a diffuse process involving both lungs. The former is by far the most common presentation of bronchiectasis, while the latter is most often associated with systemic illnesses, such as cystic fibrosis (CF), sinopulmonary disease, or both. The majority of this article will address non-CF related bronchiectasis.
Diagnosis is usually based on a compatible clinical history of chronic respiratory symptoms, such as a daily cough and viscid sputum production, and characteristic radiographic findings on CT scans, such as bronchial wall thickening and luminal dilatation.
Bronchiectasis is an abnormal dilation of the proximal and medium-sized bronchi (>2 mm in diameter) caused by weakening or destruction of the muscular and elastic components of the bronchial walls. Affected areas may show a variety of changes, including transmural inflammation, edema, scarring, and ulceration, among other findings. Distal lung parenchyma may also be damaged secondary to persistent microbial infection and frequent postobstructive pneumonia. Bronchiectasis can be congenital or acquired but is most often the latter.1
Congenital bronchiectasis usually affects infants and children and results from developmental arrest of the bronchial tree. The more commonly acquired forms occur in adults and older children and require an infectious insult, impairment of drainage, airway obstruction, and/or a defect in host defense. The tissue is also damaged in part by the host response of neutrophilic proteases, inflammatory cytokines, nitric oxide, and oxygen radicals. This results in damage to the muscular and elastic components of the bronchial wall. Additionally, peribronchial alveolar tissue may be damaged, resulting in diffuse peribronchial fibrosis.4
The result is abnormal bronchial dilatation with bronchial wall destruction and transmural inflammation. The most important functional finding of altered airway anatomy is severely impaired clearance of secretions from the bronchial tree.
Impaired clearance of secretions causes colonization and infection with pathogenic organisms, contributing to the common purulent expectoration observed in patients with bronchiectasis. The result is further bronchial damage and a vicious cycle of bronchial damage, bronchial dilation, impaired clearance of secretions, recurrent infection, and more bronchial damage
Malteser Krankenhaus St. Franziskus Hospital on October 31, 2010:
If your symptoms suggest bronchiectasis then the diagnosis can be confirmed by a CT scan. A CT scan is like a detailed X-ray test. A CT scan can determine the width of the bronchi. Widened bronchi seen on a CT scan confirm bronchiectasis.
Various other tests may be advised if an underlying cause is suspected.
What are the treatments for bronchiectasis?
Your doctor may advise one or more of the following. The treatment options chosen may depend on the severity of the condition.
Antibiotics are the mainstay of treatment. If you have mild bronchiectasis, you are likely to need a course of antibiotics every now and then to clear chest infections as and when they occur. Antibiotics may not be necessary if your sputum changes colour to green. But, if your cough or breathlessness gets worse or you start to feel generally unwell, your doctor may advise them.
If you have more severe bronchiectasis, chest infections may return quickly once you stop taking antibiotics. In this situation you may be advised to take antibiotics regularly to prevent infections from developing. One option for this is pulsed antibiotic treatment. This means regular short courses of antibiotics with breaks in treatment between the courses. Another option is to take antibiotics every day indefinitely. Recently, taking regular antibiotics by inhaler (nebuliser) rather than antibiotic tablets has become more popular. This is because it can deliver high doses of the antibiotic directly into the airways with relatively small amounts getting into the body. This reduces the problem of possible side-effects.
Physiotherapy and other exercise therapies
The aim of physiotherapy and other exercise therapies is to help you to cough up and clear the mucus and to improve your overall lung fitness. This may prevent a build-up of infected mucus, which may prevent chest infections. A physiotherapist will advise on the correct way to drain the affected areas. For example, it may mean that once or twice a day you should adopt a head down position and do some controlled breathing exercises to clear the build up of mucus. This is often combined with chest clapping by yourself, or by a relative, carer, or friend. It takes time and effort to do this properly and regularly. More recently, various devices have been designed which help to improve the drainage of mucus.
In addition, if you are able, a daily exercise activity such as running, brisk walking, swimming, dancing, aerobics, etc, may help to clear the mucus too.
Physiotherapy and exercise therapies have been a main treatment for many years. However, research studies to provide evidence in support of these therapies is variable and conflicting. More research is needed to confirm the place of these therapies.
Other medicines and immunisation
Some medicines that have an anti-inflammatory effect are sometimes advised. For example, medicines called leukotriene receptor antagonists may reduce the inflammation that occurs in the airways with bronchiectasis. Further research is needed to look into these other medicines.
Immunisation against the pneumococcus bacterium, and an annual 'flu jab' is advised. This reduces the chance of some types of chest infection developing.
Bronchodilator inhalers are commonly used in asthma to relax and open wide (dilate) the airways. They are also useful if the wheezing and breathlessness of bronchiectasis become severe (this is known as an acute attack). There are a number of types - for example, salbutamol inhaler. Steroid inhalers are no longer recommended for bronchiectasis unless there is also underlying asthma.
Your doctor may advise hospital admission if your symptoms become severe. Rapid breathing and chest pain are signs that your condition may not be responding to treatment at home.
Do not smoke
Smoking makes symptoms worse and smokers are very strongly advised to stop. Also, avoid passive smoking - that is, breathing in the smoke from people smoking nearby.
Treatment of underlying conditions
As mentioned above, about 4 in 10 people with bronchiectasis have an underlying cause. In some of these cases additional treatments may be advised, depending on the cause.
An operation may be an option if you have a small local area of lung damage causing symptoms. Cutting out the damaged airway may cure the problem. Surgery may be considered even if you have widespread bronchiectasis. This may be to cut out a particularly bad area of lung that is acting as a reservoir for mucus and infection. A lung transplant may be considered in severe cases.
What is the outlook (prognosis)?
Most people with bronchiectasis (with no underlying cause) have a good outlook. Symptoms in many affected people do not become severe. Treatment, in particular antibiotics when an infection occurs, or regularly when needed, keeps most people reasonably well.
The condition becomes worse in some cases and breathing problems may develop. In a small number of cases the condition becomes gradually worse over time as more and more of the airways become affected.
A life-threatening bleed from a damaged airway may also occur but is rare.
The outlook for people where bronchiectasis is part of another condition depends on the underlying cause.
Children's Mercy Hospitals and Clinics on October 31, 2010:
Bronchiectasis is an abnormal widening of one or more airways. Extra mucus is made in the abnormal airways which is prone to infection. The main symptom is a cough which produces a lot of sputum. Treatment often includes regular physiotherapy and courses of antibiotics. Long-term antibiotic treatment is needed in some cases. Inhalers are sometimes used. Surgery is occasionally needed. You should not smoke as smoking can make things worse. Immunisation against flu and pneumococcus are advised.
Air passes into the lungs via the windpipe (trachea) which divides into a series of branching airways called bronchi.
Air passes from the bronchi into millions of tiny air sacs (alveoli). Oxygen from the air is passed into the bloodstream through the thin walls of the alveoli.
Tiny glands in the lining of the airways make a small amount of mucus. The mucus keeps the airways moist but also traps any dust and dirt in the inhaled air. There are many tiny hairs (cilia) on the surface of the cells lining the airways. The millions of cilia lining the airways sweep the mucus to the back of the throat to form sputum (phlegm) which is swallowed. Coughing also helps to clear the airways.
What is bronchiectasis?
Bronchiectasis is a permanent abnormal widening (dilation) in one or more of the airways (bronchi). Extra mucus tends to form and pool in the parts of the airways that are widened. Widened airways with extra mucus are prone to infection.
The extent of bronchiectasis can vary greatly. There may be only one section of one airway that is widened and abnormal. At the other extreme, many airways may be widened. Many affected people fall somewhere between these extremes.
The widened parts of the airways are damaged and inflamed. This causes extra mucus to form which is less easily cleared. These parts of the airways are also more floppy and liable to collapse inwards which may affect airflow through the affected airways. The severity of symptoms depends on how many of the airways are affected and how badly.
The lung tissue next to a badly inflamed section of airway may also become inflamed and damaged.
What causes bronchiectasis?
The cause is often not clear and no cause can be found in over half of cases. An underlying cause is found in about 4 in 10 cases. Some conditions that affect or damage airways can cause bronchiectasis. Examples include the following:
Severe lung infections such as tuberculosis (TB), whooping cough, pneumonia or measles, which can damage the airways at the time of infection. Ongoing bronchiectasis may then develop.
Conditions which make a person prone to infections may also be a cause, such as deficiencies of the immune system.
Some inherited conditions. For example, a condition called primary ciliary dyskinesia affects the cilia so they do not beat correctly to clear the mucus. Cystic fibrosis is another condition that affects the lungs and causes bronchiectatic airways. Some rare immune problems can cause lung infections and damage to airways.
Inhaled objects, such as peanuts, can become stuck and block an airway. This may lead to local damage to that airway. Acid from the stomach, which is regurgitated and inhaled, can damage airways. Inhaling poisonous gases may also cause damage.
Some diseases that cause inflammation in other parts of the body can occasionally cause inflammation and damage in the bronchi and lead to bronchiectasis. For example: ulcerative colitis, Crohn's disease, coeliac disease, rheumatoid arthritis, systemic lupus erythematosus.
How common is bronchiectasis?
About one in a thousand people in the UK has bronchiectasis. In fact, the figure may be higher as it is thought that about a quarter of people diagnosed with chronic pulmonary obstructive disease (another long-term lung condition which used to be called chronic bronchitis) may actually have bronchiectasis.
What are the symptoms of bronchiectasis?
Coughing up lots of sputum is the main symptom. The amount of sputum can vary, depending on the severity. It can be very tiring to cough up large amounts of sputum each day.
Tiredness and poor concentration are common.
Wheeziness is common.
Some people become breathless, particularly when exercising or exerting themselves.
You may cough up some blood from an inflamed airway. This is typically just small amounts of blood now and then. Occasionally, a large amount of blood can be coughed up.
Some people with bronchiectasis also have chronic (persistent) sinusitis. This may cause an increase in mucus from the nose (catarrh).
Recurring chest infections are common. This is because an inflamed airway with extra mucus that does not clear easily is ideal for bacteria (germs) to grow and multiply. Sputum turns greeny/yellow when it is infected.
The severity of symptoms varies greatly. Some people have only mild symptoms and become used to an intermittent cough. They may have the occasional chest infection but a diagnosis of bronchiectasis is never made, or is made years after symptoms begin. At the other extreme, some people have severe symptoms with an almost permanent chest infection. Many people with bronchiectasis fall somewhere in between these extremes.
Leids Universitair Medisch Centrum on October 31, 2010:
Bronchiectasis (brong-ke-EK-ta-sis) is a condition in which damage to the airways causes them to widen and become flabby and scarred. The airways are tubes that carry air in and out of your lungs.
Bronchiectasis usually is the result of an infection or other condition that injures the walls of your airways or prevents the airways from clearing mucus. Mucus is a slimy substance that the airways produce to help remove inhaled dust, bacteria, and other small particles.
In bronchiectasis, your airways slowly lose their ability to clear out mucus. When mucus can't be cleared, it builds up and creates an environment in which bacteria can grow. This leads to repeated, serious lung infections.
Each infection causes more damage to your airways. Over time, the airways lose their ability to move air in and out. This can prevent enough oxygen from reaching your vital organs.
Bronchiectasis can lead to serious health problems, such as respiratory failure, atelectasis (at-eh-LEK-tah-sis), and heart failure.
Bronchiectasis can affect just one section of one of your lungs or many sections of both lungs.
The initial lung damage that leads to bronchiectasis often begins in childhood. However, symptoms may not appear until months or even years after you start having repeated lung infections.
In the United States, common childhood infections, such as whooping cough and measles, used to cause many cases of bronchiectasis. However, these causes are now less common due to the use of vaccines and antibiotics.
Now, in the United States, bronchiectasis usually is due to an underlying medical condition that injures the airway walls or prevents the airways from clearing mucus. Examples of such conditions include cystic fibrosis and primary ciliary dyskinesia (SIL-e-ar-e dis-ki-NE-ze-ah).
Bronchiectasis that occurs in only one part of the lung may be due to a blockage rather than an underlying condition.
Bronchiectasis can be congenital or acquired. Congenital bronchiectasis usually affects infants and children. It's the result of a problem with how the lungs form in a fetus.
Acquired bronchiectasis occurs as a result of another medical condition. It can affect adults and older children. This type of bronchiectasis is more common than the congenital type.
Bronchiectasis can't be cured. However, with proper care, most people who have it can enjoy a good quality of life.
Early diagnosis and treatment of bronchiectasis are important. The sooner your doctor can start treating your bronchiectasis and any underlying conditions, the better the chances of preventing further damage to your lungs.
Tricare Military Health System on October 31, 2010:
In order to prevent bronchiectasis, children should be immunized against measles, pertussis and other acute respiratory infections of childhood. While smoking has not been found to be a direct cause of bronchiectasis, it is certainly an irritant that all patients should avoid in order to prevent the development of infections (such as bronchitis) and further complications.
A healthy body mass index, vaccination (especially against pneumonia and influenza) and regular doctor visits may have beneficial effects on the prevention of progressing bronchiectasis. The presence of hypoxemia, hypercapnia, dyspnea level and radiographic extent can greatly affect the mortality rate from this disease.
Treatment of bronchiectasis includes controlling infections and bronchial secretions, relieving airway obstructions, removal of affected portions of lung by surgical removal or artery embolization and preventing complications. This includes the prolonged usage of antibiotics to prevent detrimental infections, as well as eliminating accumulated fluid with postural drainage and chest physiotherapy. Surgery may also be used to treat localized bronchiectasis, removing obstructions that could cause progression of the disease.
Inhaled steroid therapy that is consistently adhered to can reduce sputum production and decrease airway constriction over a period of time, and help prevent progression of bronchiectasis. One commonly used therapy is beclometasone dipropionate, which is also used in asthma treatment. Use of inhalers such as albuterol (salbutamol), fluticasone (Flovent/Flixotide) and ipratropium (Atrovent) may help reduce likelihood of infection by clearing the airways and decreasing inflammation.
Although not approved for use in any country, Mannitol dry inhalation powder, under the name Bronchitol, has been granted orphan drug status by the FDA for use in patients with bronchiectasis and with cystic fibrosis.
Combination therapies, long acting bronchodilators and inhaled corticosteroids such as Symbicort and Advair Diskus are also commonly used inhaled medicines which has in many cases been effective in clearing the airways, reducing sputum and reducing inflammation.
Rene Laënnec, the man who invented the stethoscope, used his invention to first discover bronchiectasis in 1819. The disease was researched in greater detail by Sir William Osler in the late 1800s; it is suspected that Osler actually died of complications from undiagnosed bronchiectasis
Avera Group on October 31, 2010:
Bronchiectasis is a disease state defined by localized, irreversible dilation of part of the bronchial tree. It is classified as an obstructive lung disease, along with emphysema, bronchitis and cystic fibrosis. Involved bronchi are dilated, inflamed, and easily collapsible, resulting in airflow obstruction and impaired clearance of secretions. Bronchiectasis is associated with a wide range of disorders, but it usually results from necrotizing bacterial infections, such as infections caused by the Staphylococcus or Klebsiella species or Bordetella pertussis.
People with bronchiectasis produce frequent green/yellow sputum (what is distinctive is that patients with bronchiectasis can fill 240ml (8 oz) glasses with their daily sputum production). There may be bad breath and, rarely, diarrhea (because patients with bronchiectasis often develop chronic pancreatitis).
The diagnosis of bronchiectasis is based on the review of clinical history and characteristic patterns in high-resolution CT scan findings. Such patterns include "tree-in-bud" abnormalities and cysts with definable borders. In one small study, CT findings of bronchiectasis and multiple small nodules were reported to have a sensitivity of 80%, specificity of 87%, and accuracy of 80% for the detection of bronchiectasis. Bronchiectasis may also be diagnosed without CT scan confirmation if clinical history clearly demonstrates frequent respiratory infections as well as confirmation of an underlying problem via blood work and sputum culture samples.
Bronchiectasis, gross pathology.
Bronchiectasis secondary to a large carcinoid tumor (not shown) that was completely obstructing the bronchus proximally. The yellowish discoloration of lung parenchyma reflects obstructive pneumonia.
Bronchiectasis has both congenital and acquired causes, with the latter more frequent.
Acquired Immune Deficiency Syndrome (AIDS) is the leading cause of bronchiectasis, especially in children. AIDS predisposes patients to a variety of pulmonary ailments, such as pneumonia and other opportunistic infections.
Tuberculosis is another major cause. Endobronchial tuberculosis commonly leads to bronchiectasis, either from bronchial stenosis or secondary traction from fibrosis.
Bronchiectasis can sometimes be an unusual complication of inflammatory bowel disease, especially ulcerative colitis. It can occur in Crohn's disease as well, but does so less frequently. Bronchiectasis in this situation usually stems from various allergic responses to inhaled fungus spores.
Recent evidence has shown an increased risk of bronchiectasis in patients with rheumatoid arthritis who smoke. One study stated a tenfold increased prevalence of the disease in this cohort. Still, it is unclear as to whether or not cigarette smoke is a specific primary cause of bronchiectasis.
Other acquired causes of bronchiectasis involving environmental exposures include respiratory infections, obstructions, inhalation and aspiration of ammonia and other toxic gases, pulmonary aspiration, alcoholism, heroin (drug use), various allergies, and allergic bronchopulmonary aspergillosis.
Kartagener syndrome, which affects the mobility of cilia in the lungs, aids in the development of the disease. Another common genetic cause is cystic fibrosis, in which a small number of patients develop severe localized bronchiectasis. Young's syndrome, which is clinically similar to cystic fibrosis, is thought to significantly contribute to the development of bronchiectasis. This is due to the occurrence of chronic, sinopulmonary infections. Patients with alpha 1-antitrypsin deficiency have been found to be particularly susceptible to bronchiectasis, for unknown reasons. Other less-common congenital causes include primary immunodeficiencies, due to the weakened or nonexistent immune system response to severe, recurrent infections that commonly affect the lung. Williams-Campbell syndrome can also lead to bronchiectasis.
Hospital Israelita Albert Einstein on October 31, 2010:
Lung abscess is treated with a combination of antibiotic drugs, oxygen therapy, and surgery. The antibiotics that are usually given for lung abscess are penicillin G, penicillin V, and clindamycin. They are given intravenously until the patient shows signs of improvement, and then continued in oral form. The patient may need to take antibiotics for a month or longer, until the chest x ray indicates that the abscess is healing. Oxygen may be given to patients who are having trouble breathing.
Most patients with lung abscess will not need surgery. About 5% of patients—usually those who do not respond to antibiotics or are coughing up large amounts of blood (500 mL or more)—may have emergency surgery for removal of the diseased part of the lung or for insertion of a tube to drain the abscess. Antibiotic treatment is considered to have failed if fever and other symptoms continue after 10-14 days of treatment; if chest x rays indicate that the abscess is not shrinking; or if the patient has pneumonia that is spreading to other parts of the lung.
Because lung abscess is a serious condition, patients need quiet and bed rest. Hospital care usually includes increasing the patient's fluid intake to loosen up the secretions in the lungs, and physical therapy to strengthen the patient's breathing muscles.
Patients with lung abscess need careful follow-up care after the acute infection subsides. Follow-up usually includes a series of chest x rays to make sure that the infection has cleared up. Treatment with antibiotics may continue for as long as four months, to prevent recurrence.
About 95% of lung abscess patients can be treated successfully with antibiotics alone. Patients who need surgical treatment have a mortality rate of 10-15%.
Some of the conditions that make people more vulnerable to lung abscess concern long-term lifestyle behaviors, such as substance abuse and lack of dental care. Others, however, are connected with chronic illness and hospitalization. Aspiration can be prevented with proper care of unconscious patients, which includes suctioning of throat secretions and positioning patients to promote drainage. Patients who are conscious can be given physical therapy to help them cough up material in their lungs and airways. Patients with weakened immune systems can be isolated from patients with pneumonia or fungal infection.
Healthline.com - Connect to Better Health
National Institutes of Health Clinical Center in Bethesda, Maryland on October 31, 2010:
Lung abscess is an acute or chronic infection of the lung, marked by a localized collection of pus, inflammation, and destruction of tissue.
Lung abscess is the end result of a number of different disease processes ranging from fungal and bacterial infections to cancer. It can affect anyone at any age. Patients who are most vulnerable include those weakened by cancer and other chronic diseases; patients with a history of substance abuse, diabetes, epilepsy, or poor dental hygiene; patients who have recently had operations under anesthesia; and stroke patients. In children, the most vulnerable patients are those with weakened immune systems, malnutrition, or blunt injuries to the chest.
Causes and symptoms
The immediate cause of most lung abscesses is infection caused by bacteria. About 65% of these infections are produced by anaerobes, which are bacteria that do not need air or oxygen to live. The remaining cases are caused by a mixture of anaerobic and aerobic (air breathing) bacteria. When the bacteria arrive in the lung, they are engulfed or eaten by special cells called phagocytes. The phagocytes release chemicals that contribute to inflammation and eventual necrosis, or death, of a part of the lung tissue. There are several different ways that bacteria can get into the lung.
Aspiration refers to the accidental inhalation of material from the mouth or throat into the airway and lungs. It is responsible for about 50% of cases of lung abscess. The human mouth and gums contain large numbers of anaerobic bacteria; patients with periodontal disease or poor oral hygiene have higher concentrations of these organisms. Aspiration is most likely to occur in patients who are unconscious or semi-conscious due to anesthesia, seizures, alcohol and drug abuse, or stroke. Patients who have problems swallowing or coughing, or who have nasogastric tubes in place are also at risk of aspiration.
The bronchi are the two branches of the windpipe that lead into the lungs. If they are blocked by tissue swelling, cancerous tumors, or foreign objects, a lung abscess may form from infection trapped behind the blockage. Spread of infection
About 20% of cases of pneumonia that cause the death of lung tissue (necrotizing pneumonia) will develop into lung abscess. Lung abscess can also be caused by the spread of other infections from the liver, abdominal cavity, or open chest wounds. Rarely, AIDS patients can develop lung abscess from Pneumocystis carinii and other organisms that take advantage of a weakened immune system.
Lung abscess is usually slow to develop. It may take about two weeks after aspiration or bronchial obstruction for an abscess to produce noticeable symptoms. The patient may be acutely ill for two weeks to three months. In the beginning, the symptoms of lung abscess are difficult to distinguish from those of severe pneumonia. Adults will usually have moderate fever (101-102°F/38-39°C), chills, chest pain, and general weakness. Children may or may not have chest pain, but usually suffer weight loss and high fevers. As the illness progresses, about 75% of patients will cough up foul or musty-smelling sputum; some also cough up blood.
Lung abscess can lead to serious complications, including emphysema, spread of the abscess to other parts of the lung, hemorrhage, adult respiratory distress syndrome, rupture of the abscess, inflammation of the membrane surrounding the heart, or chronic inflammation of the lung.
The diagnosis is made on the basis of the patient's medical history (especially recent operations under general anesthesia) and general health as well as imaging studies. Smears and cultures taken from the patient's sputum are not usually very helpful because they will be contaminated with bacteria from the mouth. The doctor will first use a bronchoscope (lighted tube inserted into the wind-pipe) to rule out the possibility of lung cancer. In some cases of serious infection, the doctor can use a fiberoptic bronchoscope with a protected specimen brush to take material directly from the patient's lungs, for identification of the organism. This technique is time-consuming and expensive, and requires the patient to be taken off antibiotics for 48 hours. It is usually used only to evaluate severely ill patients with weakened immune systems.
In most cases, the doctor will use the results of a chest x ray to help distinguish lung abscess from empyema, cancer, tuberculosis, or cysts. In patients with lung abscess, the x ray will show a thick-walled unified clear space or cavity surrounded by solid tissue. There is often a visible air-fluid level. The doctor may also order a CT scan of the chest, in order to have a clearer picture of the exact location of the abscess.
Blood tests cannot be used to make a diagnosis of lung abscess, but they can be useful in ruling out other conditions. Patients with lung abscess usually have abnormally high white blood cell counts (leukocytosis) when their blood is tested, but this condition is not unique to lung abscess.
Healthline.com - Connect to Better Health
Fondazione Salvatore Maugeri Clinica del Lavoro e della Riabilitazione on October 31, 2010:
Oxygen if required
Rehydration if indicated
Postural drainage with chest physiotherapy
Begin with intravenous treatment usually for about 2-3 weeks and follow with oral antibiotics for a further 4-8 weeks.
Recommended first-line therapy includes beta-lactam/beta-lactamase inhibitor or cephalosporin (second or third generation) plus clindamycin.3
Previously, therapy with a broad spectrum penicillin and clindamycin was used. Clindamycin had also been used alone (covers S. aureus and anaerobes and both oral and intravenous preparations exist); however, in the 1990s it was discovered that some anaerobes were resistant to both penicillin and clindamycin.
An alternative regimen is to begin with a broad spectrum cephalosporin and flucloxacillin.6
Regimen should be altered once organism is known.
If the condition fails to resolve, consider bronchoscopy/trans-thoracic drainage/cardiothoracic surgical intervention.7
Surgery is associated with a number of complications, such as empyema and bronchoalveolar air leak - especially so in children.6
Where slow resolution occurs, the possibility of malignancy or unusual organisms must be considered.
Overall 80%-90% cure rate with antibiotic therapy.7 Morbidity and mortality more likely to be associated with underlying pathology such as bronchial carcinoma. Other poor prognostic factors include pneumonia, reduced level of consciousness, anaemia and infection with P. aeruginosa, S. aureus and K. pneumoniae
Clínica Universitaria de Navarra on October 31, 2010:
Incidence and prevalence figures have not been established.
Post general anaesthesia
Penetrating pulmonary trauma1
Severe periodontal disease
Stroke/cerebral palsy/cognitive impairment/impaired consciousness leading to increased risk of aspiration
Immunosuppression, particularly chronic granulomatous disease in children
Congenital heart disease
Chronic lung disease, particularly cystic fibrosis
Hepatic abscess/chronic liver disease
Onset of symptoms is often insidious (more acute if following pneumonia)
Spiking temperature with rigors and night sweats
Cough ± phlegm production (frequently foul-tasting and foul-smelling and often blood-stained)
Pleuritic chest pain
Finger clubbing in chronic cases
Localised dullness to percussion (if consolidation also present or effusion)
Bronchial breathing and/or crepitations (if consolidation present)
Also look for signs of severe periodontal disease and infective endocarditis
Other causes of chest infection or pneumonia, e.g. TB and opportunistic mycobacteria
Neoplasia, e.g. cavitating bronchial carcinoma
Pulmonary infarction or pulmonary embolism
Vasculitis, e.g. Wegener's granulomatosis
Sarcoidosis with cavities
Infected bronchogenic cyst
FBC - normocytic anaemia or neutrophilia
Liver function tests
Blood cultures and sputum cultures (including AAFB)
ESR/C-reactive protein usually elevated
Chest X-ray - shows walled cavity, usually with a fluid level; may also be presence of an empyema or effusion
Tapping or draining of fluid or empyema with microbiology and cytology of samples
CT scan of thorax - may detect multiple small abscesses
Fibre optic bronchoscopy can exclude obstruction and provide samples for culture
Trans-thoracic biopsy/aspiration (usually with ultrasound guidance) or trans-tracheal biopsy
Peacehealth on October 31, 2010:
Severe, localised suppurative infection in the substance of the lung, associated with necrotic cavity formation. The process is usually surrounded by a fibrous reaction, forming the abscess wall. Multiple small abscess formation may occur and is sometimes referred to as necrotising pneumonia. The most frequent cause is aspiration of anaerobic organisms from the mouth in those predisposed to pulmonary aspiration, with impaired immune defences and cough reflex. A pneumonitis develops which progresses to abscess formation over a period of days or weeks.
Inhalation of foreign body
Bacteraemia seeding in the lungs
Tricuspid endocarditis leading to septic pulmonary embolus
Extension of hepatic abscess
Associated with bronchial carcinoma
Proximal to bronchial obstruction
Complication of severe or incompletely treated pneumonia (particularly staphylococci or klebsiellae)
Penetrating pulmonary trauma, e.g. stab wound
Note: lung abscesses may present acutely or more chronically.1
Types of lung abscesses
Primary abscess - occurs in previously normal lungs and may follow aspiration
Secondary abscess - occurs in patients with an underlying lung abnormality
Anaerobic bacteria are involved in about 90% of cases. About half of these are lone anaerobic infection; the other half are mixed with aerobes. Hospital-acquired infections show a higher proportion of Gram-negative bacteria and staphylococci. Similarly, community-acquired lung abscesses are caused mostly by multiple anaerobic bacteria with fewer aerobic organisms.2
Klebsiella pneumoniae - becoming more prevalent3
Burkholderia cepacia - particularly associated with cystic fibrosis
Pasteurella multocida - zoonotic infection from cats/dogs/cattle4
Burkholderia pseudomallei - soil-borne Asian/Australian infection; cases occurred following the tsunami disaster in 20045
Mycobacterial infections - predominantly tuberculosis (TB)
Fungi such as Aspergillus, Cryptococcus, Histoplasma, Blastomyces, Coccidioides species
Parasites such as Entamoeba histolytica, Paragonimus spp.
Santé Montérégie on October 31, 2010:
A lung abscess is a pus-filled cavity in the lung surrounded by inflamed tissue and caused by an infection.
A lung abscess is usually caused by bacteria that normally live in the mouth and are inhaled into the lungs.
Symptoms include fatigue, loss of appetite, sweating, fever, and a cough that brings up sputum.
Diagnosis is usually determined with a chest x-ray.
People usually need to take antibiotics for several weeks before a lung abscess disappears.
A lung abscess is usually caused by bacteria that normally live in the mouth or throat and that are aspirated into the lungs, resulting in an infection. Often, gum (periodontal) disease is the source of the bacteria that cause a lung abscess. The body has many defenses (such as a cough) to help prevent bacteria from getting into the lungs. Infection occurs primarily when a person is unconscious or very drowsy because of sedation, anesthesia, alcohol or drug abuse, or a disease of the nervous system. In people whose immune system functions poorly, a lung abscess may be caused by organisms that are not typically found in the mouth or throat, such as fungi or Mycobacterium tuberculosis (the organism that causes tuberculosis). Another cause of lung abscess is Staphylococcus aureus as well as methicillin-resistant Staphylococcus aureus (MRSA), which is a serious infection. This usually occurs in young, previously healthy adults or children, especially if they have influenza.
Obstruction of the airways also can lead to abscess formation. If the branches of the windpipe (bronchi) are blocked by a tumor or a foreign object, an abscess can form because secretions (mucus) can accumulate behind the tumor. Bacteria sometimes enter these secretions. The obstruction prevents the bacteria-laden secretions from being coughed back up through the airway.
Less commonly, abscesses result when bacteria or infected blood clots travel through the bloodstream to the lung from another infected site in the body (septic pulmonary emboli).
Usually, people develop only one lung abscess as a result of aspiration or airway obstruction. If several abscesses develop, they are usually in the same lung. When an infection reaches the lung through the bloodstream, however, many scattered abscesses may develop in both lungs. This problem is most common among people who inject drugs using dirty needles or unsterile methods.
Eventually, most abscesses rupture into an airway, producing a lot of sputum that gets coughed up. A ruptured abscess leaves a cavity in the lung that is filled with fluid and air. The cavity may become an inactive part of the lung, or it may require surgical removal. Sometimes an abscess ruptures into the space between the lungs and the chest wall (pleural space), filling the space with pus, a condition called empyema. Very rarely, if an abscess destroys a blood vessel wall, it may lead to serious bleeding.
Symptoms and Diagnosis
Symptoms most commonly start slowly. However, depending on the cause of the abscess, symptoms can occur suddenly. Early symptoms resemble those of pneumonia: fatigue, loss of appetite, sweating, fever, and a cough that brings up sputum. The sputum may be foul smelling (because bacteria from the mouth or throat tend to produce foul odors) or streaked with blood. People also may feel chest pain with breathing, especially if the lining on the outside of the lungs and inside of the chest wall (pleura) is inflamed (a condition called pleurisy—see Pleural Disorders: Symptoms). Many people have these symptoms for weeks or months before seeking medical attention. These people have chronic abscesses and, in addition to the other symptoms, lose a substantial amount of weight and have daily fever and night sweats. In contrast, lung abscesses caused by Staphylococcus aureus or MRSA can be fatal within days, sometimes even hours.
Chest x-rays nearly always reveal a lung abscess. However, how a lung abscess appears on an x-ray is sometimes similar to how other conditions, such as cancer, sarcoidosis, or Wegener's granulomatosis, appear on x-ray. Sometimes, an abscess is only found when computed tomography (CT) of the chest is done. Cultures of sputum may help identify the organism causing the abscess, but this test is usually not useful except for excluding MRSA, tuberculosis, and fungal infections.
Treatment requires antibiotics. Antibiotics are initially given intravenously in most cases and later by mouth when the person has improved and the fever has resolved. Antibiotic treatment continues until the symptoms disappear and a chest x-ray shows that the abscess has disappeared. Such improvement may require several weeks or even months of antibiotic therapy.
Bronchoscopy (see Symptoms and Diagnosis of Lung Disorders: Bronchoscopy) is often done when the abscess is thought to be the result of a tumor or a foreign object blocking the airway. Rarely, a lung abscess may have to be drained through a tube inserted through the chest wall and into the abscess, or infected lung tissue may have to be removed surgically. Sometimes an entire lobe of a lung or even an entire lung has to be removed.
Most people are cured. Treatment is less likely to be successful when the person is debilitated or has an impaired immune system, lung cancer, or a very large abscess.
H. Lee Moffitt Cancer Center & Research Institute on October 31, 2010:
Introduction of these pathogens into the lungs first causes inflammation, which leads to tissue necrosis and then abscess formation. The abscess usually ruptures into a bronchus, and its contents are expectorated, leaving an air- and fluid-filled cavity. In about one third of cases, direct or indirect extension (via bronchopleural fistula) into the pleural cavity results in empyema.
Cavitary pulmonary lesions are not always caused by infection. Noninfectious causes include the following:
Bullae with air-fluid level
Nodular silicosis nodule with central necrosis
Symptoms and Signs
Symptoms of abscess due to anaerobic bacteria or mixed anaerobic and aerobic bacteria are usually chronic (eg, over weeks or months) and include productive cough, fever, sweats, and weight loss. Severe prostration may occur. Sputum may be purulent or blood-streaked and classically smells or tastes foul. Symptoms of abscess due to aerobic bacteria develop more acutely and resemble bacterial pneumonia. Abscesses due to organisms other than anaerobes (eg, Mycobacteria , Nocardia) lack putrid respiratory secretions and may be more likely to occur in nondependent lung regions.
Signs of lung abscess, when present, are nonspecific and resemble those of pneumonia: decreased breath sounds indicating consolidation or effusion, temperature ? 38° C, crackles over the affected area, egophony, and dullness to percussion in the presence of effusion. Patients typically have signs of periodontal disease and a history of a predisposing cause of aspiration, such as dysphagia or a condition causing impaired consciousness.
CT as needed
Sputum cultures (unless anaerobic infection is very likely), including for fungi and mycobacteria
Bronchoscopy as needed to exclude cancer
Lung abscess is suspected based on history in a patient who is aspiration-prone due to altered consciousness or dysphagia and is confirmed by chest x-ray. In an anaerobic infection due to aspiration, chest x-ray classically shows consolidation with a single cavity containing an air-fluid level in portions of the lung that would be dependent when the patient is recumbent (eg, the posterior segment upper lobes or the superior or lateral basal segments of the lower lobes). This pattern helps distinguish anaerobic abscess from other causes of cavitary pulmonary disease, because diffuse or embolic pulmonary disease often causes multiple cavitations, and TB typically involves the apices.
CT is not routinely needed but may be useful when the x-ray suggests a cavitating lesion or when an underlying pulmonary mass obstructing the drainage of a lung segment is suspected.
Bronchial carcinoma can lead to obstruction that causes pneumonia and abscess formation. This should be suspected in smokers, recent smokers, and patients with unexplained cavitary lesions and no fever. Bronchoscopy is sometimes done to exclude cancer or the presence of a foreign body or to detect unusual pathogens, such as fungi.
Cultures: Anaerobic bacteria are rarely identifiable on culture because uncontaminated specimens are difficult to obtain and because most laboratories do not culture anaerobes well or often. If sputum is putrid, then anaerobic infection is assumed to be the cause. However, if empyema is present, pleural fluid provides a good source for anaerobic culture.
When clinical findings make anaerobic infection less likely, aerobic, fungal, or mycobacterial infection should be suspected, and attempts should be made to identify a pathogen. Cultures of sputum, bronchoscopic aspirates, or both are helpful. MRSA is generally found in both the sputum and blood cultures.
IV antibiotics or, for less seriously affected patients, oral antibiotics
Percutaneous drainage or surgery if empyema present or no response to antibiotics
Treatment is with antibiotics. Clindamycin
600 mg IV q 6 to 8 h is usually the drug of choice because it has excellent activity against streptococci and anaerobic organisms. The primary alternative is a combination ?-lactam/?-lactamase inhibitor (eg, ampicillin/sulbactam 1 to 2 g IV q 6 h, ticarcillin/clavulanate 3 to 6 g IV q 6 h, piperacillin/tazobactam 3 g IV q 6 h). Metronidazole
500 mg q 8 h may be used but must be combined with penicillin 2 million units q 6 h IV. Less seriously ill patients may be given oral antibiotics such as clindamycin
300 mg po q 6 h or amoxicillin/clavulanate 875/125 mg po q 12 h. IV regimens can be converted to oral ones when the patient defervesces. For very serious infections involving MSRA, the best treatment is vancomycin
Optimal duration of treatment is unknown, but common practice is to treat until the chest x-ray shows complete resolution, which generally takes 3 to 6 wk or longer. In general, the larger the abscess, the longer it will take for x-rays to demonstrate resolution.
Most authorities do not recommend chest physical therapy and postural drainage because they may cause spillage of infection into other bronchi with extension of the infection or acute obstruction. If the patient is weak or paralyzed or has respiratory failure, tracheostomy and suctioning may be necessary. Rarely, bronchoscopic aspiration helps facilitate drainage. An accompanying empyema must be drained. Percutaneous or surgical drainage of lung abscesses is necessary in the roughly 10% of patients in whom lesions do not respond to antibiotics. Resistance to antibiotic treatment is most common with large cavities and with infections that complicate obstructions.
When surgery is necessary, lobectomy is the most common procedure; segmental resection may suffice for small lesions (< 6 cm diameter cavity). Pneumonectomy may be necessary for multiple abscesses or for pulmonary gangrene unresponsive to drug therapy
Azienda Unità Sanitaria Locale di Modena on October 31, 2010:
Lung abscess is a necrotizing lung infection characterized by a pus-filled cavitary lesion. It is almost always caused by aspiration of oral secretions by patients who have impaired consciousness. Symptoms are persistent cough, fever, sweats, and weight loss. Diagnosis is based primarily on chest x-ray. Treatment usually is with clindamycin or combination ?-lactam/?-lactamase inhibitors.
Most lung abscesses develop after aspiration of oral secretions by patients with gingivitis or poor oral hygiene. Typically, patients have altered consciousness as a result of alcohol intoxication, illicit drugs, anesthesia, sedatives, or opioids. Older patients and those unable to handle their oral secretions, often because of neurologic disease, are also at risk.
A less common cause of lung abscess is necrotizing pneumonia that may develop from hematogenous seeding of the lungs due to suppurative thromboembolism (eg, septic embolism from IV drug use) or right-sided endocarditis. In contrast to aspiration, these conditions typically cause multiple rather than isolated lung abscesses.
The most common pathogens of lung abscesses due to aspiration are anaerobic bacteria, but about half of all cases involve both anaerobic and aerobic organisms (see Table 1: Lung Abscess: Infectious Causes of Cavitary Lung Lesions). The most common aerobic pathogens are streptococci and staphylococci—sometimes methicillin-resistant Staphylococcus aureus (MRSA). An unusual but very important acute and often lethal form of lung necrosis is caused by S. aureus with genes for Panton-Valentine leukocidin. Very serious and fulminant cases may be caused by MRSA (USA 300 strain), which has become a rare but very important cause of necrotizing pneumonia in young previously healthy adults and children. Occasionally, cases are due to gram-negative bacteria, especially Klebsiella. Immunocompromised patients with lung abscess may have infection with Nocardia, Mycobacteria sp, or fungi. Some people, especially those from developing countries, are at risk of abscess due to Mycobacterium tuberculosis, and rare cases are due to amebic infection (eg, with Entamoeba histolytica), paragonimiasis, or Burkholderia pseudomallei.
Society of Cardiovascular and Interventional Radiology on October 31, 2010:
Symptoms depend on whether the abscess is caused by anaerobic or other bacterial infection.
Anaerobic infection in lung abscess
Patients often present with indolent symptoms that evolve over a period of weeks to months.
The usual symptoms are fever, cough with sputum production, night sweats, anorexia, and weight loss.
The expectorated sputum characteristically is foul smelling and bad tasting.
Patients may develop hemoptysis or pleurisy
Other pathogens in lung abscess
These patients generally present with conditions that are more emergent in nature and are usually treated while they have bacterial pneumonia.
Cavitation occurs subsequently as parenchymal necrosis ensues.
Abscesses from fungi, Nocardia species, and Mycobacteria species tend to have an indolent course and gradually progressive symptoms.
The findings on physical examination of a patient with lung abscess are variable. Physical findings may be secondary to associated conditions such as underlying pneumonia or pleural effusion. The physical examination findings may also vary depending on the organisms involved, the severity and extent of the disease, and the patient's health status and comorbidities.
Patients with lung abscesses may have low-grade fever in anaerobic infections and temperatures higher than 38.5°C in other infections.
Generally, patients with in lung abscess have evidence of gingival disease.
Clinical findings of concomitant consolidation may be present (eg, decreased breath sounds, dullness to percussion, bronchial breath sounds, course inspiratory crackles).
The amphoric or cavernous breath sounds are only rarely elicited in modern practice.
Evidence of pleural friction rub and signs of associated pleural effusion, empyema, and pyopneumothorax may be present. Signs include dullness to percussion, contralateral shift of the mediastinum, and absent breath sounds over the effusion.
Digital clubbing may develop rapidly.
The bacterial infection may reach the lungs in several ways. The most common is aspiration of oropharyngeal contents.
Patients at the highest risk for developing lung abscess have the following risk factors:
Other patients at high risk for developing lung abscess include individuals with an inability to protect their airways from massive aspiration because of a diminished gag or cough reflex, caused by a state of impaired consciousness (eg, from alcohol or other CNS depressants, general anesthesia, or encephalopathy).
Infrequently, the following infectious etiologies of pneumonia may progress to parenchymal necrosis and lung abscess formation:
S aureus (may result in multiple abscesses)
An abscess may develop as an infectious complication of a preexisting bulla or lung cyst.
An abscess may develop secondary to carcinoma of the bronchus; the bronchial obstruction causes postobstructive pneumonia, which may lead to abscess formation.
Fox Chase Cancer Center on October 31, 2010:
Most frequently, the lung abscess arises as a complication of aspiration pneumonia caused by mouth anaerobes. The patients who develop lung abscess are predisposed to aspiration and commonly have periodontal disease. A bacterial inoculum from the gingival crevice reaches the lower airways, and infection is initiated because the bacteria are not cleared by the patient's host defense mechanism. This results in aspiration pneumonitis and progression to tissue necrosis 7-14 days later, resulting in formation of lung abscess.
Other mechanisms for lung abscess formation include bacteremia or tricuspid valve endocarditis, causing septic emboli (usually multiple) to the lung. Lemierre syndrome, an acute oropharyngeal infection followed by septic thrombophlebitis of the internal jugular vein, is a rare cause of lung abscesses. The oral anaerobe F necrophorum is the most common pathogen.
Because of the difficulty obtaining material uncontaminated by nonpathogenic bacteria colonizing the upper airway, lung abscesses rarely have a microbiologic diagnosis.
Published reports since the beginning of the antibiotic area have established that anaerobic bacteria are the most significant pathogens in lung abscess. In a study by Bartlett et al in 1974, 46% of patients with lung abscesses had only anaerobes isolated from sputum cultures, while 43% of patients had a mixture of anaerobes and aerobes.1 The most common anaerobes are Peptostreptococcus species, Bacteroides species, Fusobacterium species, and microaerophilic streptococci.
Aerobic bacteria that may infrequently cause lung abscess include Staphylococcus aureus, Streptococcus pyogenes, Streptococcus pneumoniae (rarely), Klebsiella pneumoniae, Haemophilus influenzae, Actinomyces species, Nocardia species, and gram-negative bacilli.
Challenging current expert opinion, a study by Wang et al suggested that the bacteriologic characteristics of lung abscess have changed.2 In a series of 90 patients with community-acquired lung abscess in Taiwan, anaerobes were recovered from just 28 patients (31%); the predominant bacterium was K pneumoniae, in 30 patients (33%). Another significant finding was that the rate of resistance of anaerobes and Streptococcus milleri to clindamycin and penicillin increased compared with previous reports.
Nonbacterial and atypical bacterial pathogens may also cause lung abscesses, usually in the immunocompromised host. These microorganisms include parasites (eg, Paragonimus and Entamoeba species), fungi (eg, Aspergillus, Cryptococcus, Histoplasma, Blastomyces, and Coccidioides species), and Mycobacterium species.
The frequency of lung abscess in the general population is not known.
Most patients with primary lung abscess improve with antibiotics, with cure rates documented at 90-95%.
Host factors associated with a poor prognosis include advanced age, debilitation, malnutrition, human immunodeficiency virus infection or other forms of immunosuppression, malignancy, and duration of symptoms greater than 8 weeks.3 The mortality rate for patients with underlying immunocompromised status or bronchial obstruction who develop lung abscess may be as high as 75%.4
Aerobic organisms, frequently hospital acquired, are associated with poor outcomes. A retrospective study reported the overall mortality rate of lung abscesses caused by mixed gram-positive and gram-negative bacteria at approximately 20%.5
A male predominance for lung abscess is reported in published case series.
Lung abscesses likely occur more commonly in elderly patients because of the increased incidence of periodontal disease and the increased prevalence of dysphagia and aspiration. However, a case series from an urban center with high prevalence of alcoholism reported a mean age of 41 years.
Albany Medical Center on October 31, 2010:
Lung abscess is defined as necrosis of the pulmonary tissue and formation of cavities containing necrotic debris or fluid caused by microbial infection. The formation of multiple small (
Hirslanden Privatklinikgruppe on October 31, 2010:
Signs and symptoms
Onset of symptoms is often gradual, but in necrotizing staphylococcal or gram-negative bacillary pneumonias patients can be acutely ill. Cough, fever with shivering and night sweats are often present. Cough can be productive with foul smelling purulent sputum (?70%) or less frequently with blood (i.e. hemoptysis in one third cases) . Affected individuals may also complain of chest pain, shortness of breath, lethargy and other features of chronic illness.
Patients are generally cachectic at presentation. Finger clubbing is present in one third of patients. Dental decay is common especially in alcoholics and children. On examination of chest there will be features of consolidation such as localised dullness on percussion, bronchial breath sound etc.
Pathology image of a lung abscess.
Chest Xray and other imaging studies
Abscess is often unilateral and single involving posterior segments of the upper lobes and the apical segments of the lower lobes as these areas are gravity dependent when lying down. Presence of air-fluid levels implies rupture into the bronchial tree or rarely growth of gas forming organism.
Raised inflammatory markers (high ESR, CRP) are usual but not specific. Examination of sputum is important in any pulmonary infections and here often reveals mixed flora. Transtracheal of Transbronchial (via bronchoscopy) aspirates can also be cultured. Fibre optic bronchoscopy is often performed to exclude obstructive lesion; it also helps in bronchial drainage of pus.
Broadspectrum antibiotic to cover mixed flora is the mainstay of treatment. Pulmonary physiotherapy and postural drainage are also important. Surgical procedures are required in selective patients for drainage or pulmonary resection.
Rare nowadays but include spread of infection to other lung segments, bronchiectasis, empyema, and bacteraemia with metastatic infection such as brain abscess.
Most cases respond to antibiotic and prognosis is usually excellent unless there is a debilitating underlying condition. Mortality from lung abscess alone is around 5% and is improving.
Universitätsklinikum Hamburg Eppendorf on October 31, 2010:
Lung abscess is necrosis of the pulmonary tissue and formation of cavities (more than 2 cm) containing necrotic debris or fluid caused by microbial infection.
This pus-filled cavity is often caused by aspiration, which may occur during altered consciousness. Alcoholism is the most common condition predisposing to lung abscesses.
Lung abscess is considered primary (60%) when it results from existing lung parenchymal process and is termed secondary when it complicates another process e.g. vascular emboli or follows rupture of extrapulmonary abscess into lung.
Conditions contributing to lung abscess
Aspiration of oropharyngeal or gastric secretion
Vasculitis: Wegener's granulomatosis
Necrotizing tumors: 8% to 18% are due to neoplasms across all age groups, higher in older people; primary squamous carcinoma of the lung is the commonest.
In the post-antibiotic era pattern of frequency is changing. In older studies anaerobes were found in up to 90% cases but they are much less frequent now.
Anaerobic bacteria: Peptostreptococcus, Bacteroides, Fusobacterium species,
Microaerophilic streptococcus : Streptococcus milleri
Aerobic bacteria: Staphylococcus, Klebsiella, Haemophilus, Pseudomonas, Nocardia, Escherichia coli, Streptococcus, Mycobacteria
Fungi: Candida, Aspergillus
Parasites: Entamoeba histolytica,
Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo on October 31, 2010:
The Pleura is a sac surrounding the lungs. It consists of two layers, one covering the lung (visceral), while the other covers the inside of the chest wall or rib cage (parietal). Between the two layers is a space in which a small amount of fluid circulates to lubricate the two surfaces as they slide over one another during breathing.
Pleurisy refers to the inflammation (irritation, swelling, stickiness) of the pleura. Pleurisy is not a disease, but a symptom of another condition (e.g., virus or bacterial infection).
Inflammation of the pleura can lead to fluid build up (between the two layers) known as pleural effusion. During inflammation, sticky, fibrous material accumulates over the pleural surfaces, resulting in the layers rubbing against (friction rub) each other during breathing, and causing chest pain. Sometimes the inflammation is dry (i.e., dry pleurisy).
Shortness of breath
Sharp chest pain with breathing. Pain can limit the movement on the side of the chest with pleurisy.
Rapid shallow breaths
Inability to take a deep breath due to chest pain
Connective tissue diseases:
Systemic Lupus Erythematosus
Liver failure -- Cirrhosis
Kidney or renal failure -- uremia, nephritic syndrome
- Pulmonary Emboli -- blood clot to the lungs
Body temperature may be elevated
Normal chest expansion during respiration is affected.
Listening to the chest with a stethoscope, the doctor will hear the friction rub (i.e., creaky, coarse, rubbing sound).
The heart sound and breath sounds may be muffled due to presence of effusion.
Blood tests may show evidence of infection -- elevated number of white blood cells and ESR.
Blood may show evidence of cancer -- elevation of CA-125 (Ovarian Cancer), CA-19-9 (Pancreatic Cancer and others)
Blood may show organ failure:
High BUN/Creatinine -- Kidney failure
High liver or pancreatic enzymes
Low oxygen levels
Chest X-Ray -- Pneumonia, asbestos
CAT scan (using computers) and Ultrasound (using sound) can show fluid or effusion.
Thoracentesis -- done by inserting a needle into the pleura. The removed effusion can then be sent to a laboratory for analysis.
Treat underlying disorder
Oxygen, if levels are low
Aspirin and other NSAIDs (e.g., Ibuprofen, Indocin, etc.) are effective in reducing the inflammation, fever, and pain.
Painkillers such as codeine can help.
In severe pain, a nerve block is performed using a numbing agent (e.g., Xylocaine) that is injected into the nerves between the ribs for temporary relief of pain.
Therapeutic Thoracentesis is done to remove the effusion, which helps breathing.
Contact a physician immediately. Call 911 if you have high fever, pain, or difficulty breathing.
National Hospital Organization on October 31, 2010:
Causes | Symptoms | Wet pleurisy | Dry pleurisy | Treatment
Pleurisy is an inflammation of the pleura- a two-ply membrane that both encloses the lung and lines the chest cavity.
People have two pleurae, one around each lung. The pleurae act as a protective wrapping, fitting snugly over your lungs. Pleurae are made up of two layers. Normally, there is no space between the inner and outer layer. The layers are joined at the edges, so that the pleura might be compared to a closed balloon, completely empty of air and wrapped tightly around the outside of each of the lungs.
Normally, there is nothing but a thin layer of Iubricating layer of fluid between the inner pleural lining and the outer one. The smooth pleura linings and lubricating fluid allow your lungs to move freely in your chest, as they do in normal breathing.
In people with pleurisy, the two layers of pleura get inflamed (red & swollen). This can create a space between the layers called the pleural cavity (cavity means space). In wet pleurisy, this space can fill up with fluid that can get infected.
What causes pleurisy?
Pleurisy can arise from various causes and take various ways to develop, sometimes with excess fluid in the pleural cavity ("wet pleurisy") and sometimes without ("dry pleurisy"), sometimes accompanied by no pain sometimes very painful.
There are two kinds of pleurisy. A "primary" pleurisy is an inflammation arising in the pleural tissues themselves, from a germ that attacked them directly, or perhaps from an injury or growth. A "secondary" pleurisy is an added effect from some other chest disease - pneumonia, for instance - in which the germs reach the pleura as well as the lungs themselves, or tuberculosis, or lung abscess, or tumour of the lung; almost anything wrong in the chest.
The symptoms and course of primary and secondary pleurisy may be exactly the same, with only the cause different.
Back to Top
Symptoms of pleurisy
People with pleurisy may experience:
pain: pain during each breath, pain when you move, and extreme pain when you cough or sneeze
shallow and difficult breathing
loss of appetite
These symptoms could be cased by pleurisy, or they could be caused by another disease; only a doctor can tell. If you have these symptoms, or if you think you have pleurisy, see your doctor right away.
Back to Top
How pleurisy happens
Pleurisy may be acute - appearing, giving trouble for a period, and then disappearing. It may be chronic - hanging on, the pain steady or recurring often, the interference with breathing and other normal activity an enduring burden. Whether acute or chronic, pleurisy can be either dry or wet, painful or free of pain.
Dry pleurisy is an inflammation that has not formed fluid. It can be very painful, especially when the swollen outer layer is stretched on breathing. A grating sensation may be distinctly felt by the victim when the two layers, both perhaps stiffened and swollen, rub against each other. Oddly enough, however, it is only the outer lining, next to the chest wall, that feels pain. The one next to the lung has no pain nerves.
Again, the inflammation of dry pleurisy may subside and the pain go away, even though the grating of the roughened tissues may still be felt. Or it may turn into wet pleurisy, with the accumulation of fluid. The fluid may be absorbed and become a dry pleurisy again.
Back to Top
Wet pleurisy (pleurisy with effusion) involves fluid that may arise from any of several causes. One may be the inflammation itself, which causes an outpouring of blood and lymph (supplementary body fluid). A chest injury with bleeding is an example of another possible source of fluid.
Back to Top
The effects of pleurisy
When dry pleurisy heals, it leaves strands of fibrous tissue (adhesions) strung between the lung and the wall of the chest, tying them together. Sometimes these adhesions are so extensive that they limit the movement of the lungs. But usually the soreness disappears and the adhesions stretch so much that they no longer cause any difficulty.
In wet pleurisy, the fluid builds up in the pleural cavity. There may be enough to restrict the movement of the lungs and therefore the ability to breathe. On the other hand, the increasing fluid may separate the linings so that movement of the chest wall and of the sensitive outer lining is limited - causing pain to subside.
A large amount of fluid displaces the heart as well as the Iung. The lung may remain compressed or displaced and fail to return to its full capacity because of thickening of the pleura after the fluid has been absorbed.
Fluid that is relatively clear may give little trouble and be readily absorbed in time. But if the fluid becomes infected - as it may do - it turns into pus and may lead to further complications. This condition, called "empyema," is very serious.
The pus sometimes breaks through the lung wall and into the air passages, where it may be coughed up. Removal of the pus by drainage is usually needed for the patient to recover.
Back to Top
How is pleurisy treated?
The inflammation of pleurisy is treated by attacking the infection that may have caused it. A pleurisy caused by some lung disease is treated, first of all, by identifying the underlying disease and giving whatever treatment is available for it.
To limit the pain of pleurisy, limiting the movement of the lungs may be desirable. The doctor may suggest lying on the sore side in a special way - for example, on a firm surface - to limit breathing movement on that side enough to reduce the stretching of the sore tissues and therefore the pain. He may also prescribe medication for the pain itself.
For dry pleurisy, such treatment is generally enough. In wet pleurisy, the doctor may decide to remove the fluid by drawing it out with a needle. Timing of this is important, since the fluid may come back if it is removed too soon. On the other hand, if it's not done early enough dense adhesions may form, resulting in permanent breathing difficulty.
Hospital for Sick Children on October 31, 2010:
Pleurisy is inflammation of the lining of the lungs and chest (the pleura) that leads to chest pain (usually sharp) when you take a breath or cough.
Pleurisy may develop when you have lung inflammation due to infections such as pneumonia or tuberculosis. It is often a sign of a viral infection of the lungs. This inflammation also causes the sharp chest pain of pleurisy.
It may also occur with:
The main symptom of pleurisy is pain in the chest. This pain most likely occurs when you take a deep breath in or out, or cough. Some people feel the pain in the shoulder.
Deep breathing, coughing, and chest movement makes the pain worse.
Pleurisy can cause fluid to collect inside the chest cavity. This can make breathing difficult and may cause the following symptoms:
Bluish skin color (cyanosis)
Shortness of breath
Rapid breathing (tachypnea)
Exams and Tests
When you have pleurisy, the normally smooth lining of the lung (the pleura) become rough. They rub together with each breath, and may produce a rough, grating sound called a "friction rub." The health care professional can hear this sound with the stethoscope, or by placing an ear against the chest.
The health care provider may perform the following tests:
Ultrasound of the chest
X-ray of the chest
The health care provider can remove fluid in the lungs by thoracentesis and check it for signs of infection.
Treatment depends on what is causing the pleurisy. Bacterial infections are treated with antibiotics. Some bacterial infections require a surgical procedure to drain all the infected fluid.
Viral infections normally run their course without medications. Patients often can control the pain of pleurisy with acetaminophen or anti-inflammatory drugs such as ibuprofen.
Recovery depends on what is causing the pleurisy.
Collapsed lung due to thoracentesis
Complications from the original illness
When to Contact a Medical Professional
Call your health care provider if you have symptoms of pleurisy. If you have breathing difficulty or your skin turns blue, seek immediate medical care.
Early treatment of bacterial respiratory infections can prevent pleurisy.
Pleuritis; Pleuritic chest pain
University of Kentucky Healthcare Good Samaritan Hospital Lexington on October 31, 2010:
Most pleurisy is caused by infection and is unavoidable. You can avoid severe pleuritic chest pain by early diagnosis and treatment with anti-inflammatory drugs.
Pleurisy caused by viral infections usually lasts about seven days and goes away. Pleurisy from other conditions, such as a cancer, may continue indefinitely.
Cleveland Clinic Lerner Research Institute Cleveland, Ohio on October 31, 2010:
Exams and Tests
The doctor will ask you many questions about the pain, such as where it is located, how long it has been there, and how you've tried to make it better. The doctor will also ask about your personal habits, especially smoking, including the use of tobacco and street drugs such as marijuana and cocaine. Do not hide any information from the doctor. Anything you say will be just between the two of you, and you won't get in trouble with the law.
The doctor will perform a complete physical examination and may do a number of tests to exclude other conditions.
A doctor or nurse will check your blood pressure, heart rate, respiratory rate, temperature, and the oxygen saturation of your blood.
The doctor will look at your skin for rashes or bruises. Infections of the skin such as shingles can cause chest pain, as can bruises.
The doctor may press on your chest. If you have pain that the doctor can duplicate, especially in front where the ribs come together on the breast bone, you may have costochondritis, which is an inflammation of the cartilage of the chest.
The doctor will listen closely with a stethoscope to your lungs. By listening, the doctor can sometimes tell whether you have other diseases of the lungs, such as asthma, emphysema, pneumonia, or a collapsed lung (pneumothorax). Some people with pleurisy develop a rubbing sound that is heard over the area that hurts. The doctor will also listen to your heart's rate and rhythm and determine whether you have any murmurs or extra heart sounds that may indicate a defect in or injury to the heart.
You may need an x-ray of your chest.
The doctor may order electrocardiogram (ECG).
Some of your blood may be sent to the lab for analysis to help rule out other causes of chest pain.
Self-Care at Home
Some chest pain is dangerous. Sometimes even an experienced doctor can't tell you the exact cause of your pain. Pleurisy is frequently diagnosed only when other more serious causes have been ruled out.
Use an anti-inflammatory medicine, such as ibuprofen (Motrin) or aspirin, to reduce the pain and inflammation.
You may have less pain if you lie on the side that hurts.
Avoid exerting yourself or doing anything that would cause you to breathe hard.
Call your doctor or go to your hospital's emergency department if you can't breathe deeply or cough because of severe pain.
When you have been diagnosed with pleurisy and more serious causes of chest pain have been ruled out, you will probably get a prescription for pain medicine.
You may be prescribed an anti-inflammatory drug such as ibuprofen (Motrin), indomethacin (Indocin), or naproxen (Naprosyn). You may even receive stronger medicine, such as codeine, hydrocodone (Vicodin), or oxycodone (Percocet).
If you have a lot of fluid in your chest, it may need to be drained.
Pleurisy can cause intense discomfort. If your pain is not well controlled, you may develop pneumonia because you can't breathe and cough appropriately. Increasing shortness of breath, coughing up more phlegm, intense shaking chills (called rigors), and high fevers should prompt reevaluation by a doctor. If the pain gets worse in spite of prescribed medication or if your symptoms do not improve after a week, you should get reevaluated by a physician.
Duke University Health System on October 31, 2010:
The pleura is a two layered sac that holds the lungs and separates them from the chest wall, diaphragm, and heart. Pleurisy results from an inflammation of this sac.
The pleura that lines the inside of the chest is called the parietal pleura. The pleura that covers the lungs is called the visceral pleura. If you are healthy, the pleura is separated by a thin layer of fluid. This lets the lungs expand and contract easily during breathing. The inflammation that occurs with pleurisy can cause pain with breathing and may even cause a large amount of fluid to collect in the pleural sac.
Pleurisy can go away on its own or worsen so that fluid has to be drained from around the lungs. Some people develop scar tissue called adhesions after they have pleurisy. They then have chronic pain or shortness of breath.
There are many causes of pleurisy.
Infectious disease caused by virus, bacteria, fungus, tuberculosis, or parasites
Cancer such as mesothelioma or spread from other areas
Collagen vascular disease such as lupus, rheumatoid arthritis, sarcoid disease, or scleroderma
Trauma from bruised or broken ribs
Gastrointestinal disease, for example pancreatitis, peritonitis, or a collection of pus under the diaphragm
Reaction to drugs such as methotrexate (Rheumatrex, Trexall) and penicillin
Blood clot in lung
Sickle cell disease
You can have many symptoms with pleurisy.
Chest pain: This is the most common symptom. The pain is generally a sharp, stabbing pain, but may also be a dull ache or a burning sensation. It is usually worse when you take a deep breath, cough, or move around. The pain is usually better if you take shallow breaths or lie on the side that hurts. Chest pain is what usually causes people with pleurisy to seek medical attention.
Cough: You may get a cough, depending on the cause of the pleurisy. Your cough may be dry or productive of sputum or blood.
Shortness of breath: The shortness of breath associated with pleurisy may be due to the underlying cause, such as a blood clot in the lungs or pneumonia, or it may be due to the chest pain caused by breathing.
Fever: You may also get a fever, depending on the cause of the pleurisy.
When to Seek Medical Care
Talk with your doctor about any chest pain that worries you. Many causes of chest pain are dangerous and even life-threatening.
If you have high fever, shaking chills, and a cough that produces thick green or yellow sputum, you may have pneumonia. Talk to your doctor, who may want you to have a chest x-ray.
Your risk of having another disease along with pleurisy increases with your age and the presence of other serious medical conditions such as heart disease, emphysema, chronic bronchitis, diabetes, and collagen vascular diseases.
If you have a swollen leg or arm along with pleurisy, you may have a deep venous thrombosis and pulmonary embolus. This is a blood clot in a vein that has broken off and traveled to your lungs.
You should almost always see a doctor for new chest pain, especially if you are very short of breath, have a high fever, or feel lightheaded or if the pain keeps you from moving around comfortably.
If you are in severe distress, call 911 immediately for ambulance transport to the nearest hospital's emergency department.
Pediatricka Klinika 2. Lekarske Fakulty on October 31, 2010:
Pleural effusion may occur without pleurisy in other conditions, such as heart failure or liver or kidney disease.
How is pleurisy diagnosed?
Many different health problems can lead to pleurisy, so your doctor will look for what is causing your inflammation. He or she will use a physical exam and a chest X-ray to look for signs of conditions that may cause pleuritic chest pain, such as:
Chest muscle strain.
Blood clots in the blood vessels of the lung (pulmonary embolism).
Inflammation around the heart (pericarditis).
Fluid in the pleura (pleural effusion).
If your doctor thinks your pleurisy may be caused by an autoimmune disease such as lupus or rheumatoid arthritis, he or she may do blood tests.
If you have pleural effusion, your doctor may use a needle to remove some of the fluid from the pleura. This procedure is called thoracentesis. The fluid is then studied, to help your doctor find out the cause of the effusion.
See pictures of pleural effusion and thoracentesis .
How is pleurisy treated?
The treatment for pleurisy depends on the cause. For example, if a bacterial infection is the cause, you will probably need an antibiotic. If a pulmonary embolism is present, you may get medicine to dissolve the clot or to prevent future blood clots (anticoagulants).
For most cases of pain caused by pleurisy, your doctor will suggest that you use aspirin, ibuprofen, or another nonsteroidal anti-inflammatory drug (NSAID). Do not give aspirin to anyone younger than 20 because of the risk of Reye syndrome. If you have severe pain, you may need prescription cough or pain medicine. You may also be able to relieve pain by lying on the painful side or pressing a pillow against it.
If you have pleural effusion, you may need to have the fluid drained through a tube that the doctor inserts in your chest.
In some cases of pleural effusion, you may need pleurodesis. During this procedure, a medicine is put into your chest cavity, which triggers an inflammatory reaction over the surface of the lung and inside the chest cavity. This in turn causes the surface of the lung to stick to the surface of the chest cavity, which prevents or reduces more fluid from building up.
Ucla University of California Los Angeles Health System, CA on October 31, 2010:
What is pleurisy?
Pleurisy is swelling (inflammation) of the thin layers of tissue (pleura) covering the lungs and the chest wall.
The outer layer of the pleura lines the inside of the chest wall, and the inner layer covers the lungs. The tiny space between the two layers is called the pleural cavity. This cavity normally contains a small amount of lubricating fluid that allows the two layers to slide over each other when you breathe.
When the pleura becomes inflamed, the layers rub together, causing chest pain. This is known as pleuritic pain.
Pleurisy is sometimes called pleuritis.
What causes pleurisy?
In young, healthy people an infection of the lower respiratory system by a virus or bacteria may cause pleurisy. Pleurisy usually lasts a few days to 2 weeks. In very rare cases, the virus or bacteria may spread and cause pleurisy in others.
Other causes of pleurisy include air leaking into the pleural cavity from a hole in a lung (pneumothorax), injury to the chest (such as a broken rib), tuberculosis or other infections, or a tumor in the pleura.
Other conditions may also cause pleurisy. These include rheumatoid arthritis, lupus, sickle cell crisis, pulmonary embolism, or pancreatitis. Pleurisy may also develop as a complication of heart surgery.
What are the symptoms?
The symptoms of pleurisy are chest pain and difficulty breathing. The chest pain usually starts suddenly. People often describe it as a stabbing pain, and it usually gets worse with breathing. The pain:
May always be present, but it usually gets worse when you breathe in. You may avoid breathing deeply to prevent the pain.
Usually is on only one side of the chest.
May extend to a shoulder or the belly.
Is usually worse when you cough, sneeze, or suddenly move.
May ease when you hold your breath or press on the painful area.
But this type of chest pain can be caused by conditions that do not affect the pleura, such as chest muscle strain and costochondritis.
If a viral infection is causing your pleurisy, you may or may not have common viral symptoms, such as fever, headache, and muscle aches.
The inflammation of the pleura sometimes causes fluid to build up in the pleural cavity (pleural effusion). You may have less pain after this happens, because the fluid prevents the two layers of the pleura from rubbing together. If there is a large amount of fluid, it may prevent the lung from expanding when you breathe in. This can make it hard to breathe. Other symptoms of pleural effusion include fever, chest pain, and a dry cough.
Multicare Health System, WA on October 31, 2010:
What Is Pleurisy?
Pleurisy, also called pleuritis, is an inflammation of the pleura, which is the moist, double-layered membrane that surrounds the lungs and lines the rib cage. The condition can make breathing extremely painful. Sometimes it is associated with another condition called pleural effusion, where excess fluid fills the area between the membrane's layers.
The double-layered pleura protects and lubricates the surface of the lungs as they inflate and deflate within the rib cage. Normally, a thin, fluid-filled gap -- the pleural space -- allows the two layers of the pleural membrane to slide gently past each other. But when these layers become inflamed, with every breath, sneeze, or cough their roughened surfaces rub painfully together like two pieces of sandpaper.
In some cases of pleurisy, excess fluid seeps into the pleural space, resulting in pleural effusion. This fluid buildup usually has a lubricating effect, relieving the pain associated with pleurisy as it reduces friction between the membrane's layers. But at the same time, the added fluid puts pressure on the lungs, reducing their ability to move freely. A large amount of fluid may cause shortness of breath. In some cases of pleural effusion, this excess liquid can become infected.
What Causes Pleurisy?
Viral infection is probably the most common cause of pleurisy. Other causes include the following:
Lung infections, such as pneumonia and tuberculosis
Other diseases such as systemic lupus erythematosus (lupus), rheumatoid arthritis, cancer, liver diseases, and pulmonary embolism
Pleurisy and pleural effusion are generally only as serious as the underlying disease causing it. If you have either of these conditions, you may already be undergoing treatment for the underlying disease; if not, seek medical attention immediately.
A pleural effusion can occur without pleurisy Kidney disease, heart failure, and liver disease can cause pleural effusion without inflammation or pain.
Metrohealth System on October 31, 2010:
Pleurisy occurs when the double membrane (pleura) that lines your chest cavity and surrounds each of your lungs becomes inflamed. Also called pleuritis, pleurisy typically causes sharp pain, almost always when you take a breath.
Pleurisy occurs as a complication of a wide variety of underlying conditions. Relieving pleurisy involves treating the underlying condition, if it's known, and taking pain relievers.
The signs and symptoms of pleurisy may include:
Chest pain when you inhale and exhale (between breaths, you feel almost no pain)
Shortness of breath
Fever and chills, depending on the cause
The sharp, fleeting pain in your chest that pleurisy causes is made worse by coughing, sneezing, moving and deep breathing. In some cases, pain may extend from your chest to your shoulder. You may find relief from pain when you hold your breath or when you apply pressure over the painful area.
When an accumulation of fluids (pleural effusion) is associated with pleurisy, the pain usually disappears because the fluid serves as a lubricant. However, if enough fluid accumulates, it puts pressure on your lungs and interferes with their normal function, causing shortness of breath. If the fluid becomes infected, the signs and symptoms of dry cough, fever and chills may appear. An infected pleural effusion is called an empyema.
When to see a doctor
Call your doctor if you experience any of the following:
Intense, persistent chest pain
Shortness of breath
These signs and symptoms point to a problem with your lungs or pleura, and may also indicate an underlying illness for which you need prompt medical care.
A double layer of membranes called pleura separate your lungs from your chest wall. One layer of the pleura overlies each lung. The other layer lines the inner chest wall. The layers are like two pieces of smooth satin rubbing against each other with almost no friction, allowing your lungs to expand and contract when you breathe without any resistance from the lining of the chest wall.
When inflamed, the two layers of the pleural membrane in the affected side of your chest rub against each other like two pieces of sandpaper, producing the pain of pleurisy when you inhale and exhale.
The underlying medical conditions that can cause pleurisy are numerous. Pleurisy causes include:
An acute viral infection, such as the flu (influenza)
Pneumonia, in those cases in which the infected portion of your lung involves the surface of the pleura
Autoimmune conditions, such as lupus, rheumatoid arthritis and autoimmune hepatitis
Tuberculosis and other infections
A clot in an artery of your lungs (pulmonary embolism)
Pleurisy can also occur as a result of trauma to your chest or after heart surgery. Rib fractures also may cause pleurisy. It's possible to fracture a rib in the absence of trauma, such as from a severe cough. In some cases, the cause of pleurisy is unknown (idiopathic).
Cancer in the lung rarely causes pleurisy. There's no relationship between smoking and pleurisy, but a "smoker's cough" will aggravate
You're likely to start by first seeing your family doctor. However, when you call to set up your appointment, you may be urged to seek immediate medical care if you are experiencing severe, lasting chest pain or difficulty breathing.
Because appointments can be brief, and because there's often a lot of ground to cover, it's a good idea to be well prepared for your appointment. Here's some information to help you get ready for your appointment, and what to expect from your doctor.
What you can do
Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance.
Write down any symptoms you've been experiencing, and for how long. Be prepared to describe your chest pain to your doctor and to pinpoint where it starts and how far it spreads.
Write down key personal information, including recent travel abroad and major life changes, both positive and negative. Your doctor may also be interested in your work history, including possible environmental exposure to asbestos over the past 20 years or longer.
Make a list of your key medical information, including other conditions you're being treated for and the names of any medications, vitamins and supplements that you're taking. Also note whether any family members or close friends have recently been ill.
Take a family member or friend along, if possible. With pleurisy, the pain you're experiencing may be too intense to safely drive. In addition, it can be difficult to soak up all the information provided to you during an appointment. Someone who accompanies you may remember something that you missed or forgot.
Write down questions to ask your doctor.
Your time with your doctor is limited, so preparing a list of questions will help you make the most of your time together. List your questions from most important to least important in case time runs out.
For pleurisy, some basic questions to ask your doctor include:
What do you think is the underlying cause of my symptoms?
Are there any other possible causes for my symptoms?
What kinds of diagnostic tests or procedures do I need?
What treatment approach do you recommend?
How soon after I begin treatment can I expect to feel better?
Are there self-care steps I can take to improve my discomfort?
Do you recommend that I stay home from work or school? For how long?
Will it help if I stop smoking?
If the people I live with smoke, will it aggravate my condition?
Am I at risk of long-term complications from this condition?
Is the underlying cause of my symptoms contagious? How can I reduce the risk of passing my illness to others?
I have these other health conditions. How can I best manage them together?
Should I see a specialist? What will that cost, and will my insurance cover seeing a specialist?
What should I do if the pain doesn't get any better?
Is there a generic alternative to the medicine you're prescribing me?
Are there any brochures or other printed material that I can take home with me? What Web sites do you recommend visiting?
In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask questions during your appointment at any time that you don't understand something.
What to expect from your doctor
Your doctor is likely to ask you a number of questions. Being ready to answer them may reserve time to go over any points you want to talk about in-depth. Your doctor may ask:
How would you describe your symptoms?
When did you first begin experiencing these symptoms?
Where is your chest pain located? Does it spread to your arms, shoulders or jaw?
Is your chest pain constant, or does it come and go?
What, if anything, makes your chest pain better? What makes it worse?
Do your symptoms include other types of pain, such as a sore throat or joint pain?
Have you been experiencing difficulty breathing?
Have you been experiencing fever or night sweats?
Have you lost weight without trying?
How does your current energy level compare to what's normal for you?
Have you been diagnosed with or treated for any other health conditions?
What medications are you currently taking?
Have you recently had any medical procedures?
Have you recently traveled to another country? Did anyone who traveled with you get sick?
Have you been involved in any work, projects or hobbies over the years that might have exposed you to asbestos?
Do or did you smoke? For how long?
What you can do in the meantime
While you wait for your appointment, over-the-counter pain relievers such as ibuprofen (Advil, Motrin, others) and acetaminophen (Tylenol, others) may make you more comfortable. Tylenol with codeine, available by prescription only, helps control both a cough and pain. Get plenty of rest. You may find that lying on the painful side of your body eases your discomfort.
Your doctor may make a diagnosis based on your signs and symptoms. When examining you and listening to your chest, your doctor may hear a "friction rub" that may sound like the crunching sound of walking on very dry snow.
Your doctor might also use the following procedures to determine
London Health Sciences Centre on October 31, 2010:
A number of alternative or complementary medicines are being investigated for their anti-inflammatory properties, and their use in pleurisy. At this time, clinical trials of these compounds have not been performed.
Extracts from the Brazilian folk remedy Wilbrandia ebracteata ("Taiuia") have been shown to reduce inflammation in the pleural cavity of mice. The extract is thought to inhibit the same enzyme, cyclooxygenase-2 (COX-2), as the non-steroidal anti-inflammatory drugs. Similarly, an extract from the roots of the Brazilian Petiveria alliacea plant reduced inflammation in a rat model of pleurisy. The extract also reduced pain sensations in the rats. An aqueous extract from Solidago chilensis has been shown to reduce inflammation in a mouse model of pleurisy.
Pleurisy root, or butterfly weed, was so named because it was used by Native Americans to treat pleurisy. The root was said to encourage coughing by thinning the mucus in the lungs. Pleurisy root is not used much today because more effective medicines are available.
Pleurisy is often associated with complications that affect the pleural space.
In some cases of pleurisy, excess fluid builds up in the pleural space. This is called a pleural effusion. The buildup of fluid usually forces the two layers of the pleura apart so they don't rub against each other when breathing. This can relieve the pain of pleurisy. A large amount of extra fluid can push the pleura against the lung until the lung, or a part of it, collapses. This can make it hard to breathe.
In some cases of pleural effusion, the extra fluid gets infected and turns into an abscess. This is called an empyema.
Pleural effusion involving fibrinous exudates in the fluid may be called fibrous pleurisy. It sometimes occurs as a later stage of pleurisy.
A person can develop a pleural effusion in the absence of pleurisy. For example, pneumonia, heart failure, cancer, or a pulmonary embolism can lead to a pleural effusion.
Air or gas also can build up in the pleural space. This is called a pneumothorax. It can result from acute lung injury or a lung disease like emphysema. Lung procedures, like surgery, drainage of fluid with a needle, examination of the lung from the inside with a light and a camera, or mechanical ventilation, also can cause a pneumothorax.
The most common symptom is sudden pain in one side of the lung and shortness of breath. A pneumothorax also can put pressure on the lung and cause it to collapse.
If the pneumothorax is small, it may go away on its own. If large, a chest tube is placed through the skin and chest wall into the pleural space to remove the air.
Blood also can collect in the pleural space. This is called hemothorax. The most common cause is injury to the chest from blunt force or surgery on the heart or chest. Hemothorax also can occur in people with lung or pleural cancer.
Hemothorax can put pressure on the lung and force it to collapse. It also can cause shock, a state of hypoperfusion in which an insufficient amount of blood is able to reach the organs.
Pleurisy and other disorders of the pleura can be serious, depending on what caused the inflammation in the pleura.
If the condition that caused the pleurisy or other pleural disorders isn't too serious and is diagnosed and treated early, one usually can expect a full recovery.
Nemours Healthcare for Children on October 31, 2010:
Treatment has several goals:
Remove the fluid, air, or blood from the pleural space
Treat the underlying condition
If large amounts of fluid, air, or blood are not removed from the pleural space, they may put pressure on the lung and cause it to collapse.
The surgical procedures used to drain fluid, air, or blood from the pleural space are as follows:
During thoracentesis, a needle or a thin, hollow, plastic tube is inserted through the ribs in the back of the chest into the chest wall. A syringe is attached to draw fluid out of the chest. This procedure can remove more than 6 cups (1.5 litres) of fluid at a time.
When larger amounts of fluid must be removed, a chest tube may be inserted through the chest wall. The doctor injects a local painkiller into the area of the chest wall outside where the fluid is. A plastic tube is then inserted into the chest between two ribs. The tube is connected to a box that suctions the fluid out. A chest x-ray is taken to check the tube's position.
A chest tube also is used to drain blood and air from the pleural space. This can take several days. The tube is left in place, and the patient usually stays in the hospital during this time.
Sometimes the fluid contains thick pus or blood clots, or it may have formed a hard skin or peel. This makes it harder to drain the fluid. To help break up the pus or blood clots, the doctor may use the chest tube to put certain medicines into the pleural space. These medicines are called fibrinolytics. If the pus or blood clots still do not drain out, surgery may be necessary.
A couple of medications are used to relieve pleurisy symptoms:
Paracetamol (acetaminophen) or anti-inflammatory agents to control pain and decrease inflammation. Only indomethacin (brand name Indocin) has been studied with respect to relief of pleurisy.
Codeine-based cough syrups to control a cough
There may be a role for the use of corticosteroids (for tuberculous pleurisy), tacrolimus (Prograf) and methotrexate (Trexall, Rheumatrex) in the treatment of pleurisy. Further studies are needed.
The following may be helpful in the management of pleurisy:
Lying on the painful side may be more comfortable
Breathing deeply and coughing to clear mucus as the pain eases. Otherwise, pneumonia may develop.
Treating the cause
Ideally, the treatment of pleurisy is aimed at eliminating the underlying cause of the disease.
If the pleural fluid is infected, treatment involves antibiotics and draining the fluid. If the infection is tuberculosis or from a fungus, treatment involves long-term use of antibiotics or antifungal medicines.
If the fluid is caused by tumors of the pleura, it may build up again quickly after it is drained. Sometimes antitumor medicines will prevent further fluid buildup. If they don't, the doctor may seal the pleural space. This is called pleurodesis. Pleurodesis involves the drainage of all the fluid out of the chest through a chest tube. A substance is inserted through the chest tube into the pleural space. This substance irritates the surface of the pleura. This causes the two layers of the pleura to squeeze shut so there is no room for more fluid to build up.
Chemotherapy or radiation treatment also may be used to reduce the size of the tumors.
If congestive heart failure is causing the fluid buildup, treatment usually includes diuretics and other medicines.
The most common and known treatment for pleurisy is generally to carry on as normal, ibuprofen and amoxicilin being common treatments prescribed by doctors. Milder forms of Pleurisy can be noticed by less inflammatres of the arms and legs. If this is the case Pleurisy will clear of all symptoms within two weeks.
University of Kentucky Healthcare Good Samaritan Hospital Lexington on October 31, 2010:
A diagnosis of pleurisy or another pleural condition is based on medical histories, physical exams, and diagnostic tests. The goals are to rule out other sources of the symptoms and to find the cause of the pleurisy so the underlying disorder can be treated.
A doctor uses a stethoscope to listen to the breathing. This detects any unusual sounds in the lungs. A person with pleurisy will have inflamed layers of the pleura that make a rough, scratchy sound as they rub against each other during breathing. This is called pleural friction rub, and it is a likely sign of pleurisy.
Depending on the results of the physical exam, diagnostic tests are sometimes performed.
A chest x-ray takes a picture of the heart and lungs. It may show air or fluid in the pleural space. It also may show what's causing the pleurisy –for example; pneumonia, a fractured rib, or a lung tumor.
Sometimes an x-ray is taken while lying on the painful side. This may show fluid that did not appear on the standard x-ray taken while standing.
Computed tomography (CT) scan
A CT scan provides a computer-generated picture of the lungs that can show pockets of fluid. It also may show signs of pneumonia, a lung abscess, or a tumor.
Ultrasonography uses sound waves to create pictures of the lungs. It may show where fluid is located in the chest. It also can show some tumors.
Magnetic resonance imaging (MRI)
Magnetic resonance imaging (MRI), also called nuclear magnetic resonance (NMR) scanning, uses powerful magnets to show pleural effusions and tumors.
Blood tests can detect bacterial or viral infection, pneumonia, rheumatic fever, a pulmonary embolism, or lupus.
Arterial blood gas
In arterial blood gas sampling, a small amount of blood is taken from an artery, usually in the wrist. The blood is then checked for oxygen and carbon dioxide levels. This test shows how well the lungs are taking in oxygen.
The illustration shows a person having thoracentesis. The person sits upright and leans on a table. Excess fluid from the pleural space is drained into a bag.
Once the presence and location of fluid is confirmed, a sample of fluid can be removed for testing. The procedure to remove fluid in the chest is called thoracentesis. The doctor inserts a small needle or a thin, hollow, plastic tube through the ribs in the back of the chest into the chest wall and draws fluid out of the chest.
Thoracentesis can be done in the doctor's office or at the hospital. Ultrasound is used to guide the needle to the fluid that is trapped in small pockets around the lungs.
Thoracentesis usually does not cause serious complications. Generally, a chest x-ray is done after the procedure to evaluate the lungs. Possible complications of thoracentesis include the following:
Pneumothorax, or buildup of air in the pleural space, with a collapsed or partially collapsed lung. Sometimes air comes in through the needle or the needle makes a hole in the lung. Usually, a hole will seal itself. But sometimes air can build up around the lung and make it collapse. A chest tube can remove the air and let the lung expand again.
Bleeding and bruising where the needle went in. In rare cases, bleeding may occur in or around the lung. The doctor can use a chest tube to drain the blood. In some cases, surgery is needed.
Infection where the needle went in.
Rarely, liver or spleen injury.
The fluid removed by thoracentesis is examined under a microscope. It is evaluated for the presence of chemicals and for its color, and texture. The clearness of the fluid is an indicator of infection, cancer, or other conditions that may be causing the buildup of fluid or blood in the pleural space.
If tuberculosis or cancer is suspected, a small piece of the pleura may be examined under a microscope to make a definitive diagnosis. This is called a biopsy.
Several approaches to taking tissue samples are available
Insertion of a needle through the skin on the chest to remove a small sample of the outer layer of the pleura.
Insertion of a small tube with a light on the end (endoscope) into tiny cuts in the chest wall in order to visualize the pleura. Small pieces of tissue can be biopsied though the endoscope.
remove a sample of the pleura through a small cut in the chest wall. This is called an open pleural biopsy. It is usually done if the sample from the needle biopsy is too small for an accurate diagnosis.
University of California San Francisco Medical Center, CA on October 31, 2010:
Pleurisy (also known as pleuritis) is an inflammation of the pleura, the lining of the pleural cavity surrounding the lungs. Among other things, infections are the most common cause of pleurisy.
The inflamed pleural layers rub against each other every time the lungs expand to breathe in air. This can cause sharp pain with inhalation (also called pleuritic chest pain).
The main symptom of pleurisy is a sharp or stabbing pain in the chest that gets worse with deep breathing, coughing or sneezing. The pain may stay in one place, or it may spread to the shoulder or back. Sometimes it becomes a fairly constant dull ache.
Depending on what's causing the pleurisy, one may have other symptoms:
Shortness of breath
Fever and chills
Rapid, shallow breathing
Unexplained weight loss
Sore throat followed by pain and swelling in the joints
Viral infection is the most common cause of pleurisy. However, many different conditions can cause pleurisy:
Bacterial infections like pneumonia and tuberculosis
Autoimmune disorders like systemic lupus erythematosus (or drug-induced lupus erythematosus) and rheumatoid arthritis
Lung cancer, including lymphoma
Other lung diseases like Cystic Fibrosis, sarcoidosis, asbestosis, lymphangioleiomyomatosis, and mesothelioma
Pulmonary embolism, a blood clot in the blood vessels that go into the lungs
Inflammatory bowel disease
Familial Mediterranean fever, an inherited condition that often causes fever and swelling in the abdomen or lung
Infection from a fungus or parasite
Heart surgery, especially coronary artery bypass grafting
High blood pressure
Can occur with no illness or infection
Some cases of pleurisy are idiopathic, meaning the cause cannot be determined.
Funom Theophilus Makama (author) from Europe on October 31, 2010:
I got something very interesting about Pleurisy.... I think, I will add to this or maybe publish a fresh hub about it....
Funom Theophilus Makama (author) from Europe on October 22, 2010:
ooooh thanks alot GusThe Redneck. What else can I say. Keep on giving your feedback...
Gustave Kilthau from USA on October 22, 2010:
HI DVD - Now THAT was a very complete and accurately done article. Thank you.
Funom Theophilus Makama (author) from Europe on October 21, 2010:
hello guys! yet another hub "Clinical details and basic medical information about Pleurisy and Pyogenic lung diseases" Enjoy and express ur views...