Cor pulmonale

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Cor pulmonale
Author Herbert P Wiedemann, MD Section Editor James K Stoller, MS, MD Deputy Editor Kevin C Wilson, MD

Last literature review for version 16.1: January 31, 2008 | This topic last updated: September 2, 2004 INTRODUCTION Cor pulmonale is the alteration of right ventricular structure or function that is due to pulmonary hypertension caused by diseases affecting the lung or its vasculature. Rightsided heart disease resulting from primary disease of the left side of the heart or from congenital heart disease is not considered within this constellation of disorders [1,2] . Although most of the conditions that cause cor pulmonale are chronic and slowly progressive, patients may also present with acute and life-threatening symptoms. Such abrupt decompensation occurs when the right ventricle is unable to compensate for the imposition of sudden additional demands, resulting either from progression of the underlying disease or a superimposed acute process. The etiology, clinical characteristics, and treatment of cor pulmonale will be reviewed here. Detailed discussions of the clinical characteristics and treatment of both primary and secondary causes of pulmonary hypertension are presented separately. (See "Overview of pulmonary hypertension", see "Diagnostic evaluation of pulmonary hypertension", see "Pathogenesis of pulmonary hypertension", and see "Treatment of pulmonary hypertension"). ETIOLOGY Cor pulmonale is a state of cardiopulmonary dysfunction that may result from several different etiologies and pathophysiologic mechanisms (show table 1). (See "Overview of pulmonary hypertension"). Possible mechanisms include [3] : Pulmonary vasoconstriction (secondary to alveolar hypoxia or blood acidosis) Anatomic reduction of the pulmonary vascular bed (emphysema, pulmonary emboli, etc) Increased blood viscosity (polycythemia, sickle-cell disease, etc) Increased pulmonary blood flow. The most frequent cause of cor pulmonale is chronic obstructive pulmonary disease (COPD) due to chronic bronchitis or emphysema [1,4,5] . In patients with COPD, an increased incidence of right ventricular involvement may correlate with increasing severity of lung dysfunction. As an example, right ventricular hypertrophy is present in 40 percent of patients with an FEV1 <1.0 L and in 70 percent of those with an FEV1 <0.6 L [1] . However, the presence of hypoxemia, hypercapnia, and polycythemia also independently predict the development of right ventricular hypertrophy in COPD, although not as strongly as abnormal pulmonary mechanics.

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PROGNOSIS The development of cor pulmonale associated with pulmonary hypertension often has important prognostic implications. In COPD, for example, the development of pulmonary hypertension and peripheral edema heralds a poor prognosis. Patients who develop peripheral edema have a five year survival of only approximately 30 percent, and those whose pulmonary vascular resistance exceeds 550 dynes-sec/cm(5) rarely survive for more than 3 years [1,5] . (See "Natural history and prognosis of COPD"). However, rather than having a substantial direct effect on mortality, the development of cor pulmonale in COPD may just reflect the severity of the underlying obstructive disease and its effect on mortality. Even in severe COPD, for example, it is uncommon to observe mean pulmonary artery pressures above 40 mmHg; such levels are far below those found in many patients with pulmonary arterial hypertension or chronic unresolved pulmonary emboli. CLINICAL CHARACTERISTICS The clinical detection and assessment of cor pulmonale are difficult due to the subtle and often nonspecific signs and symptoms. As an example, the development of peripheral edema in patients with COPD is not necessarily a reliable marker of pulmonary hypertension. Symptoms There are, however, symptoms directly attributable to pulmonary hypertension, including dyspnea on exertion, fatigue, lethargy, chest pain, and syncope with exertion. Fatigue, lethargy, and exertional syncope reflect an inability to increase cardiac output during stress because of vascular obstruction in the pulmonary arterioles. Typical exertional angina can occur in patients with primary or secondary pulmonary hypertension, even in the absence of epicardial coronary disease. The mechanism by which angina occurs is variable. Subendocardial right ventricular ischemia induced by hypoxemia and increased transmural wall tension may play a role [6] . However, angina can also be caused by dynamic compression of the left main coronary by an enlarged pulmonary artery, and this risk is greatest for patients with a pulmonary artery trunk diameter 40 mm [7,8] . Less common symptoms include cough and hemoptysis. Hoarseness can result from compression of the left recurrent laryngeal nerve by a dilated main pulmonary artery. Severe right ventricular failure leading to passive hepatic congestion may lead to complaints such as anorexia and right upper quadrant discomfort. Physical findings The physical examination should detect findings characteristic of pulmonary hypertension and right ventricular hypertrophy, sometimes accompanied by right ventricular failure. The initial physical finding of pulmonary artery hypertension is increased intensity of the pulmonic component of the second heart sound, which may even become palpable. The second heart sound may also be narrowly split, a change that will not be present if right ventricular depolarization is delayed because of concurrent right bundle branch block. Auscultation of the heart also may reveal a systolic ejection murmur and, in more severe disease, a diastolic pulmonary regurgitation murmur.

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Right ventricular hypertrophy is characterized by a prominent A wave in the jugular venous pulse, associated with a right-sided fourth heart sound and either a left parasternal heave or a downward subxiphoid thrust. Right ventricular failure leads to systemic venous hypertension. This can produce a variety of findings, such as elevated jugular venous pressure with a prominent V wave, a right ventricular third heart sound, and a high-pitched tricuspid regurgitant murmur. The right-sided murmurs and gallops are augmented with inspiration, but these findings may be obscured, depending upon the etiology of the hypertension. In severe emphysema, for example, the increased anteroposterior diameter of the chest makes auscultation difficult and changes the position of the right ventricular impulse. Although ascites is uncommon, even in severe cor pulmonale, extracardiac changes that may be seen include hepatomegaly, a pulsatile liver (if tricuspid regurgitation is prominent), and peripheral edema, which may develop or be exacerbated during a course of steroid therapy [1,5] . Edema Although some patients with severe COPD develop edema in association with clear evidence of right heart failure, other patients with edema have no hemodynamic signs of right ventricular failure, and pulmonary artery pressure and arterial blood gases are stable [9] . The pathogenesis of edema in such patients is not well understood: the cardiac output and glomerular filtration rate are usually normal or near normal, both in the resting state and with exercise [10,11] . (See "Pathophysiology and etiology of edema in adults"). Edema seems to occur primarily in patients with hypercapnia, suggesting that the high PCO2 rather than cardiac dysfunction may be responsible for the sodium retention in cor pulmonale [12] . Hypercapnia is associated with an appropriate increase in proximal bicarbonate reabsorption, which serves to minimize the fall in arterial pH, but also can contribute to edema formation, since it also promotes the passive reabsorption of NaCl and H2O [13] . (See "Chapter 3A: Cell model for proximal transport"). Another contributing factor to sodium retention may be hypoxemia. Hypoxemia can cause renal vasoconstriction, leading to a reduction in urinary sodium excretion [14] . EVALUATION Because of the nonspecificity of symptoms and signs, ancillary evaluation may be useful, including the following techniques [15] : Chest radiography Electrocardiography Two dimensional and Doppler echocardiography (which can provide an indirect measurement of pulmonary artery pressure when tricuspid regurgitation is present) Pulmonary function tests Radionuclide ventriculography Magnetic resonance imaging

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Right heart catheterization Lung biopsy Chest radiograph The characteristic chest radiograph in pulmonary arterial hypertension shows enlargement of the central pulmonary arteries (show radiograph 1). In 95 percent of patients with COPD and pulmonary hypertension, the diameter of the descending branch of the right pulmonary artery is greater than 20 mm in width. In addition, the peripheral vessels are attenuated, leading to oligemic lung fields. Right ventricular failure may result in right ventricular and right atrial dilatation on chest radiography. Right ventricular enlargement can also lead to a decrease in the retrosternal space. However, these findings may be obscured in the presence of kyphoscoliosis, hyperinflated lungs, left ventricular enlargement, or interstitial lung disease. Electrocardiogram The electrocardiogram may demonstrate signs of right ventricular hypertrophy or strain (show ECG 1). Findings that may be seen in chronic right ventricular overload include: Right axis deviation and R/S ratio greater than 1 in lead V1. Increased P wave amplitude in lead II (P pulmonale) due to right atrial enlargement (show figure 1 and show ECG 2). Incomplete or complete right bundle branch block. In acute cor pulmonale, such as occurs with acute pulmonary embolism, a classic pattern of an S wave in lead I with a Q and inverted T wave in lead III may be seen. Most electrocardiographic criteria show a high specificity (ie, the findings are absent in patients without the disease) but a low sensitivity (ie, the findings are present in patients with the disease) for the detection of RVH. The sensitivity of the electrocardiogram is even worse in patients with biventricular hypertrophy or COPD, but the presence of electrocardiographic changes of cor pulmonale in these settings connotes a poor prognosis [16,17] . (See "ECG tutorial: Chamber enlargement and hypertrophy"). Two-dimensional echocardiography Most patients with pulmonary arterial hypertension have two-dimensional echocardiographic signs of chronic right ventricular pressure overload show echocardiogram 1 show echocardiogram 2 show echocardiogram 3 show echocardiogram 4 [18] . The elevation in pressure leads to increased thickness of the right ventricle with paradoxical bulging of the septum into the left ventricle during systole (show figure 2). At a later stage, right ventricular dilatation occurs, and the septum shows abnormal diastolic flattening. Stress on the right heart initially produces hyperkinesis. However, this is eventually followed by right ventricular hypokinesis, associated with right atrial dilatation and tricuspid regurgitation. The latter is not due to an intrinsic abnormality of the tricuspid valve; it is a secondary manifestation of dilatation of the tricuspid annulus and right ventricle [19] .

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Doppler echocardiography Doppler echocardiography is the most reliable noninvasive estimation of the pulmonary artery pressure. This technique takes advantage of the functional tricuspid insufficiency usually present in PAH. The maximum tricuspid regurgitant jet velocity is recorded, and the pulmonary artery pressure (PAP) is then calculated by the modified Bernoulli equation: PAP systolic = (4 x tricuspid jet velocity squared) + RAP

where RAP is the right atrial pressure estimated from the size and respiratory variation of flow in the inferior vena cava. Other findings associated with pulmonary hypertension are pulmonic insufficiency and midsystolic closure of the pulmonic valve [20,21] . The efficacy of Doppler echocardiography may be limited by the ability to identify an adequate tricuspid regurgitant jet. It may also be less sensitive because of alterations induced by the underlying disease. For example, acoustic windows in patients with COPD may be limited by the increased anteroposterior diameter of the chest. Despite these potential problems, Doppler estimation using tricuspid regurgitation is far more sensitive than the clinical examination and can make an accurate diagnosis in the majority of patients. This is illustrated by the following observations: In one report, Doppler ultrasound examination was able to identify tricuspid regurgitation in 80 percent of 69 patients with catheterization-documented pulmonary arterial hypertension (PA systolic pressure above 35 mmHg) [22] . The accuracy was even higher in patients with more severe disease (PA systolic pressure above 50 mmHg). Tricuspid regurgitation was detected in 95 percent of these patients, and there was a 97 percent correlation with the pressure measured by catheterization. Another study of 33 patients with severe COPD compared clinical and echocardiographic criteria in establishing the diagnosis of PAH [23] . Echocardiographic cor pulmonale was said to be present when the right ventricular free wall thickness was >0.6 cm in the subxiphoid view, PA systolic pressure was greater than 40 mmHg by tricuspid jet Doppler with saline contrast, and the RV/LV ratio was increased. Clinical criteria included right ventricular hypertrophy on the electrocardiogram, enlarged pulmonary arteries on the chest radiograph, and physical findings such as a loud pulmonic heart sound, parasternal heave, jugular venous distension, edema, and hepatomegaly. Cor pulmonale was identified by clinical criteria in only 39 percent of patients versus 75 percent with echocardiography. The use of saline contrast significantly enhanced the sensitivity of Doppler ultrasound in detecting tricuspid regurgitation. Pulmonary function tests Pulmonary function tests should be performed in patients with a suggestive history of underlying lung disease and in those with normal cardiac function. It is important to appreciate that only severe interstitial lung disease (with lung volume below 50 percent of normal) produces secondary pulmonary hypertension, while a mild restrictive defect can be produced by pulmonary arterial hypertension itself. Thus, the latter finding is not indicative of interstitial lung disease as a cause of secondary pulmonary arterial hypertension. Right-sided cardiac catheterization Catheterization of the right heart is the gold standard

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for the diagnosis, quantification, and characterization of pulmonary arterial hypertension. This procedure is indicated only when the necessary information cannot be obtained with Doppler echocardiography. Current indications include: When echocardiography does not permit measurement of a tricuspid regurgitant jet, which does not exclude significant pulmonary artery hypertension [21] . When symptoms are exertional, and simultaneous measurement of left-sided pressures during exercise is also indicated. When therapy will be determined by precise measurement of pulmonary vascular resistance and the response to vasodilators. When left heart catheterization is also required, for example, in the patient over 40 years of age or with risk factors for coronary disease. Right heart catheterization can also be used to determine the potential reversibility of pulmonary arterial hypertension with vasodilators, such as sustained release calcium channel blockers [5] . Lung biopsy Pathologic assessment of pulmonary artery hypertension requires lung biopsy. Historically, pathologic examination has been used intraoperatively to look for evidence of irreversible pulmonary artery pathology. At present, right heart catheterization assessments of pulmonary vascular resistance and the vasodilator response are usually adequate to guide therapeutic decisions. TREATMENT The medical management of patients with cor pulmonale has centered upon attempts to improve oxygenation (with hypoxemic patients) or right ventricular contractility, as well as attempts to decrease pulmonary vascular resistance and vasoconstriction (primarily via vasodilators). This section will discuss the management of patients with cor pulmonale, focusing primarily on the treatment of those with cor pulmonale and COPD. Issues related to the treatment of COPD in general are discussed in detail separately. (See "Management of stable chronic obstructive pulmonary disease" and see "Treatment of pulmonary hypertension"). Oxygen therapy Long-term oxygen therapy improves the survival of hypoxemic patients with COPD; however, the mechanisms have not been fully elucidated. Two major (and not mutually exclusive) hypotheses may explain the survival benefit of oxygen therapy [4] : Oxygen therapy relieves pulmonary vasoconstriction, thereby decreasing pulmonary vascular resistance; as a result, the right ventricle increases stroke volume and cardiac output. Renal vasoconstriction also may be relieved, resulting in an increase in urinary sodium excretion [14] . Oxygen therapy improves arterial oxygen content, providing enhanced delivery to the heart, brain, and other vital organs. (See "Long-term supplemental oxygen therapy"). Diuretics If right ventricular filling volume is markedly elevated, diuretic therapy might improve the function of both right and left ventricles (the latter effect being achieved as left

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ventricular diastolic filling is enhanced through reduction in dilation of the right ventricle). As a result, diuretic therapy may improve cardiovascular performance in some patients with significant volume overload of the right ventricle. However, excessive volume depletion must be avoided, since a drop in cardiac output may result if right ventricular filling volume and pressure are reduced too dramatically in pulmonary hypertension. A simple method to assess volume status is to monitor the BUN and plasma creatinine concentration. As long as these parameters remain stable, it can be assumed that renal perfusion and therefore flow to other organs are being maintained. On the other hand, further fluid removal should cease if there is an otherwise unexplained elevation in these tests. Another potentially important complication of diuretic therapy in the patient with cor pulmonale is the development of metabolic alkalosis. (See "Causes of metabolic alkalosis"). Alkalosis suppresses ventilation, which can have important implications in patients with severe lung disease, eg, leading to difficulty weaning from a ventilator. Digoxin Except in cases of coexistent left ventricular failure, clinical studies do not support the use of digitalis in patients with cor pulmonale. Specifically, the use of digoxin in COPD patients with normal left ventricular function does not improve right ventricular ejection fraction at rest or during exercise, nor does it increase maximal exercise performance. (See "Use of digoxin in heart failure due to systolic dysfunction"). Vasodilators Several vasodilator agents (including hydralazine, nitrates, nifedipine, verapamil, and ACE inhibitors) have been utilized in an attempt to ameliorate pulmonary hypertension. In some studies, short-term but modest reductions in pulmonary artery pressure have been documented. However, vasodilators generally do not result in sustained or significant improvement and may be associated with adverse side effects: Short-term studies usually fail to document an improvement in exercise capacity or functional status, probably resulting from the fact that these patients are limited more by lung mechanics than by the degree of pulmonary hypertension [4,24,25] . Evidence of sustained efficacy (beyond three to six months) is relatively uncommon [26-28] . The use of vasodilators in patients with COPD can be associated with worsening of arterial oxygenation and/or systemic hypotension, although these effects usually are not severe. Overall, therefore, the use of vasodilator medications for patients with COPD has generally dropped from routine clinical practice. Nevertheless, patients with severe and persistent pulmonary hypertension despite oxygen and bronchodilator therapy may be candidates for a trial of vasodilator therapy. In this setting, right heart catheterization is recommended during the initial administration of the vasodilator; either sustained release nifedipine (30 to 240 mg/d orally, sustained release) or diltiazem (120 to 720 mg/d orally, sustained release) is recommended in order to objectively assess efficacy and detect possible adverse hemodynamic consequences. A reduction in pulmonary vascular resistance of

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more than 20 percent (provided that cardiac output does not decrease and pulmonary artery pressure does not increase) is a reasonable criterion that has been suggested as evidence of efficacy [5] . (See "Treatment of pulmonary hypertension"). Theophylline and the sympathomimetic amines Theophylline and the sympathomimetic amines (terbutaline, etc) may have salutary effects not related to bronchodilation. Specifically, these agents may [4] : Improve myocardial contractility Provide some degree of pulmonary vasodilation Enhance diaphragm endurance Such effects may explain why some patients treated with theophylline, for example, experience a reduction in dyspnea (as documented in at least one double-blind trial) even without a reduction in airflow obstruction [29] . As a result, it is reasonable to consider the use of theophylline as adjunctive therapy in the management of chronic or decompensated cor pulmonale in patients with COPD, at least until further evidence either supporting or refuting this approach is available. (See "Role of methylxanthines in the treatment of COPD"). Almitrine The well documented ability of almitrine to improve arterial PO2 in patients with COPD led to initial enthusiasm regarding the potential clinical utility of this agent [30] . However, almitrine appears to improve gas exchange largely by enhancing hypoxic pulmonary vasoconstriction, thereby worsening pulmonary hypertension, especially during exercise [4,31] . This may account for the dyspnea experienced by some patients receiving this agent, despite enhanced arterial oxygenation. As a result, the use of almitrine in the management of COPD is not currently recommended. Phlebotomy In patients with severe polycythemia (hematocrit above 55 percent), phlebotomy (to achieve a hematocrit of about 50 percent) is associated with a decrease in mean pulmonary artery pressure and pulmonary vascular resistance, as well as an improvement in exercise performance [4] . However, the application of continuous oxygen therapy in appropriately selected patients should reduce the number of COPD patients who become severely polycythemic. Overall, therefore, the use of phlebotomy should generally be reserved as adjunctive therapy in acute management of the markedly polycythemic patient who has an acute decompensation of cor pulmonale, or for the rare patient who remains significantly polycythemic despite appropriate long-term oxygen therapy.

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REFERENCES
1. 2. MacNee, W. State of the art: Pathophysiology of cor pulmonale in chronic obstructive pulmonary disease (Parts 1 & 2). Am J Respir Crit Care Med 1994; 150:833. Wiedemann, HP, Matthay, RA. Cor pulmonale. In: Decision Making in Emergency Medicine, Callaham, ML, Barton, CW, Schumaker, HM (Eds), Philadelphia, BC Decker, Inc 1990. p.120.

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Wiedemann, HP, Matthay, RA. The management of acute and chronic cor pulmonale. In: Heart-Lung Interactions in Health and Disease, Scharf, SM, Cassidy, SS (Eds), Marcel Dekker, Inc, New York, 1989, p. 915. Wiedemann, HP, Matthay, RA. Cor pulmonale in chronic obstructive pulmonary disease: circulatory pathophysiology and management. Clin Chest Med 1990; 11:523. Salvaterra, CG, Rubin, LJ. Investigation and management of pulmonary hypertension in chronic obstructive pulmonary disease. Am Rev Respir Dis 1993; 148:1414. Morrison, DA, Klein, C, Welsh, C. Relief of right ventricular angina and increased exercise capacity with long term oxygen therapy. Chest 1991; 100:534. Mesquita, SM, Castro, CR, Ikari, NM, et al. Likelihood of left main coronary artery compression based on pulmonary trunk diameter in patients with pulmonary hypertension. Am J Med 2004; 116:369. Kawut, SM, Silvestry, FE, Ferrari, VA, et al. Extrinsic compression of the left main coronary artery by the pulmonary artery in patients with long-standing pulmonary hypertension. Am J Cardiol 1999; 83:984. Weitzenblum, E, Apprill, M, Oswald, M, et al. Pulmonary hemodynamics in patients with chronic obstructive pulmonary disease before and during an episode of peripheral edema. Chest 1994; 105:1377. Campbell, EJM, Short, DS. The cause of oedema in cor pulmonale. Lancet 1960; 1:1184. Richens, JM, Howard, P. Oedema in cor pulmonale. Clin Sci 1982; 62:255. Farber, MO, Roberts, LR, Weinberger, MH, et al. Abnormalities of sodium and H2O handling in chronic obstructive lung disease. Arch Intern Med 1982; 142:1326. Schafer, JA. Mechanism coupling the absorption of solutes and water in the proximal nephron. Kidney Int 1984; 25:708. Reihman, DH, Farber, MO, Weinberger, MH, et al. Effect of hypoxemia on sodium and water excretion in chronic obstructive lung disease. Am J Med 1985; 78:87. Wiedemann, HP, Matthay, RA. Cor pulmonale. In: Heart Disease: Textbook of Cardiovascular Medicine, 5th ed, Braunwald, E (Ed), WB Saunders, Philadelphia, 1997, p. 1604. Klinger, JR, Hill, NS. Right ventricular dysfunction in chronic obstructive pulmonary disease. Chest 1991; 99:715. Incalzi, RA, Fuso, L, De Rosa, M, et al. Electrocardiographic signs of chronic cor pulmonale: A negative prognostic finding in chronic obstructive pulmonary disease. Circulation 1999; 99:1600. Jardin, F, Dubourg, O, Bourdarias, JP. Echocardiographic pattern of acute cor pulmonale. Chest 1997; 111:209. Mikami, T, Kudo, T, Sakurai, N, et al. Mechanisms for development of functional tricuspid regurgitation determined by pulsed Doppler and two dimensional echocardiography. Am J Cardiol 1984; 53:160. Yock, PG, Popp, RL. Noninvasive estimation of right ventricular systolic pressure by doppler ultrasound in patients with tricuspid regurgitation. Circulation 1984; 70:657. Otto, C. Textbook of clinical echocardiography. In: Pearlman, S (Ed), Saunders, Philadelphia, 1995. Berger, M, Haimowitz, A, Van Tosh, A, et al. Quantitative assessment of pulmonary hypertension in patients with tricuspid regurgitation using continuous wave doppler ultrasound. J Am Coll Cardiol 1985; 6:359. Himelman, RB, Struve, SN, Brown, JK, et al. Improved recognition of cor pulmonale in patients with severe chronic obstructive pulmonary disease. Am J Med 1988; 84:891. Singh, H, Ebejer, MJ, Higgens, DA, et al. Acute hemodynamic effects of nifedipine at rest and during maximal exercise in patients with chronic cor pulmonale. Thorax 1985; 40:910.

4. 5. 6. 7.

8.

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10. 11. 12. 13. 14. 15.

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20. 21. 22.

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25.

Dal Nogare, AR, Rubin, LJ. The effects of hydralazine on exercise capacity in pulmonary hypertension secondary to chronic obstructive pulmonary disease. Am Rev Respir Dis 1986; 133:385. Agostoni, P, Doria, E, Galli, C, et al. Nifedipine reduces pulmonary pressure and vascular tone during short- but not long-term treatment of pulmonary hypertension in patients with chronic obstructive pulmonary disease. Am Rev Respir Dis 1989; 139:120. Biernacki, W, Prince, K, Whyte, K, et al. The effect of six months of daily treatment with the beta-2 agonist oral pirbuterol on pulmonary hemodynamics in patients with chronic hypoxic cor pulmonale receiving long-term oxygen therapy. Am Rev Respir Dis 1989; 139:492. Bratel, T, Hedenstierna, G, Nyquist, O, Ripe, E. Long-term treatment with a new calcium antagonist, felodipine, in chronic obstructive lung disease. Eur J Respir Dis 1986; 68:351. Mahler, DA, Matthay, RA, Snyder, PE, et al. Sustained-release theophylline reduces dyspnea in non-reversible obstructive airway disease. Am Rev Respir Dis 1985; 131:22. Bell, RC, Mullins RC III, West LG, et al. The effect of almitrine bismesylate on hypoxemia in chronic obstructive pulmonary disease. Ann Intern Med 1986; 105:342. MacNee, W, Connaughton, JJ, Rhind, GB, et al. A comparison of the effects of almitrine or oxygen breathing on pulmonary arterial pressure and right ventricular ejection fraction in hypoxic chronic bronchitis and emphysema. Am Rev Respir Dis 1986; 134:559.

26.

27.

28.

29. 30. 31.

GRAPHICS
Major causes of cor pulmonale
Lung disease

Chronic obstructive pulmonary disease Cystic fibrosis Interstitial lung diseases

Disorders of the pulmonary circulation

Pulmonary thromboembolism Primary pulmonary hypertension Tumor emboli Sickle cell anemia Schistosomiasis Pulmonary veno-occlusive disease
Neuromuscular diseases

Amyotrophic lateral sclerosis Myasthenia gravis Poliomyelitis Guillain-Barr syndrome Spinal cord lesions Bilateral diaphragmatic paralysis
Thoracic cage deformities

Kyphoscoliosis
Disorders of ventilatory control

Primary central hypoventilation Sleep apnea syndromes

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Pulmonary artery hypertension

Chest radiograph in PA view showing enlarged pulmonary arteries (arrows) due to pulmonary hypertension induced by anomalous pulmonary venous drainage. Courtesy of Sven Paulin, MD.

Right ventricular hypertrophy

Right ventricular hypertrophy due, in this case, to primary pulmonary hypertension. The characteristic features include marked right axis deviation (+210 which is equal to -150), tall R wave in V1 (as part of a qR complex), delayed precordial transition zone with prominent S waves in leads V5 and V6, inverted T waves and ST depression in V1 to V3 consistent with right ventricular "strain", and peaked P waves in lead II consistent with concomitant right atrial enlargement. Courtesy of Ary Goldberger, MD.

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Right atrial enlargement

The left panel shows the normal pattern of atrial activation as it would appear on lead II of the electrocardiogram: activation of the right atrium (RA) occurs first, followed by left atrial (LA) activation. The right panel shows the pattern in a patient with right atrial enlargement: delayed activation of the right atrium, due to dilatation, hypertrophy, scarring, or a conduction abnormality, results in simultaneous activation of the right and left atria. The synchronous electrical activity has an additive effect upon the surface ECG, resulting in a relatively narrow P wave which is of increased amplitude (P pulmonale). The amplitude of the P wave may become significantly increased in the presence of right atrial hypertrophy; it also becomes peaked due to the increase in amount of depolarized tissue.

Atrial enlargement

P wave morphology with atrial enlargement in leads I, II, and V1. The P waves in left atrial enlargement (left panel) are wide (>0.12 sec) and notched in leads I and II and the terminal segment has a negative deflection that is deep and delayed in V1. In right atrial enlargement (middle panel), the P wave amplitude is increased (0.28 mV) in lead II. Biatrial enlargement (right panel) has characteristics of both atrial abnormalities: the P wave amplitude (0.22 mV) and duration (0.12 sec) are increased in lead II and there is deep terminal negativity in V1. Courtesy of Morton Arnsdorf, MD.

Representation of echocardiographic findings in secondary pulmonary hypertension

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Schematic representation of two-dimensional echocardiographic changes in secondary pulmonary hypertension. The main findings are right ventricular enlargement (RVE), right ventricular hypertrophy (RVH), right atrial enlargement (RAE), functional tricuspid regurgitation (TR) with a detectable regurgitant jet, and a mid-systolic notch on the pulmonary artery flow tracing. There is also paradoxic bulging of the septum into the left ventricle during systole. Adapted from Otto, C.
Cardiomyopathies, hypertensive and pulmonary heart disease. In: Textbook of Clinical Echocardiography, Otto, C, Pearlman, AS (Eds), Saunders, Philadelphia, 1995.

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