Cor Pulmonale

Definition
Dilation and hypertrophy of the right ventricle (RV) in response to diseases of the pulmonary vasculature and/or lung parenchyma Sometimes leads to RV failure with elevation of transmural RV end-diastolic pressure Often referred to as pulmonary heart disease Historically, definition has excluded congenital heart disease and those diseases in which the right heart fails secondary to dysfunction of the left side of the heart.

Epidemiology
Prevalence o In the U.S.: 510% of adult heart diseases o Chronic obstructive pulmonary disease (COPD) and chronic bronchitis are responsible for approximately 50% percent of the cases in North America. Sex o More common in men than in women

Risk Factors

Smoking Predisposition for venous thrombosis Residence at high altitude

Etiology
Develops in response to acute or chronic changes in the pulmonary vasculature and/or the lung parenchyma that are sufficient to cause pulmonary hypertension o The common pathophysiologic mechanism in each case is pulmonary hypertension that is sufficient to lead to RV dilation, with or without the development of concomitant RV hypertrophy. o The most common mechanisms that lead to pulmonary hypertension include vasoconstriction, activation of the clotting cascade, and obliteration of pulmonary arterial vessels. Etiology of acute cor pulmonale o Massive or multiple pulmonary emboli Etiology of chronic cor pulmonale o Diseases leading to hypoxic vasoconstriction Chronic bronchitis COPD Cystic fibrosis Chronic hypoventilation o Obesity o Neuromuscular disease

Chest wall dysfunction Living at high altitude o Diseases that cause occlusion of the pulmonary vascular bed Recurrent pulmonary thromboembolism Primary pulmonary hypertension Venocclusive disease Collagen vascular disease Drug-induced lung disease o Diseases that lead to parenchymal disease Chronic bronchitis COPD Bronchiectasis Cystic fibrosis Pneumoconiosis Sarcoidosis Idiopathic pulmonary fibrosis
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Associated Conditions

Obstructive sleep apnea o Sleep-disordered breathing, once thought to be a major mechanism for cor pulmonale, is rarely the sole cause of pulmonary hypertension and RV failure. o The combination of COPD and associated daytime hypoxemia is required to cause sustained pulmonary hypertension in obstructive sleep apnea.

Symptoms & Signs

Acute cor pulmonale o Sudden onset of severe dyspnea and cardiovascular collapse Occurs in the setting of massive pulmonary embolism o Pallor o Sweating o Hypotension o Rapid pulse of small amplitude o Neck vein distention o Pulsatile distended, tender liver o Systolic murmur of tricuspid regurgitation along the left sternal border o Presystolic (S4) gallop Chronic cor pulmonale o Dyspnea: characteristic feature o Tachypnea: characteristic feature o Nonproductive cough o Anterior chest pain o Hepatomegaly

Lower extremity edema Cyanosis due to arterial hypoxemia and low cardiac output o RV heave: palpable along the left sternal border or in the epigastrium o High-pitched pulmonary ejection click may be audible to the left of the upper sternum. o Fixed, narrow splitting of the second heart sound (S2) o Right ventricular protodiastolic gallop (S3) increasing during inspiration o Systolic murmur of tricuspid regurgitation augmented by inspiration o Diastolic murmur of pulmonary regurgitation o Prominent "a" and "v" waves in the jugular venous pulse The onset of RV failure is reflected in: o Increase of venous pressure o Development of larger "v" waves in jugular venous pulse with increasing tricuspid regurgitation o Positive hepatojugular reflux o Gallop rhythm with third and fourth heart sounds (S3 and S4)
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Differential Diagnosis

RV myocardial infarction Left-sided heart failure Congenital heart disease with left-to-right shunting Constrictive pericarditis

Diagnostic Approach
Evaluate the patient for left ventricular systolic and diastolic dysfunction. o Most common cause of right heart failure is not pulmonary parenchymal or vascular disease, but left heart failure. This is not referred to as cor pulmonale. History and physical examination, to determine etiology of cor pulmonale Appropriate laboratory and imaging based on the above o Chest radiography and CT to evaluate pulmonary disease o Brain natriuretic peptide (BNP) o Pulmonary function studies

Laboratory Tests

Acute cor pulmonale o Arterial blood gas Reduced partial pressure of arterial oxygen (PaO2) due to ventilationperfusion mismatch

Low partial pressure of carbon dioxide (PaCO2) due to hyperventilation Chronic cor pulmonale o Complete blood count Elevated hematocrit o Arterial blood gas Reduced PaO2 Elevated PaCO2 due to impaired ventilation BNP and N-terminal BNP levels are: o Elevated in patients with cor pulmonale secondary to RV stretch o May be dramatically elevated in acute pulmonary embolism

Imaging
Radiologic examination o Pulmonary trunk and hilar vessels enlarged o Enlarged descending right pulmonary artery Spiral CT of the chest o Useful in diagnosing acute thromboembolic disease Ventilation-perfusion lung scan o Remains reliable in most centers for establishing the diagnosis of chronic thromboembolic disease High-resolution CT scan of the chest o Most accurate means of diagnosing emphysema and interstitial lung disease Systemic venography to show deep venous thrombosis Echocardiography o Measurement of the thickness of the RV wall, diameter of RV cavity, RV ejection fraction and anatomy of the pulmonary and tricuspid valves o Interventricular septum may be displaced to the left and may move paradoxically during cardiac cycle. o Doppler echocardiography, to estimate pulmonary artery and RV systolic pressure MRI o Useful for assessing RV structure and function (RV mass, wall thickness, cavity volume, and ejection fraction) o Particularly useful in patients who are difficult to image with 2-D echocardiography because of severe lung disease

Diagnostic Procedures

Electrocardiography o P pulmonale: tall, peaked P waves o Right-axis deviation o RV hypertrophy

o Supraventricular tachyarrhythmias are common. Pulmonary function may confirm underlying lung disease. Cardiac catheterization o Right-heart catheterization is useful for confirming the diagnosis of pulmonary hypertension and for excluding elevated left-heart pressures (measured as the pulmonary capillary wedge pressures) as a cause for right heart failure. o Allows precise measurement of pulmonary vascular pressures o Allows calculation of pulmonary vascular resistance o Shows responses of pulmonary vasculature to oxygen and vasodilators o Sometimes helpful to exclude congenital and left heart diseases o Allows pulmonary angiography to confirm the nature of the pulmonary vascular obstruction Lung biopsy o Rarely useful in demonstrating vasculitis in some types of pulmonary vascular disease, such as collagen vascular diseases, rheumatoid arthritis, and Wegeners granulomatosis

Treatment Approach
Acute cor pulmonale o Treatment of pulmonary embolism o Cautious expansion of blood volume to maintain cardiac output o Inhalation of 100% oxygen Chronic cor pulmonale o Treat the underlying disorder.

Specific Treatments
Acute cor pulmonale Treatment of massive pulmonary embolus or multiple pulmonary emboli (See Pulmonary Thromboembolism for detailed treatment information.) o Primary therapy Clot dissolution with thrombolysis or Removal of pulmonary embolus by embolectomy o Secondary prevention Anticoagulation with heparin and warfarin and/or Placement of an inferior vena cava filter

Chronic cor pulmonale Treat the underlying disorder. General principles of treatment o Decrease work of breathing. Noninvasive mechanical ventilation Bronchodilation Steroids o Treat any underlying infection. o Provide adequate oxygenation (oxygen saturation 9092%). o Transfuse packed red blood cells, if patient is anemic. o Phlebotomy, if the hematocrit exceeds 65% o Diuretics Effective in the treatment of RV failure Use judiciously: Chronic diuretic use may lead to contraction alkalosis and worsening hypercapnia. o Digoxin Uncertain benefit May lead to arrhythmias in the setting of tissue hypoxia and acidosis If administered, give at low doses and monitor carefully. Treat RV failure. Agents undergoing evaluation to reduce pulmonary hypertension o Infusion of prostacyclin o Oral endothelin antagonists o Inhalation of nitric oxide

Monitoring
Acute cor pulmonale o Monitoring in the intensive care unit Chronic cor pulmonale o Periodic outpatient follow-up with assessment of pulmonary function and right heart failure o Pulmonary rehabilitation

Complications

Hypoxia Peripheral edema Hepatic congestion/ascites Syncope Death

Prognosis

Once patients with chronic pulmonary or pulmonary vascular disease develop cor pulmonale, their prognosis worsens. Acute cor pulmonale o In the U.S.: 50,000 deaths annually from pulmonary thromboembolism Probably half of persons die within the first hour from acute right heart failure due to massive or multiple emboli Chronic cor pulmonale o 5-year mortality rate: 1050% Improved with supplemental oxygen o Prognosis is poor for patients with respiratory disease, which reflects the seriousness of the underlying pulmonary disease. COPD with cor pulmonale o 3-year mortality rate: 60%

Prevention
Preventive measures are aimed at the underlying cause of cor pulmonale. o Warfarin therapy (secondary prevention of thromboembolism) o Surgical pulmonary thromboendarterectomy for patients with cor pulmonale, pulmonary hypertension from prior pulmonary emboli o Surgical correction of congenital heart disease o Cessation of intravenous drug use o Cessation of smoking o Weight reduction o Treatment of sleep apnea Continuous positive airway pressure Tracheostomy Dental appliances Surgical interventions

ICD-9-CM
416.0 Primary pulmonary hypertension (Includes acute cor pulmonale) 416.9 Chronic pulmonary heart disease, unspecified (includes chronic cor pulmonale)

See Also

Dyspnea Pulmonary Pulmonary Pulmonary Pulmonary

Function Tests Arterial Hypertension, Primary Arterial Hypertension, Secondary Thromboembolism

Internet Sites

Professionals o Homepage American Heart Association Patients o Cor pulmonale MedlinePlus

General Bibliography
Ferretti GR et al: Severity assessment of acute pulmonary embolism: role of CT angiography. Semin Roentgenol 40:25, 2005 [PMID:15732558] Han MK et al: Pulmonary diseases and the heart. Circulation116:2992, 2007 [PMID:18086941] Kessler R et al: "Natural history" of pulmonary hypertension in a series of 131 patients with chronic obstructive lung disease. Am J Respir Crit Care Med 164:219, 2001 [PMID:11463591] Penaloza D, Arias-Stella J: The heart and pulmonary circulation at high altitudes: healthy highlanders and chronic mountain sickness. Circulation115:1132, 2007 [PMID:17339571] Pengo V et al: Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med 350:2257, 2004 [PMID:15163775] Rapaport E: Cor pulmonale, in Textbooks of Respiratory Medicine, JF Murray, JA Nadel (eds). Philadelphia, Saunders, 2000 Rich S, McLaughlin VV: Pulmonary hypertension, in Braunwalds Heart Disease, 8th ed, P Libby et al (eds). Philadelphia, Saunders, 2008 Vieillard-Baron A et al: Acute cor pulmonale in massive pulmonary embolism: incidence, echocardiographic pattern, clinical implications and recovery rate. Intensive Care Med 27:1481, 2001 [PMID:11685341] Weitzenblum E: Chronic cor pulmonale. Heart 89:225, 2003 [PMID:12527688] This topic is based on Harrisons Principles of Internal Medicine, 17th edition, chapter 227, Heart Failure and Cor Pulmonale by DL Mann.