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For further information, see CMDT Part 10-54: Pulmonary Heart Disease (Cor Pulmonale)

Key Features

  • Right ventricular (RV) systolic and diastolic failure from lung disease or pulmonary vascular disease

  • Most common causes: pulmonary hypertension, chronic obstructive pulmonary disease (COPD), or idiopathic pulmonary fibrosis

  • Less common causes

    • Pneumoconiosis

    • Kyphoscoliosis

Clinical Findings

  • Predominant symptoms—intensified with RV failure—are related to the underlying pulmonary disorder

    • Chronic productive cough

    • Exertional dyspnea

    • Wheezing

    • Easy fatigability

    • Weakness

  • Other possible findings

    • Dependent edema

    • Right upper quadrant pain (hepatic congestion)

    • Cyanosis

    • Clubbing

    • Distended neck veins

    • RV heave

    • Gallop

    • Ascites

  • Severe lung disease can be a cause of low cardiac output by reducing LV filling and subsequently LV preload and stroke volume

  • Polycythemia is often present

  • Arterial oxygen saturation often < 85%

Diagnosis

  • Symptoms and signs of COPD with elevated jugular venous pressure, parasternal lift, edema, hepatomegaly, ascites

  • ECG

    • Tall, peaked P waves (P pulmonale), right axis deviation, and RV hypertrophy

    • Q waves in leads II, III, and aVF may mimic myocardial infarction

    • Frequent, nonspecific supraventricular arrhythmias

  • Chest radiograph

    • Enlarged RV and pulmonary artery

    • Possible signs of pulmonary parenchymal disease

  • Pulmonary function tests to confirm underlying lung disease

  • Echocardiogram should demonstrate normal LV size and function but with RV and right atrial dilation and RV dysfunction

  • Multi-slice CT scan to exclude pulmonary emboli

  • Serum B-type natriuretic peptide (BNP) level may be elevated from RV dysfunction

Treatment

  • Treat underlying lung disease

  • Oxygen, salt and fluid restriction, and diuretics, often in combination

  • Inotropic agents are useful when acute decompensation occurs

  • Compensated cor pulmonale has the same prognosis as the underlying lung disease

  • Average life expectancy is 2–5 years when signs of heart failure appear, but survival is significantly longer when uncomplicated emphysema is the cause

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