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