Cor Pulmonale – Overview 

Definition: 

Right ventricular enlargement (hypertrophy and/or dilation) caused by pulmonary hypertension secondary to lung disease. 

Aetiology 

Chronic lung diseases (most common): 
COPD (chronic bronchitis > emphysema) 
Interstitial lung disease 
Cystic fibrosis 

Acute causes: 
Massive pulmonary embolism (acute cor pulmonale) 

Other: 
Sleep apnoea 
Pulmonary vascular diseases 

Pathophysiology 

Chronic hypoxia → pulmonary vasoconstriction 
Pulmonary hypertension → ↑ right ventricular afterload 
RV hypertrophy (chronic) → dilation → right heart failure 

Note: Chronic hypoxia → pulmonary vasoconstriction → cor pulmonale. 

Clinical Features 

Symptoms: 
Dyspnoea on exertion → progresses to at rest 
Fatigue, weakness 
Peripheral oedema, ankle swelling 
Syncope in severe cases 

Signs: 
Elevated JVP, right-sided S3 (heart sound) 
Right ventricular heave 
Loud P2 
Hepatomegaly ± ascites 
Cyanosis (late) 

Investigations 

Chest X-ray: Enlarged pulmonary arteries, RV enlargement, maybe COPD signs 
ECG: Right axis deviation, RV hypertrophy, peaked P waves 
Echocardiography: RV enlargement/dysfunction, pulmonary pressures 
Pulmonary function tests: Underlying lung disease 
Right heart catheterization: Gold standard for measuring pulmonary pressures 

Management 

1. Treat underlying lung disease: 
Bronchodilators, corticosteroids for COPD 
Oxygen therapy for chronic hypoxemia (↓ pulmonary vasoconstriction) 

2. Manage heart failure: 
Diuretics for volume overload 
Avoid excessive fluid restriction 

3. Pulmonary vasodilators: 
Only in selected patients with severe pulmonary hypertension 
Note: Oxygen is the only therapy shown to improve survival in chronic cor pulmonale due to COPD. 

Complications 

Right heart failure 
Arrhythmias 
Hepatic congestion 
Sudden death (rare, usually acute cor pulmonale from a massive PE)