Cor Pulmonale: Clinical Features and Diagnosis

Introduction

Definition: Cor pulmonale is right ventricular (RV) dysfunction or failure secondary to pulmonary hypertension caused by diseases of the lungs, pulmonary vasculature, or chest wall (❌ not due to left-sided heart disease).

Clinical Presentation

1. Symptoms

Early / Compensated stage

• Dyspnea on exertion (most common)

• Fatigue, reduced exercise tolerance

• Chest discomfort (due to RV ischemia)

• Palpitations

• Syncope or presyncope (suggests severe pulmonary hypertension)

Late / Decompensated stage (Right heart failure)

• Peripheral edema (ankles → generalized)

• Abdominal distension, ascites

• Right upper quadrant pain (hepatic congestion)

• Anorexia, early satiety

• Oliguria (low cardiac output)
  

2. Signs (Physical Examination)

General

• Central cyanosis (chronic hypoxemia)

• Clubbing (if underlying chronic lung disease)

• Cachexia (advanced COPD)

Cardiovascular

• Raised JVP (prominent a wave)

• Parasternal heave (RV hypertrophy)

• Loud P2 (pulmonary hypertension)

• Right-sided S3 or S4

• Tricuspid regurgitation murmur (holosystolic, ↑ with inspiration – Carvallo sign)

Respiratory

• Signs of underlying lung disease:

  • Wheeze (COPD, asthma)

  • Crackles (ILD)

  • Reduced breath sounds

Abdomen & Peripheral

• Hepatomegaly (tender)

• Ascites

• Bilateral pitting edema

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Diagnostic Criteria for Cor Pulmonale

Core Concept (VERY IMPORTANT FOR EXAMS)

You must prove 2 things:1️⃣ Pulmonary hypertension2️⃣ Right ventricular dysfunctionAND exclude left heart disease

1. Evidence of Pulmonary Hypertension

Echocardiography (first-line, non-invasive)

• Elevated estimated pulmonary artery systolic pressure (PASP)

• RV hypertrophy or dilation

• Interventricular septal flattening (“D-shaped LV”)

• Tricuspid regurgitation

Right Heart Catheterization (Gold Standard)

• Mean pulmonary artery pressure (mPAP) ≥ 20 mmHg

• Pulmonary capillary wedge pressure (PCWP) ≤ 15 mmHg→ confirms pre-capillary PH

2. Evidence of Right Ventricular Dysfunction

Echocardiography

• RV dilation

• Reduced TAPSE (<17 mm)

• Reduced RV fractional area change

ECG

• Right axis deviation

• P pulmonale (peaked P in II)

• RV hypertrophy

• RBBB

Chest X-ray

• Enlarged right heart border

• Prominent pulmonary arteries

• Underlying lung pathology

3. Evidence of Underlying Lung / Pulmonary Disease

Depending on cause:

• COPD → hyperinflation, ↓ DLCO

  • DLCO = Diffusing Capacity of the Lung for Carbon Monoxide, a test of gas transfer across the alveolar-capillary membrane.

• ILD → restrictive pattern

• Pulmonary embolism → CT pulmonary angiography

• OSA → sleep study

4. Exclusion of Left-Sided Heart Disease (MANDATORY)

❌ No LV systolic or diastolic dysfunction ❌ No significant mitral or aortic valve disease ❌ Normal PCWP on right heart cath

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Common Causes (High-yield)

Chronic

• COPD (most common)

• Interstitial lung disease

• Obstructive sleep apnea

• Pulmonary hypertension (Group 3)

• Kyphoscoliosis

Acute (Acute cor pulmonale)

• Massive pulmonary embolism

• Severe ARDS

• Acute severe asthma
  

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One-line Exam Diagnosis Statement

“Cor pulmonale is diagnosed by evidence of pulmonary hypertension with right ventricular dysfunction caused by lung or pulmonary vascular disease, in the absence of left-sided heart disease.”

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Exam Pearls

✅ Loud P2 = pulmonary hypertension ✅ Edema + JVP + dyspnea + COPD = think cor pulmonale ❌ LV failure = NOT cor pulmonale ⭐ Gold standard for PH = right heart catheterization