Cardiology > Cor Pulmonale 

   Background 

Cor Pulmonale is defined as right ventricular (RV) enlargement and/or failure resulting from pulmonary hypertension secondary to diseases of the lung, vasculature, or chest wall. It excludes RV failure due to left heart disease or congenital heart disease. Chronic hypoxic pulmonary vasoconstriction is the key mechanism leading to increased pulmonary vascular resistance and RV afterload. 

II) Classification or Types

• • Acute Cor Pulmonale: Sudden RV dysfunction, usually due to massive pulmonary embolism. 

• • Chronic Cor Pulmonale: Gradual RV remodeling and failure due to long-standing pulmonary hypertension, often from COPD or interstitial lung diseases. 

III) Epidemiology 

• • Sex: More common in men due to higher COPD prevalence. 

• • Age: Typically >50 years, related to chronic lung disease progression. 

• • Race/Region: Higher prevalence in populations with high smoking rates and environmental pollutants. 

• • Social Status: Low socioeconomic groups are at higher risk due to poor access to healthcare and higher exposure to indoor air pollution.

     

   Etiology

I) What causes it

• • Chronic obstructive pulmonary disease (COPD) – most common cause 

• • Interstitial lung diseases (e.g., idiopathic pulmonary fibrosis) 

• • Obstructive sleep apnea (OSA) 

• • Pulmonary embolism (acute or chronic thromboembolic disease) 

• • High altitude exposure (chronic mountain sickness) 

• • Chest wall deformities (e.g., kyphoscoliosis) 

• • Neuromuscular diseases (e.g., amyotrophic lateral sclerosis) 

• II) Risk Factors

• • Smoking 

• • Chronic hypoxia (e.g., due to OSA or lung disease) 

• • Recurrent pulmonary emboli 

• • Untreated obstructive or restrictive lung disease 

• • Poor treatment adherence in chronic lung conditions 

    Clinical Presentation

I) History (Symptoms)

• • Exertional dyspnea – most common early symptom 

• • Fatigue and weakness 

• • Chest discomfort or pressure (due to RV strain) 

• • Palpitations (due to arrhythmias) 

• • Syncope or near-syncope (especially with exertion) 

• • Signs of right heart failure: 

• • Peripheral edema 

• • Abdominal discomfort or fullness (from hepatomegaly) 

• • Ascites 

• • Weight gain due to fluid retention 

• II) Physical Exam / Signs

Vital Signs: 

• • May show tachypnea, hypoxia, or signs of pulmonary hypertension 

Cardiovascular: 

• • Elevated jugular venous pressure (JVP) 

• • Right ventricular heave or lift 

• • Loud P2 (pulmonic component of second heart sound) 

• • Tricuspid regurgitation murmur 

• • S3 gallop (RV origin) 

Pulmonary: 

• • Signs of underlying lung disease (e.g., wheezing in COPD, dry crackles in ILD) 

• • Cyanosis in advanced disease 

Abdomen: 

• • Hepatomegaly 

• • Hepatojugular reflux 

• • Ascites 

Peripheral: 

• • Pitting peripheral edema 

• • Cool extremities (low cardiac output) 

   Differential Diagnosis (DDx)

• • Left-sided heart failure 

• • Primary pulmonary hypertension 

• • Pulmonary embolism 

• • Constrictive pericarditis 

• • Liver disease (cirrhosis causing ascites and edema) 

• • Nephrotic syndrome 

    Diagnostic Tests

Initial Tests: 

• • Electrocardiogram (ECG): 

• • • Right axis deviation 

• • • RV hypertrophy 

• • • R/S ratio >1 in V1 

• • • P pulmonale (peaked P waves) 

• • Chest X-ray: 

• • • Enlarged pulmonary arteries 

• • • RV enlargement 

• • • Signs of underlying lung pathology (e.g., hyperinflation in COPD) 

• • Echocardiography: 

• • • RV dilation or hypertrophy 

• • • Elevated RV systolic pressure 

• • • Septal flattening (D-shaped LV) 

• • • Estimate pulmonary artery pressure 

• • Pulmonary Function Tests (PFTs): 

• • • To assess for obstructive or restrictive lung disease 

• • Arterial Blood Gas (ABG): 

• • • Hypoxemia and/or hypercapnia 

• • BNP or NT-proBNP: 

• • • May be elevated in RV dysfunction 

• • Right Heart Catheterization (gold standard): 

• • • Confirms pulmonary hypertension (mean PAP ≥ 20 mmHg) 

• • • Helps exclude left heart disease 

   Treatment

I) Treat Underlying Cause:

• • Smoking cessation 

• • Oxygen therapy (if chronic hypoxia) 

• • Bronchodilators, steroids, pulmonary rehab in COPD 

• • CPAP for obstructive sleep apnea 

• • Anticoagulation for chronic thromboembolic pulmonary hypertension 

• • Treat infections or interstitial lung diseases

     

• II) Right Heart Failure Management:

• • Diuretics (furosemide, torsemide): for peripheral edema 

• • • Caution: over-diuresis can reduce RV preload and cardiac output 

• • Pulmonary Vasodilators (in select cases of pulmonary arterial hypertension): 

• • • PDE5 inhibitors (sildenafil) 

• • • Endothelin receptor antagonists (bosentan) 

• • • Prostacyclin analogs 

III) Lifestyle and Supportive Measures: 

• • Salt restriction 

• • Exercise as tolerated (cardiopulmonary rehab) 

• • Immunizations: influenza and pneumococcal vaccines 

• • Avoid high altitudes and respiratory depressants (e.g., opioids, benzodiazepines) 

   Patient Education, Screening, Vaccines

• • Education on oxygen use and smoking cessation 

• • Early recognition of worsening dyspnea or edema 

• • Adherence to pulmonary meds and devices (e.g., CPAP) 

• • Pneumococcal and influenza vaccines annually 

• • COVID-19 vaccination as per guidelines 

• • Depression screening in chronic lung disease

     

    Consults/Referrals

• • Pulmonology: for workup and management of lung disease 

• • Cardiology: for RV dysfunction, PH, and echo interpretation 

• • Sleep medicine: for suspected sleep apnea 

• • Social work: support with oxygen therapy access, home care 

• • Palliative care: for advanced cases with poor prognosis 

    Follow-Up

• • Monitor symptoms and oxygen saturation regularly 

• • Reassess RV function and pulmonary pressures via echocardiography 

• • Monitor weight, volume status, and renal function (if on diuretics) 

• • Evaluate adherence and complications from treatment 

• • Adjust therapies based on underlying cause progression