Pulmonary hypertension (PH) is abnormally high pressure in the pulmonary arteries. The current hemodynamic definition (2022 European guideline, retained by the 7th World Symposium on Pulmonary Hypertension in 2024) is a mean pulmonary artery pressure (mPAP) > 20 mmHg at rest, measured by right heart catheterization. (The older threshold was ≥ 25 mmHg.) Pre-capillary PH — the type caused by disease of the pulmonary arteries or lungs — also has a pulmonary artery wedge pressure ≤ 15 mmHg and pulmonary vascular resistance > 2 Wood units.

Clinical groups

Mechanism: vasoconstriction, endothelial dysfunction (↓nitric oxide and prostacyclin, ↑endothelin), vessel wall thickening, and in situ thrombosis raise right ventricular (RV) afterload. The RV first hypertrophies, then dilates and fails.

Cor pulmonale is RV hypertrophy, dilation, and eventually failure caused by disease of the lungs, chest wall/ventilation, or pulmonary vasculature — it excludes RV failure caused by left heart disease or congenital heart disease.

• Chronic cor pulmonale: most often from COPD (chronic hypoxemia → vasoconstriction → PH), also ILD, sleep apnea, kyphoscoliosis
• Acute cor pulmonale: sudden RV failure, most often from a large acute pulmonary embolism with shock (high-risk PE; US Clinical Category E)
• Chronic hypoxemia also causes secondary polycythemia, which raises blood viscosity and RV workload

Early / PH

• Progressive exertional dyspnea (most common), fatigue, weakness
• Exertional chest pain (RV ischemia), palpitations
• Exertional syncope or near-syncope — an ominous sign of a fixed, low cardiac output
• Hoarseness (compressed recurrent laryngeal nerve by enlarged pulmonary artery) — uncommon

Right-sided heart failure (cor pulmonale)

• Jugular venous distension, positive hepatojugular reflux
• Hepatomegaly, right upper quadrant discomfort, ascites
• Dependent peripheral edema, weight gain
• Loud pulmonic component of S2, right-sided S3 or S4, left parasternal (RV) heave, murmur of tricuspid regurgitation
• Cyanosis, clubbing (with underlying lung disease), anorexia and early satiety (gut congestion)

Acute large PE with shock (acute cor pulmonale): sudden chest pain, severe dyspnea, syncope, hypotension, JVD.

For suspected acute PE, the approach starts with clinical probability: in low or intermediate probability a D-dimer is the first test (normal result rules out PE); high probability → CT pulmonary angiography directly.

Treat the underlying cause first (groups 2–5): optimize heart failure and valve disease, COPD and ILD therapy, CPAP for sleep apnea.

Supportive therapy (all groups)

• Long-term oxygen therapy for chronic hypoxemia — corrects hypoxic vasoconstriction; in COPD it slows progression of cor pulmonale and improves survival when used ≥ 15 hours/day
• Diuretics (loop diuretics, spironolactone) for fluid overload — monitor potassium, creatinine, and blood pressure; avoid excessive diuresis because the RV depends on adequate filling
• Anticoagulation: lifelong for CTEPH; not routine in most other PH
• Phlebotomy for symptomatic severe polycythemia
• Digoxin occasionally for atrial arrhythmias (toxicity risk with hypokalemia)

PAH-specific therapy (group 1; some agents in CTEPH and selected group 3) — goals are pulmonary vasodilation, improved endothelial function, reduced RV afterload, and slowed remodeling. Increasing cardiac contractility is not a primary goal (inotropes are only used in acute RV failure).

Interventional/surgical: pulmonary endarterectomy (CTEPH, potentially curative), balloon pulmonary angioplasty, atrial septostomy, lung or heart–lung transplantation.

Listed in priority order.

1. Oxygenation
   • Give oxygen to the prescribed target; maintain long-term oxygen therapy consistently — this is the core intervention for chronic cor pulmonale
   • In COPD, titrate to SpO₂ 88–92%; in PAH, avoid hypoxemia (commonly SpO₂ ≥ 90%)
   • Position upright; monitor SpO₂, RR, mental status
2. Continuous infusion safety (epoprostenol/treprostinil)
   • Never stop or flush the line; never draw blood or give other drugs through it; keep backup supplies; know the facility's emergency plan if the line fails
3. Fluid balance and right heart failure
   • Daily weight (same scale, same time), intake and output, edema, JVD, abdominal girth
   • Sodium restriction (commonly < 2 g/day) and fluid restriction only as prescribed
   • Elevate edematous legs when resting; skin care over edematous areas
   • Electrolytes with diuretics
4. Medication monitoring — BP (vasodilators), liver tests and pregnancy tests (ERAs), CBC (sotatercept, ERAs), INR or anticoagulant safety (CTEPH)
5. Activity
   • Pace activities, rest periods, avoid both prolonged bed rest and strenuous exertion; supervised pulmonary or cardiac rehabilitation
   • Stop activity for chest pain, dizziness, or near-syncope
6. Avoid triggers of acute RV failure
   • Hypoxemia, infection, dehydration or fluid overload, anemia, dysrhythmias, Valsalva/straining (stool softeners), sudden vasodilation
7. Psychosocial — chronic, progressive disease; anxiety and depression; palliative care as appropriate

• Use oxygen as prescribed, including during sleep and exertion; oxygen safety (no smoking, no open flames)
• Vaccinations (influenza, pneumococcal, COVID-19, RSV) — respiratory infections are a major cause of deterioration
• Weigh daily; report a gain of 2–3 lb (about 1 kg) in 1 day or 5 lb (about 2 kg) in 1 week, increasing swelling, or worsening breathlessness
• Limit salt; follow any fluid limit
• Report fainting, chest pain, or dizziness immediately
• Avoid: high altitude and unpressurized flights without oxygen planning, hot tubs and saunas (vasodilation and hypotension), heavy lifting, straining, smoking
• Avoid over-the-counter decongestants (e.g., pseudoephedrine) and check all new medications with the provider — interactions with PAH drugs are common; never use nitrates or erectile dysfunction drugs with PDE-5 inhibitors or riociguat
• Pregnancy carries a very high risk of death in PAH — use reliable contraception, and two methods with teratogenic drugs if required
• Home infusion clients: sterile line care, carry backup pump and drug, know emergency contacts
• Elevate legs to reduce edema; stay active within limits

• PH = mPAP > 20 mmHg at rest (right heart catheterization); pre-capillary PH also has PVR > 2 Wood units
• Left heart disease is the most common cause of PH; cor pulmonale by definition excludes left-sided causes
• Cor pulmonale = RV failure due to lung disease, most commonly COPD
• Signs: JVD, hepatomegaly, peripheral edema, loud P2
• ECG: RV hypertrophy, right atrial enlargement (P pulmonale), right axis deviation
• BNP/NT-proBNP reflects RV strain
• Long-term oxygen is the key therapy for chronic cor pulmonale
• PAH drugs aim for vasodilation, endothelial repair, afterload reduction — not increased contractility
• Never interrupt epoprostenol; no nitrates with PDE-5 inhibitors or riociguat; ERAs are teratogenic and hepatotoxic
• Vaccination prevents deteriorations
• Acute large PE with shock → sudden chest pain, dyspnea, syncope; heparin → aPTT monitoring
• Exertional syncope is an ominous sign