What is Pulmonary Hypertension

It is defined as mean pulmonary arterial pressure > 25 mm Hg at rest and > 30 mm Hg during

Causes of Pulmonary Hypertension

1. Idiopathic pulmonary hypertension—no underlying cause. May have genetic predisposition.
2. Secondary pulmonary hypertension—it is more common than idiopathic pulmonary hypertension.
3. Respiratory—COPD, emphysema, chronic bronchitis, DPLD, pulmonary thromboembolism.
4. Cardiac—Left-sided heart failure, mitral stenosis, reversal of shunt (ASD, VSD, PDA).
5. Connective tissue disorders—scleroderma, SLE.
6. Sleep apnoea syndrome and other sleep disorders.
7. Drugs—such as cocaine.


• May be asymptomatic.
• Shortness of breath (orthopnea or paroxysmal nocturnal dyspnea).
• Chest pain, palpitations, cough.
• Haemoptysis (rarely).
• Fatigue.
• Ankle swelling.
• Dizziness, syncope.


• Palpable P2.
• Prominent ‘a’ wave in JVP.
• Left parasternal heave (indicates RVH).
• Epigastric pulsation (indicates RVH).
• Loud P2 on auscultation.
• Early diastolic murmur (Graham–Steel murmur due to pulmonary regurgitation).


• Right-sided heart failure and cor pulmonale.
• Arrhythmia.


• CXR PA View (shows enlargement of pulmonary arteries).
• Doppler echocardiography.
• Pulmonary function test.
• Ventilation-perfusion (V/Q) scan.
• CT scan of chest.
• Other investigations according to suspicion of cause.
• Open-lung biopsy.
• Genetic tests.

Treatment of Pulmonary Hypertension

1. General—reduction of weight, avoid heavy exercise, smoking must be stopped.
2. Oxygen.
3. Diuretics.
4. Digoxin
5. Vasodilator drugs—ACE inhibitors: Sildenafil and Tadalafil.
6. Endothelin receptor antagonists—Bosentan, Ambrisentan.
7. Calcium channel blockers—Amlodipine, Nifedipine.
8. Anticoagulants—Warfarin.
9. Surgery—
–– Atrial septostomy.
–– Heart-lung transplantation in selected cases.
–– Pulmonary thromboendarterectomy.
–– Cardiac arrest.