Pulmonary hypertension is defined as an mPAP of more than 25 mmHg at rest, as measured on right heart catheterisation

Aetiology

• Primary arterial hypertension: primary disease of the pulmonary arteries/arterioles
• Secondary arterial hypertension: occurs due to underlying disease or known risk factors, most commonly heart disease (e.g. congenital shunts) and lung fibrosis (e.g. COPD, OSA, pulmonary fibrosis)

Clinical features

Symptoms

• Dyspnoea
• Fatigue
• Weakness
• Angina
• Syncope
• Abdominal distension

Signs

• Left parasternal heave
• Loud P2 heart sound
• Soft pansystolic murmur with tricuspid regurgitation or early diastolic murmur with pulmonary regurgitation

Investigations

• Routine bloods: include FBC, renal and liver biochemistry, thyroid function tests, and serological assays (for underlying autoimmune rheumatic diseases, HIV, hepatitis)
• CXR: shows enlargement of the pulmonary arteries and major branches, may be right atrial and left ventricular enlargement
• ECG: P pulmonale, right ventricular hypertrophy, right axis deviation