Pulmonary Hypertension

Increased pressure in the pulmonary arteries. Diagnosed when mean pulmonary artery pressure ≥ 25 mmHg at rest.

• Group 1: Pulmonary arterial hypertension (PAH) – idiopathic, hereditary, drug and toxin-induced, associated with other conditions.
• Group 2: Due to left heart disease.
• Group 3: Due to lung diseases and/or hypoxia.
• Group 4: Chronic thromboembolic pulmonary hypertension (CTEPH).
• Group 5: Unclear/multifactorial mechanisms.

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• Idiopathic (unknown cause).
• Heart diseases: left heart failure, valvular disease.
• Lung diseases: COPD, interstitial lung disease.
• Recurrent pulmonary embolism.
• Connective tissue disorders: systemic sclerosis, lupus.
• HIV, liver diseases, congenital heart diseases.

• Dyspnoea on exertion
• Chest pain, often angina-like
• Palpitations
• Syncope (late-stage)

• Signs of right heart failure: Ankle swelling, raised JVP, hepatic enlargement.

• Echocardiography: Estimates pulmonary artery pressure, assesses RV function.
• Right heart catheterisation: Gold standard for diagnosis.
• ECG: Right ventricular hypertrophy, right axis deviation.
• Pulmonary function tests: To exclude lung disease.
• V/Q (Ventilation/Perfusion) scan: To rule out CTEPH.
• CT pulmonary angiography: To assess pulmonary vasculature and exclude other diseases.

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• General measures: Oxygen therapy, diuretics for right heart failure, anticoagulation.
• Specific PAH therapies: Calcium channel blockers, endothelin receptor antagonists, phosphodiesterase type-5 inhibitors, prostacyclin analogues, soluble guanylate cyclase stimulators.
• Surgical: Atrial septostomy, lung transplantation.

• Depends on cause, severity, and response to treatment.
• Idiopathic PAH has a poorer prognosis.
• Monitoring is crucial: functional class, exercise capacity, and echocardiographic findings.

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