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Pulmonary hypertension

Definition
  • Increased pressure in the pulmonary arteries. Diagnosed when mean pulmonary artery pressure ā‰„ 25 mmHg at rest.
Classification
  • 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.
Etiology
  • 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.
Clinical Presentation
  • Dyspnoea on exertion.
  • Chest pain, often angina-like.
  • Palpitations.
  • Signs of right heart failure: ankle swelling, raised JVP, hepatic enlargement.
  • Syncope (late-stage).
Investigations
  • 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.
Management
  • 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.
Prognosis
  • 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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