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Pulmonary Hypertension

Background Knowledge ๐Ÿง 

Definition

Increased pressure in the pulmonary arteries. Diagnosed when mean pulmonary artery pressure โ‰ฅ 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.

Pathophysiology

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Aetiology

  • 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 Features ๐ŸŒก๏ธ

Symptoms

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

Signs

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

Investigations ๐Ÿงช

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.

Diagnostic tests

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Management ๐Ÿฅผ

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.

Complications

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Key Points

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