Pulmonary Hypertension

• Pulmonary hypertension refers to increased resistance and pressure in the pulmonary arteries. It causes strain on the right side of the heart as it tries to pump blood through the lungs. There is back pressure through the right side of the heart and into the systemic venous system.
• Pulmonary hypertension is defined as a mean pulmonary arterial pressure of more than 20 mmHg.
• Causes of Pulmonary Hypertension


  • Group 1 - Idiopathic pulmonary hypertension or connective tissue disease (e.g., systemic lupus erythematous)
  • Group 2 - Left heart failure, usually due to myocardial infarction or systemic hypertension
  • Group 3 - Chronic lung disease (e.g., COPD or pulmonary fibrosis)
  • Group 4 - Pulmonary vascular disease (e.g., pulmonary embolism)
  • Group 5 - Miscellaneous causes such as sarcoidosis, glycogen storage disease and haematological disorders
• Shortness of breath is a common symptom of pulmonary hypertension
• Groups of pulmonary hypertension

  • Group 3 - Chronic lung disease (e.g., COPD or pulmonary fibrosis)
  • Group 4 - Pulmonary vascular disease (e.g., pulmonary embolism)
  • Group 5 - Miscellaneous causes such as sarcoidosis, glycogen storage disease, and haematological disorders
• Signs and Symptoms of pulmonary hypertension

  • Shortness of breath is the main presenting symptom
  • Syncope (loss of consciousness)
  • Tachycardia
  • Raised jugular venous pressure (JVP)
  • Hepatomegaly
  • Peripheral oedema
• Investigations for pulmonary hypertension

  1. ECG changes indicate right-sided heart strain: P pulmonale (peaked P waves), Right ventricular hypertrophy (tall R waves in V1 and V2 and deep S waves in V5 and V6), Right axis deviation, Right bundle branch block

  2. Chest x-ray changes include Dilated pulmonary arteries, Right ventricular hypertrophy

  3. Other investigations include Raised NT‑proBNP blood test result indicates right ventricular failure, Echocardiogram can be used to estimate the pulmonary artery pressure
• Management of idiopathic pulmonary hypertension

  • The prognosis is poor, with a mean survival of 2-3 years after diagnosis if untreated
  • Treatment options include Calcium channel blockers, Intravenous prostaglandins (e.g., epoprostenol), Endothelin receptor antagonists (e.g., macitentan), Phosphodiesterase-5 inhibitors (e.g., sildenafil)
• Management of secondary pulmonary hypertension

  1. Treating the underlying cause such as pulmonary embolism, COPD, or systemic lupus erythematosus

  2. Supportive treatments (e.g., oxygen and diuretics) are used for complications such as respiratory failure, oedema, and arrhythmias