classification of pulmonary hypertension

Last reviewed 01/2018

The clinical classification is helpful in understanding the different aetiology and determining treatment

    • Group 1 Pulmonary arterial hypertension (PAH)
      • idiopathic PAH
      • PAH associated with connective tissue disease: predominantly seen with scleroderma

    • Group 2 Left heart disease
      • PH associated with left heart dysfunction, systolic, diastolic or valve disease

    • Group 3 Lung disease
      • PH in patients with COPD or interstitial lung disease

    • Group 4 Chronic thromboembolic pulmonary hypertension

    • Group 5 Unclear and multifactorial mechanisms
      • Rare diseases

  • Groups 2 and 3 contain the majority of patients, but in these patients with common medical diseases the underlying condition needs treatment, usually by cardiologists and respiratory physicians respectively. Patients in groups 1 and 4 should be referred for specific treatment by PH specialists in the UK national designated centres


  • 1) British Heart Foundation (August 2012). Factfile: pulmonary hypertension