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Classification of pulmonary hypertension

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

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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

Reference:

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

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