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Classification of pulmonary hypertension

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

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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