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

Reference:

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