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. 2019 Jan 31;2019(1):CD012621. doi: 10.1002/14651858.CD012621.pub2

1. World Health Organisation/World Symposium classification of pulmonary hypertension.

WHO group Classification
Group 1 Pulmonary arterial hypertension
  • idiopathic PAH

  • PAH with vasoreactivity

  • heritable PAH

  • drugs and toxins

  • associated with:

    • connective tissue disease;

    • HIV;

    • portal hypertension;

    • congenital heart disease;

    • schistosomiasis.


Pulmonary veno‐occlusive disease/pulmonary capillary haemangiomatosis
Persistent pulmonary hypertension of the newborn
Group 2 Pumonary hypertension due to left heart disease
  • due to left heart failure with preserved ejection fraction

  • due to left heart failure with reduced ejection fraction


Valvular heart disease
Congenital post‐capillary obstructive lesions
Group 3 Pulmonary hypertension due to chronic lung disease or chronic hypoxaemia, or both
  • obstructive lung disease

  • restrictive lung disease

  • other lung diseases with a mixed obstructive/restrictive pattern

  • sleep disordered breathing

  • alveolar hypoventilation disorders

  • chronic exposures to high altitudes

  • developmental lung diseases

Group 4 Pulmonary hypertension due to pulmonary artery obstruction
  • chronic thromboembolic pulmonary hypertension

  • other pulmonary artery obstructions

Group 5 Pulmonary hypertension with unclear mechanisms
  • haematologic disorders (chronic haemolytic anaemia, myeloproliferative disorders, splenectomy)

  • systemic disorders (sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis, neurofibromatosis)

  • metabolic disorders (glycogen storage disease, Gaucher disease, thyroid disorders)

  • others (pulmonary tumoral thrombotic microangiopathy, fibrosing mediastinitis, chronic renal failure (with/without dialysis), segmental pulmonary hypertension)

PAH: pulmonary arterial hypertension