Updated clinical classification of pulmonary hypertension (from the fifth World Symposium on pulmonary hypertension, Nice, 2013)

Pulmonary arterial hypertension (PAH)1
 Idiopathic PAH1.1
  ALK1, endoglin (with or without hereditary haemorrhagic telangiectasia), SMAD9, CAV1, KCNH31.2.2
 Drug and toxin-induced1.3
  Associated with:1.4
  Connective tissue diseases1.4.1
  HIV infection1.4.2
  Portal hypertension1.4.3
  Congenital heart diseases1.4.4
 Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis1′
 Persistent pulmonary hypertension of the newborn1″
Pulmonary hypertension owing to left heart disease2
 Left ventricular systolic dysfunction2.1
 Left ventricular diastolic dysfunction2.2
 Valvular disease2.3
 Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies2.4
Pulmonary hypertension owing to lung diseases and/or hypoxia3
 Chronic obstructive pulmonary disease3.1
 Interstitial lung disease3.2
 Other pulmonary diseases with mixed restrictive and obstructive pattern3.3
 Sleep disordered breathing3.4
 Alveolar hypoventilation disorders3.5
 Chronic exposure to high altitude3.6
 Developmental abnormalities3.7
Chronic thromboembolic pulmonary hypertension4
Pulmonary hypertension with unclear multifactorial mechanisms5
 Haematological disorders: chronic haemolytic anaemia, myeloproliferative disorders, splenectomy5.1
 Systemic disorders: sarcoidosis, pulmonary Langerhans cell histocytosis, lymphangioleiomyomatosis, neurofibromatosis, vasculitis5.2
 Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders5.3
 Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure, segmental PH5.4
  • BMPR2: bone morphogenetic protein receptor protein type 2; ALK1: activin receptor-like kinase type 1; CAV1: caveolin-1. Reproduced from [4] with permission from the publisher.