1 Pulmonary arterial hypertension |
1.1 Idiopathic |
1.2 Heritable |
1.2.1 Bone morphogenetic protein receptor type II |
1.2.2 Activin receptor-like kinase 1, endoglin (with or without hereditary haemorrhagic telangiectasia) |
1.2.3 Unknown |
1.3 Drug and toxin induced |
1.4 Associated pulmonary arterial hypertension |
1.4.1 Connective tissue disease |
1.4.2 HIV infection |
1.4.3 Portal hypertension |
1.4.4 Congenital heart disease |
1.4.5 Schistosomiasis |
1.4.6 Chronic haemolytic anaemia |
1.5 Persistent pulmonary hypertension of the newborn |
1′ Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis |
2 Pulmonary hypertension due to left heart disease |
2.1 Systolic dysfunction |
2.2 Diastolic dysfunction |
2.3 Valvular disease |
3 Pulmonary hypertension due to lung diseases and/or hypoxia |
3.1 Chronic obstructive pulmonary disease |
3.2 Interstitial lung disease |
3.3 Other pulmonary diseases with mixed restrictive and obstructive pattern |
3.4 Sleep-disordered breathing |
3.5 Alveolar hypoventilation disorders |
3.6 Chronic exposure to high altitude |
3.7 Developmental abnormalities |
4 Chronic thromboembolic pulmonary hypertension |
5 Pulmonary hypertension with unclear and/or multifactorial mechanisms |
5.1 Haematological disorders: myeloproliferative disorders, splenectomy |
5.2 Systemic disorders: sarcoidosis, pulmonary Langerhans’ cell histiocytosis, lymphangioleiomyomatosis, neurofibromatosis, vasculitis |
5.3 Metabolic disorder: glycogen storage disease, Gaucher disease, thyroid disorders |
5.4 Others: tumoural obstruction, fibrosing mediastinitis, chronic renal failure on dialysis |