1 PAH |
1.1 Idiopathic |
1.2 Heritable |
1.2.1 BMPR2 |
1.2.2 ALK-1, endoglin (with or without hereditary haemorrhagic telangiectasia) |
1.2.3 Unknown |
1.3 Drugs and toxins induced |
1.4 Associated with (APAH) |
1.4.1 Connective tissue diseases |
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 PH due to left heart disease |
2.1 Systolic dysfunction |
2.2 Diastolic dysfunction |
2.3 Valvular disease |
3 PH 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 PH |
5 PH 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 disorders: glycogen storage disease, Gaucher disease, thyroid disorders |
5.4 Others: tumoural obstruction, fibrosing mediastinitis, chronic renal failure on dialysis |
PAH: pulmonary arterial hypertension; BMPR2: bone morphogenetic protein receptor, type 2; ALK-1: activin receptor-like kinase 1; APAH: associated pulmonary arterial hypertension. Reproduced from 11 with permission from the publisher.