1 Pulmonary arterial hypertension |
1.1 Idiopathic |
1.2 Heritable |
1.2.1 BMPR2 mutation |
1.2.2 ALK1, ENG, SMAD9, CAV1 and KCNK3 mutation |
1.2.3 Unknown |
1.3 Drug and toxin induced |
1.4 Associated with |
1.4.1 Connective tissue disease |
1.4.2 HIV infection |
1.4.3 Portal hypertension |
1.4.4 Congenital heart diseases |
1.4.5 Schistosomiasis |
1′ Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis |
1′.1 Idiopathic |
1′.2 Heritable |
1′.2.1 EIF2AK4 mutation |
1′.2.2 Other mutations |
1′.3 Drug, toxin and radiation induced |
1′.4 Associated with |
1′.4.1 Connective tissue disease |
1′.4.2 HIV infection |
1″ Persistent PH of the newborn |
2 PH due to left heart disease |
2.1 Left ventricular systolic dysfunction |
2.2 Left ventricular diastolic dysfunction |
2.3 Valvular disease |
2.4 Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies |
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 lung diseases |
4 Chronic thromboembolic PH |
5 PH with unclear multifactorial mechanisms |
5.1 Haematological disorders: chronic haemolytic anaemia, myeloproliferative disorders and splenectomy |
5.2 Systemic disorders: sarcoidosis, pulmonary histiocytosis and lymphangioleiomyomatosis |
5.3 Metabolic disorders: glycogen storage disease, Gaucher disease and thyroid disorders |
5.4 Others: tumoural obstruction, fibrosing mediastinitis, chronic renal failure and segmental PH |
BMPR: bone morphogenic protein receptor type II; ALK1: activin receptor-like kinase 1; ENG: endoglin; CAV1: caveolin-1; KCNK3: potassium channel, two pore domain subfamily K, member 3; EIF2AK4: eukaryotic translation initiation factor 2α kinase 4. Reproduced and modified from [1].