1 Pulmonary arterial hypertension |
1.1 Idiopathic |
1.2 Heritable |
1.2.1 BMPR2 mutation |
1.2.2 Other mutations |
1.3 Drugs and toxins 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 disease |
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 Drugs, toxins and radiation induced |
1′.4 Associated with: |
1′.4.1 Connective tissue disease |
1′.4.2 HIV infection |
1′′ Persistent pulmonary hypertension of the newborn |
2 Pulmonary hypertension 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 |
2.5 Congenital/acquired pulmonary vein stenosis |
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 lung diseases |
4 Chronic thromboembolic pulmonary hypertension and other pulmonary artery obstructions |
4.1 Chronic thromboembolic pulmonary hypertension |
4.2 Other pulmonary artery obstructions |
4.2.1 Angiosarcoma |
4.2.2 Other intravascular tumours |
4.2.3 Arteritis |
4.2.4 Congenital pulmonary arteries stenoses |
4.2.5 Parasites (hydatidosis) |
5 Pulmonary hypertension with unclear and/or multifactorial mechanisms |
5.1 Haematological disorders: chronic haemolytic anaemia, myeloproliferative disorders, splenectomy |
5.2 Systemic disorders, sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis, neurofibromatosis |
5.3 Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders |
5.4 Others: pulmonary tumoral thrombotic microangiopathy, fibrosing mediastinitis, chronic renal failure (with/without dialysis), segmental pulmonary hypertension |