1. Pulmonary arterial hypertension (PAH) |
âââ1.1 Idiopathic |
âââ1.2 Heritable |
1.2.1 BMPR2 |
1.2.2 ALK1, endoglin (with or without hereditary hemorrhagic teleangiectasia) |
1.2.3 Unknown |
âââ1.3 Drugs and toxins induced |
âââ1.4 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 anemia |
âââ1.5 Persistent pulmonary hypertension of the newborn |
1' Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis |
2. Pulmonary hypertension due to left heart disease |
âââ2.1 Sysytolic dysfunction |
âââ2.2 Diastolic dysfunction |
âââ2.3 Valvular disease |
3. Pulmonary hypertension due to lung diseases and/or hypoxemia |
âââ3.1 Chronic obstructive pulmonary disease |
âââ3.2 Interstitial lung disease |
âââ3.3 Other pulmonary diseases with mixed restrictive and obstructive pattern |
âââ3.4 Sleep-disordered breathing |
âââ3.5 Alveolar hypoventilation disorders |
âââ3.6 Chronic exposure to high altitude |
âââ3.7 Developmental abnormalities |
4. Chronic thromboembolic pulmonary hypertension |
5. Pulmonary hypertension with unclear and/or multifactorial mechanisms |
âââ5.1 Hematological disorders: myeloproliferative disorders, splenectomy |
âââ5.2 Systemic disorders: sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, neurofibromatosis, vasculitis |
âââ5.3 Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders |
âââ5.4 Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure on dialysis |