|
| (1) Pulmonary artery hypertension | |
| (1.1) Idiopathic (IPAH) | |
| (1.2) Hereditary (HPAH)—BMPR2, ALK-1, endoglin | |
| (1.3) Drug and toxin induced | |
| (1.4) Associated pulmonary artery hypertension (APAH) | |
| (1.4.1) Connective tissue disorders | |
| (1.4.2) HIV infection | |
| (1.4.3) Portal hypertension | |
| (1.4.4) Congenital heart diseases | |
| (1.4.5) Schistosomiasis | |
| (1.4.6) Chronic hemolytic anemia | |
| (1.4.7) Persistent newborn pulmonary hypertension | |
| (1.5) Pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis (PCH) | |
| (2) Pulmonary hypertension caused by left-heart disease | |
| (2.1) Systolic dysfunction | |
| (2.2) Diastolic dysfunction | |
| (2.3) Valve disease | |
| (3) Pulmonary hypertension secondary to pulmonary diseases and/or hypoxemia | |
| (3.1) Chronic obstructive pulmonary disease | |
| (3.2) Interstitial pulmonary disease | |
| (3.3) Other pulmonary diseases with mixed restrictive and obstructive patterns | |
| (3.4) Sleep-disordered breathing | |
| (3.5) Alveolar hypoventilation disorders | |
| (3.6) Chronic high-altitude exposure | |
| (3.7) Developmental abnormalities | |
| (4) Chronic thromboembolic pulmonary hypertension (CTEPH) | |
| (5) Pulmonary hypertension with unclear or multifactorial mechanisms | |
| (5.1) Hematological disorders: myeloproliferative and splenectomy | |
| (5.2) Systemic disorders: sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, neurofibromatosis, and vasculitis | |
| (5.3) Metabolic disorders: glycogen storage disease, Gaucher's disease, and thyroid disorders | |
| (5.4) Others: tumoral obstruction, fibrous mediastinitis, and chronic renal failure with dialysis | |
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