|
| (I) Pulmonary arterial hypertension (PAH) |
| (1) Idiopathic PAH |
| (2) Heritable |
| (i) BMBR2 |
| (ii) ALK1, endoglin (with or without hereditary hemorrhagic telangiectasia) |
| (iii) Unknown |
| (3) Drug and toxin induced |
| (4) Associated with |
| (i) Connective tissue diseases |
| (ii) HIV infection |
| (iii) Portal hypertension |
| (iv) Congenital heart diseases |
| (v) Schistosomiasis |
| (vi) Chronic hemolytic anemia |
| (5) Persistent pulmonary hypertension of the newborn |
| (II) Pulmonary hypertension due to left heart disease |
| (1) Systolic dysfunction |
| (2) Diastolic dysfunction |
| (3) Valvular disease |
| (III) Pulmonary hypertension due to lung diseases and/or hypoxia |
| (1) Chronic obstructive pulmonary disease |
| (2) Interstitial lung disease |
| (3) Other pulmonary diseases with mixed restrictive and obstructive patterns |
| (4) Sleep-disordered breathing |
| (5) Alveolar hypoventilation to high altitude |
| (6) Developmental abnormalities |
| (IV) Chronic thromboembolic pulmonary hypertension (CTEPH) |
| (V) PH with unclear multifactorial mechanisms |
| (1) Hematologic disorders: myeloproliferative disorders and splenectomy |
| (2) Systemic disorders: sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, neurofibromatosis, and vasculitis |
| (3) Metabolic disorders: glycogen storage disease, Gaucher disease, and thyroid disorders |
| (4) Others: tumoral obstruction, fibrosing mediastinitis, and chronic renal failure (on dialysis) |
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