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(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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