|
| (1) Group 1 pulmonary arterial hypertension |
| (1.1) Idiopathic (IPAH) |
| (1.2) Heritable |
| (1.2.1) BMPR2 |
| (1.2.2) AKL1, endoglin (with or without heredity hemorrhagic telangiectasia) |
| (1.2.3) Unknown |
| (1.3) Drug and toxin induced |
| (1.4) Associates with (APAH) |
| (1.4.1) Connective tissue disease |
| (1.4.2) Human immunodeficiency virus (HIV) infection |
| (1.4.3) Portal hypertension |
| (1.4.4) Congenital heart disease |
| (1.4.5) Schistosomiasis |
| (1.4.6) Chronic hemolytic anemia |
| (1.5) Persistent pulmonary hypertension of the newborn |
| 1′ Pulmonary veno-occulsive disease (PVOD) and/or pulmonary capillary hemangiomatosis |
| Group 2 Pulmonary hypertension due to left heart disease |
| (2.1) Systolic dysfunction |
| (2.2) Diastolic dysfunction |
| (2.3)Valvular disease |
| Group 3 Pulmonary hypertension due to lung disease 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 altitudes |
| (3.7) Developmental abnormalities |
| Group 4 Chronic thromboembolic pulmonary hypertension |
| Group 5 pulmonary hypertension due to unclear multifactorial mechanisms |
| (5.1) Hematological disorders: myeloproliferative disorders, splenectomy |
| (5.2) Systemic disorders: sarcoidosis, pulmonary langerhans cell histiocytosis, lymphangioleiomyomatosis, neurofibromatosis, vasculitis. |
| (5.3) Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders. |
| (5.4) Other: tumor obstruction, fibrosing mediastinitis, chronic renal failure on dialysis. |
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