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(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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