Group 1: PAH |
1.1 Idiopathic PAH |
1.2 Heritable PAH |
1.2.1 Bone morphogenic protein receptor type II |
1.2.2 ALK-1, ENG, SMAD9, CAV1, KCNK3 |
1.2.3 Unknown |
1.3 Drug and toxin induced (eg anorexigens, amphetamines) |
1.4 Associated with: |
1.4.1 connective tissue disease |
1.4.2 HIV infection |
1.4.3 portal hypertension |
1.4.4 congenital heart disease |
1.4.5 schistosomiasis |
1’ Pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis |
1’’ Persistent pulmonary hypertension of the newborn |
Group 2: Pulmonary hypertension due to left heart disease |
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Group 3: Pulmonary hypertension due to lung diseases and/or hypoxia |
3.1 Chronic obstructive pulmonary disease |
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Group 4: Chronic thromboembolic pulmonary hypertension |
Group 5: Pulmonary hypertension with unclear multifactorial mechanisms |
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HIV = human immunodeficiency virus; PAH = pulmonary arterial hypertension; PH = pulmonary hypertension. |