1. Pulmonary arterial hypertension |
1.1 Idiopathic pulmonary arterial hypertension |
1.2 Heritable |
1.2.1. BMPR2 |
1.2.2. ALK1, endoglin (with or without hereditary haemorrhagic telangiectasia) |
1.2.3 Unknown |
1.3 Drug- and toxin-induced |
1.4 Associated with: |
1.4.1 Connective tissue diseases |
1.4.2 HIV infection |
1.4.3 Portal hypertension |
1.4.4 Congenital heart diseases |
1.4.5 Schistosomiasis |
1.4.6 Chronic haemolytic anaemia |
1.5 Persistent pulmonary hypertension of the newborn |
1'. Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis |
2. Pulmonary hypertension due to left heart disease |
2.1 Systolic dysfunction |
2.2 Diastolic dysfunction |
2.3 Valvular disease |
3. Pulmonary hypertension due to lung diseases 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 altitude |
3.7 Developmental abnormalities |
4. Chronic thromboembolic pulmonary hypertension |
5. Pulmonary hypertension with unclear and/or multifactorial mechanisms |
5.1 Haematological 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 Others: tumoural obstruction, fibrosing mediastinitis, chronic renal failure on dialysis |
BMPR2: bone morphogenetic protein receptor, type II; ALK1: activin receptor-like kinase 1 gene. Reproduced from [2] with permission from the publisher.