ESC/ERS guidelines: updated clinical classification of pulmonary hypertension (PH)
| 1 PAH |
| 1.1 Idiopathic |
| 1.2 Heritable |
| 1.2.1 BMPR2 |
| 1.2.2 ALK-1, endoglin (with or without hereditary haemorrhagic telangiectasia) |
| 1.2.3 Unknown |
| 1.3 Drugs and toxins induced |
| 1.4 Associated with (APAH) |
| 1.4.1 Connective tissue diseases |
| 1.4.2 HIV infection |
| 1.4.3 Portal hypertension |
| 1.4.4 Congenital heart disease |
| 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 PH due to left heart disease |
| 2.1 Systolic dysfunction |
| 2.2 Diastolic dysfunction |
| 2.3 Valvular disease |
| 3 PH 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 PH |
| 5 PH 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 |
PAH: pulmonary arterial hypertension; BMPR2: bone morphogenetic protein receptor, type 2; ALK-1: activin receptor-like kinase 1; APAH: associated pulmonary arterial hypertension. Reproduced from 11 with permission from the publisher.