Classification of pulmonary hypertension (5th World Symposium on Pulmonary Hypertension, Nice 2013)5
| 1. Pulmonary arterial hypertension (PAH) | |
| 1.1. Idiopathic PAH | |
| 1.2. Heritable PAH | 1.2.1. BMPR2 1.2.2. ALK-1, ENG, SMAD9, CAV1, KCNK3 1.2.3. Unknown |
| 1.3. Drug and toxin induced | |
| 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 diseases 1.4.5. Schistosomiasis |
| 1′ Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis | |
| 1″ Persistent pulmonary hypertension of the newborn | |
| 2. Pulmonary hypertension due to left heart disease | 2.1. LV systolic dysfunction 2.2. LV diastolic dysfunction 2.3. Valvular disease 2.4. Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies |
| 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 lung diseases |
| 4. Chronic thromboembolic pulmonary hypertension | |
| 5. Pulmonary hypertension with unclear multifactorial mechanisms | 5.1. Haematological disorders: chronic haemolytic anaemia, myeloproliferative disorders, splenectomy 5.2. Systemic disorders: sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis 5.3. Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders 5.4. Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure, segmental pulmonary hypertensive |