1. PAH |
1.1. Idiopathic PAH |
1.2. Heritable PAH |
1.2.1. BMPR2 |
1.2.2. ALK1, 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 PH of the newborn |
2. PH due to left heart disease |
2.1. Left ventricular systolic dysfunction |
2.2. Left ventricular diastolic dysfunction |
2.3. Valvular disease |
2.4 Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies |
3. PH due to lung diseases and/or hypoxia |
3.1. COPD |
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. CTEPH |
5. PH 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: tumoural obstruction, fibrosing mediastinitis, chronic renal failure, segmental PH |