1.Pulmonary arterial hypertension (PAH) |
| 1.1 Idiopathic (IPAH) |
| 1.2 Heritable |
| 1.3 Drug- or toxin-induced |
| 1.4 Associated PAH (APAH) associated with: |
| | 1.4.1 Connective tissue disease |
| | 1.4.2 HIV infection |
| | 1.4.3 Portal hypertension |
| | 1.4.4 Congenital heart defect |
| | 1.4.5 Schistosomiasis |
| 1´. Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis |
| 1´´. Persistent PH of the newborn |
2. Increased pulmonary blood pressure due to left heart disease |
| 2.1 Systolic dysfunction |
| 2.2 Diastolic dysfunction |
| 2.3 Valvular disease |
| 2.4 Congenital/acquired obstruction of the cardiac left in-/outflow tract and congenital cardiomyopaties |
| 2.5 Congenital/acquired stenosis of pulmonary veins |
3. Increased pulmonary blood pressure due to lung disease and/or states causing hypoxaemia |
| 3.1 Chronic obstructive pulmonary disease |
| 3.2 Interstitial lung disease |
| 3.3 Other lung disease associated with both restrictive and obstructive hypoventilation |
| 3.4 Sleep-disordered breathing |
| 3.5 States causing alveolar hypoventilation |
| 3.6 Extended stay in high altitudes |
| 3.7 Developmental disorder |
4. Chronic thromboembolic PH |
| 4.1 CTEPH |
| 4.2 Other conditions causing obstruction of pulmonary arteries (e.g. angiosarcoma, other intravascular tumours, arteritis) |
5. Increased pulmonary blood pressure developing by undefined and/or multiple mechanisms |
| 5.1 Haematological states: chronic haemolytic anaemia, myeloproliferative disorders, status post splenectomy |
| 5.2 Systemic diseases: sarcoidosis, pulmonary, histiocytosis, lymphangioleiomyomatosis, neurofibromatosis |
| 5.3 Metabolic disorders, e.g. Gaucher's disease, thyroid disorders |
| 5.4 Other: e.g. mediastinal fibrosis, chronic renal failure |