1 Pulmonary arterial hypertension | 75 (87.2) | 85 (67.5) | ×1.2 |
1.1 Idiopathic | 15 (17.5) | 17 (13.5) | |
1.2 Heritable | 2 (2.3) | 4 (3.2) | |
1.2.1 BMPR2 | | | |
1.2.2 ALK1, endoglin | | | |
1.2.3 Unknown | | | |
1.3 Drugs and toxins 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 disease | 58 (67.5) | 64 (51) | |
1.4.5 Schistosomiasis | | | |
1.4.6 Chronic haemolytic anaemia | | | |
1.5 Persistent PH of the newborn | | | |
1′ Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis | | 1 | |
2 PH due to left heart disease | 2 (2.3) | 4 (3.2) | ×2 |
3 PH due to lung disease and/or hypoxia | 6 (7) | 24 (19) | ×4 |
3.1 Chronic obstructive pulmonary disease | | | |
3.2 Interstitial lung disease | | 5 | |
3.3 Other pulmonary diseases with mixed restrictive and obstructive pattern | | 4 | |
3.4 Sleep-disordered breathing | | | |
3.5 Alveolar hypoventilation disorders | 2 | 11 | |
3.6 Chronic exposure to high altitude | | | |
3.7 Developmental abnormalities | 4 | 4 | |
4 Chronic thromboembolic PH | | | |
5 PH with unclear and/or multifactorial mechanisms | 3 (3.5) | 13 (10.3) | ×4.3 |
5.1 Haematological disorders: myeloproliferative disorders and splenectomy | 3 | 11 | |
5.2 Systemic disorders: sarcoidosis, pulmonary Langerhans’ cell histiocytosis, lymphangioleiomyomatosis, neurfibromatosis and vasculitis | | 2 | |
5.3 Metabolic disorders: glycogen storage disease, Gaucher disease and thyroid disorders | | | |
5.4 Others: tumoral obstruction, fibrosing medistinitis and chronic renal failure on dialysis | | | |