Aetiology of pulmonary hypertension(PH) in children
| Data source | Clinical study | Post-marketing surveillance | National registry | National registry |
| First author [Ref.] | Rosenzweig [2] | Beghetti [3] | Fasnacht [4] | Haworth [5] |
| Enrolled patients | 86 | 146 | 23 | 216 |
| Females | 49 (57) | 71 (49) | 11 (48) | 117 (54) |
| Age yrs | 0–18 | 2–11 | 0–18 | 0–19 |
| PH aetiology | ||||
| IPAH | 36 (42) | 59 (40) | 8 (35)# | 60 (28) |
| FPAH | ||||
| PAH–mixed CTD | 2 (2.3) | 1 (0.7) | 9 (4.2) | |
| PAH–scleroderma | 3 (2.1) | |||
| PAH–CHD | 48 (56) | 66 (45) | 12 (52) | 104 (48)¶ |
| PAH–other | 13 (8.9) | 14 (6.5) | ||
| CTEPH | 1 (0.7) | |||
| PH–pulmonary disease | 2 (1.4) | 3 (13) | 29 (13) | |
| PPH | 1 (0.7) |
Data are presented as n, range or n (%). PAH: pulmonary arterial hypertension; IPAH: idiopathic PAH; FPAH: familial PAH; CTD: connective tissue disease; CHD: congenital heart disease; CTEPH: chronic thromboembolic PH; PPH: portopulmonary hypertension; #: includes familial PH; ¶: includes 47 patients with post-operative PH.