First author [Ref.] | Patients n | Description | Age of patients yrs | End-points | Results |
Oliveira [30] | 6 | Case series of children with IPAH treated with sildenafil | 10 (3–19) | Haemodynamics6MWDWHO FC | Improved WHO FC and systemic arterial oxygen saturation in all patients |
Oxygen saturation | Some improvements in haemodynamics and exercise capacity | ||||
Karatza [31] | 3 | Case series investigating oxyhaemoglobin saturations and exercise tolerance in children treated with sildenafil | 10.4 (6–13) | Haemodynamics6MWDWHO FC | Improved WHO FC and exercise capacity observed in all three patients |
Humpl [32] | 14 | Open-label study of children with IPAH and PAH-CHD sildenafil | 10.5 (5–18) | 6MWDHaemodynamics | Improvements in haemodynamics and exercise capacity observed |
Data for age is presented as mean (range). IPAH: idiopathic PAH; CHD: congenital heart disease; 6MWD: 6-min walk distance; WHO FC: World Health Organization functional class.