title: Treatment of primary pulmonary hypertension with oral sildenafil.
abstract: Sildenafil, a selective phosphodiesterase-5 inhibitor, was administered orally to a 14-year-old girl with primary pulmonary hypertension (PPH) at a dose of 0.5 mg/kg/4 h on a daily basis, increased to doses of 1 and 2 mg/kg at monthly intervals. Following therapy, oxygen saturation increased and exercise capacity improved significantly, but with no change in pulmonary haemodynamics. No side-effects were noted. Sildenafil may be beneficial in children with PPH, but the mechanism of benefit is unclear.
authors.affiliation: Department of Paediatric Cardiology, Royal Brompton Hospital, London, UK.
authors.forename: Ageliki A
authors.initials: AA
authors.lastname: Karatza
authors.forename: Indra
authors.initials: I
authors.lastname: Narang
authors.forename: Mark
authors.initials: M
authors.lastname: Rosenthal
authors.forename: Andrew
authors.initials: A
authors.lastname: Bush
authors.forename: Alan G
authors.initials: AG
authors.lastname: Magee
mesh_terms: D000284:Administration, Oral; D001794:Blood Pressure / Q000187:drug effects; D002648:Child; D017079:Exercise Tolerance / Q000187:drug effects; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000503:physiopathology; D010726:Phosphodiesterase Inhibitors / Q000008:administration & dosage*; D010879:Piperazines / Q000008:administration & dosage*; D011659:Pulmonary Gas Exchange / Q000187:drug effects; D011687:Purines; D000068677:Sildenafil Citrate; D013450:Sulfones; D016896:Treatment Outcome
chemical_list: D010726:Phosphodiesterase Inhibitors; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D000068677:Sildenafil Citrate