Preliminary experience with bosentan as initial therapy in childhood idiopathic pulmonary arterial hypertension.
Since September 2001, 7 consecutive patients with childhood idiopathic pulmonary arterial hypertension (IPAH), a rapidly progressive and fatal condition, have been treated with combinations of bosentan, and other therapies (sildenafil/warfarin/epoprostenol), at our institution. Survival and clinical status in these patients were compared with a group of 12 historic control patients who were diagnosed prior to 1997 and received only conventional medical therapy. Survival in the bosentan-treated subjects was better than among historic controls with comparable disease severity (log rank, p = 0.04). Our findings indicate treatment with bosentan permits a delay in IPAH disease progression and, in combination with other therapies, improves survival compared with historic control patients.
Department of Cardiology, The Royal Children's Hospital, Melbourne, Victoria, Australia.
Catherine M
CM
Simpson
Daniel J
DJ
Penny
Andrew D
AD
Cochrane
Andrew M
AM
Davis
Michelle L
ML
Rose
Sarah E
SE
Wilson
Robert G
RG
Weintraub
D000367:Age Factors; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D008297:Male; D013449:Sulfonamides / Q000627:therapeutic use*; D016019:Survival Analysis
D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan