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Preliminary experience with bosentan as initial therapy in childhood idiopathic pulmonary arterial hypertension.

Simpson CM, Penny DJ, Cochrane AD, Davis AM, Rose ML, Wilson SE, Weintraub RG

Department of Cardiology, The Royal Children's Hospital, Melbourne, Victoria, Australia.

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.

Published 27 March 2006 in J Heart Lung Transplant, 25(4): 469-73.
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