Simpson Catherine M, Penny Daniel J, Cochrane Andrew D, Davis Andrew M, Rose Michelle L, Wilson Sarah E, Weintraub Robert G
Department of Cardiology, The Royal Children's Hospital, Melbourne, Victoria, Australia.
J Heart Lung Transplant. 2006 Apr;25(4):469-73. doi: 10.1016/j.healun.2005.11.438. Epub 2006 Feb 28.
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.
