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  • J. Heart Lung Transplant. · Apr 2006

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

    • Catherine M Simpson, Daniel J Penny, Andrew D Cochrane, Andrew M Davis, Michelle L Rose, Sarah E Wilson, and Robert G Weintraub.
    • Department of Cardiology, The Royal Children's Hospital, Melbourne, Victoria, Australia.
    • J. Heart Lung Transplant. 2006 Apr 1;25(4):469-73.

    AbstractSince 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.

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