Transition of Stable Pediatric Patients With Pulmonary Arterial Hypertension from Intravenous Epoprostenol to Intravenous Treprostinil

2007 
Intravenous epoprostenol was the first agent approved by the United States Food and Drug Administration for the management of pulmonary arterial hypertension (PAH). However, epoprostenol therapy carries the risks of a short half-life ( 1 year. Children were transitioned in the hospital over 24 hours using a rapid or slow strategy. The children were a mean age of 11 years (range 3 to 17) and were transitioned to treprostinil from August 2004 to August 2005. The baseline 6-minute walking distance was on average 516 ± 115 m (n = 9) and did not change after transition. Patients were treated with treprostinil for 1.1 ± 0.5 years. There were 2 deaths, and 2 patients transitioned to other therapy. Seven patients experienced ≥1 central-line infection. Despite a higher dose of treprostinil, the side effects were subjectively diminished. In conclusion, treprostinil provides an alternative therapy in children with PAH, with fewer side effects. However, evaluation regarding rates of infection requires further exploration.
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