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

https://doi.org/10.1016/j.amjcard.2006.09.119Get rights and content

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 (<6 minutes) and side effects, including jaw pain, flushing, and headache. Recently, intravenous treprostinil has been studied, primarily in adults with PAH, and found to provide effective therapy. The effects of continuous intravenous treprostinil were retrospectively evaluated in 13 children with stable PAH who had been treated with epoprostenol for >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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Method and Results

This was a retrospective analysis designed to evaluate the safety and tolerability of transitioning pediatric patients with PAH receiving stable intravenous epoprostenol doses to intravenous treprostinil. All patients were enrolled in an institutional review board–approved protocol, A Prospective Evaluation of Adolescents and Children With Pulmonary Arterial Hypertension (PEACH). This protocol allows follow-up analysis of pediatric patients with PAH receiving different therapies. Patients

Discussion

The purpose of this retrospective analysis was to evaluate the tolerability and safety of transitioning stable pediatric patients with PAH from epoprostenol to treprostinil. Stable patients were safely transitioned in the hospital with close observation, with further dose escalation of treprostinil on an outpatient basis. Although rapid and slow transitions were well tolerated, slow transition theoretically may be safer for patients with more severe disease. Assessments at 6 and 12 months after

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This study was supported in part by Grant M01-RR00069 from the General Clinical Research Center, National Center for Research Resources, National Institutes of Health, Bethesda, Maryland.

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