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An Adolescent Patient With Idiopathic Pulmonary Arterial Hypertension Weaned Off Intravenous Epoprostenol Following Treatment With Selexipag: A Case Report

Chida-Nagai A, Tsujioka T, Sasaki D, Izumi G, Yamazawa H, Takeda A

Frontiers in pediatrics, 2022

2 citations

Abstract

Idiopathic pulmonary arterial hypertension (PAH) is a rare, progressive disease affecting the pulmonary arteries. Epoprostenol, a synthetic prostaglandin analog, is the most potent pharmacological treatment modality used in patients with PAH. However, it requires continuous intravenous infusion, which negatively impacts the patient's quality of life and frequently results in complications, such as catheter-related bloodstream infection. We weaned an adolescent female patient off epoprostenol by gradually introducing oral selexipag over a sustained period, following many years of continuous intravenous epoprostenol use alone. Oral selexipag might have an efficacy comparable to epoprostenol in young patients with PAH.

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Evidence Hub reads this as a case report, done in people. Its evidence level is 5 of 5 (Very low · case / narrative / animal / in vitro / in silico). Case reports, narrative reviews, and animal, cell or computer-model studies give the weakest evidence for human health.

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