Why the study?
Continuous intravenous epoprostenol infusion negatively impacts quality of life and frequently causes complications such as catheter-related bloodstream infections in patients with PAH.
Can oral selexipag be used to successfully wean an adolescent patient with idiopathic PAH off continuous intravenous epoprostenol?
Can oral selexipag be used to successfully wean an adolescent patient with idiopathic PAH off continuous intravenous epoprostenol?
Oral selexipag may be an effective alternative to continuous intravenous epoprostenol in young patients with PAH, potentially improving quality of life and reducing catheter-related complications.
Successful epoprostenol weaning feasible in one adolescent idiopathic PAH case; hypothesis-generating and should not yet change practice.
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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Nagai et al. (2022) studied this question.
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