Use of sotatercept in an adolescent patient with idiopathic pulmonary arterial hypertension

Abstract

An adolescent boy with idiopathic pulmonary arterial hypertension (iPAH) presented with persistent pulmonary hypertension with right ventricular dysfunction despite upfront oral triple combination therapy. Additional sotatercept treatment led to a striking improvement in pulmonary hemodynamics and right ventricular function without adverse side effects. Sotatercept, recently approved for use in adult patients with PAH, may be an effective and safe therapeutic option for pediatric patients with iPAH. Further studies are needed to confirm the long-term efficacy and safety of this treatment strategy.

Learning objective

Sotatercept may be considered as an additional therapy for pediatric patients with idiopathic pulmonary arterial hypertension. Sotatercept treatment in children with idiopathic pulmonary arterial hypertension should be evaluated further.