Kohei Kurosaki, Yohei Akazawa, Tomoyasu Yuki, Kohta Takei, Kiyohiro Takigiku
Nagano Children’s Hospital.
Japan
Journal of Cardiology Cases
J Cardiol Cases 2026; 34: 173-176
DOI: 10.1016/j.jccase.2026.06.010
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.
Category
Class I. Idiopathic Pulmonary Hypertension
Medical Therapy. Efficacy or Lack of Efficacy
Medical Therapy. Adverse Effects or Lack of Adverse Effects
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes