Oktay Tutarel, Oliver Miera, Felix Berger
Deutsches Herzzentrum der Charité, Freie Universität Berlin and Humboldt-Universität zu Berlin.
Germany
Pulmonary Circulation
Pulm Circ 2026; 16:
DOI: 10.1002/pul2.70283
Abstract
Eisenmenger syndrome represents a complex and severe form of pulmonary arterial hypertension (PAH) associated with congenital heart disease. Sotatercept, a first-in-class activin-signaling inhibitor, offers a novel therapeutic approach in the treatment of PAH, and has emerged as a promising therapeutic agent. However, patients with Eisenmenger syndrome were not included in its pivotal trials. Therefore, data regarding its effect in this patient cohort is lacking. We present two cases of patients with Eisenmenger syndrome treated with sotatercept. Sotatercept improved the subjective and objective exercise capacity in both patients without any significant adverse side effects.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Symptoms and Findings Associated with Pulmonary Vascular Disease
Medical Therapy. Efficacy or Lack of Efficacy
Medical Therapy. Adverse Effects or Lack of Adverse Effects
Age Focus: Adult 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