Association of Pericardial Tamponade With Initiation of Sotatercept in a Patient With Eisenmenger Syndrome

Khalifah A. Aldawsari, Charles T. Simpkin, Benjamin S. Frank, David Dunbar Ivy

https://doi.org/10.1002/pul2.70300 

Abstract

Eisenmenger syndrome (ES) represents an advanced stage of pulmonary arterial hypertension (PAH) associated with congenital heart disease. It is characterized by elevated pulmonary vascular resistance, with a right-to-left shunt and resting hypoxemia. ES is associated with high morbidity and mortality. Sotatercept, an activin-signaling inhibitor recently approved for PAH, has demonstrated meaningful hemodynamic and clinical benefits; however, post-approval experience has raised concern for an association with pericardial effusion. We report a 21-year-old woman with ES secondary to an unrepaired patent ductus arteriosus, who developed a hemodynamically significant pericardial effusion 84 days after initiating sotatercept. She presented with tamponade physiology requiring urgent pericardiocentesis and drain placement. An extensive evaluation revealed no obvious alternative etiology. A review of the published literature identified 39 cases of pericardial effusion associated with sotatercept, with more than half occurring in patients with connective tissue–associated PAH. This case highlights potential susceptibility in complex physiologic states such as ES and emphasizes the importance of close surveillance.

Read the full case report