Salvage Use of Sotatercept for Potts Shunt in Refractory Pulmonary Hypertension
TL;DR
It is suggested that short‐term perioperative sotatercept can serve as an effective acute rescue therapy to safeguard high‐risk surgical interventions in critical pediatric PAH.
Abstract
ABSTRACT The reverse Potts shunt (RPS) is a promising palliative intervention for pediatric patients with drug‐refractory severe pulmonary arterial hypertension (PAH), yet it carries substantial risks of perioperative mortality and hemodynamic instability. We report two high‐risk pediatric female patients who developed acute hemodynamic instability and recurrent pulmonary hypertensive crises following successful percutaneous RPS creation. Despite maximal targeted triple therapy and vasoactive support, no sustained improvement was observed. Following multidisciplinary consensus, a single salvage dose of sotatercept (0.3 mg/kg) was administered. Both patients demonstrated rapid, hemodynamic stabilization within 48 h, enabling the swift withdrawal of vasoactive support and inhaled nitric oxide within 1 week. Follow‐up echocardiography revealed significant recovery of right ventricular function, and both patients were successfully weaned off treprostinil. These findings suggest that short‐term perioperative sotatercept can serve as an effective acute rescue therapy to safeguard high‐risk surgical interventions in critical pediatric PAH.