Anesthesia for Potts Shunt in a Child with Severe Refractory Idiopathic Pulmonary Arterial Hypertension.

Childhood idiopathic pulmonary arterial hypertension is a progressive and fatal disease. When pulmonary artery pressures become suprasystemic and refractory to medical management, atrial septostomy can be recommended for bridging patients to lung transplantation. Recently, a surgical Potts shunt has been recommended as an alternative rescue therapy, and initial outcome data are promising. The placement of a Potts shunt converts the child to Eisenmenger physiology, which is anticipated to provide an improved quality and duration of life. We present the first description of anesthetic management of a child undergoing surgical Potts shunt for pulmonary arterial hypertension and summarize the multiple, unique intraoperative considerations.