Intraoperative Hemodynamic Instability in a Patient With Ebstein's Anomaly Complicated With Eisenmenger Syndrome

Ebstein's anomaly is a rare congenital displacement of the tricuspid valve resulting in atrialization of the right ventricle. About half of the patients with Ebstein's anomaly also have atrial septal defects, which may lead to chronic shunting and development of Eisenmenger syndrome. We describe a case of a sexagenarian male patient with a history of Ebstein's anomaly complicated with Eisenmenger syndrome undergoing robotic laparoscopic adrenalectomy who presented hemodynamic instability, hypoxemia, and likely right-to-left shunting intraoperatively, as well as the actions taken to correct it and have a successful outcome. Perioperative management of adult patients with congenital heart defects is complex and requires careful monitoring. When available, intraoperative transesophageal echocardiography is strongly recommended. To prevent right-to-left shunting, maintaining elevated systemic vascular resistance with the use of vasopressors and low positive end-expiratory pressure (PEEP) ventilation is critical.