Anesthetic Management of Pheochromocytoma Resection in Adults with Single Ventricle Physiology

Survival rates for patients with palliated congenital heart disease are increasing, and an increasing number of adults with cyanotic congenital heart disease (CCHD) might require surgical resection of pheochromocytoma-paraganglioma (PHEO-PGL). A recent study supports the idea that patients with a history of CCHD and current or historical cyanosis might be at increased risk for developing PHEO-PGL. We review the anesthetic management of two adults with single-ventricle physiology following Fontan palliation presenting for PHEO-PGL resection and review prior published case reports. We found the use of epidural analgesia to be safe and effective in the operative and postoperative management of our patients.