Perioperative Management of the Fontan Patient for Cardiac and Noncardiac Surgery.

The Fontan circulation is the single-ventricle approach to surgical palliation of complex congenital heart disease wherein biventricular separation and function cannot be safely achieved. Incremental improvements in this surgical technique, along with improvements in the long-term medical management of these patients, have led to greater survival of these patients and a remarkably steady increase in the number of adults living with this unusual circulation and physiology. This has implications for healthcare providers who now have a greater chance of encountering Fontan patients during the course of their practice. This has particularly important implications for anesthesiologists because the effects of their interventions on the finely balanced Fontan circulation may be profound. The American Heart Association and American College of Cardiology recommend that, when possible, elective surgery should be performed in an adult congenital heart disease center, although this may not be feasible in the provision of true emergency care. This review article summarizes the pathophysiology pertinent to the provision of anesthesia in this complex patient group and describes important modifications to anesthetic technique and perioperative management.