[Perioperative Management of Fontan Operation]

Surgical results of Fontan operation has been improved over the decades due to the introduction of the staged operations and some modifications of Fontan route from the classical atrio-pulmonary connection to total cavo-pulmonary connection. However, issues remain because of the single ventricular physiology of Fontan circulation. This article explains about the preoperative checklist for Fontan operation and tips on the postoperative management including an early extubation, use of inhaled nitric oxide after extubation, anticoagulation therapy, and efficacy of angiotensin converting enzyme inhibitor, supported by the cutting-edge evidence. Some patients who underwent Fontan operation, however, suffer from protein-losing enteropathy, heart failure, and thus are classified as failing Fontan. Treatment for these patients with failed Fontan is an unsolved problem in the state where heart transplantation is inadequately available in Japan.