ABSTRACT
A 37-year-old male patient who had previously undergone left original Blalock-Taussig shunt, original Glenn shunt, left pulmonary artery patch plasty, and a Björk procedure was referred to our hospital due to protein-losing enteropathy. Because he suffered from severe low-cardiac output syndrome immediately after the Björk procedure, mechanical circulatory support and construction of the bypass between the right atrial appendage and the innominate vein using an artificial graft were required. We performed a Fontan-revision operation : total cavopulmonary connection with extra-cardiac conduit, right atrial ablation, pacemaker lead implantation, construction of fenestration between the conduit and the atrium, and reconstruction of the left pulmonary artery in front of the ascending aorta successfully. His postoperative course was uneventful and protein-losing enteropathy had not recurred 3 years after the operation.