ABSTRACT
As the operative mortality has diminished and the number of survivors has increased after Fontan procedure, morbidities related to the unnatural physiology of cavopulmonary flow have developed. One of the complications by the hemodynamic derangement after Fontan procedure is a protein-losing enteropathy. This is a rare but life-threatening complication after the Fontan operation. Treatment strategies are highly variable. But, reports on successful management are limited. We experienced three cases of protein-losing enteropathy after the Fontan operation. We report that the conversion to the total extracardiac or intracardiac cavopulmonary connection with fenestration is a satisfactory treatment modality for protein- losing enteropathy after the Fontan operation.