PROTEIN-LOSING ENTEROPATHY AFTER THE FONTAN OPERATION

Patients were observed after the Fontan operation to determine the fre quency and severity of protein-losing enteropathy. A total of 427 pati ents who survived for 30 days after the Fontan operation, performed be tween 1973 and January 1987, were analyzed and, thus far, protein-losi ng enteropathy has developed in 47 of 427, The cumulative risk for the development of protein-losing enteropathy by 10 years was 13.4% among 30-day survivors, and 5-year survival after the diagnosis was 46%, He modynamic studies done coincident with the diagnosis of protein-losing enteropathy have shown increased systemic venous pressure, decreased cardiac index, increased pulmonary vascular resistance, and increased ventricular end-diastolic pressure, Medical management of protein-losi ng enteropathy was only partially successful, Statistical analysis has shown that factors related to protein-losing enteropathy were ventric ular anatomy, increased preoperative ventricular end-diastolic pressur e, longer operative bypass time, increased length of hospital stay, an d postoperative renal failure, This study suggests that scrupulous sel ection of cases for the Fontan operation is mandatory and that certain perioperative factors may predispose to this serious complication of the Fontan procedure.