PROTEIN-LOSING ENTEROPATHY AFTER THE FONTAN OPERATION - AN INTERNATIONAL MULTICENTER STUDY

Objective: This multicenter study retrospectively analyzes the data on 114 patients with protein-losing enteropathy after Fontan-type surger y, Special attention was given to the different treatment strategies u sed and their effect on outcome. Methods and results: In 35 participat ing centers 3029 Fontan operations were performed. The incidence of pr otein-losing enteropathy in the survivors was 3.7%. The median age at Fontan-type surgery was 8.2 years (range: 0.6 to 32.9 years). Median a ge at diagnosis of protein-losing enteropathy was 11.7 years with a me dian time interval between surgery and diagnosis of 2.7 years (range: 0.1 to 16.4 years). Most patients had edema (79%) and effusions (75%). Hemodynamic data revealed a mean right atrial pressure of 17 +/- SD 5 .3 mm kg with a cardiac index, of 2.4 +/- 0.8 L/min/m(2). Medical trea tment only (n = 52) resulted in a complete resolution of symptoms in 2 5%, no improvement in 29%, and death in 46%. Surgical treatment (n = 5 2) was associated with relief of protein-losing enteropathy in 19%, no improvement in 19%, and death in 62%. In 13 patients 16 percutaneous interventions were performed. This resulted in symptomatic improvement after 12 interventions and no improvement after 4 interventions. Conc lusions: We conclude that the current treatment of protein-losing ente ropathy after Fontan operation is associated with a very high mortalit y and morbidity rate. Preventive strategies and new therapeutic approa ches are necessary.