Relation of mesenteric vascular resistance after Fontan operation and protein-losing enteropathy

Abstract

Early survival after the Fontan operation for single ventricle has improved substantially since its inception1–3; however, late-term complications continue to be problematic.4 One such complication, protein-losing enteropathy (PLE), is seen in 3% to 15% of patients after the Fontan operation, with a mortality rate of 50% at 5 years from time of initial diagnosis.5,6 The pathophysiology of this disorder is poorly understood. Although postulated as being related to the potentially deleterious effects of elevated systemic venous pressure, these pressures are frequently no different in patients with PLE than in those without PLE after the Fontan operation.6 To date, no specific circulatory derangement has been identified; thus, the mechanism of the disease remains a mystery. To obtain a better understanding of the pathophysiology of PLE after the Fontan operation, we investigated whether increased mesenteric vascular resistance (MVR) is present in subjects after the Fontan operation.