“…Despite favorable short-term outcomes and lowered mortality rates (1,13,14,17,19), numerous long-term complications have been reported (8) including ventricular dysfunction and limited ventricular power (28), increased pulmonary vascular resistance (4), arrhythmias (6), severely diminished exercise capacity (21, 24, 31), protein losing enteropathy (22), and hepatic complications (4, 9). Although considerable advances have been made in understanding total cavopulmonary connection (TCPC) hemodynamics (16,18,20,29,30) and in pre-operative surgical planning (26), therapies for improving cardiac hemodynamics and averting end-stage complications in the growing population of Fontan patients are currently lacking.Of specific interest to this study are the effects of Fontan circulation on the liver and gastrointestinal (GI) tract. The altered series arrangement of the systemic and pulmonary circulations in the Fontan patient, together with the lack of a functional right heart to pump blood to the lungs, predisposes the systemic veins to higher pressures and in turn elevates hepatic venous pressure.…”