Right Ventricular Assist Device as a Bridge to Recovery Postheart Transplantation for Failed Fontan Circulation

Background: Long-term outcome for children with functionally univentricular hearts has improved. Failure of Fontan circulation and protein loosing enteropathy (PLE) leaves cardiac transplantation as ultimate option. Posttransplantation, these patients are at risk for elevated pulmonary resistance, right ventricular (RV) failure, and early mortality. Supporting the RV with a mechanical circulatory device may allow for recovery of function and hemodynamics.

Clinical Case: A 4-year-old girl with failing Fontan circulation and PLE required urgent Fontan take down for clinical deterioration. She eventually received cardiac transplantation. The postoperative course was complicated by right ventricular failure, high pulmonary pressure, and obstruction of aortic arch. Despite inotropic support, right ventricular function deteriorated requiring implantation of a short-term mechanical circulatory pump. After reconstruction of the aortic arch, several attempts to wean mechanical circulatory support failed. Subsequently, she was switched to a Berlin Heart right ventricular device (RVAD) and listed for second cardiac transplantation. She gradually improved right ventricular function. Moreover, pulmonary hypertension and PLE resolved. Consequently, RVAD support was weaned and the assist device was successfully explanted 15 months posttransplant. PLE recurred 21 months after transplant. RV function remained normal with estimated systolic pressures in the upper normal range.

Conclusion: Heart transplantation is a high-risk procedure in patients with failing Fontan circulation and PLE. RVAD support may facilitate recovery of right ventricular function and improve pulmonary resistance. Altered mesenteric circulation and lymphangiectasia may persist and predisposes to relapsing or persisting PLE.