Abstract

Patients with congenital heart disease (CHD) are living longer due to advances in cardiovascular medicine. We sought to review the physiology and evaluation of these CHD patients who demonstrate the emerging need for combined heart and liver transplantation. The principal population affected by failing cardiohepatic physiology are those with single-ventricle physiology palliated to Fontan. Patients are evaluated by a multidisciplinary team including transplant cardiology and hepatology to determine the degree of liver damage and necessity of combined heart and liver transplantation. While still only performed at a limited number of centers, early data suggest good graft and patient survival. Combined heart and liver transplantation is warranted in a specific subset of patients with CHD. En bloc heart and liver transplantation may offer benefits including reduction in ischemia times. As experience increases, additional guidelines to help direct care for these patients will be warranted.

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