The Emerging Need for Combined Heart and Liver Transplantation in Congenital Heart Disease
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LIVER TRANSPLANTATION (D MULLIGAN AND R BATRA, SECTION EDITORS)
The Emerging Need for Combined Heart and Liver Transplantation in Congenital Heart Disease Peter A. Than 1 & Aleah L. Brubaker 1 & Noelle H. Ebel 2 & Elizabeth L. Profita 3 & Carlos O. Esquivel 1
# Springer Nature Switzerland AG 2020
Abstract Purpose of Review 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. Recent Findings 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. Summary 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. Keywords Congenital heart disease . Fontan . Heart transplant . Liver transplant
Background Congenital heart disease (CHD) encompasses a broad range of structural anomalies, including single-ventricle physiology, biventricular physiology with right heart failure, valvular anomalies, and vascular transposition. Advances in pediatric cardiology and congenital cardiac surgery have resulted in dramatically improved survival and a subsequent increase in the number of people living with CHD [1, 2]. The downstream effect of this has been the emergence of delayed complications, such as heart failure, arrhythmias, valvular heart disease, multi-system organ failure, and other causes of late morbidity This article is part of the Topical Collection on Liver Transplantation * Carlos O. Esquivel [email protected] 1
Division of Abdominal Transplantation, Department of Surgery, Stanford University School of Medicine, Stanford, CA, USA
2
Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Stanford University School of Medicine, Stanford, CA, USA
3
Division of Cardiology, Department of Pediatrics, Stanford University School of Medicine, Stanford, CA, USA
and mortality. Some CHD anomalies can be managed with corrective heart surgery, and early intervention may prevent patients from developing end organ sequelae of cardiac dysfunction. In other cases, such as Alagille’s syndrome, congenital structural anomalies in the heart and the liver may be treated with corrective heart surgery followed by liver transplant and rarely require combined heart and liver transplantation. Still other forms of CHD, such as single-ventricle physiology, can be surgically palliated but ulti
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