Liver transplantation after stage II palliation for hypoplastic left heart syndrome

Abstract

The association of biliary atresia (BA) with congenital heart diseases has been extensively described, and there are a number of reports on the outcomes of patients in this group who undergo liver transplantation (LT). The intraoperative management and the timing of LT for patients with end-stage liver disease are matters of debate, especially when complex heart diseases are involved. This report describes the outcome after LT for a pediatric recipient with BA and hypoplastic left heart syndrome. The patient underwent Norwood-Sano and Glenn procedures for heart palliation before LT. He was cyanotic, was severely malnourished, and had complications secondary to chronic liver failure. At the time of transplantation, the child was 16 months old and weighed 5175 g. Despite the critical clinical scenario and the long hospitalization period, there were no cardiac, vascular, or biliary complications after LT. At the age of 48 months, the patient was awaiting the final cardiac repair. In conclusion, the presence of complex cardiac malformations may not be a contraindication to LT. An experienced surgical team and a multidisciplinary approach are key to a successful outcome.

Outcomes of liver transplantation in pediatric recipients with cardiovascular disease

“…Bacha et al 10 concluded that patients with end-stage liver disease presented with higher morbidity and mortality in open-heart surgery with cardiopulmonary bypass than children with no liver disease, but the intervention represents a reasonable option for a chance at LT and/or long-term survival. Six publications describe LT in patients with a wide range of complex congenital heart defects 1,9,[11][12][13][14]. The heart procedures were performed before LT in five patients, during LT in one patient (patent ductus arteriosus), and after LT on two occasions.…”

Successful management of living donor liver transplantation for biliary atresia with single ventricle physiology—from peri‐transplant through total cavopulmonary connection: A case report

“…Three LTxs under single ventricle physiology have been reported to date; one was carried out after PA banding (stage I palliation), one after stage II palliation, and the other after Fontan completion . The case most similar to ours was the first one in 2006 in which a 13‐month‐old girl with single atrium, common atrioventricular valve, and intermediate type of ventricular septal defect underwent deceased donor LTx post‐PA banding.…”

“…Although many reports have described successful LTx in pediatric patients with congenital heart diseases, very few such transplantations have been performed in children with single ventricle physiology at various stages . Single ventricle physiology is a condition in which only one functional ventricular chamber pumps blood to the pulmonary and systemic circulation.…”