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Published on: May 7, 2015
Cardiac surgery in children with end-stage liver disease awaiting liver transplantation
Jonah N K Odim1, Jeffrey Wu, Hillel Laks
1Division of Cardiothoracic Surgery, David Geffen School of Medicine, University of California-Los Angeles, California 90095-1741, USA. jodim@mednet.ucla.edu
Insights
Pediatric cardiac repair using cardiopulmonary bypass (CPB) is safe for children awaiting liver transplants. These crucial surgeries can reduce risks before liver transplantation, improving outcomes for critically ill children.
Area of Science:
- Pediatric Cardiology
- Hepatology
- Congenital Heart Disease
Background:
- Children awaiting liver transplantation often have complex congenital heart disease (CHD).
- Cardiac repair in these patients presents unique surgical challenges.
- The safety of cardiopulmonary bypass (CPB) for these children was previously uncertain.
Purpose of the Study:
- To evaluate the safety and outcomes of cardiac repair in infants with end-stage liver disease (ESLD) requiring liver transplantation.
- To test the hypothesis that CPB operations are not associated with prohibitive morbidity and mortality in this population.
Main Methods:
- A retrospective review of five infants with ESLD awaiting liver transplantation who underwent cardiac surgery between 1994 and 2004.
- Assessment of mortality and morbidity as primary and secondary endpoints.
- Utilized the pediatric end-stage liver disease (PELD) score to quantify liver disease severity.
Main Results:
- Five infants (3 male, 2 female) with a mean age of 8.6 months and mean PELD score of 18.0 required CPB for cardiac repair.
- One early mortality occurred due to cardiac arrest during central shunt creation in a patient with myocardial hypertrophy.
- All other patients survived cardiac surgery and subsequently underwent successful liver transplantation.
Conclusions:
- Cardiac repair is a feasible and safe procedure for children with significant CHD awaiting liver transplantation.
- Judicious perioperative support is crucial for minimizing risks associated with cardiac surgery in this vulnerable group.
- Successful cardiac repair can mitigate risks prior to liver transplantation.
Background:
Cardiac repair for congenital heart disease in children awaiting liver transplantation presents unique therapeutic challenges and dilemmas. We tested the hypothesis that operations in these children requiring cardiopulmonary bypass (CPB) were not associated with prohibitive morbidity and mortality.
Methods:
Over the last 10 years (1994-2004), five infants were identified in our database with end-stage liver disease and awaiting liver transplantation that required cardiac surgery. Primary end point for the study was mortality. Secondary end points included morbidity and time to liver transplantation. The new pediatric end-stage liver disease (PELD) model was used to score liver disease severity.
Results:
Three boys and two girls with mean age of 8.6 months (range, 1.5-21 months) and mean PELD of 18.0 (range, 10-29) required CPB for repair. The only early mortality in the series occurred after cardiac arrest during creation of a central shunt. The child expired two days later despite extracorporeal membrane oxygenation support. The patient had important myocardial hypertrophy. All other patients survived and underwent successful liver transplantation.
Conclusions:
Children with significant congenital heart disease awaiting liver transplantation can undergo safe cardiac repair with judicious perioperative support thereby reducing the risks of subsequent liver transplantation.
Related Concept Videos
Kidney Transplant I: Introduction
Kidney Transplant II: Surgical Procedure

