Outcomes of Fontan Patients Undergoing Combined Heart - Liver Transplantation in Pediatric Hospitals Across the U.S.
Timestops
0:00
Introduction to Fontan Procedure and Liver Disease
This chapter introduces the challenges faced by Fontan patients who require both heart and liver transplants, highlighting the rising incidence of liver disease among these individuals.
0:11
Study Overview and Patient Demographics
An overview of the study examining combined heart-liver transplants in children and young adults, detailing the demographics and complexity of the patient population.
0:23
Survival Rates Post-Transplant
Discussion of the survival rates following combined heart-liver transplantation, emphasizing the 85% one-year survival rate and comparable three-year outcomes.
0:35
Long-Term Outcomes and Effectiveness
Exploration of the long-term outcomes for Fontan patients post-transplant, reinforcing the effectiveness of combined heart-liver transplantation.
0:47
Implications for Pediatric Cardiology
Analysis of the implications of these findings for pediatric cardiology and the management of complex congenital heart defects.
0:59
Future Directions in Transplantation
Discussion on future trends in combined organ transplantation and the importance of ongoing research in this field.
Topic overview
Grant Chappell, Amir Mehdizadeh-Shrifi, Darren Turner, Alexander Bondoc, Suzanne Evans, Alexander G Miethke, Gregory Tiao, Meghan M Chlebowski, Alexander R Opotowsky, David Lehenbauer, Marco Ricci, Awais Ashfaq, David L S Morales
Background: With increasing numbers of patients surviving Fontan palliation, there is a rise in Fontan-associated liver disease and transplantation strategies inclusive of combined heart-liver transplantation (CHLT). Therefore, we reviewed a combined dataset assessing outcomes of pediatric patients undergoing CHLT.
Methods: Patients undergoing Fontan CHLT between 2010 and 2024 at pediatric hospitals were queried from a merged dataset of the Pediatric Health Information System and United Network for Organ Sharing. Matching (2:1) was completed with Fontan heart-only transplant recipients.
Results: A total of 34 patients underwent Fontan CHLT at 9 pediatric hospitals between 2010 and 2024, 82% (n = 28) after 2019. Almost one-half (47%; n = 16) were age ≤18 years at the time of CHLT, with a median age of 19 years (interquartile range, 15-24 years). More than two-thirds (68%) had hypoplastic left heart syndrome, 38% had double-inlet left ventricle, 26% had double-outlet right ventricle, and 18% had tricuspid atresia. One-third of the patients (n = 11) had a Model for End-Stage Liver Disease/Pediatric End-Stage Liver Disease score of ≤10 at CHLT, with 91% hospital survival, and those with a score >10 had 82% survival. One-year survival was 85% overall, 94% for those age >18 years, and 75% for those age ≤18 years. All deaths occurred before hospital discharge and within 6 months of transplantation, with a mean follow-up of 4 years. Three-year survival was similar in the CHLT and heart transplant -only (HT) groups (85% vs 84%; P = .96, log-rank test).
Conclusions: This multi-institutional analysis demonstrates the rapidly increasing numbers of Fontan palliated patients successfully undergoing CHLT in pediatric hospitals. Conditional on surviving to discharge, long-term survival has been excellent, demonstrating a success story in transplantation management for this complex population.
Intended audience: Healthcare professionals and clinicians.
Categories
Disease/Condition
Anatomy/Organ System
Procedure/Intervention
Population
Topic Format
Clinical Task
Keywords
Fontan procedure
heart transplantation
liver transplantation
hypoplastic left heart syndrome
pediatric cardiology
liver disease
combined transplant
survival rates
long-term outcomes
adolescent health
adult congenital heart disease
complex congenital heart defects
post-operative care
transplant eligibility
multi-organ transplant
clinical outcomes
pediatric surgery
transplantation trends
medical research
patient care
Hashtags
#FontanProcedure
#HeartTransplant
#LiverTransplant
#PediatricCardiology
#LiverDisease
#CombinedTransplant
#SurvivalRates
#CongenitalHeartDisease
#PediatricSurgery
#Transplantation
#AdolescentHealth
#ClinicalOutcomes
#MedicalResearch
#PatientCare
#MultiOrganTransplant
#HeartHealth
#ChildHealth
#TransplantEligibility
#ComplexCongenitalDefects
#PostOperativeCare
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