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Adult Congenital Heart Disease Transplantation: Does Univentricle Physiology Impact Early Mortality?
Elizabeth H Stephens1, Joseph A Dearani1, Elaine M Griffeth1
1Department of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota.
Insights
Heart transplantation outcomes for adult congenital heart disease (ACHD) patients show survival is similar beyond one year, despite higher initial risks for univentricular (UniV) physiology patients. This impacts ACHD heart failure management.
Area of Science:
- Cardiology
- Transplantation Medicine
- Adult Congenital Heart Disease
Background:
- Growing population of adult congenital heart disease (ACHD) patients experiencing heart failure.
- Limited data on heart transplantation efficacy in the ACHD population.
- Increasing need for advanced treatment options for ACHD patients.
Purpose of the Study:
- Evaluate heart transplantation outcomes in adult congenital heart disease (ACHD) patients.
- Compare survival and complication rates between biventricular (BiV) and univentricular (UniV) ACHD physiology.
- Identify factors influencing transplantation success in ACHD.
Main Methods:
- Retrospective review of 77 ACHD patients undergoing heart transplantation (1990-2023).
- Kaplan-Meier and competing risk analyses for survival.
- Subgroup analysis comparing BiV and UniV physiology.
Main Results:
- Univentricular (UniV) patients had higher rates of cirrhosis and protein-losing enteropathy pre-transplant.
- Multiorgan transplantation was more frequent in UniV patients (50% vs 23%).
- Survival rates were similar beyond one year post-transplant for both UniV and biventricular (BiV) ACHD patients, despite initial trends.
Conclusions:
- Adult congenital heart disease (ACHD) patients with univentricular (UniV) physiology face higher preoperative risks, including liver disease.
- Heart transplantation is a viable option for ACHD patients, with comparable long-term survival for UniV and BiV physiology.
- Further research is needed to optimize outcomes for UniV ACHD patients undergoing heart transplantation.
Background:
With patients with congenital heart disease increasingly living into adulthood, there is a growing population of patients with adult congenital heart disease (ACHD) who have heart failure. Limited data exist on evaluating heart transplantation in this population.
Methods:
A retrospective review was performed of patients with ACHD who underwent heart transplantation from November 1990 to January 2023. Kaplan-Meier, cumulative incidence accounting for competing risk of death, and subgroup analyses comparing those patients with biventricular (BiV) and univentricular (UniV) physiology were performed. Data are presented as median (interquartile range [IQR]) or counts (%).
Results:
A total of 77 patients with a median age of 36 years (IQR, 27-45 years) were identified, including 57 (74%) BiV and 20 (26%) UniV patients. Preoperatively, UniV patients were more likely to have cirrhosis (9 of 20 [45.0%] vs 4 of 57 [7.0%]; P < .001) and protein losing enteropathy (4 of 20 [20.0%] vs 1 of 57 [1.8%]; P = .015). Multiorgan transplantation was performed in 23 patients (30%) and more frequently in UniV patients (10 [50%] vs 13 [23%]; P = .04). Operative mortality was 6.5%, 2 of 20 (10%) among UniV patients and 2 of 57 (4%) among BiV patients (P = .276). Median clinical follow-up was 6.0 years (IQR, 1.4-13.1 years). Survival tended to be lower among UniV patients compared with BiV patients, particularly within the first year (P = .09), but it was similar for survivors beyond 1 year. At 5 years, the incidence of rejection was 28% (IQR, 17%-38%) and that of coronary allograft vasculopathy was 16% (IQR, 7%-24%).
Conclusions:
Underlying liver disease and the need for heart-liver transplantation were significantly higher among UniV patients. Survival tended to be lower among UniV patients, particularly within the first year, but it was similar for survivors beyond 1 year.
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