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Updated: Sep 10, 2025
Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
Outcomes After Heart Transplantation in Adults With Congenital Heart Disease-A Single-Center Experience
Ahmed Younis1, Sara ElZalabany2, William R Miranda2
1Department of Pediatrics and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota, USA.
Insights
Adults with congenital heart disease (CHD) show improved survival after heart transplantation. Mortality is linked to operative factors, not CHD type, highlighting the need for updated risk assessment.
Area of Science:
- Cardiology
- Transplantation Medicine
- Congenital Heart Disease
Background:
- Adult congenital heart disease (CHD) presents unique challenges for heart transplantation.
- High-volume centers are crucial for managing complex CHD cases.
Purpose of the Study:
- To evaluate outcomes of heart transplantation in adult patients with congenital heart disease (CHD).
- To analyze survival rates and predictors of mortality in this population.
Main Methods:
- Retrospective cohort study of 89 adult CHD patients undergoing heart transplantation.
- Data collected from a high-volume transplant center between 2003 and 2024.
Main Results:
- Overall survival rates (97% at 30 days, 91% at 1 year, 87% at 5 years) exceeded national estimates.
- Fontan physiology patients had lower survival but it wasn't an independent mortality predictor.
- Longer cardiopulmonary bypass, ventilation, and mechanical support predicted mortality.
Conclusions:
- Heart transplantation survival in adults with CHD is improving and surpasses historical data.
- Mortality is associated with perioperative factors, not the underlying CHD physiology.
- Increasing Fontan physiology and multi-organ transplant rates necessitate refined risk stratification and referral strategies.
Background:
The purpose of this study was to describe outcomes after heart transplantation in adults with congenital heart disease (CHD) based on the experience from a high-volume transplant centre.
Method:
We undertook a retrospective cohort study of adults with CHD who underwent heart transplantation at Mayo Clinic, Rochester, Minnesota (2003-2024).
Results:
Of 89 patients (median age 40 years [interquartile range 9-66 years], 52% male) who underwent heart transplantation, 67 (75%) had biventricular physiology and 22 (25%) had Fontan physiology. Fifty (56%) and 39 (44%) received single organ vs multi-organ transplants, respectively. The proportion of patients with Fontan palliation undergoing heart transplantation was higher in the late era (after December 31, 2013): 31% (21/67) vs 5% (1/22); P = 0.005. The 30-day, 1-year, and 5-year survival rates were 97% (95% CI 97%-99%), 91% (95% CI 87%-95%), and 87% (95% CI 82%-92%), respectively, and these rates were higher than the estimates from national registries. Patients with Fontan physiology had lower post-transplantation survival compared with those with biventricular physiology, but Fontan physiology was not an independent predictor of mortality. The predictors of post-transplantation mortality were longer cardiopulmonary bypass time, mechanical ventilation duration, and postoperative mechanical circulatory support.
Conclusions:
We observed a higher post-transplantation survival compared with historical estimates. Post-transplantation mortality was related to operative and postoperative factors, and not to CHD physiology. There was a high proportion of patients requiring multi-organ transplantation, and a rising proportion of patients with Fontan physiology who underwent transplantation in the later part of the study. These findings highlight important demographic changes, the importance of institutional expertise, and the need for improvements in risk stratification and referral patterns to align with these changes.
