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Updated: Jan 10, 2026
Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
Challenges and Growing experience in Heart Transplantation in Adult Congenital Heart Disease
Ashish H Shah1, Darren H Freed2, Ananthraman Rajaraman3
1Section of Cardiology, Department of Internal Medicine, Rady Faculty of Health Sciences, University of Manitoba, Winnipeg, Manitoba, Canada.
Insights
Adults with congenital heart disease (CHD) face heart failure (HF) as a leading cause of death. Heart transplantation offers a final treatment option for end-stage HF in CHD patients, requiring specialized multidisciplinary evaluation.
Area of Science:
- Cardiology
- Adult Congenital Heart Disease
- Heart Transplantation
Background:
- Advances in congenital heart disease (CHD) management have increased survival into adulthood.
- Heart failure (HF) is the primary cause of mortality in the adult CHD population.
- Unique HF characteristics in CHD patients complicate diagnosis and treatment response.
Purpose of the Study:
- To review current understanding of heart transplantation in adult CHD patients.
- To highlight challenges and considerations specific to CHD in heart transplantation.
- To discuss outcomes, surgical aspects, immunology, organ allocation, and Fontan patient considerations.
Main Methods:
- Review of current literature on heart transplantation for congenital heart disease.
- Synthesis of information on historical outcomes, surgical techniques, and immunological factors.
- Analysis of organ allocation policies and special considerations for the Fontan population.
Main Results:
- Heart transplantation outcomes in CHD patients have improved over time.
- Complex anatomical and physiological features necessitate expert multidisciplinary evaluation.
- Special considerations exist for the Fontan population, including combined heart-liver transplantation.
Conclusions:
- Heart transplantation is a viable option for end-stage heart failure in adult CHD patients.
- Comprehensive evaluation at specialized centers is crucial for successful transplantation.
- Ongoing research and specialized approaches are needed for optimal outcomes in this complex population.
Abstract:
With advances in congenital heart disease (CHD) management, most infants now survive into adulthood. Heart failure (HF) remains the leading cause of death in this growing adult cohort. CHD patients can present with unique characteristics of failure, not only making diagnosis more challenging but also limiting the response to guideline-directed HF strategies. Heart transplantation in those with end-stage failure remains the final treatment option. Distinct from the acquired HF population, CHD patients often present with complex anatomical and physiological features, and comprehensive multidisciplinary evaluation of potential transplant candidates in an expert adult CHD transplant centre is recommended. This review summarizes the current understanding of heart transplantation in CHD patients, with a focus on historical perspective and improved outcomes, surgical considerations, immunological challenges, organ allocation policies, and special considerations in the Fontan population, including the emerging role of combined heart-liver transplantation.
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