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Updated: May 12, 2026

Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
Heart transplantation in congenital heart disease: in whom to consider and when?
Christine H Attenhofer Jost1, Dörthe Schmidt, Michael Huebler
1Division of Cardiac Surgery, University Hospital Zurich, Zurich, Switzerland.
Insights
Adults with complex congenital heart disease (CHD) increasingly need heart transplantation (HTx) due to end-stage heart failure. While HTx in CHD carries risks, outcomes have improved, remaining a vital treatment option.
Area of Science:
- Cardiology
- Adult Congenital Heart Disease
- Transplantation Medicine
Background:
- Improvements in surgical repair allow complex congenital heart disease (CHD) patients to reach adulthood.
- These patients face a high risk of end-stage heart failure, increasing the need for heart transplantation (HTx).
Purpose of the Study:
- To summarize the evolving causes of heart failure deaths in adults with CHD.
- To discuss reasons for, contraindications to, and risks associated with HTx in CHD patients.
- To review outcomes and future trends in HTx for complex CHD.
Main Methods:
- Review of current literature on heart transplantation for congenital heart disease.
- Discussion of etiologies of heart failure in adult CHD.
- Presentation of case vignettes illustrating challenges and outcomes of HTx in CHD.
Main Results:
- HTx for CHD presents higher perioperative death and rejection risks than for acquired heart disease.
- Outcomes for HTx in complex CHD have improved significantly over the last two decades.
- Heart-lung transplantation is less frequently needed, while lung transplantation with intracardiac repair is an option for specific defects.
Conclusions:
- Heart transplantation remains a crucial treatment for end-stage heart failure in adults with complex CHD.
- Mechanical circulatory support may reduce waiting list mortality.
- Continued advancements are improving HTx outcomes for CHD patients.
Abstract:
Due to impressive improvements in surgical repair options, even patients with complex congenital heart disease (CHD) may survive into adulthood and have a high risk of end-stage heart failure. Thus, the number of patients with CHD needing heart transplantation (HTx) has been increasing in the last decades. This paper summarizes the changing etiology of causes of death in heart failure in CHD. The main reasons, contraindications, and risks of heart transplantation in CHD are discussed and underlined with three case vignettes. Compared to HTx in acquired heart disease, HTx in CHD has an increased risk of perioperative death and rejection. However, outcome of HTx for complex CHD has improved over the past 20 years. Additionally, mechanical support options might decrease the waiting list mortality in the future. The number of patients needing heart-lung transplantation (especially for Eisenmenger's syndrome) has decreased in the last years. Lung transplantation with intracardiac repair of a cardiac defect is another possibility especially for patients with interatrial shunts. Overall, HTx will remain an important treatment option for CHD in the near future.
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