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Updated: Jul 1, 2025
Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
Adults with Congenital Heart Disease and Transplant: Challenges, Opportunities, and Policy
Nicole Herrick1, Marcus Urey1, Laith Alshawabkeh2
1Division of Cardiovascular Medicine, Department of Medicine, University of Calilfornia San Francisco, 505 Parnassus Avenue, San Francisco, CA 94143, USA.
Insights
Heart transplantation for adults with congenital heart disease (ACHD) is increasing. While initial survival is challenging, long-term outcomes improve significantly after the first year for ACHD patients.
Area of Science:
- Cardiology
- Transplantation Medicine
- Adult Congenital Heart Disease
Background:
- The incidence of heart transplantation in adults with congenital heart disease (ACHD) is growing.
- Post-transplant mortality in ACHD patients remains higher than in non-ACHD recipients, particularly within the first year.
Purpose of the Study:
- To review the challenges and strategies for heart transplantation in ACHD patients.
- To emphasize the importance of specialized care and allocation considerations for this population.
Main Methods:
- Review of current literature and clinical practices regarding heart transplantation in ACHD.
- Analysis of survival data and allocation system priorities for ACHD patients.
Main Results:
- ACHD patients face higher early mortality but demonstrate superior long-term survival after the first post-transplant year compared to non-ACHD patients.
- Optimized pre-transplant evaluation and perioperative planning can reduce risks.
- The current organ allocation system provides priority, but exceptions are crucial for complex cases like Fontan circulation.
Conclusions:
- Despite higher initial risks, heart transplantation offers improved long-term survival for ACHD patients.
- Specialized ACHD centers are essential for managing these complex patients.
- Careful patient selection, risk mitigation strategies, and appropriate organ allocation are vital for successful outcomes.
Abstract:
The rate of heart transplantation in adults with congenital heart disease (ACHD) is rising, but the 1-year mortality posttransplantation remains higher than non-ACHD patients. A robust pretransplant assessment and operative and postoperative planning can mitigate much of the perioperative risk. Importantly, ACHD patients who survive the first year have significantly better 10-year survival compared with non-ACHD patients. The current allocation system gives ACHD patients a relatively high priority, but providers must use the prespecified exception requests for higher status, especially for patients with the Fontan circulation. It is vital that ACHD patients with end-stage heart failure are cared for at centers with ACHD subspecialty care.
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