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Updated: May 1, 2026
Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
Heart transplantation in children for end-stage congenital heart disease
Anees J Razzouk1, Leonard L Bailey1
1Department of Cardiovascular and Thoracic Surgery, Loma Linda University Children's Hospital, Loma Linda, CA.
Insights
Heart transplantation (HT) is now rare for congenital heart disease (CHD). However, HT is increasingly needed for end-stage CHD after surgery, presenting unique challenges but offering comparable long-term survival for children.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Surgery
- Transplantation Medicine
Background:
- Congenital heart disease (CHD) survival has improved with reconstructive surgery, leading to a growing population with end-stage disease.
- End-stage CHD, often due to myocardial dysfunction or circulatory failure post-surgery, is an emerging indication for heart transplantation (HT).
- Children with CHD present unique challenges for HT due to complex physiology and prior surgical interventions.
Purpose of the Study:
- To review the evolving role of heart transplantation (HT) in pediatric congenital heart disease (CHD) management.
- To identify challenges and critical factors for improving outcomes in CHD patients undergoing HT.
- To assess the long-term survival of pediatric CHD recipients post-heart transplantation.
Main Methods:
- Review of current clinical practices and outcomes for heart transplantation in pediatric congenital heart disease.
- Analysis of factors influencing early mortality and long-term survival in this specific patient population.
- Emphasis on recipient selection, optimization, and perioperative management strategies.
Main Results:
- Heart transplantation (HT) as primary therapy for congenital heart disease (CHD) is now uncommon.
- Congenital heart disease (CHD) remains a significant risk factor for early mortality after heart transplantation (HT), particularly in Fontan physiology cases.
- Early referral, careful patient selection, and meticulous management are crucial for improving outcomes in pediatric CHD recipients.
Conclusions:
- While less common as primary therapy, heart transplantation (HT) is increasingly vital for end-stage pediatric congenital heart disease (CHD).
- Optimizing recipient selection and perioperative care is essential to mitigate the risks associated with HT in CHD patients.
- Pediatric congenital heart disease (CHD) survivors of heart transplantation (HT) can achieve long-term survival rates comparable to other recipients.
Abstract:
Heart transplantation (HT) as primary therapy for children with congenital heart disease (CHD) has become unusual. With improved early results of reconstructive surgery, the population of children and adults surviving with CHD is expanding. End-stage CHD related to myocardial dysfunction or circulation failure after prior surgery is becoming more common as an indication for HT. This heterogeneous group of CHD recipients referred for HT presents unique decision-making, technical, and physiologic challenges. Historically, a diagnosis of CHD has been a major risk factor for early mortality after HT. Rescue HT, especially in the setting of failing Fontan physiology, has the worst outcome. Early referral (before end-organ damage), proper selection, and optimization of recipients, as well as meticulous intra- and postoperative management are crucial to improving early outcomes of HT in this population. Beyond the early post-HT period, children with end-stage CHD experience long-term survival comparable to most other non-CHD recipients.
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