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Transplantation in complex congenital heart disease
1Division of Cardiothoracic Surgery, Loma Linda University Medical Center, 11175 Campus Street, 92354, Loma Linda, CA, USA
Insights
Pediatric heart transplantation, especially for congenital heart disease, shows promising survival rates. Neonatal transplants offer a survival advantage over infant transplants, highlighting the importance of careful management.
Area of Science:
- Pediatric Cardiology
- Cardiothoracic Surgery
- Transplantation Medicine
Background:
- Orthotopic heart transplantation is a critical intervention for pediatric patients with end-stage heart disease.
- Congenital heart disease (CHD) is the predominant indication for heart transplantation in children, particularly in neonates and infants.
- Complexities in CHD necessitate specialized surgical and management strategies for successful transplantation.
Purpose of the Study:
- To analyze the outcomes of pediatric orthotopic heart transplantation at a single institution.
- To evaluate the impact of patient demographics, primary diagnosis, and prior procedures on transplant success.
- To identify key factors contributing to perioperative survival and long-term success in pediatric heart recipients.
Main Methods:
- Retrospective review of 345 pediatric orthotopic heart transplantations performed between January 1990 and August 1999.
- Analysis of patient data including age, diagnosis (especially CHD and hypoplastic left heart syndrome), previous surgeries, and transplant outcomes.
- Focus on surgical techniques including en bloc organ removal, low-flow bypass, and limited circulatory rest, alongside multidisciplinary care.
Main Results:
- 75% of transplants were for congenital heart disease, with 49% diagnosed with hypoplastic left heart syndrome.
- Neonatal heart transplants demonstrated a higher survival rate (77%) compared to infant transplants (63%).
- Congenital heart transplantation was associated with low perioperative risk due to technical considerations.
Conclusions:
- Pediatric heart transplantation, even in high-risk cases of congenital heart disease, can achieve high perioperative survival and long-term success.
- Neonatal heart transplantation appears to confer a survival advantage over infant transplantation.
- Meticulous attention to recipient selection, surgical technique, and multidisciplinary management is fundamental for optimal outcomes.
Abstract:
Three hundred and forty-five pediatric patients underwent orthotopic heart transplantation at Loma Linda University Medical Center throughout August 1999. Seventy-five percent of these patients had the diagnosis of congenital heart disease as the primary indication for transplantation. Two hundred and fifty-seven were infants and of these, 91 were neonates. Forty-nine percent had the primary diagnosis of hypoplastic left heart syndrome or its equivalent. Thirty patients had variant situs and 15 of these had situs inversus. Seventy-seven children had undergone one or more previous cardiac and/or thoracic procedures. There appeared to be a survival advantage with neonatal as compared to infant heart transplants (77% vs. 63%). Technical considerations for reconstruction have made congenital heart transplantation a low perioperative risk factor as compared to the child with cardiomyopathy. Attention to (1) recipient selection; (2) adequate en bloc removal of the heart and accompanying vessels; (3) use of low-flow bypass techniques with limited circulatory rest; and (4) multidisciplinary management have been the fundamental approach at this institution. Although challenging, high perioperative survival and long-term success can be achieved with heart transplantation in patients with high-risk or inoperable congenital heart disease.