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Transplantation as a primary treatment for hypoplastic left heart syndrome: intermediate-term results
A J Razzouk1, R E Chinnock, S R Gundry
1Department of Surgery, Loma Linda University School of Medicine, California, USA.
Insights
Cardiac transplantation offers good outcomes for infants with hypoplastic left heart syndrome. Improving donor availability and managing rejection are key to enhancing survival rates for this complex congenital heart defect.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Transplant Immunology
Background:
- Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect with high mortality.
- Orthotopic cardiac transplantation has become a primary treatment for infants diagnosed with HLHS.
- This study reviews a decade of experience with cardiac transplantation for HLHS.
Purpose of the Study:
- To evaluate the outcomes of cardiac transplantation in infants with hypoplastic left heart syndrome.
- To identify factors influencing survival and complications post-transplantation.
- To assess the feasibility and effectiveness of cardiac transplantation as a treatment for HLHS.
Main Methods:
- A cohort of 176 infants with HLHS underwent cardiac transplant protocol between 1985 and 1995.
- Interventional procedures included stenting the ductus arteriosus and enlarging interatrial communication.
- Data collected included age at transplant, graft ischemic time, and hypothermic circulatory arrest duration.
Main Results:
- 142 infants received cardiac transplants; 34 died awaiting transplant.
- Actuarial survival rates at 7 years reached 70%.
- Late deaths were primarily due to graft rejection (50%) and graft vasculopathy (8 patients).
Conclusions:
- Cardiac transplantation is a viable option for infants with HLHS, yielding good short- and intermediate-term results.
- Enhanced donor availability and improved management of rejection and graft vasculopathy are crucial for better long-term survival.
- Further research into immunosuppression and prevention of graft deterioration is warranted.
Background:
Hypoplastic left heart syndrome is a lethal malformation. For the last 10 years, orthotopic cardiac transplantation has been our preferred treatment for infants with hypoplastic left heart syndrome.
Methods:
One hundred seventy-six infants with hypoplastic left heart syndrome were entered into a cardiac transplant protocol between November 1985 and November 1995. Interventional procedures to stent the ductus arteriosus or enlarge the interatrial communication were performed in 8 and 35 patients, respectively. Thirty-four patients (19%) died during the waiting period, and 142 infants underwent cardiac transplantation. Age at cardiac transplantation ranged from 1.5 hours to 6 months (median, 29 days). The majority of grafts were oversized, and the median graft ischemic time was 273 minutes (range, 60 to 576 minutes). The implantation procedure used a period of hypothermic circulatory arrest ranging from 23 to 110 minutes (median, 53 minutes). Repair of other significant defects included interrupted aortic arch and total or partial anomalous pulmonary venous connection.
Results:
There were 13 early and 22 late deaths. Patient actuarial survival at 1 month and at 1, 5 and 7 years was 91%, 84%, 76%, and 70% respectively. Half of the late deaths were due to rejection. Severe graft vasculopathy was confirmed in 8 patients. Retransplantation was performed in 5 patients for graft vasculopathy 4 and rejection 1. Lymphoblastic leukemia developed in 1 patient 3 years after cardiac transplantation.
Conclusions:
Cardiac transplantation can be performed in infants with hypoplastic left heart syndrome with good operative and intermediate-term results. Improved survival can be achieved with increased donor availability, better management of rejection, and control of graft vasculopathy.