Related Experiment Videos
Cardiac transplantation for infants with hypoplastic left-heart syndrome
M Chiavarelli1, S R Gundry, A J Razzouk
1Department of Surgery, Loma Linda University, CA.
Insights
Cardiac transplantation significantly improves survival for infants with hypoplastic left-heart syndrome. This intervention offers a better long-term outlook compared to the natural progression of this severe congenital heart defect.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Transplantation Medicine
Background:
- Hypoplastic left-heart syndrome (HLHS) is a severe congenital heart defect with a uniformly lethal natural history.
- Limited treatment options historically led to poor outcomes for infants with HLHS.
Purpose of the Study:
- To evaluate the impact of cardiac transplantation on the natural history of infants diagnosed with HLHS.
- To analyze outcomes based on the pretransplant period and waiting mortality.
Main Methods:
- Retrospective cohort study analyzing data from 111 infants with HLHS.
- Data collected pretransplant, during hospitalization, and up to 6 years post-transplant.
- Statistical analysis included product-limit estimates and log rank tests.
Main Results:
- Cardiac transplantation was performed in 84 infants (76%).
- Five-year actuarial survival for transplant recipients was 81%, significantly higher than the natural history.
- Operative mortality was 13%, with 89% freedom from reoperation at 5 years.
Conclusions:
- Cardiac transplantation offers a significant survival benefit for infants with HLHS.
- This intervention positively alters the previously uniformly fatal course of HLHS.
Objective:
To determine whether cardiac transplantation improves the natural history of infants with hypoplastic left-heart syndrome and to examine differences in outcome as a function of the pretransplant period.
Design:
Retrospective cohort study. Historical, clinical, and laboratory data were collected during the pretransplant period, the in-hospital period, and for up to 6 years following transplantation. Data were analyzed using the product-limit estimate and the log rank test.
Setting:
A tertiary, acute care, university teaching hospital.
Patients:
All 111 infants with hypoplastic left-heart syndrome who entered and completed a protocol leading to transplantation from November 19, 1985, to December 31, 1991. Infants who died while waiting for transplantation were included.
Intervention:
Orthotopic cardiac allotransplantation.
Main Outcome Measures:
Pretransplant waiting mortality and its influence on posttransplant survival, operative (in-hospital or within 30 postoperative days in discharged patients) and intermediate-term mortality (5 years), and reoperation rates for cardiac surgery.
Results:
Transplantation procedures were performed in 84 infants (76%; 95% confidence interval [CI], 66% to 83%) ranging in age from 3 hours to 151 days. Twenty-seven infants registered for transplantation died while awaiting a donor heart. Operative mortality was 13% (CI, 7% to 23%), and 69 patients were late survivors (62% [CI, 52% to 71%] of the study group and 82% [CI, 72% to 89%] of the transplant recipients). Overall 5-year actuarial survival was 61% (CI, 52% to 70%). Transplant recipients had a 5-year survival of 81% (CI, 71% to 88%). Freedom from reoperation was 89% (CI, 76% to 95%) at 5 years.
Conclusions:
Cardiac transplantation for hypoplastic left-heart syndrome has a significant positive impact on the natural history of this uniformly lethal lesion.