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  • Title: Cardiac transplantation for infants with hypoplastic left-heart syndrome.
    Author: Chiavarelli M, Gundry SR, Razzouk AJ, Bailey LL.
    Journal: JAMA; ; 270(24):2944-7. PubMed ID: 8254855.
    Abstract:
    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.
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