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.(JAMA. 1993;270:2944-2947)