Survival analysis and risk factors for mortality in transplantation and staged surgery for hypoplastic left heart syndrome

OBJECTIVES We compared survival in treatment strategies and determined risk factors for one-year mortality for hypoplastic left heart syndrome (HLHS) using intention-to-treat analysis. BACKGROUND Staged revision of the native heart and transplantation as treat ments for HLHS have been compared in treatment-received analyses, which can bias results. METHODS Data on 231 infants with HLHS, born between 1989 and 1994 and inten ded for surgery, were collected from four pediatric cardiac surgical center s. Status at last contact for survival analysis and mortality at one year f or risk factor analysis were the outcome measures. RESULTS Survival curves showed improved survival for patients intended for transplantation over patients intended for staged surgery. One-year surviva l was 61% for transplantation and 42% for staged surgery (p < 0.01); five-y ear survival was 55% and 38%, respectively (p < 0.01). Survival curves adju sted for preoperative differences were also significantly different (p < 0. 001). Waiting-list mortality accounted for 63% of first-year deaths in the transplantation group. Mortality with stage 1 surgery accounted for 86% of that strategy's first-pear mortality. Birth weight <3 kg (odds ratio [OR] 2 .4), highest creatinine greater than or equal to 2 mg/dL (OR 4.7), restrict ive atrial septal defect (OR 2.7) and, in staged surgery, atresia of one (O R 4.2) or both (OR 11.0) left-sided valves produced a higher risk for one-y ear mortality. CONCLUSIONS Transplantation produced significantly higher survival at all a ges up to seven years. Patients with atresia of one or both valves do poorl y in staged surgery and have significantly higher survival with transplanta tion. This information may be useful in directing patients to the better st rategy for them. CT Am Coil Cardiol 2000;36:1178-85) (C) 2000 by the Americ an College of Cardiology.