Early survival of infants weighing 2.5 Kilograms or less undergoing first-stage reconstruction for hypoplastic left heart syndrome

Background - Results of staged palliation for hypoplastic left heart syndrome (HLHS) have improved in recent years; however, certain risk factors have been associated with decreased survival rates. Methods and Results - We retrospectively reviewed the medical records of 67 patients weighing ≤2.5 kg undergoing the first stage of reconstructive surgery at our institution between January 1, 1990, and December 31, 1997. HLHS was present in 45 patients, complex double-outlet right ventricle in 10, unbalanced AV canal in 5, tricuspid atresia with transposition of the great vessels in 4, and other diagnoses in 3. Mean age at surgery was 10.1 ± 10.7 days (median, 8 days), and mean weight was 2.2±0.3 kg (median, 2.2 kg). Fourteen patients weighed ≤2.0 kg, and 2 patients weighed ≤ 1.5 kg. Early mortality (death within 30 days or before hospital discharge) was 51% (34 of 67). No patient, procedural, or time-related variables correlated with increased mortality. However, there was a trend toward increased mortality with increased cardiopulmonary bypass time (P=0.076) and decreased preoperative ventricular performance (P=0.139). Conclusions - These findings suggest that low weight alone in a patient with HLHS or an anatomic variant should not be considered a contraindication to staged reconstructive surgery.