Background: We reviewed 212 consecutive patients who underwent stage I palliative surgery for hypoplastic left heart syndrome (HLHS) at our institution between January 1983 and June 1993.
Methods and results: Six surgeons participated in the care of these patients. Follow-up is 97% complete. Preoperative anatomic and physiological factors and procedural features of the stage I operation were analyzed for impact on stage I mortality, survival to stage II palliation, and actuarial survival. Hospital mortality was not significantly lower during the second half of the study period (P = .242). Operative mortality was 46.2%. Multivariate analysis revealed improved stage I operative survival in patients with mitral stenosis (MS) and aortic stenosis (AS; P = .006). Additional risk factors for stage I mortality were a lower immediately pre-stage I pH (P = .034) and weight < 3 kg (P = .015). Overall first-year actuarial survival for MS/AS was 59%, and it was 33% for all others (P = .001). Among stage I survivors, patients with MS/AS were more likely to survive to stage II palliation (P = .031). Analysis of actuarial survival of stage I survivors showed that a smaller ascending aorta (P < .001), aortic atresia (P < .001), and mitral atresia (P = .002) were all risk factors for intermediate death.
Conclusions: Preoperative anatomic and physiological state are predictors of stage I mortality. HLHS anatomic subtype also influences intermediate outcome, most notably pre-stage II attrition. These data may be useful in choosing initial management for patients with HLHS.