Ten-Year Institutional Experience With Palliative Surgery for Hypoplastic Left Heart Syndrome

Abstract

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 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.