Management of 239 patients with hypoplastic left heart syndrome and related malformations from 1993 to 2007

Abstract

Background: We reviewed our entire programmatic operative experience with children with hypoplastic left heart syndrome (HLHS) and related malformations.

Methods: As of October 1, 2007, 239 patients with HLHS and related malformations underwent surgical treatment at the Congenital Heart Institute of Florida. This manuscript focuses on the 199 initially treated with Norwood stage 1.

Results: One hundred and ninety-nine patients were initially treated with Norwood stage 1. Univariate analysis demonstrated the following significant predictors of mortality: right ventricular dominance (p = 0.0023), mechanical circulatory support before stage 1 (p = 0.0192), and significant noncardiac abnormality or syndrome, including Down syndrome, Turner syndrome, heterotaxy, asplenia, polysplenia, biliary atresia, or other chromosomal abnormality (p < 0.0001). Multivariable logistic regression analysis revealed the presence of a significant noncardiac abnormality or syndrome or prematurity less than 35 weeks or mechanical circulatory support before stage 1 to be a significant predictor of mortality (p < 0.0001). Over the 14 years of this patient series, survival for the 157 "low-risk" patients managed with Norwood staged palliation (those patients without significant noncardiac abnormality or syndrome or prematurity less than 35 weeks or mechanical circulatory support prior to Stage 1) was 86%, 80%, and 69% at 30 days after Stage 1, hospital discharge after Stage 1, and 1 year of after Stage 1, respectively.

Conclusions: Several treatment options are available for HLHS and related malformations. The appropriate treatment strategy must be matched to the individual patient, taking into consideration anatomic variables as well as other patient-specific characteristics. The majority of patients with HLHS and related malformations can undergo successful staged palliation with risk that varies according to several documented anatomic and patient-specific variables.