Original Articles

Improvements in Survival and Neurodevelopmental Outcomes in Surgical Treatment of Hypoplastic Left Heart Syndrome: A Meta-Analytic Review

Address correspondence to: Joseph J. Sistino, PhD, CCP, Chief Perfusionist, Medical University of South Carolina, 151B Rutledge Avenue, Charleston, SC 29425. E-mail: sistinoj@musc.edu

Received: 18 April 2011
Accepted: 20 September 2012

Abstract

The purpose of this study was to analyze the changes over the past two decades in hospital survival and neurodevelopmental outcomes after surgical treatment for hypoplastic left heart syndrome. The hypothesis for this study is that increasing hospital survival following the Stage I Norwood (S1N) procedure is associated with improvements in neurodevelopmental outcomes. Studies included in the meta-analysis were identified by searching Ovid MEDLINE® from January 1980 to October 2010. A total of 72 articles were identified. Fifteen single-center study articles were appropriate for obtaining survival data and 14 were used for neurodevelopmental outcomes. Wechsler Intelligence Test IQ scores and the Bayley II Mental Development (MDI) and Psychomotor Development Indices (PDI) were the primary neurodevelopmental outcomes included in this meta-analysis. Metaregression analysis using a mixed-effects model compared the percent survival and neurodevelopmental scores with the year of surgery. Hospital survival for the S1N operation increased significantly from 1996 to 2007 (p < .05). The overall mean survival during this time period was 80.05% (95% confidence interval [CI], 76.4–84.0%). Standardized Wechsler IQ scores increased significantly from 1989 to 1999 (p < .05) and the mean IQ was 85.09 (95% CI, 82.3–89.5). The Bayley II MDI increased significantly from 1998 to 2005 (p < .05) with a mean MDI of 86.9 (95% CI, 84.9–88.9). The Bayley II PDI increased significantly from 1998 to 2005 (p < .05) with a mean PDI of 73.4 (95% CI, 71.2–75.5). Increased survival has been associated with improved but below normal neurodevelopmental outcomes.