Hypoplastic Left Heart Syndrome is not Associated with Worse Clinical or Neurodevelopmental Outcomes Than Other Cardiac Pathologies After the Norwood–Sano Operation

Abstract

There is evidence to suggest that patients undergoing a Norwood for non-HLHS anatomy may have lower mortality than classic HLHS, but differences in neurodevelopmental outcome have not been assessed. Our objective was to compare survival and neurodevelopmental outcome during the same surgical era in a large, well-described cohort. All subjects who underwent a Norwood-Sano operation between 2005 and 2014 were included. Follow-up clinical, neurological, and developmental data were obtained from the Western Canadian Complex Pediatric Therapies Follow-up Program database. Developmental outcomes were assessed at 2 years of age using the Bayley Scales of Infant and Toddler Development (Bayley-III). Survival was assessed using Kaplan-Meier analysis. Baseline characteristics, survival, and neurodevelopmental outcomes were compared between those with HLHS and those with non-HLHS anatomy (non-HLHS). The study comprised 126 infants (75 male), 87 of whom had HLHS. Five-year survival was the same for subjects with HLHS and those with non-HLHS (HLHS 71.8%, non-HLHS 76.9%; p = 0.592). Ninety-three patients underwent neurodevelopmental assessment including Bayley-III scores. The overall mean cognitive composite score was 91.5 (SD 14.6), language score was 86.6 (SD 16.7) and overall mean motor composite score was 85.8 (SD 14.5); being lower than the American normative population mean score of 100 (SD 15) for each (p-value for each comparison, <0.0001). None of the cognitive, language, or motor scores differed between those with HLHS and non-HLHS (all p > 0.05). In the generalized linear models, dominant right ventricle anatomy (present in 117 (93%) of patients) was predictive of lower language and motor scores. Comparative analysis of the HLHS and non-HLHS groups undergoing single ventricle palliation including a Norwood-Sano, during the same era, showed comparable 2-year survival and neurodevelopmental outcomes.

Outcomes of Preterm Infants With Congenital Heart Defects After Early Surgery: Defining Risk Factors at Different Time Points During Hospitalization

“…This study did not compare CCHD children with and without prematurity, however, mean scores for functional and neurodevelopmental outcomes of preterm neonates with CCHD in this study tend to be within the lower range of published results for preterm neonates (33,35,36). Particularly, low scores for self-care skills and language abilities indicate the need for early developmental testing and appropriate interventions for these children (38)(39)(40). Among the predictive factors, the presence of syndromic diagnoses adversely affected neurodevelopmental outcome, as previously reported (41).…”

Developmental outcomes after early surgery for complex congenital heart disease: a systematic review and meta‐analysis

“…Of the infant set of papers, 17 (six single‐ventricle studies, eight two‐ventricle studies, three mixed studies) reported the associations of ‘clinical illness’ and developmental outcome. Of the single‐ventricle studies, two stated significant associations between ‘clinical illness’ and developmental outcome: one reported worse motor and cognitive outcome,45 the other stated worse cognition and language;112 one study reported no association between ‘clinical illness’ and developmental outcome;54 and three stated inconsistent results 21,34,119…”

“…Of the four single-ventricle studies, one reported a significant association between lactate levels and worse developmental outcome, 26 one reported mixed results between worse cognition with a variable of hypoxemia and no association with another variable of hypoxemia, 34 and two studies did not find significant associations. 21,112 Of the two-ventricle studies, one found a significant association between hypoxemia and worse motor and cognitive outcome, 19 four reported inconsistent results of worse motor and behavioral adaptive skills yet preserved cognition, 22,41,50,51 and the remaining study reported low predictive value of hypoxemia and disability. 33 Of the studies with mixed lesions, one reported varied results, 30 and the other reported no association between hypoxemia and developmental outcome.…”

The Neurodevelopmental Outcomes of Patients With Single Ventricles Across the Lifespan

“…Neurodevelopmental outcomes for individuals with single-ventricle hearts, including those with HLHS, are comparable, 6 and thus included together in this focused review.…”