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.
Neurocognitive deficits Outcomes CHD Hypoplastic left heart syndrome Norwood operation
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We thank Irina Dinu for statistical support. The Complex Pediatric Therapies Follow-up Program has been supported over the years by Alberta Health, the Stollery Children’s Hospital, and the Glenrose Rehabilitation Hospital. The Women and Children’s Health Research Institute has funded registration and acute care data collection starting in 2014. These funding agencies had no role in the design and conduct of the study; analysis or interpretation of the data; preparation, review, or approval of the manuscript; or the decision to submit the manuscript for publication.
While the registry was funded as above, this study was not specifically funded.
Compliance with Ethical Standards
Conflict of Interest
No authors have any conflicts of interest relevant to this study.
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.
Dulfer K, Bossers SSM, Utens EMWJ et al (2016) Does functional health status predict health-related quality of life in children after Fontan operation?. Cardiol Young 26:459–468CrossRefPubMedGoogle Scholar
Goldberg CS, Schwartz EM, Brunberg JA et al (2000) Neurodevelopmental outcome of patients after the Fontan operation: a comparison between children with hypoplastic left heart syndrome and other functional single ventricle lesions. J Pediatr 137:646–652CrossRefPubMedGoogle Scholar
Bellinger DC, Jonas RA, Rappaport LA et al (1995) Developmental and neurologic status of children after heart surgery with hypothermic circulatory arrest or low-flow cardiopulmonary bypass. N Engl J Med 332:549–555CrossRefPubMedGoogle Scholar
Terplan K (1973) Patterns of brain damage in infants and children with congenital heart disease: association with catheterization and surgical procedures. Am J Dis Child 125:175–195CrossRefGoogle Scholar
Licht DJ, Shera DM, Clancy RR et al (2009) Brain maturation is delayed in infants with complex congenital heart defects. J Thorac Cardiovasc Surg 137:529–537CrossRefGoogle Scholar
Alsoufi B, Slesnick T, Mccracken C et al (2015) Current outcomes of the Norwood operation in patients with single-ventricle malformations other than hypoplastic left heart syndrome. World J Pediatric Congenit Heart Surg 6:46–52CrossRefGoogle Scholar
Daebritz SH, Nollert GDA, Zurakowski D et al (2000) Results of Norwood stage I operation: comparison of hypoplastic left heart syndrome with other malformations. J Thorac Cardiovasc Surg 119:358–367CrossRefPubMedGoogle Scholar
Fortuna RS, Ruzmetov M, Geiss DM (2013) Outcomes of the modified Norwood procedure: hypoplastic left heart syndrome versus other single-ventricle malformations. Pediatr Cardiol 35:96–102CrossRefPubMedGoogle Scholar
Robertson CMT, Sauve RS, Joffe AR et al (2011) The Registry and Follow-up of Complex Pediatric Therapies Program of Western Canada: a mechanism for service, audit, and research after life-saving therapies for young children. Cardiol Res Pract 2011:11Google Scholar
Robertson CMT, Joffe AR, Sauve RS et al (2004) Outcomes from an interprovincial program of newborn open heart surgery. J Pediatr 144:86–92CrossRefPubMedGoogle Scholar
Blishen B, Carroll W, Moore C (1987) The 1981 socioeconomic index for occupations in Canada. Can Rev Sociol Anthropol 24:465–488CrossRefGoogle Scholar
Bayley N (2006) Bayley scales of infant and toddler development (Bayley-III). Harcourt Assessment Inc, San Antonio, TXGoogle Scholar
Rogers BT, Msall ME, Buck GM et al (1995) Neurodevelopmental outcome of infants with hypoplastic left heart syndrome. J Pediatr 126:496–498CrossRefPubMedGoogle Scholar
Wernovsky G, Stiles KM, Gauvreau K et al (2000) Cognitive development after the Fontan operation. Circulation 102:883–889CrossRefPubMedGoogle Scholar
Creighton DE, Robertson CMT, Sauve RS et al (2007) Neurocognitive, functional, and health outcomes at 5 years of age for children after complex cardiac surgery at 6 weeks of age or younger. Pediatrics 120:478–486CrossRefGoogle Scholar
Mahle W, Clancy R, Moss E et al (2000) Neurodevelopmental outcome and lifestyle assessment in school-aged and adolescent children with hypoplastic left heart syndrome. Pediatrics 105:1082–1089CrossRefPubMedGoogle Scholar
Gaynor JW, Mahle WT, Cohen MI et al (2002) Risk factors for mortality after the Norwood procedure. Eur J Cardiothorac Surg 22:82–89CrossRefPubMedGoogle Scholar
Alsoufi B, Mori M, Gillespie S et al (2015) Impact of patient characteristics and anatomy on results of Norwood operation for hypoplastic left heart syndrome. Ann Thorac Surg 100:591–598CrossRefPubMedGoogle Scholar