Longitudinal assessment of growth in hypoplastic left heart syndrome: Results from the Single Ventricle Reconstruction Trial

• Type of Material: Article
• Authors: Phillip T. Burch, University of Utah; Eric Gerstenberger, New England Research Institutes; Chitra Ravishankar, The Children's Hospital of Philadelphia; David A. Hehir, Children's Hospital of Wisconsin Wauwatosa; Rtan R. Davies, Alfred I. duPont Hospital for Children; Steven D. Colan, Children's Hospital Boston; Lynn A. Sleeper, New England Research Institutes; Jane W. Newburger, Children's Hospital Boston; Martha Clabby, Emory University; Ismee A Williams, Columbia University Medical Center; Jennifer S. Li, Duke University School of Medicine; Karen Uzark, University of Michigan Medical School; David S. Cooper, University of Cincinnati; Linda M. Lambert, University of Utah; Victoria L. Pemberton, National Institutes of Health, Bethesda; Nancy A. Pike, University of California, Los Angeles; Jeffrey B. Anderson, University of Cincinnati; Carolyn Dunbar-Masterson, Children's Hospital Boston; Svetlana Khaikin, University of Toronto; Sinai C. Zyblewski, Medical University of South Carolina; L. LuAnn Minich, University of Utah School of Medicine
• Language: English
• Date: 2014-01-01
• Publisher: Wiley Open Access: Creative Commons Attribution Non-Commercial
• Publication Version: Final Published Version
• Copyright Statement: © 2014 The Authors.
• License: Creative Commons Attribution-Noncommercial 3.0 Unported
• Final Published Version (URL): http://dx.doi.org/10.1161/JAHA.114.000079
• Title of Journal or Parent Work: Journal of the American Heart Association
• ISSN: 2047-9980
• Volume: 3
• Issue: 3
• Start Page: e000079
• End Page: e000079
• Grant/Funding Information: Supported by U01 grants from the National Heart, Lung, and Blood Institute (HL068269, HL068270, HL068279, HL068281, HL068285, HL068292, HL068290, HL068288, HL085057).
• Keywords: Infant, Newborn; Hypoplastic Left Heart Syndrome; Child Development; risk factors; Age Factors; Norwood Procedures; Treatment Outcome; Humans; hypoplastic left heart syndrome; Male; pediatrics; Female; growth; Body Height; Child, Preschool; Infant; Weight Gain
• Research Categories: Health Sciences, Medicine and Surgery; Health Sciences, General

Abstract

Background-We sought to characterize growth between birth and age 3 years in infants with hypoplastic left heart syndrome who underwent the Norwood procedure. Methods and Results-We performed a secondary analysis using the Single Ventricle Reconstruction Trial database after excluding patients < 37 weeks gestation (N=498). We determined length-for-age z score (LAZ) and weight-for-age z score (WAZ) at birth and age 3 years and change in WAZ over 4 clinically relevant time periods. We identified correlates of change in WAZ and LAZ using multivariable linear regression with bootstrapping. Mean WAZ and LAZ were below average relative to the general population at birth (P < 0.001, P=0.05, respectively) and age 3 years (P < 0.001 each). The largest decrease in WAZ occurred between birth and Norwood discharge; the greatest gain occurred between stage II and 14 months. At age 3 years, WAZ and LAZ were < -2 in 6% and 18%, respectively. Factors associated with change in WAZ differed among time periods. Shunt type was associated with change in WAZ only in the Norwood discharge to stage II period; subjects with a Blalock-Taussig shunt had a greater decline in WAZ than those with a right ventricle-pulmonary artery shunt (P=0.002). Conclusions-WAZ changed over time and the predictors of change in WAZ varied among time periods. By age 3 years, subjects remained small and three times as many children were short as were underweight (> 2 SD below normal). Failure to find consistent risk factors supports the strategy of tailoring nutritional therapies to patient- and stage-specific targets.