Longitudinal assessment of growth in hypoplastic left heart syndrome: results from the single ventricle reconstruction trial.

Phillip T Burch, Eric Gerstenberger, Chitra Ravishankar, David A Hehir, Ryan R Davies, Steven D Colan, Lynn A Sleeper, Jane W Newburger, Martha L Clabby, Ismee A Williams, Jennifer S Li, Karen Uzark, David S Cooper, Linda M Lambert, Victoria L Pemberton, Nancy A Pike, Jeffrey B Anderson, Carolyn Dunbar-Masterson, Svetlana Khaikin, Sinai C Zyblewski, L LuAnn Minich

Journal: Journal of the American Heart Association 2015;3(3):e000079

PMID: 24958780

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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.

CLINICAL TRIAL REGISTRATION URL

http://clinicaltrials.gov/. Unique identifier: NCT00115934.

Address: Department of Surgery, University of Utah, Salt Lake City, UT (P.T.B., L.M.L.).; New England Research Institutes, Watertown, MA (E.G., L.A.S.).; The Children's Hospital of Philadelphia, Philadelphia, PA (C.R.).; The Children's Hospital of Wisconsin, Milwaukee, WI (D.A.H.).; Nemours/A.I. DuPont Hospital for Children, Wilmington, DE (R.R.D.).; Children's Hospital Boston and Harvard Medical School, Boston, MA (S.D.C., J.W.N., C.D.M.).; The Hospital for Sick Children, Toronto, Ontario, Canada (M.L.C., S.K.).; Columbia University Medical Center, New York, NY (I.A.W.).; Duke University Medical Center, Durham, NC (J.S.L.).; University of Michigan Medical School, Ann Arbor, MI (K.U.).; University of Cincinnati, Cincinnati, OH (D.S.C., J.B.A.).; National Institutes of Health, Bethesda, MD (V.L.P.).; University of California Los Angeles, Los Angeles, CA (N.A.P.).; Medical University of South Carolina, Charleston, SC (S.C.Z.).; Department of Pediatrics, University of Utah, Salt Lake City, UT (L.A.M.).

DOI: 10.1161/JAHA.114.000079

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MeSH Terms: Age Factors, Body Height, Child Development, Child, Preschool, Female, Humans, Hypoplastic Left Heart Syndrome, Infant, Infant, Newborn, Male, Norwood Procedures, Treatment Outcome, Weight Gain