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Research Publications

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

Burch PT1, Gerstenberger E2, Ravishankar C3, Hehir DA4, Davies RR5, Colan SD6, Sleeper LA2, Newburger JW6, Clabby ML7, Williams IA8, Li JS9, Uzark K10, Cooper DS11, Lambert LM1, Pemberton VL12, Pike NA13, Anderson JB11, Dunbar-Masterson C6, Khaikin S7, Zyblewski SC14, Minich LL15; Pediatric Heart Network Investigators.

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.

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