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DOI: 10.1055/s-0035-1555982
Long-Term Neurodevelopmental Outcome of Hypoplastic Left Heart Syndrome
Background: Data on long-term neurodevelopmental outcome of children treated for hypoplastic left heart syndrome (HLHS) comparing Hybrid and Norwood procedure are needed.
Methods: Neurodevelopmental outcome after 1 (Bayley Scales III) and 4 years (Wechsler Primary Preschool Intelligence Scale-III, and Movement-ABC 2) after Hybrid (2006–2008) and Norwood procedure (2004–2008) for HLHS resp. early (within first year after implementation) versus late period (after first year).
Results: Twenty of 31 (18 males) infants treated with Hybrid (n = 13) and Norwood (n = 18) procedure survived until age of 1 year. At 1 year, motor (PDI) and cognitive (MDI) outcome were significantly impaired compared with the norm [PDI 57 (49–99), p < 0.001; MDI 91 (65–109), p = 0.002], but not different comparing Hybrid (n = 9) and Norwood (n = 11). At 4 years, 16 of 20 children (late death n = 1, lost to follow up n = 3) both cognitive and motor performance was poorer than the norm [IQ: 89 (76–116), p = 0.02; motor outcome p = 0.002], but not different between Hybrid (n = 7) and Norwood (n = 9)[IQ: Norwood 92 (80–104) versus Hybrid 88 (76–116), p = 1.0; motor outome: p > 0.8]. However, cognitive performance of children treated in the early period was lower compared with children treated in the late period independent of performed procedure [IQ early 87 (76–101) versus late period 96 (80–116), p = 0.03].
Conclusion: Overall, cognitive and motor functions are impaired at 1 and 4 years of age in children with HLHS, independent of performed procedure, but affected by an institutional learning curve.