Thorac Cardiovasc Surg 2015; 63 - V0026
DOI: 10.1055/s-0035-1555982

Long-Term Neurodevelopmental Outcome of Hypoplastic Left Heart Syndrome

W. Knirsch 1, 2, R. Liamlahi 1, 2, 3, R. Prêtre 4, H. Dave 2, 4, V. Bernet 2, 5, B. Latal 2, 3, O. Kretschmar 1, 2
  • 1Cardiology, University Children’s Hospital Zurich, Switzerland
  • 2Children’s Research Center, University Children’s Hospital Zurich, Switzerland
  • 3Child Development Center, University Children’s Hospital Zurich, Switzerland
  • 4Congenital Cardiovascular Surgery, University Children’s Hospital Zurich, Switzerland
  • 5Neonatology and Pediatric Intensive Care Unit, University Children’s Hospital Zurich, Switzerland

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.