title: Mortality and neurodevelopmental outcome at 1 year of age comparing hybrid and Norwood procedures.
abstract: OBJECTIVE
  Neonates with hypoplastic left heart syndrome (HLHS) are at risk of high mortality and neurodevelopmental morbidity. As an alternative to Norwood-type stage I palliation, the hybrid procedure has been developed. It consists of bilateral pulmonary artery banding, catheter-based stenting of the arterial duct and balloon atrioseptostomy and delays open-heart surgery. Thus, it may be associated with a better outcome. The aim of this study was to determine the mortality and neurodevelopmental outcome in patients with HLHS and other univentricular heart (UVH) defects treated with hybrid or Norwood procedures.
  
  
  METHODS
  Thirty-one children (18 males) with HLHS and other UVH defects undergoing Norwood or hybrid procedure between 2004 and 2008 were consecutively enrolled. Mortality and neurodevelopmental outcome at 1 year of age were determined.
  
  
  RESULTS
  One-year mortality was 36% (31% in the hybrid vs. 39% in the Norwood group, P=0.71). Predictors of mortality were lower birth weight (P=0.02), older age at first procedure (P=0.02) and smaller size of ascending aorta (P=0.05). Overall, median psychomotor development index (PDI) and mental development index (MDI) of the Bayley Scales of Infant Development II were lower than the norm of 100 [PDI 57 (49-99), P<0.001; MDI 91 (65-109), P=0.002]. No effect of surgical treatment on neurodevelopmental outcome was found. Predictors of impaired motor outcome were length of hospital stay (LOHS) (P=0.01), lower body weight at second procedure (P=0.004) and female sex (P=0.01). Predictors of impaired cognitive outcome were longer mechanical ventilation time (P=0.03), intensive care unit stay (P=0.04) and LOHS (P<0.001), respectively.
  
  
  CONCLUSIONS
  Mortality at 1 year of age is comparable between patients undergoing hybrid and Norwood procedures. Early neurodevelopmental outcome is significantly impaired in patients with both HLHS and other UVH defects. Multicentre randomized studies are needed to determine the long-term neurodevelopmental outcome of children treated with the hybrid procedure.
authors.affiliation: Division of Paediatric Cardiology, University Children's Hospital Zurich, Zurich, Switzerland. walter.knirsch@kispi.uzh.ch
authors.forename: Walter
authors.initials: W
authors.lastname: Knirsch
authors.forename: Rabia
authors.initials: R
authors.lastname: Liamlahi
authors.forename: Maja I
authors.initials: MI
authors.lastname: Hug
authors.forename: Ricarda
authors.initials: R
authors.lastname: Hoop
authors.forename: Michael
authors.initials: M
authors.lastname: von Rhein
authors.forename: René
authors.initials: R
authors.lastname: Prêtre
authors.forename: Oliver
authors.initials: O
authors.lastname: Kretschmar
authors.forename: Beatrice
authors.initials: B
authors.lastname: Latal
mesh_terms: D006348:Cardiac Surgical Procedures / Q000379:methods*; D002315:Cardiopulmonary Bypass; D002658:Developmental Disabilities / Q000209:etiology*; D005260:Female; D005500:Follow-Up Studies; D006352:Heart Ventricles / Q000002:abnormalities / Q000601:surgery; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000150:complications / Q000401:mortality / Q000601:surgery*; D007223:Infant; D007231:Infant, Newborn; D008607:Intellectual Disability / Q000209:etiology*; D008297:Male; D009483:Neuropsychological Tests; D058327:Norwood Procedures; D011446:Prospective Studies; D011596:Psychomotor Disorders / Q000209:etiology*; D011788:Quality of Life; D012307:Risk Factors; D015996:Survival Rate; D016896:Treatment Outcome