title: Interstage somatic growth in children with hypoplastic left heart syndrome after initial palliation with the hybrid procedure.
abstract: Introduction The hybrid procedure is one mode of initial palliation for hypoplastic left heart syndrome. Subsequently, patients proceed with either the "three-stage" pathway - comprehensive second stage followed by Fontan completion - or the "four-stage" pathway - Norwood procedure, hemi-Fontan, or Fontan completion. In this study, we describe somatic growth patterns observed in the hybrid groups and a comparison primary Norwood group.
  
  
  METHODS
  A retrospective analysis of patients who have undergone hybrid procedure and Fontan completion was performed. Weight-for-age and height-for-age z-scores were recorded at each operation.
  
  
  RESULTS
  We identified 13 hybrid patients - eight in the three-stage pathway and five in the four-stage pathway - and 49 Norwood patients. Weight: three stage: weight decreased from hybrid procedure to comprehensive second stage (-0.4±1.3 versus -2.3±1.4, p<0.01) and then increased to Fontan completion (-0.4±1.5 versus -0.6±1.4, p<0.01); four stage: weight decreased from hybrid procedure to Norwood (-2.0±1.4 versus -3.3±0.9, p=0.06), then stabilised to hemi-Fontan. Weight increased from hemi-Fontan to Fontan completion (-2.7±0.6 versus -1.0±0.7, p=0.01); primary Norwood group: weight decreased from Norwood to hemi-Fontan (p<0.001) and then increased to Fontan completion (p<0.001). Height: height declined from hybrid procedure to Fontan completion in the three-stage group. In the four-stage group, height decreased from hybrid to hemi-Fontan, and then increased to Fontan completion. The Norwood group decreased in height from Norwood to hemi-Fontan, followed by an increase to Fontan completion.
  
  
  CONCLUSIONS
  In this study, we show that patients undergoing the hybrid procedure have poor weight gain before superior cavopulmonary connection, before returning to baseline by Fontan completion. This study identifies key periods to target poor somatic growth, a risk factor of morbidity and worse neurodevelopmental outcomes.
authors.affiliation: Department of Congenital Heart Disease,Evelina London Children's Hospital,London,United Kingdom.
authors.forename: Fiona T S
authors.initials: FT
authors.lastname: Chan
authors.affiliation: Department of Congenital Heart Disease,Evelina London Children's Hospital,London,United Kingdom.
authors.forename: Hannah R
authors.initials: HR
authors.lastname: Bellsham-Revell
authors.affiliation: Department of Congenital Heart Disease,Evelina London Children's Hospital,London,United Kingdom.
authors.forename: Hannah
authors.initials: H
authors.lastname: Duggan
authors.affiliation: Department of Congenital Heart Disease,Evelina London Children's Hospital,London,United Kingdom.
authors.forename: John M
authors.initials: JM
authors.lastname: Simpson
authors.affiliation: Department of Congenital Heart Disease,Evelina London Children's Hospital,London,United Kingdom.
authors.forename: Tony
authors.initials: T
authors.lastname: Hulse
authors.affiliation: Department of Congenital Heart Disease,Evelina London Children's Hospital,London,United Kingdom.
authors.forename: Aaron J
authors.initials: AJ
authors.lastname: Bell
mesh_terms: D002657:Child Development / Q000502:physiology*; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000379:methods*; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000503:physiopathology* / Q000601:surgery; D007223:Infant; D008297:Male; D058327:Norwood Procedures / Q000379:methods*; D010166:Palliative Care; D011184:Postoperative Period; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: Hypoplastic left heart syndrome; hybrid procedure; somatic growth