title: Neurodevelopmental Outcome in Children after Fetal Cardiac Intervention for Aortic Stenosis with Evolving Hypoplastic Left Heart Syndrome.
abstract: To characterize neurodevelopmental outcomes after fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome and determine the risk factors for adverse neurodevelopment.
  
  
  
  Questionnaires were mailed to families of children who underwent fetal aortic valvuloplasty from 2000 to 2012, and medical records were reviewed retrospectively. The primary outcome was the General Adaptive Composite score of the Adaptive Behavior Assessment System Questionnaire-Second Edition. Other questionnaires included the Behavior Assessment System for Children, Behavior Rating Inventory of Executive Function, Ages and Stages, and Pediatric Quality of Life Inventory.
  
  
  
  Among 69 eligible subjects, 52 (75%) completed questionnaires at median age of 5.5 (range 1.3-12) years; 30 (58%) had biventricular status circulation. The General Adaptive Composite mean score (92 ± 17) was lower than population norms (P < .001) and similar to published reports in patients with hypoplastic left heart syndrome without fetal intervention; scores in the single ventricular versus biventricular group were 97 ± 19 vs 89 ± 14, respectively (P = .10). On multivariable analysis, independent predictors of a lower General Adaptive Composite score were total hospital duration of stay in the first year of life (P = .001) and, when forced into the model, biventricular status (P = .02). For all other neurodevelopmental questionnaires (Behavior Assessment System for Children, Behavior Rating Inventory of Executive Function, Ages and Stages, Pediatric Quality of Life Inventory), most subscale scores for patients with biventricular and single ventricular status were similar.
  
  
  
  Children who underwent fetal aortic valvuloplasty have neurodevelopmental delay, similar to patients with hypoplastic left heart syndrome without fetal intervention. Achievement of biventricular circulation was not associated with better outcomes. We infer that innate patient factors and morbidity during infancy have the greatest effect on neurodevelopmental outcomes.
authors.affiliation: Department of Cardiology, Boston Children's Hospital, Boston, MA; Department of Pediatrics, Harvard Medical School, Boston, MA.
authors.forename: Kristin
authors.initials: K
authors.lastname: Laraja
authors.affiliation: Department of Psychiatry, Boston Children's Hospital, Boston, MA; Department of Psychiatry, Harvard Medical School, Boston, MA.
authors.forename: Anjali
authors.initials: A
authors.lastname: Sadhwani
authors.affiliation: Department of Cardiology, Boston Children's Hospital, Boston, MA; Department of Pediatrics, Harvard Medical School, Boston, MA.
authors.forename: Wayne
authors.initials: W
authors.lastname: Tworetzky
authors.affiliation: Department of Cardiology, Boston Children's Hospital, Boston, MA; Department of Pediatrics, Harvard Medical School, Boston, MA.
authors.forename: Audrey C
authors.initials: AC
authors.lastname: Marshall
authors.affiliation: Department of Cardiology, Boston Children's Hospital, Boston, MA; Department of Pediatrics, Harvard Medical School, Boston, MA.
authors.forename: Kimberlee
authors.initials: K
authors.lastname: Gauvreau
authors.affiliation: Department of Cardiology, Boston Children's Hospital, Boston, MA; Department of Pediatrics, Harvard Medical School, Boston, MA.
authors.forename: Lindsay
authors.initials: L
authors.lastname: Freud
authors.affiliation: Department of Cardiology, Boston Children's Hospital, Boston, MA.
authors.forename: Cara
authors.initials: C
authors.lastname: Hass
authors.affiliation: Department of Cardiology, Boston Children's Hospital, Boston, MA.
authors.forename: Carolyn
authors.initials: C
authors.lastname: Dunbar-Masterson
authors.affiliation: Department of Psychiatry, Harvard Medical School, Boston, MA; Developmental Medicine Center, Boston Children's Hospital, Boston, MA.
authors.forename: Janice
authors.initials: J
authors.lastname: Ware
authors.affiliation: Department of Cardiology, Boston Children's Hospital, Boston, MA.
authors.forename: Terra
authors.initials: T
authors.lastname: Lafranchi
authors.affiliation: Department of Obstetrics and Gynecology, Brigham and Women's Hospital, Boston, MA; Department of Obstetrics and Gynecology, Harvard Medical School, Boston, MA.
authors.forename: Louise
authors.initials: L
authors.lastname: Wilkins-Haug
authors.affiliation: Department of Cardiology, Boston Children's Hospital, Boston, MA; Department of Pediatrics, Harvard Medical School, Boston, MA.
authors.forename: Jane W
authors.initials: JW
authors.lastname: Newburger
mesh_terms: D001024:Aortic Valve Stenosis / Q000150:complications* / Q000601:surgery*; D063126:Balloon Valvuloplasty; D006348:Cardiac Surgical Procedures / Q000379:methods; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005333:Fetus / Q000601:surgery*; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000150:complications*; D007223:Infant; D065886:Neurodevelopmental Disorders / Q000453:epidemiology*; D011183:Postoperative Complications / Q000453:epidemiology*; D011247:Pregnancy; D012189:Retrospective Studies; D012307:Risk Factors
keywords: child development; congenital heart disease; fetal cardiac intervention; neurodevelopmental outcomes