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Neurodevelopmental Outcome in Children after Fetal Cardiac Intervention for Aortic Stenosis with Evolving Hypoplastic
Kristin Laraja1, Anjali Sadhwani2, Wayne Tworetzky1
1Department of Cardiology, Boston Children's Hospital, Boston, MA; Department of Pediatrics, Harvard Medical School, Boston, MA.
Insights
Children undergoing fetal aortic valvuloplasty for hypoplastic left heart syndrome experience neurodevelopmental delays. Infant morbidity and innate patient factors significantly impact outcomes, not biventricular circulation achievement.
Area of Science:
- Pediatric Cardiology
- Neurodevelopmental Pediatrics
- Congenital Heart Disease Research
Background:
- Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect requiring complex management.
- Fetal aortic valvuloplasty (FAV) aims to improve left heart development in utero.
- Neurodevelopmental outcomes following fetal interventions for HLHS require thorough characterization.
Purpose of the Study:
- To assess neurodevelopmental outcomes in children after fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome.
- To identify risk factors associated with adverse neurodevelopmental trajectories in this population.
Main Methods:
- Retrospective review of medical records and mailed questionnaires (Adaptive Behavior Assessment System, Behavior Assessment System for Children, Behavior Rating Inventory of Executive Function, Ages and Stages, Pediatric Quality of Life Inventory).
- Study included 52 children who completed assessments at a median age of 5.5 years.
- Multivariable analysis was used to identify predictors of neurodevelopmental outcomes.
Main Results:
- Children who underwent FAV had lower General Adaptive Composite (GAC) scores than population norms, comparable to HLHS patients without fetal intervention.
- Independent predictors of lower GAC scores included total hospital duration of stay in the first year of life and biventricular status.
- Most other neurodevelopmental questionnaire subscale scores were similar between single and biventricular circulation groups.
Conclusions:
- Children treated with fetal aortic valvuloplasty exhibit neurodevelopmental delays, similar to those with hypoplastic left heart syndrome managed postnatally.
- Achieving biventricular circulation post-intervention did not correlate with improved neurodevelopmental outcomes.
- Innate patient-related factors and infant morbidity appear to be the primary determinants of neurodevelopmental outcomes in this cohort.
Objective:
To characterize neurodevelopmental outcomes after fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome and determine the risk factors for adverse neurodevelopment.
Study Design:
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.
Results:
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.
Conclusion:
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.
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