Early Results of Neurodevelopment Following Hybrid Stage I for Hypoplastic Left Heart Syndrome

Abstract

Motor skills and neurodevelopment in infants with hypoplastic left heart syndrome (HLHS) who have undergone Hybrid Stage I palliation is unknown. The purpose of this study is to assess early neurodevelopment in infants with HLHS after Hybrid Stage I palliation. Developmental assessment was performed in HLHS infants who underwent Hybrid Stage I palliation at 2 and 4 months of age using the Test of Infant Motor Performance, and at 6 months of age, prior to undergoing the second staged surgery, using the Bayley Scales of Infant and Toddler Development, 3rd edition (Bayley-III). Results were compared to healthy control subjects and norm-referenced data. The HLHS group scored between -1 and -2 standard deviations (SD) below the mean at 2 months of age (p = 0.002), and within -1 SD of the mean, at 4 months of age (p = 0.0019), on the TIMP. Compared to the control group, composite motor skills were significantly lower at 6 months of age on the Bayley-III in the HLHS group (p = 0.0489), however, not significant for cognitive (p = 0.29) or language (p = 0.68). Percentile rank motor scores were 17 ± 20 % in the HLHS group compared to 85 ± 12 % for the healthy age-matched control group. Infants with HLHS who undergo Hybrid Stage I palliation score lower on standardized motor skill tests compared to healthy age-matched controls and the norm-referenced population. This suggests that infants with HLHS have poorer motor skill performance than typically developing infants at 6 months of age.

Clinical feasibility of pre‐operative neurodevelopmental assessment of infants undergoing open heart surgery

“…reported that 76% of their study cohort did not complete pre‐operative neurobehavioural assessment due to medical contraindications. Numerous other prospective cohort studies have reported similar challenges to obtaining baseline data consequently limiting their ability to determine the prevalence of abnormalities prior to surgery and the impact of intra‐operative risk factors. The ACC score correlates with post‐operative length of stay and mortality prior to discharge .…”

“…Additionally, participant attrition and missing data are universal threats to longitudinal studies. Studies with small sample sizes and missing data limit the predictive validity of findings and create significant challenges for investigators . These challenges are amplified when studying medically fragile, at‐risk infants during the vulnerable time between birth and open heart surgery.…”

The Evolution of Therapeutic Strategies: Niche Apportionment for Hybrid Palliation

“…Knirsch and colleagues [61] could not identify any effect of the respective hybrid versus Norwood surgical strategy on neurodevelopmental outcome assessed with standardized neuromotor examination and the Bayley Scales of Infant Development II through follow-up at age 4 years [63]. Similar to Norwood patients, hybrid patients showed signs of significant neurodevelopmental delay before [62] or after comprehensive stage II [61]. So far, there is no evidence that hybrid palliation has a protective or detrimental effect on the neonatal brain compared with Norwood palliation.…”

“…Several studies have assessed neurodevelopmental outcome of patients palliated with the hybrid strategy [61,62]. Knirsch and colleagues [61] could not identify any effect of the respective hybrid versus Norwood surgical strategy on neurodevelopmental outcome assessed with standardized neuromotor examination and the Bayley Scales of Infant Development II through follow-up at age 4 years [63].…”

Understanding the Hybrid Stage I Approach for Hypoplastic Left Heart Syndrome

“…Similar to findings in previous developmental studies in children who had a Norwood palliation, motor impairment was high. 40 In another study, 41 infants with HLHS who underwent hybrid stage I palliation and normal, healthy, age-matched controls were compared. The HLHS group scored lower than the control group in motor skills, language, and cognitive development as indicated by scores on the Bayley Scales of Infant and Toddler Development.…”