The Association Between Family Resources and Language Among Young Children Who are Deaf and Hard of Hearing.

OBJECTIVE: Our study (1) examined demographic factors in families with children with bilateral hearing loss and how they relate to Family Resource Scale (FRS) questionnaire data and (2) examined correlations between FRS data and measures of language. METHODS: Children aged 6 months to 10 years with bilateral hearing loss were enrolled. Parents completed the FRS questionnaire to assess their access to socioeconomic resources at the first language measurement visit. Assessments measured receptive and expressive language, nonverbal intelligence quotient, and adaptive functioning. RESULTS: Among the 85 children included in the analysis, approximately 40% had hearing loss classified as mild to moderate and 25% had a cochlear implant. Participants' mean FRS score was 130 (SD 16.6) (with the highest possible score of 150 and indicating better access to resources). Significant positive correlations ( p -value ≤ 0.05) were found between maternal education, paternal education, and family income and several FRS subscales (Growth and Support, Necessities in Health, Childcare, Personal Resources). Significant positive correlations were found between the Necessities in Health subscale and all the language measurements. CONCLUSION: Children whose parents reported better access to socioeconomic resources related to health care had higher language performance scores. Although early access to intervention services has improved for deaf or hard-of-hearing children, there are other variables contributing to language development, including access to socioeconomic resources. This study highlights the need for further research addressing more specific and modifiable resources to improve language performance for deaf or hard-of-hearing children.

A Randomized Clinical Trial Targeting Daily Living Skills in Autistic Adolescents Without an Intellectual Disability Before the Transition to Adulthood.

OBJECTIVES: In the United States, more than 75,000 autistic adolescents graduate from high school each year, and many lack the skills to successfully transition to college, work, and independent living. Daily living skills (DLS) in autistic adolescents without an intellectual disability (ID) fall 6 to 8 years behind peers. Better DLS are linked to more positive adult outcomes for autistic individuals. Surviving and Thriving in the Real World (STRW) is the only known evidence-based intervention that targets age-appropriate DLS in autistic adolescents without ID. The study objective was to evaluate STRW's efficacy compared with an active comparator (Program for the Evaluation and Enrichment of Relational Skills [PEERS]). METHOD: Autistic adolescents in their last 2 years of high school were randomized to STRW or PEERS. Outcome measures were the Vineland Adaptive Behavior Scales, Third Edition (VABS-3), DLS domain and subdomains (Personal, Domestic, Community), and DLS Goal Attainment Scaling (DLS-GAS) areas (Morning Routine, Cooking, Laundry, Money). RESULTS: Adolescents were randomly assigned to STRW (n = 34) or PEERS (n = 30). Owing to COVID-19, 28.1% received in-person intervention and 71.9% received telehealth intervention. STRW youth made significant gains on the VABS-3 DLS domain ( p = 0.01) and Domestic subdomain ( p = 0.005) and DLS-GAS Total, Laundry, and Money areas (all p 's < 0.05) compared with PEERS. CONCLUSION: STRW shows promise for acquiring age-appropriate DLS compared with PEERS. Adolescents in STRW progressed toward achieving DLS in the average range and closing the gap between chronological age and DLS. By improving DLS, STRW may facilitate more successful adult outcomes.

Caregiver experiences helping children with Down syndrome use positive airway pressure to treat obstructive sleep apnea.

BACKGROUND/OBJECTIVE: While positive airway pressure (PAP) is an efficacious intervention for the treatment of obstructive sleep apnea syndrome (OSAS) in children with Down syndrome (DS), implementation and consistent use can be difficult. Caregiver perspectives and experiences using PAP are described with the aim of informing clinical practice. METHODS: Qualitative semi-structured phone interviews were conducted with 40 caregivers (i.e., mothers) of children with DS and OSAS treated with PAP for at least 6 months. Content analysis was used to identify themes associated with adherence and non-adherence. RESULTS: Respondents indicated variability in caregiver experience with the adoption of PAP and observed benefits of PAP. Varied experiences were attributed to several themes including accessing supplies, interactions with the medical team and equipment company, and patients' unique needs and behaviors, including the child's willingness and ability to adapt to PAP, sensory sensitivities, keeping the mask on all night, and differences in daytime behavior. Many families reported that desensitization with a reward system and trust within the caregiver-patient relationship were helpful. Caregiver suggestions for improving PAP adherence for families of children with DS included improving communication with the medical team and medical equipment company, emphasizing patience, using visual supports, and social support and education for extended family. CONCLUSIONS: Although family experiences varied, several actionable strategies by both the medical team and families emerged for improving the experience of and adherence to PAP in children with DS.

Stimulant Medication Treatment in Children with Congenital Heart Disease and Attention-Deficit/Hyperactivity Disorder: Cardiovascular Outcomes.

OBJECTIVE: Children with congenital heart disease (CHD) are at increased risk for attention-deficit/hyperactivity disorder (ADHD). The aim of this study was to determine whether children with CHD and ADHD clinically treated with stimulant medication were at increased risk for changes in cardiovascular parameters or death compared with CHD-matched controls. METHODS: In this retrospective cohort study, patients with CHD + ADHD treated with stimulant medication (exposed group [EG]) were matched by CHD diagnosis and visit age to patients not on stimulants (nonexposed group [NEG]). Cardiovascular parameters (heart rate [HR] and systolic and diastolic blood pressure [SBP and DBP]) and electrocardiograms (ECGs) from medical records over 12 months were compared using mixed effects models. RESULTS: Cardiovascular parameters for 151 children with CHD (mean age 8 ± 4 years) were evaluated (N = 46 EG and N = 105 NEG). Stimulant medication use was not associated with sudden cardiac death. HR and SBP did not significantly change over time in the EG and remained similar between groups. EG children had higher DBP compared with NEG children over time ( p = 0.001). Group × time interactions for HR, SBP, and DBP were not different between the EG and NEG. QTc was not significantly different between the EG and NEG (447 ms vs 439 ms, p = 0.23). EG children demonstrated improvement in ADHD symptoms. CONCLUSION: Stimulant medication use in children with CHD was not associated with clinically significant changes in cardiovascular parameters compared with controls. Stimulants should be considered for ADHD treatment in children with CHD when prescribed with appropriate monitoring and coordination with the cardiologist.

Does the History of Tracheoesophageal Fistula Repair Alter Outcomes of Laryngeal Cleft Repair?

OBJECTIVE: Tracheoesophageal fistula and esophageal atresia (TEA) and laryngeal cleft (LC) can coexist in some patients. The surgery-specific success rate of LC repair in children with associated TEA has not been well described. The aim of the study is to determine if the history of TEA alters the LC repair outcomes. STUDY DESIGN: Case series with chart review. SETTING: Single-institution academic medical center. METHOD: A retrospective review was conducted of patients with LC with and without TEA repair between January 2001 and November 2020. Data collected and analyzed included demographics and clinical characteristics, LC type, and LC with TEA timing of repairs. RESULTS: An overall 282 patients met the inclusion criteria of LC repair: LC (n = 242, 85.8%) and LC + TEA (n = 40, 14.2%). Revision repair was required in 43 patients (15.2%) with 8 (2.8%) needing a second revision repair. The first LC revision rate in the LC group was 36/242 (14.9%) as compared with 7/40 (17.5%) in the LC + TEA group (P = .67). The second LC revision rate in the LC and LC + TEA groups was 7 (2.9%) and 1 (2.4%), respectively. The median time to revision was 5.1 months (interquartile range, 3.45-10.6) in the LC group as compared with 29.2 months (interquartile range, 4.8-44.2) in the LC + TEA group (P = .06). CONCLUSION: The incidence of TEA and LC was 14.2% in our study. Based on our findings, history of TEA repair is not associated with a higher revision rate vs LC alone. The history of TEA repair did not alter the outcomes of LC repair.

An outbreak of Burkholderia contaminans at a quaternary children's hospital linked to equipment reprocessing.

Burkholderia cepacia complex (BCC) has been increasingly implicated in local and multistate outbreaks in both adult and pediatric healthcare settings. However, a lack of source identification may be common for BCC outbreak investigations. We describe, in detail, the investigation of an outbreak of BCC (B. contaminans) among pediatric patients at a large quaternary-care children's hospital and our system-level changes and outcomes.

Within- and across-frequency temporal processing and speech perception in cochlear implant users.

OBJECTIVE: Cochlear implant (CI) recipient's speech perception performance is highly variable and is influenced by temporal processing abilities. Temporal processing is commonly assessed using a behavioral task that requires the participant to detect a silent gap with the pre- and post-gap stimuli of the same frequency (within-frequency gap detection) or of different frequencies (across-frequency gap detection). The purpose of the study was to evaluate behavioral and electrophysiological measures of within- and across-frequency temporal processing and their correlations with speech perception performance in CI users. DESIGN: Participants included 11 post-lingually deafened adult CI users (n = 15 ears; Mean Age = 50.2 yrs) and 11 age- and gender-matched normal hearing (NH) individuals (n = 15 ears; Mean Age = 49.0 yrs). Speech perception was assessed with Consonant-Nucleus-Consonant Word Recognition (CNC), Arizona Biomedical Sentence Recognition (AzBio), and Bamford-Kowal-Bench Speech-in-Noise Test (BKB-SIN) tests. Within- and across-frequency behavioral gap detection thresholds (referred to as the GDTwithin and GDTacross) were measured using an adaptive, two-alternative, forced-choice procedure. Cortical auditory evoked potentials (CAEPs) were elicited using within- and across-frequency gap stimuli under four gap duration conditions (no gap, GDT, sub-threshold GDT, and supra-threshold GDT). Correlations among speech perception, GDTs, and CAEPs were examined. RESULTS: CI users had poorer speech perception scores compared to NH listeners (p < 0.05), but the GDTs were not different between groups (p > 0.05). Compared to NH peers, CI users showed increased N1 latency in the CAEPs evoked by the across-frequency gap stimuli (p < 0.05). No group difference was observed for the CAEPs evoked by the within-frequency gap (p > 0.05). Three CI ears showing the longest GDTwithin also showed the poorest performance in speech in noise. The within-frequency CAEP increased in amplitude with the increase of gap duration; while the across-frequency CAEP displayed a similar amplitude for all gap durations. There was a significant correlation between speech scores and within-frequency CAEP measures for the supra-threshold GDT condition, with CI users with poorer speech performance having a smaller N1-P2 amplitude and longer N1 latency. No correlations were found among GDTacross, speech perception, and across-frequency CAEP measures. CONCLUSIONS: Within- and across-frequency gap detection may involve different neural mechanisms. The within-frequency gap detection task can help identify CI users with poor speech performance for rehabilitation. The within-frequency CAEP is a better predictor for speech perception performance than the across-frequency CAEP.

Factors Associated with Early Intervention Intensity for Children Who Are Deaf or Hard of Hearing.

We quantified the intensity of early intervention (EI) services allocated to 1262 children who were deaf or hard of hearing (DHH) within a state program and identified factors associated with intervention intensity. Child specific data were collected on children born between 2008 and 2014. Data from Individualized Family Service Plans of children enrolled in Part C EI programming were evaluated for the type and duration of services during their EI enrollment. Associations between EI intensity and child/family variables were examined. Median age of EI enrollment was 5.3 months. The most frequently received services included primary service coordination, specialized DHH service, special instruction, language therapy, and family training; 60% of children received 4 or more different EI services. The median service intensity was 138.1 min per month across all EI years. The factors associated with higher EI intensity included severe hearing loss, bilateral hearing loss and presence of a disability. Children enrolled in EI at later ages received higher intensity of specialized DHH services, suggesting a need to "catch up" due to late acquisition of services. Evaluating EI service intensity broadens our understanding of effective components of state-based programs that support the developmental needs of children who are DHH.

Collaborative biostatistics and epidemiology in academic medical centres: A survey to assess relationships with health researchers and ethical implications.

The role of collaborative biostatisticians and epidemiologists in academic medical centers and how their degree type, supervisor type, and sex influences recognition and feelings of respect is poorly understood. We conducted a cross-sectional survey of self-identified biostatisticians and epidemiologists working in academic medical centers in the US or Canada. The survey was sent to 341 contacts at 125 institutions who were asked to forward the survey invitation to faculty and staff at their institution and posted on Community sections of the American Statistical Association website. Participants were asked a variety of questions including if they felt pressured to produce specific results, whether they had intellectual and ethical freedom to pursue appropriate use of statistical methods in collaborative research, and if they felt their contributions were appropriately recognized by collaborators. We received responses from 314 biostatisticians or related methodologists. A majority were female (59%), had a doctorate degree (52%), and reported a statistician or biostatistician supervisor (69%). Overall, most participants felt valued by their collaborators, but that they did not have sufficient calendar time to meet deadlines. Doctoral-level participants reported more autonomy in their collaborations than master's level participants. Females were less likely to feel recognized and respected compared to males. The survey results suggest that while most respondents felt valued by their collaborators, they have too many projects and need more time to critically review research. Further research is needed to understand why response differs by sex and how these responses fluctuate over time.

Association of Age of Enrollment in Early Intervention with Emergent Literacy in Children Who Are Deaf or Hard of Hearing.

OBJECTIVE: Literacy skill development in deaf or hard of hearing (DHH) children is essential for success in school and beyond. Our objective was to evaluate the association between age of early intervention (EI) enrollment for DHH children and emergent literacy in preschool. METHODS: This was a population-based study that leveraged state public health and education data on all children identified with hearing loss. The sample included children born between 2008 and 2014 enrolled in EI for hearing loss who received preschool supports (years 2011-2014) through the Ohio Department of Education. The Get it! Got it! Go!, measuring emergent literacy domains of picture naming, rhyming, and alliteration, was administered during preschool in fall and spring. Exposure was enrollment into EI before age 6 months (early) versus at/after 6 months (later). Propensity score matching and mixed model analyses were used to examine associations between EI enrollment and outcomes over time. Model least square means with 95% confidence intervals (CIs) were reported. RESULTS: One hundred two successful matches were made for 256 preschoolers. Children enrolled in EI early had significantly higher mean scores (mean [95% CI]) over time for emerging literacy domains of picture naming (2.42 [0.47-4.37]), rhyming (1.2 [0.35-2.06]), and alliteration (0.61 [0.15-1.07]) compared with later enrolled children. Children enrolled early had significantly higher emergent literacy scores at entry, although literacy development was similar between groups. CONCLUSION: Children enrolled in EI before age 6 months had consistently higher scores in emergent literacy components over time compared with children enrolled at/after age 6 months.

A Pilot Randomized Controlled Trial of a Daily Living Skills Intervention for Adolescents with Autism.

Adolescents with autism spectrum disorder (ASD) without an intellectual disability have daily living skills (DLS) impairments. An initial feasibility pilot of Surviving and Thriving in the Real World (STRW), a group intervention that targets DLS, demonstrated significant improvements. A pilot RCT of STRW was conducted to extend these findings. Twelve adolescents with ASD were randomized to the treatment or waitlist groups. The treatment group had significant DLS improvements on the Vineland Adaptive Behavior Scales, 3rd Edition and the DLS goal attainment scale. Four adolescents from the waitlist crossed over and completed STRW. Entire sample analyses with 10 participants demonstrated large DLS gains. Results provide further evidence of the efficacy of STRW for closing the gap between DLS and chronological age.

Technology assisted language intervention (TALI) for children who are deaf/hard of hearing: promising impact on pragmatic skills.

Children who are deaf and hard of hearing (DHH) often demonstrate pragmatic language difficulties which can impact academic and social outcomes. This randomized control trial for DHH children, ages 3-12 years, explored the Technology-Assisted Language Intervention (TALI), incorporating augmentative and alternative communication technology (AAC) into traditional speech/language therapy, compared to treatment-as-usual (TAU) to determine impact on pragmatics. Pragmatic outcome measures included parent reported Pragmatics Profile of the CELF-5 (for children age ≥5 years) and CELF-P Descriptive Pragmatics Profile (for children <5 years) in addition to parent reported Social and Communication domains of the Vineland Adaptive Behavior Scales,Third Edition (VABS). Over 24 weeks, children ≥5 years in the TALI made significantly more progress (increase in raw scores) on the Pragmatics Profile compared to children in TAU (12.7 points vs. -6.0 points; p = 0.04) and also showed significant gains on two of the three subscales. For children ≥5 years, no significant VABS changes were seen in either intervention group. For children <5 years, there were no statistically significant differences in growth on the CELF-P total pragmatics raw score or on any subdomain. However, children in TALI had significant increases in the mean VABS Communication (86.7-99.1) and Social domain standard scores (91.8-97.4;p = 0.01), while gains for children in TAU on the Communication and Social domain standard scores were not statistically significant. These promising results support the need for additional research exploring the effectiveness of AAC supported speech/language therapy to enhance DHH children's pragmatic language skills. Trial registration: ClinicalTrials.gov identifier: NCT02998164.

Comparison of children receiving emergent sepsis care by mode of arrival.

OBJECTIVE: To determine if differences in patient characteristics, treatments, and outcomes exist between children with sepsis who arrive by emergency medical services (EMS) versus their own mode of transport (self-transport). METHODS: Retrospective cohort study of patients who presented to the Emergency Department (ED) of two large children's hospitals and treated for sepsis from November 2013 to June 2017. Presentation, ED treatment, and outcomes, primarily time to first bolus and first parental antibiotic, were compared between those transported via EMS versus patients who were self-transported. RESULTS: Of the 1813 children treated in the ED for sepsis, 1452 were self-transported and 361 were transported via EMS. The EMS group were more frequently male, of black race, and publicly insured than the self-transport group. The EMS group was more likely to have a critical triage category, receive initial care in the resuscitation suite (51.9 vs. 22%), have hypotension at ED presentation (14.4 vs. 5.4%), lactate >2.0 mmol/L (60.6 vs. 40.8%), vasoactive agents initiated in the ED (8.9 vs. 4.9%), and to be intubated in the ED (14.4 vs. 2.8%). The median time to first IV fluid bolus was faster in the EMS group (36 vs. 57 min). Using Cox LASSO to adjust for potential covariates, time to fluids remained faster for the EMS group (HR 1.26, 95% CI 1.12, 1.42). Time to antibiotics, ICU LOS, 3- or 30-day mortality rates did not differ, yet median hospital LOS was significantly longer in those transported by EMS versus self-transported (6.5 vs. 5.3 days). CONCLUSIONS: Children with sepsis transported by EMS are a sicker population of children than those self-transported on arrival and had longer hospital stays. EMS transport was associated with earlier in-hospital fluid resuscitation but no difference in time to first antibiotic. Improved prehospital recognition and care is needed to promote adherence to both prehospital and hospital-based sepsis resuscitation benchmarks.

The Impact of Socioeconomic Status on Time to Decannulation Among Children With Tracheostomies.

OBJECTIVE: To determine if time to tracheostomy decannulation differs among children by socioeconomic status. STUDY DESIGN: Case series with chart review. SETTING: Tertiary pediatric medical center. METHODS: Patients (≤21 years old) who underwent tracheostomy from January 1, 2011, to December 31, 2016. Patients were divided into 2 groups based on their socioeconomic status (SES), low SES and high SES. Principal components analysis was used to create an index for SES using census data obtained by the US Census Bureau's American Community Survey 5 year data profile from 2013 to 2017. Statistical analysis was performed using a χ2 for categorical variables and Wilcoxon rank-sum test for continuous variables. A general linear model was constructed to control for clinical factors to understand the independent effect of SES on time to decannulation. RESULTS: In total, 215 patients were included; of these patients, 111 patients (52%) were included in the high-SES group and 104 patients (48%) were included in the low-SES group. There was a significant difference in the time to decannulation for children based on SES status, with those children in the low-SES group taking on average 10 months longer to decannulate (38.7 vs 28.0 months, P = .0007). Median follow-up was 44.1 months (interquartile range, 29.6-61.3 months). CONCLUSION: Health care disparities appear to exist among children undergoing decannulation of their tracheostomy tube. Patients with lower SES had a significantly longer time to decannulation than those with higher SES.

A Technology-Assisted Language Intervention for Children Who Are Deaf or Hard of Hearing: A Randomized Clinical Trial.

BACKGROUND AND OBJECTIVES: Children who are deaf or hard of hearing (DHH) often have persistent language delays despite early identification and interventions. The technology-assisted language intervention (TALI), which incorporates augmentative and alternative communication technology into a speech-language therapy model, was designed to support language learning. The study objective was to evaluate the impact of the TALI on spoken language outcomes in DHH children. METHODS: Children aged 3 to 12 years with mild to profound bilateral hearing loss were enrolled in a single-site randomized controlled trial. Children were randomly assigned to receive the TALI or treatment as usual (TAU) (with no change in current care) and were followed for 24 weeks. Primary outcomes included spoken language measures elicited from language samples. Secondary outcomes included standardized assessments. Intention-to-treat analyses were used. RESULTS: Analyses focused on 41 children randomly assigned to TALI (n = 21) or TAU (n = 20). Among all participants, mean age was 6.3 years (SD 2.5). Over 24 weeks, children in the TALI group, compared with those in the TAU group, had significantly greater increases in the length of phrases they used to express themselves (ß = .91 vs .15, respectively; P< .0001). Similar findings were seen with conversational turn-taking and number of different words spoken. CONCLUSIONS: Providing visual supports for language concepts that are typically challenging for DHH children to acquire allowed children to process and comprehend spoken language more fully. Such strategies can mitigate persistent language delays with the goal of improving lifelong outcomes and independence across settings.

Demographic and Clinical Characteristics Associated With Adherence to Guideline-Based Polysomnography in Children With Down Syndrome.

OBJECTIVES: To compare the demographic and clinical characteristics of children with Down syndrome who did and did not receive polysomnography to evaluate for obstructive sleep apnea after publication of the American Academy of Pediatrics' guidelines recommending universal screening by age 4 years. STUDY DESIGN: Retrospective cohort study. SETTING: Single tertiary pediatric hospital. METHODS: Review was conducted of children with Down syndrome born between 2007 and 2012. Children who obtained polysomnography were compared with children who did not, regarding demographic data, socioeconomic status, and comorbidities. RESULTS: We included 460 children with Down syndrome; 273 (59.3%) received at least 1 polysomnogram, with a median age of 3.6 years (range, 0.1-8.9 years). There was no difference in the distribution of sex, insurance status, or socioeconomic status between children who received polysomnography and those who did not. There was a significant difference in race distribution (P = .0004) and distance from home to the medical center (P < .0001) between groups. Among multiple medical comorbidities, only children with a history of hypothyroidism (P = .003) or pulmonary aspiration (P = .01) were significantly more likely to have obtained polysomnography. CONCLUSIONS: Overall, 60% of children with Down syndrome obtained a polysomnogram. There was no difference between groups by payer status or socioeconomic status. A significant difference in race distribution was noted. Proximity to the medical center and increased medical need appear to be associated with increased likelihood of obtaining a polysomnogram. This study illustrates the need for improvement initiatives to increase the proportion of patients receiving guideline-based screening.

Sleep Architecture in Children With Down Syndrome With and Without Obstructive Sleep Apnea.

OBJECTIVE: To characterize polysomnographic sleep architecture in children with Down syndrome and compare findings in those with and without obstructive sleep apnea. STUDY DESIGN: Case series with retrospective review. SETTING: Single tertiary pediatric hospital (2005-2018). METHODS: We reviewed the electronic health records of patients undergoing polysomnography who were referred from a specialized center for children with Down syndrome (age, ≥12 months). Continuous positive airway pressure titration, oxygen titration, and split-night studies were excluded. RESULTS: A total of 397 children were included (52.4% male, 81.6% Caucasian). Mean age at the time of polysomnography was 4.7 years (range, 1.4-14.7); 79.4% had obstructive sleep apnea. Sleep variables were reported as mean (SD) values: sleep efficiency, 85% (11%); sleep latency, 29.8 minutes (35.6); total sleep time, 426 minutes (74.6); rapid eye movement (REM) latency, 126.8 minutes (66.3); time spent in REM sleep, 22% (7%); arousal index, 13.3 (5); and time spent supine, 44% (28%). There were no significant differences between those with obstructive sleep apnea and those without. Sleep efficiency <80% was seen in 32.5%; 34.3% had a sleep latency >30 minutes; 15.9% had total sleep time <360 minutes; and 75.6% had an arousal index >10/h. Overall, 69.2% had ≥2 metrics of poor sleep architecture. REM sleep time <20% was seen in 35.3%. REM sleep time decreased with age. CONCLUSION: In children with Down syndrome, 32.5% had sleep efficiency <80%; 75.6% had an elevated arousal index; and 15.9% had total sleep time <360 minutes. More than a third of the patients had ≥3 markers of poor sleep architecture. There was no difference in children with or without obstructive sleep apnea.

Pattern of head circumference growth following bidirectional Glenn in infants with single ventricle heart disease.

BACKGROUND: Infants with single ventricle congenital heart disease demonstrate increasing head growth after bidirectional Glenn; however, the expected growth trajectory has not been well described. AIMS: 1) We will describe the pattern of head circumference growth in the first year after bidirectional Glenn. 2) We will determine if head growth correlates with motor developmental outcomes approximately 12 months after bidirectional Glenn. METHODS: Sixty-nine single ventricle patients underwent bidirectional Glenn between 2010 and 2016. Patients with structural brain abnormalities, grade III-IV intra-ventricular haemorrhage, significant stroke, or obstructive hydrocephalus were excluded. Head circumference and body weight measurements from clinical encounters were evaluated. Motor development was measured with Psychomotor Developmental Index of the Bayley Scales of Infant Development, Third Edition. Generalised estimating equations assessed change in head circumference z-scores from baseline (time of bidirectional Glenn) to 12 months post-surgery. RESULTS: Mean age at bidirectional Glenn was 4.7 (2.3) months and mean head circumference z-score based on population-normed data was -1.13 (95% CI -1.63, -0.63). Head circumference z-score increased to 0.35 (95% CI -0.20, 0.90) (p < 0.0001) 12 months post-surgery. Accelerated head growth, defined as an increase in z-score of >1 from baseline to 12 months post-surgery, was present in 46/69 (66.7%) patients. There was no difference in motor Psychomotor Developmental Index scores between patients with and without accelerated head growth. CONCLUSION: Single ventricle patients demonstrated a significant increase in head circumference after bidirectional Glenn until 10-12 months post-surgery, at which time growth stabilised. Accelerated head growth did not predict sub-sequent motor developmental outcomes.