Skip to main content Accessibility help
Hostname: page-component-55597f9d44-xbgml Total loading time: 0.438 Render date: 2022-08-18T02:14:32.706Z Has data issue: true Feature Flags: { "shouldUseShareProductTool": true, "shouldUseHypothesis": true, "isUnsiloEnabled": true, "useRatesEcommerce": false, "useNewApi": true } hasContentIssue true

Implementing standard screening for autism spectrum disorder in CHD

Published online by Cambridge University Press:  25 June 2020

Alexander Tan
Department of Neuropsychology, Children’s Healthcare of Atlanta, Atlanta, GA, USA
Eric S. Semmel
Department of Psychology and Neuroscience Institute, Georgia State University, Atlanta, GA, USA
Ian Wolf
Department of Neuropsychology, Children’s Healthcare of Atlanta, Atlanta, GA, USA
Bailee Hammett
Department of Neuropsychology, Children’s Healthcare of Atlanta, Atlanta, GA, USA
Dawn Ilardi*
Department of Neuropsychology, Children’s Healthcare of Atlanta, Atlanta, GA, USA Department of Rehabilitation Medicine, Emory University, Atlanta, GA, USA
Author for correspondence: Dawn Ilardi, 1400 Tullie Road, NE Atlanta, GA30329, USA. Phone: +1 404-785-5894; Fax: +1 404-785-0978. E-mail:



While the overall prevalence of autism is 1.7% in the United States of America, research has demonstrated a two- to five-fold increase in CHD. The Cardiac Neurodevelopmental Outcome Collaborative recommends screening for autism from infancy through adolescence. This study investigated the frequency of autism concerns at a single Cardiac Neurodevelopmental Program and examined current clinical practice as a way to improve quality of care.

Materials and methods:

Patients (n = 134; mean age = 9.0 years) included children with high-risk CHD who completed a neurodevelopmental evaluation following a formalised referral to the Cardiac Neurodevelopmental Program between 2018 and 2019. Retrospective chart review included parent report on the Behaviour Assessment System for Children-3 and Adaptive Behaviour Assessment System-3. Descriptive and correlation analyses were completed.


In this sample, 11.2% presented with autism-related concerns at referral, 2 were diagnosed with autism, 9 were referred to an autism specialist (6 confirmed diagnosis; 3 not completed). Thus, at least 5.9% of the sample were diagnosed with autism following thorough clinical evaluation. Analyses showed atypicality, along with deficient adaptability, leisure, social, and communication skills. Frequency of early intervention, school supports, and relation with comorbidities are reported.


Prior to assessment recommendations by the Cardiac Neurodevelopmental Outcome Collaborative, autism screening may not be completed systematically in clinical care for CHD. The current sample demonstrates a high frequency of autism in the typically referred clinical sample. Commonly used parent-report measures may reveal concerns but will not help diagnosis. Systematic use of an autism screener is essential.

Original Article
© The Author(s), 2020. Published by Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)


Hoffman, JI, Kaplan, S, Liberthson, RR.Prevalence of congenital heart disease. Am Heart J 2004; 147: 425439.10.1016/j.ahj.2003.05.003CrossRefGoogle ScholarPubMed
Marelli, A, Miller, SP, Marino, BS, Jefferson, AL, Newburger, JW.Brain in congenital heart disease across the lifespan: the cumulative burden of injury. Circulation 2016; 133: 19511962.CrossRefGoogle ScholarPubMed
Mebius, MJ, Kooi, EMW, Bilardo, CM, Bos, AF.Brain injury and neurodevelopmental outcome in congenital heart disease: a systematic review. Pediatrics 2017; 140: e20164055.CrossRefGoogle ScholarPubMed
Mahle, WT, Clancy, RR, Moss, EM, Gerdes, M, Jobes, DR, Wernovsky, G.Neurodevelopmental outcome and lifestyle assessment in school-aged and adolescent children with hypoplastic left heart syndrome. Pediatrics 2000; 105: 10821089.CrossRefGoogle ScholarPubMed
Sun, L, Macgowan, CK, Sled, JG, et al.Reduced fetal cerebral oxygen consumption is associated with smaller brain size in fetuses with congenital heart disease. Circulation 2015; 131: 13131323.10.1161/CIRCULATIONAHA.114.013051CrossRefGoogle ScholarPubMed
Wray, J.Congenital heart disease and cardiac surgery in childhood: effects on cognitive function and academic ability. Heart 2001; 85: 687691.10.1136/heart.85.6.687CrossRefGoogle ScholarPubMed
Wypij, D, Newburger, JW, Rappaport, LA, et al.The effect of duration of deep hypothermic circulatory arrest in infant heart surgery on late neurodevelopment: the Boston circulatory arrest trial. J Thorac Cardiovasc Surg 2003; 126: 13971403.CrossRefGoogle ScholarPubMed
Mahle, WT, Tavani, F, Zimmerman, RA, et al.An MRI study of neurological injury before and after congenital heart surgery. Circulation 2002; 106(12 Suppl 1): 109114.Google ScholarPubMed
Cassidy, AR, Ilardi, D, Bowen, SR, et al.Congenital heart disease: a primer for the pediatric neuropsychologist. Child Neuropsychol 2017; 24: 144.Google ScholarPubMed
Bean Jaworski, JL, White, MT, DeMaso, DR, Newburger, JW, Bellinger, DC, Cassidy, AR.Visuospatial processing in adolescents with critical congenital heart disease: organization, integration, and implications for academic achievement. Child Neuropsychol 2018; 24: 451468.10.1080/09297049.2017.1283396CrossRefGoogle ScholarPubMed
Ilardi, D, Ono, KE, McCartney, R, Book, W, Stringer, AY.Neurocognitive functioning in adults with congenital heart disease. Congenit Heart Dis 2017; 12: 166173.CrossRefGoogle ScholarPubMed
Sanz, JH, Berl, MM, Armour, AC, Wang, J, Cheng, YI, Donofrio, MT.Prevalence and pattern of executive dysfunction in school age children with congenital heart disease. Congenit Heart Dis 2017; 12: 202209.CrossRefGoogle ScholarPubMed
Bellinger, DC, Watson, CG, Rivkin, MJ, et al.Neuropsychological status and structural brain imaging in adolescents with single ventricle who underwent the Fontan procedure. J Am Heart Assoc 2015; 4: pii: e002302.CrossRefGoogle ScholarPubMed
Mussatto, KA, Hoffmann, RG, Hoffman, GM, et al.Risk and prevalence of developmental delay in young children with congenital heart disease. Pediatrics 2014; 133: e570e577.CrossRefGoogle ScholarPubMed
Semmel, ES, Dotson, VM, Burns, TG, Mahle, WT, King, TZ.Posterior cerebellar volume and executive function in young adults with congenital heart disease. J Int Neuropsychol Soc 2018; 24: 939948.10.1017/S1355617718000310CrossRefGoogle ScholarPubMed
King, TZ, Smith, KM, Burns, TG, et al.fMRI investigation of working memory in adolescents with surgically treated congenital heart disease. Appl Neuropsychol Child 2016; 6: 721.CrossRefGoogle ScholarPubMed
Brewster, RC, King, TZ, Burns, TG, Drossner, DM, Mahle, WT.White matter integrity dissociates verbal memory and auditory attention span in emerging adults with congenital heart disease. J Int Neuropsychol Soc 2015; 21: 2233.10.1017/S135561771400109XCrossRefGoogle ScholarPubMed
Rollins, CK, Asaro, LA, Akhondi-Asl, A, et al.White matter volume predicts language development in congenital heart disease. J Pediatr 2017; 181: 42.e248.e2.CrossRefGoogle ScholarPubMed
Bellinger, DC.Are children with congenital cardiac malformations at increased risk of deficits in social cognition? Cardiol Young 2008; 18: 39.10.1017/S104795110700176XCrossRefGoogle ScholarPubMed
Calderon, J, Bonnet, D, Courtin, C, Concordet, S, Plumet, MH, Angeard, N.Executive function and theory of mind in school-aged children after neonatal corrective cardiac surgery for transposition of the great arteries. Dev Med Child Neurol 2010; 52: 11391144.10.1111/j.1469-8749.2010.03735.xCrossRefGoogle ScholarPubMed
Association, AP.Diagnostic and Statistical Manual of Mental Disorders (DSM-5®). Arlington, VA: American Psychiatric Pub; 2013.10.1176/appi.books.9780890425596CrossRefGoogle Scholar
Ganz, ML.The lifetime distribution of the incremental societal costs of autism. Arch Pediatr Adolesc Med 2007; 161: 343349.10.1001/archpedi.161.4.343CrossRefGoogle ScholarPubMed
Baio, J, Wiggins, L, Christensen, DL, et al.Prevalence of autism spectrum disorder among children aged 8 years—autism and developmental disabilities monitoring network, 11 sites, United States, 2014. MMWR Surveill Summa 2018; 67: 1.CrossRefGoogle Scholar
Bean Jaworski, JL, Flynn, T, Burnham, N, et al.Rates of autism and potential risk factors in children with congenital heart defects. Congenit Heart Dis. 2017; 12: 421429.10.1111/chd.12461CrossRefGoogle ScholarPubMed
Razzaghi, H, Oster, M, Reefhuis, J.Long-term outcomes in children with congenital heart disease: national health interview survey. J Pediatr 2015; 166: 119124.CrossRefGoogle ScholarPubMed
Tsao, PC, Lee, YS, Jeng, MJ, et al.Additive effect of congenital heart disease and early developmental disorders on attention-deficit/hyperactivity disorder and autism spectrum disorder: a nationwide population-based longitudinal study. Eur Child Adolesc Psychiatry 2017; 26: 13511359.CrossRefGoogle ScholarPubMed
Bellinger, DC, Rivkin, MJ, DeMaso, D, et al.Adolescents with tetralogy of Fallot: neuropsychological assessment and structural brain imaging. Cardiol Young. 2015; 25: 338347.CrossRefGoogle ScholarPubMed
Bellinger, DC, Wypij, D, Rivkin, MJ, et al.Adolescents with d-transposition of the great arteries corrected with the arterial switch procedure: neuropsychological assessment and structural brain imaging. Circulation 2011; 124: 13611369.CrossRefGoogle ScholarPubMed
Dawson, G, Rogers, S, Munson, J, et al.Randomized, controlled trial of an intervention for toddlers with autism: the early start Denver model. Pediatrics 2010; 125: e17e23.CrossRefGoogle ScholarPubMed
Thabtah, F, Peebles, D.Early autism screening: a comprehensive review. Int J Environ Res Public Health 2019; 16: 3502.CrossRefGoogle ScholarPubMed
Kentrou, V, de Veld, DM, Mataw, KJ, Begeer, S.Delayed autism spectrum disorder recognition in children and adolescents previously diagnosed with attention-deficit/hyperactivity disorder. Autism 2019; 23: 10651072.CrossRefGoogle ScholarPubMed
Davidovitch, M, Levit-Binnun, N, Golan, D, Manning-Courtney, P.Late diagnosis of autism spectrum disorder after initial negative assessment by a multidisciplinary team. J Dev Behav Pediatr 2015; 36: 227234.CrossRefGoogle ScholarPubMed
Antshel, KM, Aneja, A, Strunge, L, et al.Autistic spectrum disorders in velo-cardio facial syndrome (22q11.2 deletion). J Autism Dev Disord 2007; 37: 17761786.10.1007/s10803-006-0308-6CrossRefGoogle Scholar
Marino, BS, Lipkin, PH, Newburger, JW, et al.Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation 2012; 126: 11431172.10.1161/CIR.0b013e318265ee8aCrossRefGoogle ScholarPubMed
Harrison, P, Oakland, T.Adaptive Behavior Assessment System—Third edition (ABAS-3). The Psychological Corporation, San Antonio, TX, 2015.Google Scholar
Reynolds, C, Kamphaus, R.Behavior Assessment System for Children–Third edition (BASC-3). Pearson, Bloomington, MN, 2015.Google Scholar
Constantino, JN, Gruber, CP.Social Responsiveness Scale Second Edition (SRS-2). Western Psychological Services (WPS), Torrance, CA, 2012.Google Scholar
Lord, C, Rutter, M, DiLavore, P, Risi, S, Gotham, K, Bishop, S.Autism diagnostic observation schedule–2nd edition (ADOS-2). Western Psychological Services (WPS), Los Angeles, CA, 2012.Google Scholar
McCormick, CEB, Kavanaugh, BC, Sipsock, D, et al.Autism heterogeneity in a densely sampled U.S. population: results from the first 1,000 participants in the RI-CART study. Autism Res. 2020; 13: 474488.CrossRefGoogle Scholar
Lukmanji, S, Manji, SA, Kadhim, S, et al.The co-occurrence of epilepsy and autism: a systematic review. Epilepsy Behav 2019; 98: 238248.10.1016/j.yebeh.2019.07.037CrossRefGoogle ScholarPubMed
Delobel-Ayoub, M, Klapouszczak, D, van Bakel, MME, et al.Prevalence and characteristics of autism spectrum disorders in children with cerebral palsy. Dev Med Child Neurol 2017; 59: 738742.10.1111/dmcn.13436CrossRefGoogle ScholarPubMed
Kanne, SM, Gerber, AJ, Quirmbach, LM, Sparrow, SS, Cicchetti, DV, Saulnier, CA.The role of adaptive behavior in autism spectrum disorders: implications for functional outcome. J Autism Dev Disord 2011; 41: 10071018.CrossRefGoogle ScholarPubMed
Farmer, C, Swineford, L, Swedo, SE, Thurm, A.Classifying and characterizing the development of adaptive behavior in a naturalistic longitudinal study of young children with autism. J Neurodev Disord 2018; 10: 1.CrossRefGoogle Scholar
Bradstreet, LE, Juechter, JI, Kamphaus, RW, Kerns, CM, Robins, DL.Using the BASC-2 Parent Rating Scales to screen for autism spectrum disorder in toddlers and preschool-aged children. J Abnorm Child Psychol 2017; 45: 359370.10.1007/s10802-016-0167-3CrossRefGoogle ScholarPubMed
Shattuck, PT, Durkin, M, Maenner, M, et al.Timing of identification among children with an autism spectrum disorder: findings from a population-based surveillance study. J Am Acad Child Adolesc Psychiatry 2009; 48: 474483.CrossRefGoogle ScholarPubMed
Zwaigenbaum, L, Bauman, ML, Fein, D, et al.Early screening of autism spectrum disorder: recommendations for practice and research. Pediatrics 2015; 136(Suppl 1): S41S59.10.1542/peds.2014-3667DCrossRefGoogle ScholarPubMed
Smith-Young, J, Chafe, R, Audas, R.“Managing the wait”: parents’ experiences in accessing diagnostic and treatment services for children and adolescents diagnosed with autism spectrum disorder. Health Serv Insights 2020; 13: 1178632920902141.Google ScholarPubMed
Swanson, AR, Warren, ZE, Stone, WL, Vehorn, AC, Dohrmann, E, Humberd, Q.The diagnosis of autism in community pediatric settings: does advanced training facilitate practice change? Autism 2014; 18: 555561.CrossRefGoogle Scholar
Richards, C, Jones, C, Groves, L, Moss, J, Oliver, C.Prevalence of autism spectrum disorder phenomenology in genetic disorders: a systematic review and meta-analysis. Lancet Psychiatry 2015; 2: 909916.CrossRefGoogle ScholarPubMed
Kuzniewicz, MW, Wi, S, Qian, Y, Walsh, EM, Armstrong, MA, Croen, LA.Prevalence and neonatal factors associated with autism spectrum disorders in preterm infants. J Pediatr 2014; 164: 2025.CrossRefGoogle ScholarPubMed
Valicenti-McDermott, M, Hottinger, K, Seijo, R, Shulman, L.Age at diagnosis of autism spectrum disorders. J Pediatr 2012; 161: 554556.10.1016/j.jpeds.2012.05.012CrossRefGoogle ScholarPubMed
Elder, JH, Kreider, CM, Brasher, SN, Ansell, M.Clinical impact of early diagnosis of autism on the prognosis and parent-child relationships. Psychol Res Behav Manag 2017; 10: 283292.CrossRefGoogle ScholarPubMed
Rotholz, DA, Kinsman, AM, Lacy, KK, Charles, J.Improving early identification and intervention for children at risk for autism spectrum disorder. Pediatrics 2017; 139: e20161061.CrossRefGoogle ScholarPubMed
Cited by

Save article to Kindle

To save this article to your Kindle, first ensure is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the or variations. ‘’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Implementing standard screening for autism spectrum disorder in CHD
Available formats

Save article to Dropbox

To save this article to your Dropbox account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you used this feature, you will be asked to authorise Cambridge Core to connect with your Dropbox account. Find out more about saving content to Dropbox.

Implementing standard screening for autism spectrum disorder in CHD
Available formats

Save article to Google Drive

To save this article to your Google Drive account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you used this feature, you will be asked to authorise Cambridge Core to connect with your Google Drive account. Find out more about saving content to Google Drive.

Implementing standard screening for autism spectrum disorder in CHD
Available formats

Reply to: Submit a response

Please enter your response.

Your details

Please enter a valid email address.

Conflicting interests

Do you have any conflicting interests? *