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Original article

Vol. 152 No. 3536 (2022)

ORCHID (Outcome Registry for CHIldren with severe congenital heart Disease) a Swiss, nationwide, prospective, population-based, neurodevelopmental paediatric patient registry: framework, regulations and implementation

  • Julia Natterer 
  • Juliane Schneider
  • Nicole Sekarski
  • Verena Rathke
  • Mark Adams
  • Beatrice Latal
  • Cristina Borradori-Tolsa
  • Maya Bouhabib
  • Katharina Fuhrer Kradolfer
  • Martin Glöckler
  • Damian Hutter
  • Janet Kelly
  • Christelle L’Ebraly
  • Marc R. Pfluger
  • Angelo Polito
  • Michael von Rhein
  • Walter Knirsch 
Cite this as:
Swiss Med Wkly. 2022;152:w30217


INTRODUCTION: Congenital heart disease (CHD) is the most frequent birth defect. As survival has significantly improved, attention has turned to neurodevelopmental outcomes of children undergoing heart surgery in early infancy. Since multiple risk factors contribute to neurodevelopmental alterations, a nationwide registry collecting data on medical characteristics, interventions, clinical course and neurodevelopment until school-age is needed to improve the quality of management, identify risk- and protective factors affecting neurodevelopment, and facilitate multicentre trials.

METHODS AND ANALYSIS: The Swiss Outcome Registry for CHIldren with severe congenital heart Disease (ORCHID) is a nationwide, prospective, population-based patient registry developed (1) to collect baseline characteristics and clinical data of CHD patients operated with bypass-surgery or hybrid procedures in the first 6 weeks of life in Switzerland, (2) to monitor long-term neurodevelopment, and (3) to relate clinical characteristics and neurodevelopment to identify risk and protective factors in these children. This registry started data collection relating to pregnancy, birth, preoperative course, catheter-based and surgical treatment, postoperative course and reinterventions in 2019. The primary outcome includes standardised neurodevelopmental assessments at 9 to 12 months, 18 to 24 months and 5.5 to 6 years. We expect to include 80 to 100 children per year. Correlation and regression analyses will be used to investigate risk- and protective factors influencing neurodevelopment.

ETHICS AND DISSEMINATION OF RESULTS: Swiss ORCHID received support by the Accentus Charitable Foundation, the Anna Mueller Grocholoski Stiftung, the Swiss Society of Paediatric Cardiology, the Verein Kinderherzforschung, and the Corelina – Stiftung für das Kinderherz, and was approved by the cantonal ethics committees. Findings will be presented at national and international scientific meetings, and published in peer-reviewed journals. Results will also be shared with patient organizations, primary health care providers, and public health stakeholders to ensure a widespread dissemination of the results.


  1. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002 Jun;39(12):1890–900.
  2. Lytzen R, Vejlstrup N, Bjerre J, Petersen OB, Leenskjold S, Dodd JK, et al. Live-Born Major Congenital Heart Disease in Denmark: Incidence, Detection Rate, and Termination of Pregnancy Rate From 1996 to 2013. JAMA Cardiol. 2018 Sep;3(9):829–37.
  3. Wernovsky G. Current insights regarding neurological and developmental abnormalities in children and young adults with complex congenital cardiac disease. Cardiol Young. 2006 Feb;16(S1 Suppl 1):92–104.
  4. Brosig C, Butcher J, Butler S, Ilardi DL, Sananes R, Sanz JH, et al. Monitoring developmental risk and promoting success for children with congenital heart disease: recommendations for cardiac neurodevelopmental follow-up programs. Clin Pract Pediatr Psychol. 2014;2(2):153–65.
  5. Martin GR, Jonas RA. Surgery for Congenital Heart Disease: improvements in Outcomes. Am J Perinatol. 2018 May;35(6):557–60.
  6. Bouma BJ, Mulder BJ. Changing Landscape of Congenital Heart Disease. Circ Res. 2017 Mar;120(6):908–22.
  7. Marino BS, Lipkin PH, Newburger JW, Peacock G, Gerdes M, Gaynor JW, et al.; American Heart Association Congenital Heart Defects Committee, Council on Cardiovascular Disease in the Young, Council on Cardiovascular Nursing, and Stroke Council. Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation. 2012 Aug;126(9):1143–72.
  8. Bolduc ME, Rennick JE, Gagnon I, Majnemer A, Brossard-Racine M. Canadian Developmental Follow-up Practices in Children With Congenital Heart Defects: A National Environmental Scan. CJC Pediatric and Congenital Heart Disease. 2022;1(1):3–10.
  9. Latal B. Neurodevelopmental Outcomes of the Child with Congenital Heart Disease. Clin Perinatol. 2016 Mar;43(1):173–85.
  10. Liamlahi R, Latal B. Neurodevelopmental outcome of children with congenital heart disease. Handb Clin Neurol. 2019;162:329–45.
  11. Wernovsky G. Outcomes regarding the central nervous system in children with complex congenital cardiac malformations. Cardiol Young. 2005 Feb;15(S1 Suppl 1):132–3.
  12. Feldmann M, Bataillard C, Ehrler M, Ullrich C, Knirsch W, Gosteli-Peter MA, et al. Cognitive and Executive Function in Congenital Heart Disease: A Meta-analysis. Pediatrics. 2021 Oct;148(4):e2021050875.
  13. Naef N, Liamlahi R, Beck I, Bernet V, Dave H, Knirsch W, et al. Neurodevelopmental Profiles of Children with Congenital Heart Disease at School Age. J Pediatr. 2017 Sep;188:75–81.
  14. Feldmann M, Guo T, Miller SP, Knirsch W, Kottke R, Hagmann C, et al. Delayed maturation of the structural brain connectome in neonates with congenital heart disease. Brain Commun. 2020 Nov;2(2):fcaa209.
  15. Massaro AN, El-Dib M, Glass P, Aly H. Factors associated with adverse neurodevelopmental outcomes in infants with congenital heart disease. Brain Dev. 2008 Aug;30(7):437–46.
  16. Chock VY, Reddy VM, Bernstein D, Madan A. Neurologic events in neonates treated surgically for congenital heart disease. J Perinatol. 2006 Apr;26(4):237–42.
  17. Howell HB, Zaccario M, Kazmi SH, Desai P, Sklamberg FE, Mally P. Neurodevelopmental outcomes of children with congenital heart disease: A review. Curr Probl Pediatr Adolesc Health Care. 2019 Oct;49(10):100685.
  18. Sun L, van Amerom JF, Marini D, Portnoy S, Lee FT, Saini BS, et al. MRI characterization of hemodynamic patterns of human fetuses with cyanotic congenital heart disease. Ultrasound Obstet Gynecol. 2021 Dec;58(6):824–36.
  19. Peyvandi S, Latal B, Miller SP, McQuillen PS. The neonatal brain in critical congenital heart disease: insights and future directions. Neuroimage. 2019 Jan;185:776–82.
  20. Stegeman R, Feldmann M, Claessens NH, Jansen NJ, Breur JM, de Vries LS, et al.; European Association Brain in Congenital Heart Disease Consortium. A Uniform Description of Perioperative Brain MRI Findings in Infants with Severe Congenital Heart Disease: results of a European Collaboration. AJNR Am J Neuroradiol. 2021 Nov;42(11):2034–9.
  21. Algra SO, Haas F, Poskitt KJ, Groenendaal F, Schouten AN, Jansen NJ, et al. Minimizing the risk of preoperative brain injury in neonates with aortic arch obstruction. J Pediatr. 2014 Dec;165(6):1116–1122.e3.
  22. Lynch JM, Buckley EM, Schwab PJ, McCarthy AL, Winters ME, Busch DR, et al. Time to surgery and preoperative cerebral hemodynamics predict postoperative white matter injury in neonates with hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2014 Nov;148(5):2181–8.
  23. Petit CJ, Rome JJ, Wernovsky G, Mason SE, Shera DM, Nicolson SC, et al. Preoperative brain injury in transposition of the great arteries is associated with oxygenation and time to surgery, not balloon atrial septostomy. Circulation. 2009 Feb;119(5):709–16.
  24. Dimitropoulos A, McQuillen PS, Sethi V, Moosa A, Chau V, Xu D, et al. Brain injury and development in newborns with critical congenital heart disease. Neurology. 2013 Jul;81(3):241–8.
  25. Claessens NH, Chau V, de Vries LS, Jansen NJ, Au-Young SH, Stegeman R, et al. Brain Injury in Infants with Critical Congenital Heart Disease: Insights from Two Clinical Cohorts with Different Practice Approaches. J Pediatr. 2019 Dec;215:75–82.e2.
  26. Atallah J, Garcia Guerra G, Joffe AR, Bond GY, Islam S, Ricci MF, et al.; Western Canadian Complex Pediatric Therapies Follow‐up Program*. Survival, Neurocognitive, and Functional Outcomes After Completion of Staged Surgical Palliation in a Cohort of Patients With Hypoplastic Left Heart Syndrome. J Am Heart Assoc. 2020 Feb;9(4):e013632.
  27. Newburger JW, Wypij D, Bellinger DC, du Plessis AJ, Kuban KC, Rappaport LA, et al. Length of stay after infant heart surgery is related to cognitive outcome at age 8 years. J Pediatr. 2003 Jul;143(1):67–73.
  28. Gaynor JW, Stopp C, Wypij D, Andropoulos DB, Atallah J, Atz AM, et al.; International Cardiac Collaborative on Neurodevelopment (ICCON) Investigators. Neurodevelopmental outcomes after cardiac surgery in infancy. Pediatrics. 2015 May;135(5):816–25.
  29. Beckmann A, Dittrich S, Arenz C, Krogmann ON, Horke A, Tengler A, et al. German Registry for Cardiac Operations and Interventions in Patients with Congenital Heart Disease: Report 2020-Comprehensive Data from 6 Years of Experience. Thorac Cardiovasc Surg. 2021;69(S 03):e21-e31.
  30. Alsaied T, Allen KY, Anderson JB, Anixt JS, Brown DW, Cetta F, et al. The Fontan outcomes network: first steps towards building a lifespan registry for individuals with Fontan circulation in the United States. Cardiol Young. 2020 Aug;30(8):1070–5.
  31. Marino BS, Sood E, Cassidy AR, Miller TA, Sanz JH, Bellinger D, et al. The origins and development of the cardiac neurodevelopment outcome collaborative: creating innovative clinical, quality improvement, and research opportunities. Cardiol Young. 2020 Nov;30(11):1597–602.
  32. Bates KE, Yu S, Mangeot C, Shea JA, Brown DW, Uzark K. Identifying best practices in interstage care: using a positive deviance approach within the National Pediatric Cardiology Quality Improvement Collaborative. Cardiol Young. 2019 Mar;29(3):398–407.
  33. Svensson B, Idvall E, Nilsson F, Liuba P. Health-related quality of life in children with surgery for CHD: a study from the Swedish National Registry for Congenital Heart Disease. Cardiol Young. 2017 Mar;27(2):333–43.
  34. Olsen M, Christensen TD, Pedersen L, Johnsen SP, Hjortdal VE. Late mortality among Danish patients with congenital heart defect. Am J Cardiol. 2010 Nov;106(9):1322–6.
  35. Robertson CM, Sauve RS, Joffe AR, Alton GY, Moddemann DM, Blakley PM, et al. The registry and follow-up of complex pediatric therapies program of Western Canada: a mechanism for service, audit, and research after life-saving therapies for young children. Cardiol Res Pract. 2011;2011:965740.
  36. Ware J, Butcher JL, Latal B, Sadhwani A, Rollins CK, Brosig Soto CL, et al. Neurodevelopmental evaluation strategies for children with congenital heart disease aged birth through 5 years: recommendations from the cardiac neurodevelopmental outcome collaborative. Cardiol Young. 2020 Nov;30(11):1609–22.
  37. Fourdain S, Simard MN, Dagenais L, Materassi M, Doussau A, Goulet J, et al. Gross Motor Development of Children with Congenital Heart Disease Receiving Early Systematic Surveillance and Individualized Intervention: brief Report. Dev Neurorehabil. 2021 Jan;24(1):56–62.
  38. Adams MB, Bucher HU. Un début précoce dans la vie: qu’apporte un registre national? Forum Med Suisse. 2013;13(3):35–7.
  39. McQuillen PS, Miller SP. Congenital heart disease and brain development. Ann N Y Acad Sci. 2010 Jan;1184(1):68–86.
  40. Adams MB, Bickle Graz M, Grunt S, Weber P, Capone Mori A, Bauder F, et al. Follow-up assessment of high-risk newborns in Switzerland. Recommendations of the Swiss Society of Neonatology, the Swiss Society of Developmental Pediatrics and the Swiss Society of Neuropediatrics. Paediatrica. 2014;25(5):6–10.
  41. Albers CA, Grieve AJ. Test Review: Bayley, N. (2006). Bayley Scales of Infant and Toddler Development– Third Edition. San Antonio, TX: Harcourt Assessment. 2007;25(2):180-90.
  42. Palisano R, Rosenbaum P, Walter S, Russell D, Wood E, Galuppi B. Development and reliability of a system to classify gross motor function in children with cerebral palsy. Dev Med Child Neurol. 1997 Apr;39(4):214–23.
  43. Bain SK, Gray R. Test Reviews: Kaufman, A. S., & Kaufman, N. L. (2004). Kaufman Assessment Battery for Children, Second edition. Circle Pines, MN: AGS. 2008;26(1):92-101.
  44. Largo RH, Caflisch JA, Hug F, Muggli K, Molnar AA, Molinari L, et al. Neuromotor development from 5 to 18 years. Part 1: timed performance. Dev Med Child Neurol. 2001 Jul;43(7):436–43.

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