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Deterioration of functional abilities in children surviving the Fontan operation

Published online by Cambridge University Press:  25 April 2018

M. Florencia Ricci ,
Billie-Jean Martin ,
Ari R. Joffe ,
Irina A. Dinu ,
Gwen Y. Alton ,
Gonzalo G. Guerra ,
Charlene M. T. Robertson  and
For the Western Canadian Complex Pediatric Therapies Follow-Up Program
M. Florencia Ricci*
Affiliation:
Child Development Clinic, Department of Pediatrics and Child Health, University of Manitoba, Winnipeg, Canada
Billie-Jean Martin
Affiliation:
Department of Cardiothoracic Surgery, Stanford University, Stanford, CA, USA
Ari R. Joffe
Affiliation:
Department of Pediatrics, Division of Pediatric Critical Care, University of Alberta, Edmonton, Canada
Irina A. Dinu
Affiliation:
School of Public Health, University of Alberta, Edmonton, Canada
Gwen Y. Alton
Affiliation:
Pediatric Rehabilitation, Glenrose Rehabilitation Hospital, University of Alberta, Edmonton, Canada
Gonzalo G. Guerra
Affiliation:
Department of Pediatrics, Division of Pediatric Critical Care, University of Alberta, Edmonton, Canada
Charlene M. T. Robertson
Affiliation:
Pediatric Rehabilitation, Glenrose Rehabilitation Hospital, University of Alberta, Edmonton, Canada Department of Pediatrics, Division of Developmental Pediatrics, University of Alberta, Edmonton, Canada
*
Author for correspondence: M. Florencia Ricci, MD, PhD Candidate, SSCY Center, 1155 Notre Dame Ave, Winnipeg, MB R3E 3G1, Canada. Tel: +1 204 258 6631; Fax: +1 204 258 6798; E-mail: fricci@rccinc.ca
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Abstract

Functional abilities are needed for activities of daily living. In general, these skills expand with age. We hypothesised that, in contrast to what is normally expected, children surviving the Fontan may have deterioration of functional abilities, and that peri-Fontan stroke is associated with this deterioration. All children registered in the Western Canadian Complex Pediatric Therapies Follow-up Program who survived a Fontan operation in the period 1999–2016 were eligible for inclusion. At the age of 2 years (pre-Fontan) and 4.5 years (post-Fontan), the Adaptive Behavior Assessment System-II general adaptive composite score was determined (population mean: 100, standard deviation: 15). Deterioration of functional abilities was defined as ⩾1 standard deviation decrease in pre- to post-Fontan scores. Perioperative strokes were identified through chart review. Multivariable logistic regression analysis determined predictors of deterioration of functional abilities. Of 133 children, with a mean age at Fontan of 3.3 years (standard deviation 0.8) and 65% male, the mean (standard deviation) general adaptive composite score was 90.6 (17.5) at 2 years and 88.3 (19.1) at 4.5 years. After Fontan, deterioration of functional abilities occurred in 34 (26%) children, with a mean decline of 21.8 (7.1) points. Evidence of peri-Fontan stroke was found in 10 (29%) children who had deterioration of functional abilities. Peri-Fontan stroke (odds ratio 5.00 (95% CI 1.74, 14.36)) and older age at Fontan (odds ratio 1.67 (95% CI 1.02, 2.73)) predicted functional deterioration. The trajectory of functional abilities should be assessed in this population, as more than 25% experience deterioration. Efforts to prevent peri-Fontan stroke, and to complete the Fontan operation at an earlier age, may lead to reduction of this deterioration.

Keywords

ChildFontan procedureactivities of daily livingstroke
Type
Original Articles
Information
Cardiology in the Young , Volume 28 , Issue 6 , June 2018 , pp. 868 - 875
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Copyright
© Cambridge University Press 2018 

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References

1. Ringle, ML, Wernovsky, G. Functional, quality of life, and neurodevelopmental outcomes after congenital cardiac surgery. Semin Perinatol 2016; 40: 556–570.Google Scholar
2. Limperopoulos, C, Majnemer, A, Shevell, MI, et al. Functional limitations in young children with congenital heart defects after cardiac surgery. Pediatrics 2001; 108: 1325–1331.Google Scholar
3. Harrison, P, Oakland, T. Manual of the Adaptive Behaviour Assessment System II. Psychological Corp, San Antonio, TX, 2003.Google Scholar
4. Oakland, T, Algina, J. Adaptive Behavior Assessment System-II parent/primary caregiver form: ages 0–5: its factor structure and other implications for practice. J Appl Sch Psychol 2011; 27: 103–117.CrossRefGoogle Scholar
5. Gaynor, JW, Ittenbach, RF, Gerdes, M, et al. Neurodevelopmental outcomes in preschool survivors of the Fontan procedure. J Thorac Cardiovasc Surg 2014; 147: 1276–1283.e5.CrossRefGoogle ScholarPubMed
6. Downing, TE, Allen, KY, Glatz, AC, et al. Long-term survival after the Fontan operation: twenty years of experience at a single center. J Thorac Cardiovasc Surg 2017; 154: 243–253.e2.CrossRefGoogle ScholarPubMed
7. Alsaied, T, Bokma, JP, Engel, ME, et al. Factors associated with long-term mortality after Fontan procedures: a systematic review. Heart 2017; 103: 104–110.Google Scholar
8. Rempel, G, Magill-Evans, J, Wiart, L, et al. There is so much more to a child than their heart; Supports and Services for Children with Complex Congenital Heart Disease and Their Parents. Final Report. Alberta Centre for Child, Family and Community Research, Edmonton, AB, 2014.Google Scholar
9. Barker, PCA, Nowak, C, King, K, Mosca, RS, Bove, EL, Goldberg, CS. Risk factors for cerebrovascular events following Fontan palliation in patients with a functional single ventricle. Am J Cardiol 2005; 96: 587–591.CrossRefGoogle ScholarPubMed
10. 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: e002302.CrossRefGoogle ScholarPubMed
11. Chun, DS, Schamberger, MS, Flaspohler, T, et al. Incidence, outcome, and risk factors for stroke after the Fontan procedure. Am J Cardiol 2004; 93: 117–119.CrossRefGoogle ScholarPubMed
12. du Plessis, AJ, Chang, AC, Wessel, DL, et al. Cerebrovascular accidents following the Fontan operation. Pediatr Neurol 1995; 12: 230–236.CrossRefGoogle ScholarPubMed
13. Gordon, AL, Ganesan, V, Towell, A, Kirkham, FJ. Functional outcome following stroke in children. J Child Neurol 2002; 17: 429–434.CrossRefGoogle ScholarPubMed
14. Robertson, CMT, Sauve, RS, Joffe, AR, 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.CrossRefGoogle Scholar
15. Ricci, MF, Andersen, JC, Joffe, AR, et al. Chronic neuromotor disability after complex cardiac surgery in early life. Pediatrics 2015; 136: e922–933.CrossRefGoogle ScholarPubMed
16. Blishen, BR, Carroll, WK, Moore, C. The 1981 socioeconomic index for occupations in Canada. Can Rev Soc Anthropol 1987; 24: 465–488.CrossRefGoogle Scholar
17. Wernovsky, G, Wypij, D, Jonas, RA, et al. Postoperative course and hemodynamic profile after the arterial switch operation in neonates and infants. A comparison of low-flow cardiopulmonary bypass and circulatory arrest. Circulation 1995; 92: 2226–2235.Google Scholar
18. Alton, GY, Rempel, GR, Robertson, CMT, Newburn-Cook, CV, Norris, CM. Functional outcomes after neonatal open cardiac surgery: comparison of survivors of the Norwood staged procedure and the arterial switch operation. Cardiol Young 2010; 20: 668–675.Google Scholar
19. Alton, GY, Taghados, S, Joffe, AR, et al. Prediction of preschool functional abilities after early complex cardiac surgery. Cardiol Young 2015; 25: 655–662.Google Scholar
20. Brosig, CL, Bear, L, Allen, S, et al. Preschool neurodevelopmental outcomes in children with congenital heart disease. J Pediatr 2017; 183: 80–86.e1.Google Scholar
21. Willard, VW, Qaddoumi, I, Chen, S, et al. Developmental and adaptive functioning in children with retinoblastoma: a longitudinal investigation. J Clin Oncol 2014; 32: 2788–2793.Google Scholar
22. Chapieski, L, Brewer, V, Evankovich, K, Culhane-Shelburne, K, Zelman, K, Alexander, A. Adaptive functioning in children with seizures: impact of maternal anxiety about epilepsy. Epilepsy Behav 2005; 7: 246–252.CrossRefGoogle ScholarPubMed
23. Warren, SF, Brady, N, Fleming, KK, Hahn, LJ. The longitudinal effects of parenting on adaptive behavior in children with fragile X syndrome. J Autism Dev Disord 2017; 47: 768–784.CrossRefGoogle ScholarPubMed
24. Oakland, T, Harrison, PL. Adaptive Behavior Assessment System II: Clinical Use and Interpretation. Elsevier/Academic Press, San Diego, CA, 2011.Google Scholar
25. Laraja, K, Sadhwani, A, Tworetzky, W, et al. Neurodevelopmental outcome in children after fetal cardiac intervention for aortic stenosis with evolving hypoplastic left heart syndrome. J Pediatr 2017; 184: 130–136.e4.CrossRefGoogle ScholarPubMed
26. Heber, R. Terminology and the classification of mental retardation. Am J Ment Defic 1958; 63: 214–219.Google ScholarPubMed
27. Pike, NA, Evangelista, LS, Doering, LV, Eastwood, J-A, Lewis, AB, Child, JS. Quality of life, health status, and depression: comparison between adolescents and adults after the Fontan procedure with healthy counterparts. J Cardiovasc Nurs 2012; 27: 539–546.CrossRefGoogle ScholarPubMed
28. Ponsford, J, Cameron, P, Fitzgerald, M, Grant, M, Mikocka-Walus, A. Long-term outcomes after uncomplicated mild traumatic brain injury: a comparison with trauma controls. J Neurotrauma 2011; 28: 937–946.Google Scholar
29. Catroppa, C, Anderson, VA, Morse, SA, Haritou, F, Rosenfeld, JV. Outcome and predictors of functional recovery 5 years following pediatric traumatic brain injury (TBI). J Pediatr Psychol 2008; 33: 707–718.CrossRefGoogle ScholarPubMed
30. Cheng, HH, Rajagopal, S, McDavitt, E, et al. Stroke in acquired and congenital heart disease patients and its relationship to hospital mortality and lasting neurologic deficits. Pediatr Crit Care Med 2016; 17: 976–983.Google Scholar
31. Everts, R, Pavlovic, J, Kaufmann, F, et al. Cognitive functioning, behavior, and quality of life after stroke in childhood. Child Neuropsychol 2008; 14: 323–338.Google Scholar
32. Long, B, Anderson, V, Jacobs, R, et al. Executive function following child stroke: the impact of lesion size. Dev Neuropsychol 2011; 36: 971–987.Google Scholar
33. Greenham, M, Gordon, A, Anderson, V, Mackay, MT. Outcome in childhood stroke. Stroke 2016; 47: 1159–1164.CrossRefGoogle ScholarPubMed
34. Rempel, GR, Harrison, MJ, Williamson, DL. Is “treat your child normally” helpful advice for parents of survivors of treatment of hypoplastic left heart syndrome? Cardiol Young 2009; 19: 135–144.CrossRefGoogle Scholar
35. Shiraishi, S, Yagihara, T, Kagisaki, K, et al. Impact of age at Fontan completion on postoperative hemodynamics and long-term aerobic exercise capacity in patients with dominant left ventricle. Ann Thorac Surg 2009; 87: 555–561.CrossRefGoogle ScholarPubMed
36. Martin, B-J, Ricci, MF, Atallah, J, et al. Neurocognitive abilities in children who undergo the Fontan operation: the association between hypoplastic left heart syndrome and outcomes. J Am Coll Cardiol 2016; 67: 946.Google Scholar
37. Dixon, SD, Stein, MT. Encounters With Children: Pediatric Behavior and Development. Mosby Elsevier, Philadelphia, PA, 2006.Google Scholar
38. Bass, JL, Corwin, M, Gozal, D, et al. The effect of chronic or intermittent hypoxia on cognition in childhood: a review of the evidence. Pediatrics 2004; 114: 805–816.Google Scholar
39. Norman, G, Sloan, J, Wyrwich, K. Interpretation of changes in health-related quality of life: the remarkable universality of half a standard deviation. Med Care 2003; 41: 582–592.Google Scholar
40. 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: e17–e23.CrossRefGoogle ScholarPubMed