Hostname: page-component-cd9895bd7-jn8rn Total loading time: 0 Render date: 2024-12-22T23:23:31.788Z Has data issue: false hasContentIssue false
  • English
  • Français

Does functional health status predict health-related quality of life in children after Fontan operation?

Published online by Cambridge University Press:  23 April 2015

Karolijn Dulfer ,
Sjoerd S. M. Bossers ,
Elisabeth M. W. J. Utens ,
Nienke Duppen ,
Irene M. Kuipers ,
Livia Kapusta ,
Gabrielle van Iperen ,
Michiel Schokking ,
Arend D. J. ten Harkel  and
Tim Takken
...Show all authors
Karolijn Dulfer
Affiliation:
Department of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Centre–Sophia Children’s Hospital, Rotterdam, the Netherlands
Sjoerd S. M. Bossers
Affiliation:
Department of Paediatrics, Division of Cardiology, Erasmus Medical Centre – Sophia Children’s Hospital, Rotterdam, the Netherlands
Elisabeth M. W. J. Utens
Affiliation:
Department of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Centre–Sophia Children’s Hospital, Rotterdam, the Netherlands
Nienke Duppen
Affiliation:
Department of Paediatrics, Division of Cardiology, Erasmus Medical Centre – Sophia Children’s Hospital, Rotterdam, the Netherlands
Irene M. Kuipers
Affiliation:
Department of Paediatrics, Division of Cardiology, Academic Medical Centre, Amsterdam, the Netherlands
Livia Kapusta
Affiliation:
Department of Paediatrics, Division of Cardiology, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands Department of Paediatrics, Pediatric Cardiology Unit, Tel-Aviv Sourasky Medical Centre, Tel Aviv, Israel
Gabrielle van Iperen
Affiliation:
Department of Paediatrics, Division of Cardiology, University Medical Centre Utrecht – Wilhelmina Children’s Hospital, Utrecht, the Netherlands
Michiel Schokking
Affiliation:
Department of Paediatrics, Division of Cardiology, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands
Arend D. J. ten Harkel
Affiliation:
Department of Paediatrics, Division of Cardiology, Leiden University Medical Centre, Leiden, the Netherlands
Tim Takken
Affiliation:
Child Development and Exercise Centre, University Medical Centre Utrecht – Wilhelmina Children’s Hospital, Utrecht, the Netherlands
Willem A. Helbing*
Affiliation:
Department of Paediatrics, Division of Cardiology, Erasmus Medical Centre – Sophia Children’s Hospital, Rotterdam, the Netherlands
*
Correspondence to: W. A. Helbing, Department of Pediatric Cardiology, Erasmus Medical Centre – Sophia Children’s Hospital, Sp-2429, PO Box 2060, 3000 CB Rotterdam, the Netherlands. Tel: +31 10 7036264; Fax: +31 10 7036772; E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Purpose

It is important to identify those children with a Fontan circulation who are at risk for impaired health-related quality of life. We aimed to determine the predictive value of functional health status – medical history and present medical status – on both physical and psychosocial domains of health-related quality of life, as reported by patients themselves and their parents.

Methods

We carried out a prospective cross-sectional multi-centre study in Fontan patients aged between 8 and 15, who had undergone staged completion of total cavopulmonary connection according to a current technique before the age of 7 years.

Functional health status was assessed as medical history – that is, age at Fontan, type of Fontan, ventricular dominance, and number of cardiac surgical procedures – and present medical status – assessed with magnetic resonance imaging, exercise testing, and rhythm assessment. Health-related quality of life was assessed with The TNO/AZL Child Questionnaire Child Form and Parent Form.

Results

In multivariate prediction models, several medical history variables, such as more operations post-Fontan completion, lower age at Fontan completion, and dominant right ventricle, and present medical status variables, such as smaller end-diastolic volume, a higher score for ventilatory efficiency, and the presence of sinus node dysfunction, predicted worse outcomes on several parent-reported and self-reported physical as well as psychosocial health-related quality of life domains.

Conclusions

Medical history and worse present medical status not only predicted worse physical parent-reported and self-reported health-related quality of life but also worse psychosocial health-related quality of life and subjective cognitive functioning. These findings will help in identifying patients who are at risk for developing impaired health-related quality of life.

Keywords

Fontan circulationcongenital heart diseasequality of life
Type
Original Articles
Information
Cardiology in the Young , Volume 26 , Supplement 3 , March 2016 , pp. 459 - 468
Check for updates
Copyright
© Cambridge University Press 2015 

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.)

Footnotes

*

Both authors contributed equally.

References

1. d’Udekem, Y, Iyengar, AJ, Cochrane, AD, et al. The Fontan procedure: contemporary techniques have improved long-term outcomes. Circulation 2007; 116: I157–I164.Google Scholar
2. Robbers-Visser, D, Miedema, M, Nijveld, A, et al. Results of staged total cavopulmonary connection for functionally univentricular hearts; comparison of intra-atrial lateral tunnel and extracardiac conduit. Eur J Cardiothorac Surg 2010; 37: 934–941.Google Scholar
3. Anderson, PA, Sleeper, LA, Mahony, L et al. Contemporary outcomes after the Fontan procedure: a Pediatric Heart Network multicenter study. J Am Coll Cardiol 2008; 52: 85–98.Google Scholar
4. Marino, BS, Shera, D, Wernovsky, G, et al. The development of the pediatric cardiac quality of life inventory: a quality of life measure for children and adolescents with heart disease. Qual Life Res 2008; 17: 613–626.Google Scholar
5. Idorn, L, Jensen, AS, Juul, K, et al. Quality of life and cognitive function in Fontan patients, a population-based study. Int J Cardiol 2013; 168: 3230–3235.CrossRefGoogle ScholarPubMed
6. Dulfer, K, Helbing, WA, Duppen, N, Utens, EM. Associations between exercise capacity, physical activity, and psychosocial functioning in children with congenital heart disease: a systematic review. Eur J Prev Cardiol 2014; 21: 1200–1215.Google Scholar
7. McCrindle, BW, Zak, V, Breitbart, RE, et al. The Relationship of patient medical and laboratory characteristics to changes in functional health status in children and adolescents after the Fontan procedure. Pediatr Cardiol 2014; 35: 632–640.CrossRefGoogle ScholarPubMed
8. McCrindle, BW, Zak, V, Pemberton, VL, et al. Functional health status in children and adolescents after Fontan: comparison of generic and disease-specific assessments. Cardiol Young 2013: 1–9.Google Scholar
9. Czosek, RJ, Bonney, WJ, Cassedy, A, et al. Impact of cardiac devices on the quality of life in pediatric patients. Circ Arrhythm Electrophysiol 2012; 5: 1064–1072.Google Scholar
10. Robbers-Visser, D, Jan Ten Harkel, D, Kapusta, L, et al. Usefulness of cardiac magnetic resonance imaging combined with low-dose dobutamine stress to detect an abnormal ventricular stress response in children and young adults after Fontan operation at young age. Am J Cardiol 2008; 101: 1657–1662.Google Scholar
11. Luijnenburg, SE, Robbers-Visser, D, Moelker, A, Vliegen, HW, Mulder, BJ, Helbing, WA. Intra-observer and interobserver variability of biventricular function, volumes and mass in patients with congenital heart disease measured by CMR imaging. Int J Cardiovasc Imaging 2010; 26: 57–64.CrossRefGoogle ScholarPubMed
12. Bossers, SS, Helbing, WA, Duppen, N, et al. Exercise capacity in children after total cavopulmonary connection: lateral tunnel versus extracardiac conduit technique. J Thorac Cardiovasc Surg 2014; 148: 1490–1497.CrossRefGoogle ScholarPubMed
13. Ten Harkel, AD, Takken, T, Van Osch-Gevers, M, Helbing, WA. Normal values for cardiopulmonary exercise testing in children. Eur J Cardiovasc Prev Rehabil 2011; 18: 48–54.Google Scholar
14. Cohen, MI, Bridges, ND, Gaynor, JW, et al. Modifications to the cavopulmonary anastomosis do not eliminate early sinus node dysfunction. J Thorac Cardiovasc Surg 2000; 120: 891–900.Google Scholar
15. Mason, JW, Ramseth, DJ, Chanter, DO, Moon, TE, Goodman, DB, Mendzelevski, B. Electrocardiographic reference ranges derived from 79,743 ambulatory subjects. J Electrocardiol 2007; 40: 228–234.CrossRefGoogle ScholarPubMed
16. Rijnbeek, PR, Witsenburg, M, Schrama, E, Hess, J, Kors, JA. New normal limits for the paediatric electrocardiogram. Eur Heart J 2001; 22: 702–711.Google Scholar
17. Salameh, A, Gebauer, RA, Grollmuss, O, Vit, P, Reich, O, Janousek, J. Normal limits for heart rate as established using 24-hour ambulatory electrocardiography in children and adolescents. Cardiol Young 2008; 18: 467–472.Google Scholar
18. Epstein, AE, DiMarco, JP, Ellenbogen, KA, et al. ACC/AHA/HRS 2008 Guidelines for device-based Therapy of Cardiac Rhythm Abnormalities: a report of the American College of Cardiology/American Heart Association Task Force on practice guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices): developed in collaboration with the American Association for Thoracic Surgery and Society of Thoracic Surgeons. Circulation 2008; 117: e350–e408.Google Scholar
19. Deal, BJ. Late arrhythmias following Fontan surgery. World J Pediatr Congenit Heart Surg 2012; 3: 194–200.Google Scholar
20. Cohen, MI, Wernovsky, G, Vetter, VL, et al. Sinus node function after a systematically staged Fontan procedure. Circulation 1998; 98: II352–II358; discussion II358–II359.Google ScholarPubMed
21. Dilawar, M, Bradley, SM, Saul, JP, Stroud, MR, Balaji, S. Sinus node dysfunction after intraatrial lateral tunnel and extracardiac conduit Fontan procedures. Pediatr Cardiol 2003; 24: 284–288.CrossRefGoogle ScholarPubMed
22. Vogels, T, Bruil, J, Koopman, H, Fekkes, M, Verrips, GHW. TACQOL CF 12-15 Manual. Developed by Leiden Center for Child Health and Pediatrics LUMC-TNO 2004.Google Scholar
23. Verrips, GH, Vogels, AG, den Ouden, AL, Paneth, N, Verloove-Vanhorick, SP. Measuring health-related quality of life in adolescents: agreement between raters and between methods of administration. Child Care Health Dev 2000; 26: 457–469.CrossRefGoogle ScholarPubMed
24. Khairy, P, Fernandes, SM, Mayer, JE Jr., et al. Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation 2008; 117: 85–92.Google Scholar
25. McCrindle, BW, Zak, V, Sleeper, LA, et al. Laboratory measures of exercise capacity and ventricular characteristics and function are weakly associated with functional health status after Fontan procedure. Circulation 2010; 121: 34–42.CrossRefGoogle ScholarPubMed
26. Appelhans, BM, Luecken, LJ. Heart rate variability as an index of regulated emotional responding. Rev General Psychol 2006; 10: 229.Google Scholar
27. van den Bosch, AE, Roos-Hesselink, JW, Van Domburg, R, Bogers, AJ, Simoons, ML, Meijboom, FJ. Long-term outcome and quality of life in adult patients after the Fontan operation. Am J Cardiol 2004; 93: 1141–1145.Google Scholar
28. Khairy, P, Poirier, N, Mercier, LA. Univentricular heart. Circulation 2007; 115: 800–812.Google Scholar
29. McCrindle, BW, Williams, RV, Mitchell, PD, et al. Relationship of patient and medical characteristics to health status in children and adolescents after the Fontan procedure. Circulation 2006; 113: 1123–1129.Google Scholar
30. Moons, P, Van Deyk, K, Budts, W, De Geest, S. Caliber of quality-of-life assessments in congenital heart disease: a plea for more conceptual and methodological rigor. Arch Pediatr Adolesc Med 2004; 158: 1062–1069.Google Scholar
31. McCrindle, BW, Williams, RV, Mital, S, et al. Physical activity levels in children and adolescents are reduced after the Fontan procedure, independent of exercise capacity, and are associated with lower perceived general health. Arch Dis Child 2007; 92: 509–514.Google Scholar
32. Snookes, SH, Gunn, JK, Eldridge, BJ, et al. A systematic review of motor and cognitive outcomes after early surgery for congenital heart disease. Pediatrics 2010; 125: e818–e827.Google Scholar
33. 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: 1143–1172.Google Scholar
34. Saiki, H, Kurishima, C, Masutani, S, Senzaki, H. Cerebral circulation in patients with Fontan circulation: assessment by carotid arterial wave intensity and stiffness. Ann Thorac Surg 2014; 97: 1394–1399.Google Scholar
35. Blaufox, AD, Sleeper, LA, Bradley, DJ, et al. Functional status, heart rate, and rhythm abnormalities in 521 Fontan patients 6 to 18 years of age. J Thorac Cardiovasc Surg 2008; 136: 100–107; 107 e101.Google Scholar
36. Sarajuuri, A, Jokinen, E, Mildh, L, et al. Neurodevelopmental burden at age 5 years in patients with univentricular heart. Pediatrics 2012; 130: e1636–e1646.Google Scholar
37. Sugimoto, A, Ota, N, Ibuki, K, et al. Risk factors for adverse neurocognitive outcomes in school-aged patients after the Fontan operation. Eur J Cardiothorac Surg 2013; 44: 454–461.Google Scholar
38. Goldberg, CS, Schwartz, EM, Brunberg, JA, et al. Neurodevelopmental outcome of patients after the Fontan operation: a comparison between children with hypoplastic left heart syndrome and other functional single ventricle lesions. J Pediatr 2000; 137: 646–652.Google Scholar
39. Blaufox, AD, Sleeper, LA, Bradley, DJ, et al. Functional status, heart rate, and rhythm abnormalities in 521 Fontan patients 6 to 18 years of age. J Thorac Cardiovasc Surg 2008; 136: 100–107; e101.CrossRefGoogle ScholarPubMed
40. Williams, IA, Sleeper, LA, Colan, SD, et al. Functional state following the Fontan procedure. Cardiol Young 2009; 19: 320–330.Google Scholar
41. Jenkins, PC, Chinnock, RE, Jenkins, KJ, et al. Decreased exercise performance with age in children with hypoplastic left heart syndrome. J Pediatr 2008; 152: 507–512.CrossRefGoogle ScholarPubMed
42. Hager, A, Hess, J. Comparison of health related quality of life with cardiopulmonary exercise testing in adolescents and adults with congenital heart disease. Heart 2005; 91: 517–520.Google Scholar
Supplementary material: File

Dulfer supplementary material

Dulfer supplementary material 1

Download Dulfer supplementary material(File)
File 70.9 KB