Skip to main content

Advertisement

Log in

Clinical Profile and Quality of Life of Adult Patients After the Fontan Procedure

  • Original Article
  • Published:
Pediatric Cardiology Aims and scope Submit manuscript

Abstract

Increasingly, more patients with univentricular heart reach adulthood. Therefore, long-term psychological features are an important concern. The aim of this study was to evaluate the clinical and psychological profile of post-Fontan adult patients and to identify the most significant determinants of quality of life. In this retrospective cross-sectional study, we reviewed the surgical and medical history of post-Fontan adult patients. Patients underwent a 24-h electrocardiogram, echocardiography and exercise testing. Self-report questionnaires were used to assess the Work Ability Index, quality of life (Satisfaction with Life Scale), perceived health status (SF-36 questionnaire), coping strategies (Brief Cope questionnaire) and presence of mood disorders (Hospital Anxiety and Depression Scale). Thirty-nine patients aged between 18 and 48 years (mean 27.5 years) were enrolled. The mean follow-up was 21.5 years. Most patients were unmarried (82.9 %), had a high school diploma (62.9 %) and were employed (62.9 %). Twenty-nine patients (82.3 %) had at least one long-term complication. The median single ventricle ejection fraction was 57 %, and the median maximal oxygen consumption was 26.8 ml/min/kg. This population tended to be anxious and to use adaptive coping strategies. Quality of life was perceived as excellent or good in 57.2 % of cases and was not related to either cardiac function or exercise capacity. Both quality of life and SF-36 domains were related to the Work Ability Index. This cohort of post-Fontan adult patients enjoyed a good quality of life irrespective of disease severity.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. American College of Sports Medicine (2000) ACSM’s guidelines for exercise testing and prescription, sixth edition. 77, tables 4 and 5. ed. Philadelphia: Lippincott Williams &Wilkins

  2. Bridges ND, Lock JE, Castaneda AR (1990) Baffle fenestration with subsequent transcatheter closure. Modification of the Fontan operation for patients at increased risk. Circulation 82(5):1681–1689

    Article  CAS  PubMed  Google Scholar 

  3. Carver CS (1997) You want to measure coping but your protocol’s too long: consider the brief COPE. Int J Behav Med 4(1):92–100

    Article  CAS  PubMed  Google Scholar 

  4. De Leval MR, Kilner P, Gewillig M, Bull C (1988) Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience. J Thorac Cardiovasc Surg 96(5):682–695

    PubMed  Google Scholar 

  5. Deanfield J, Thaulow E, Warnes C, Webb G, Kolbel F, Hoffman A et al (2003) Management of grown up congenital heart disease. Eur Heart J 24(11):1035–1084

    Article  PubMed  Google Scholar 

  6. Diener E, Emmons RA, Larsen RJ, Griffin S (1985) The Satisfaction with Life Scale. J Pers Assess 49(1):71–75

    Article  CAS  PubMed  Google Scholar 

  7. d’Udekem Y, Cheung MM, Setyapranata S, Iyengar AJ, Kelly P, Buckland N et al (2009) How good is a good Fontan? quality of life and exercise capacity of Fontans without arrhythmias. Ann Thorac Surg 88(6):1961–1969

    Article  PubMed  Google Scholar 

  8. Gewillig M (2005) The Fontan circulation. Heart 91(6):839–846

    Article  PubMed Central  PubMed  Google Scholar 

  9. Hager A, Hess J (2005) Comparison of health related quality of life with cardiopulmonary exercise testing in adolescents and adults with congenital heart disease. Heart 91(4):517–520

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  10. Harrison DA, Liu P, Walters JE, Goodman JM, Siu SC, Webb GD et al (1995) Cardiopulmonary function in adult patients late after Fontan repair. J Am Coll Cardiol 26(4):1016–1021

    Article  CAS  PubMed  Google Scholar 

  11. Idorn L, Juul K, Jensen AS, Hanel B, Nielsen KG, Andersen H et al (2013) Arrhythmia and exercise intolerance in Fontan patients: current status and future burden. Int J Cardiol

  12. Ilmarinen J (2007) The work ability index (WAI). Occup Med (Oxford, England) 57:160

    Article  Google Scholar 

  13. Italia in cifre, 1st ed (2012). http://www.istat.it. p 15–20

  14. Kamphuis M, Ottenkamp J, Vliegen HW, Vogels T, Zwinderman KH, Kamphuis RP et al (2002) Health related quality of life and health status in adult survivors with previously operated complex congenital heart disease. Heart 87(4):356–362

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  15. Kovacs AH, Saidi AS, Kuhl EA, Sears SF, Silversides C, Harrison JL et al (2009) Depression and anxiety in adult congenital heart disease: predictors and prevalence. Int J Cardiol 137(2):158–164

    Article  PubMed  Google Scholar 

  16. Loup O, von Weissenfluh C, Gahl B, Schwerzmann M, Carrel T, Kadner A (2009) Quality of life of grown-up congenital heart disease patients after congenital cardiac surgery. Eur J Cardiothorac Surg 36(1):105–111

    Article  PubMed  Google Scholar 

  17. Marcelletti C, Corno A, Giannico S, Marino B (1990) Inferior vena cava-pulmonary artery extracardiac conduit. A new form of right heart bypass. J Thorac Cardiovasc Surg 100(2):228–232

    CAS  PubMed  Google Scholar 

  18. McHorney CA, Ware JE Jr, Raczek AE (1993) The MOS 36-item short-form health survey (SF-36): II. Psychometric and clinical tests of validity in measuring physical and mental health constructs. Med Care 31(3):247–263

    Article  CAS  PubMed  Google Scholar 

  19. Milanesi O, Stellin G, Colan SD, Facchin P, Crepaz R, Biffanti R et al (2002) Systolic and diastolic performance late after the Fontan procedure for a single ventricle and comparison of those undergoing operation at 12 months of age. Am J Cardiol 89(3):276–280

    Article  PubMed  Google Scholar 

  20. Moons P, Van Deyk K, De Geest S, Gewillig M, Budts W (2005) Is the severity of congenital heart disease associated with the quality of life and perceived health of adult patients? Heart 91(9):1193–1198

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  21. Moons P, Bovijn L, Budts W, Belmans A, Gewillig M (2010) Temporal trends in survival to adulthood among patients born with congenital heart disease from 1970 to 1992 in belgium. Circulation 122(22):2264–2272

    Article  PubMed  Google Scholar 

  22. Overgaard D, Schrader AM, Lisby KH, King C, Christensen RF, Jensen HF et al (2011) Patient-reported outcomes in adult survivors with single-ventricle physiology. Cardiology 120(1):36–42

    Article  PubMed  Google Scholar 

  23. Pike NA, Evangelista LS, Doering LV, Eastwood JA, Lewis AB, Child JS (2012) Quality of life, Health status, and depression: comparison between adolescents and adults after the Fontan procedure with healthy counterparts. J Cardiovasc Nurs 27(6):539–546

    Article  PubMed Central  PubMed  Google Scholar 

  24. Saliba Z, Butera G, Bonnet D, Bonhoeffer P, Villain E, Kachaner J et al (2001) Quality of life and perceived health status in surviving adults with univentricular heart. Heart 86(1):69–73

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  25. Spijkerboer AW, De Koning WB, Duivenvoorden HJ, Bogers AJ, Verhulst FC, Helbing WA et al (2010) Medical predictors for long-term behavioral and emotional outcomes in children and adolescents after invasive treatment of congenital heart disease. J Pediatr Surg 45(11):2146–2153

    Article  PubMed  Google Scholar 

  26. Zigmond AS, Snaith RP (1983) The Hospital Anxiety and Depression Scale. Acta Psychiatr Scand 67(6):361–370

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgments

We thank Jean Ann Gilder (Scientific Communication srl., Naples, Italy) for substantive editing of the text.

Conflict of interest

The authors have no conflicts of interest to declare.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Giulia Bordin.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Bordin, G., Padalino, M.A., Perentaler, S. et al. Clinical Profile and Quality of Life of Adult Patients After the Fontan Procedure. Pediatr Cardiol 36, 1261–1269 (2015). https://doi.org/10.1007/s00246-015-1156-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-015-1156-y

Keywords

Navigation