Skip to main content

Advertisement

Log in

Long-Term Results of the Fontan Operation

Pediatric Cardiology Aims and scope Submit manuscript

Abstract

Modifications of the Fontan operation can afford excellent palliation for many patients with a variety of forms of single ventricle. In properly selected patients who are good candidates, early and late survival can be as high as 95–97%. However, mortality is considerably higher for patients with risk factors for poor outcome. For high-risk patients, inclusion of a fenestration may reduce morbidity and mortality. Important long-term undesirable events include the ongoing risk of death, arrhythmias, protein-losing enteropathy, and cardiac failure. The key to excellent long-term outcome is proper selection of patients for the operation.

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

Fig. 1

Similar content being viewed by others

References

  1. Bridges N, Lock J, Castaneda A (1990) Baffle fenestration with subsequent transcatheter closure: modification of the Fontan operation for patients at increased risk. Circulation 82:1681–1689

    PubMed  CAS  Google Scholar 

  2. Canobbio M, Mair D, Van Der Velde M, Koos B (1996) Pregnancy outcomes after the Fontan repair. J Am Coll Cardiol 28:763–767

    Article  PubMed  CAS  Google Scholar 

  3. Earing MG, Cetta F, Driscoll DJ, Mair DD, Hodge DO, Dearani JA, Puga FJ, Danielson GK, O'Leary PW (2005) Long-term results of the Fontan operation for double-inlet left ventricle. Am J Cardiol 96(2):291–298

    Article  PubMed  Google Scholar 

  4. Choussat A (1978) Selection criteria for Fontan’s procedure. In: Anderson RH, Shinebourne EA (eds) Paediatric Cardiology 1977. Churchill Livingstone, Edinburgh, UK, pp 559–566

    Google Scholar 

  5. Driscoll DJ (1986) Exercise tolerance and cardiorespiratory response to exercise after the Fontan operation for tricuspid atresia or functional single ventricle. J Am Coll Cardiol 7:1087-1094

    Article  PubMed  CAS  Google Scholar 

  6. Driscoll DJ, Offord K, Feldt R, et al. (1992) 5–15 Year follow-up after Fontan operation. Circulation 85:469–496

    PubMed  CAS  Google Scholar 

  7. Driscoll DJ, Staats B, Heise C, et al. (1984) Functional single ventricle: cardiorespiratory response to exercise. J Am Coll Cardiol 4:337–342

    Article  PubMed  CAS  Google Scholar 

  8. Durongpisitkul K, Driscoll D, Mottram C, et al. (1997) Cardiopulmonary responses to exercise after the modified Fontan operation: a study of determinants of performance. J Am Coll Cardiol 29:785–790

    Article  PubMed  CAS  Google Scholar 

  9. Durongpisitkul K, Porter C, Cetta F, et al. (1998) Predictors of early- and late-onset supraventricular tachyarrhythmias after the Fontan operation. Circulation 98:1099–1107

    PubMed  CAS  Google Scholar 

  10. Farrell P, Chang A, Murdison K, et al. (1992) Outcome and assessment after the modified Fontan procedure for hypoplastic left heart syndrome. Circulation 1992:85

    Google Scholar 

  11. Feldt R, Driscoll D, Offord K, et al. (1996) Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg 112:672–680

    Article  PubMed  CAS  Google Scholar 

  12. Fontan F, Baudet E (1971) Surgical repair of tricuspid atresia. Thorax 26:240–248

    PubMed  CAS  Google Scholar 

  13. Fontan F, Kirklin J, Fernandez G, et al. (1990) Outcome after a “perfect” Fontan operation. Circulation 81:1520–1536

    PubMed  CAS  Google Scholar 

  14. Fredriksen P, Therrien J, Veldtman G, et al. (2001) Lung function and aerobic capacity in adult patients following modified Fontan procedure. Heart 85:295–299

    Article  PubMed  CAS  Google Scholar 

  15. Gaynor W, Bridges N, Choen M, et al. (2002) Predictors of outcome after the Fontan operation: is hypoplastic left heart syndrome still a factor? J Thorac Cardiovasc Surg 123:237–245

    Article  PubMed  Google Scholar 

  16. Gelatt M, Hamilton R, McCrindle W, et al. (1994) Risk factors for atrial tachyarrhythmias after the Fontan operation. J Am Coll Cardiol 24:1735–1741

    Article  PubMed  CAS  Google Scholar 

  17. Gildein P, Mocellin R, Kaufmehl K (1994) Oxygen uptake transient kinetics during constant-load exercise in children after operations of ventricular septal defect, tetralogy of Fallot, transposition of the great arteries, or tricuspid valve atresia. Am J Cardiol 74:166–169

    Article  PubMed  CAS  Google Scholar 

  18. Grant G, Mansell A, Garofano R, et al. (1988) Cardiorespiratory response to exercise after the Fontan procedure for tricuspid atresia. Pediatr Res 24:1–5

    Article  PubMed  CAS  Google Scholar 

  19. Harrison D, Liu P, Walters J, et al. (1995) Cardiopulmonary function in adult patients late after Fontan repair. J Am Coll Cardiol 26:1016–1021

    Article  PubMed  CAS  Google Scholar 

  20. Hsu D, Quaegebeur J, Ing F, et al. (1997) Outcome after the single stage, non-fenestrated Fontan operation. Circulation 96:II-335–II-340

    PubMed  CAS  Google Scholar 

  21. Julsrud P, Wiegel TJ, Edwards W, et al. (2000) The influence of ventricular morphology on outcome after the Fontan procedure. Am J Cardiol 86:319–323

    Article  PubMed  CAS  Google Scholar 

  22. Mahle W, Clancy R, Moss E, et al. (2000) Neurodevelopmental outcome and life style assessment in school-aged and adolescent children with hypoplastic left heart syndrome. Pediatrics 105:1082–1089

    Article  PubMed  CAS  Google Scholar 

  23. Mair D, Puga F, Danielson G (2001) The Fontan procedure for tricuspid atresia: early and late results of a 25-year experience with 216 patients. J Am Coll Cardiol 37:933–939

    Article  PubMed  CAS  Google Scholar 

  24. Minamisawa S, Nakazawa M, Momma K, Imai Y, Saatomi G (2001) Effect of aerobic training on exercise performance in patients after the Fontan operation. Am J Cardiol 88:695–698

    Article  PubMed  CAS  Google Scholar 

  25. Monagle P, Cochrane A, McCrindle B, et al. (1998) Thromboembolic complications following Fontan procedures: a critical review of the literature. J Thorac Cardiovasc Surg 115:493–498

    Article  PubMed  CAS  Google Scholar 

  26. Nir A, Driscoll D, Mottram C, et al. (1993) Cardiorespiratory response to exercise after the Fontan operation; a serial study. J Am Coll Cardiol 22:216–220

    Article  PubMed  CAS  Google Scholar 

  27. Ohuchi H, Arakaki Y, Hiraumi Y, Tasato H, Kamiya T (1998) Cardiorespiratory response during exercise in patients with cyanotic congenital heart disease with and without a Fontan operation and in patients with congestive heart failure. Int J Cardiol 66:241–251

    Article  PubMed  CAS  Google Scholar 

  28. Ohuchi H, Yasuda K, Hasegawa S, et al. (2001) Influence of ventricular morphology on aerobic exercise capacity in patients after the Fontan operation. J Am Coll Cardiol 37:1967–1674

    Article  PubMed  CAS  Google Scholar 

  29. Rhode J, Garofano R, Bowman F, et al. (1990) Effect of right ventricular anatomy on the cardiopulmonary response to exercise. Circulation 81:1811–1817

    Google Scholar 

  30. Stamm C, Friehs I, Duebener L, et al. (2002) Improving results of the modified Fontan operation in patients with heterotaxy syndrome. Ann Thorac Surg 74:1967–1978

    Article  PubMed  Google Scholar 

  31. Stamm C, Friehs I, Mayer J, et al. (2001) Long-term results of the lateral tunnel Fontan operation. J Thorac Cardiovasc Surg 121:28–41

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to D. J. Driscoll.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Driscoll, D.J. Long-Term Results of the Fontan Operation. Pediatr Cardiol 28, 438–442 (2007). https://doi.org/10.1007/s00246-007-9003-4

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-007-9003-4

Keywords

Navigation