Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Review Article
  • Published:

The Fontan circulation: a challenge to William Harvey?

Nature Clinical Practice Cardiovascular Medicine volume 2, pages 202–208 (2005)Cite this article

Abstract

The Fontan operation, which places the systemic and pulmonary circulations in series and is driven by a single ventricular chamber, is the treatment of choice for patients born with one ventricle. Its introduction 35 years ago was the result of a flurry of experimental and clinical research that had started in the 1940s. A large number of children have benefited and continue to benefit from the Fontan operation, but there is a genuine concern that, despite the refinement of the surgical procedures in the past 20 years, continuing attrition might be inevitable. This adverse effect can lead to a decline in functional capacity, and premature late death.

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Figure 1: The different types of Fontan circulation.
Figure 2: Schematic model of the Fontan circulation.
Figure 3: Path lines of intracardiac and extracardiac total cavopulmonary connection.
Figure 4: The circulation of the lung.

Similar content being viewed by others

References

  1. Harvey W (Translated by Keynes G, 1978) Exercitatio Anatomica de Motu Cordis et Sanguinus in Animalibus. Birmingham, AL: LB Adams, The Classics of Medicine Library

  2. Rodbard S and Wagner D (1949) Bypassing the right ventricle. Proc Soc Exp Biol Med 71: 69–70

    Article  CAS  Google Scholar 

  3. Robicsek F (1982) An epitaph for cavopulmonary anastomosis. Ann Thorac Surg 34: 208–220

    Article  CAS  Google Scholar 

  4. Sade RM and Castaneda AR (1975) The dispensable right ventricle. Surgery 77: 624–631

    CAS  PubMed  Google Scholar 

  5. Nuland SB et al. (1958) Circulatory bypass of the right heart. III. Some observations on long-term survivors. Surgery 43: 184–201

    CAS  PubMed  Google Scholar 

  6. Haller JA Jr et al. (1966) Experimental studies on permanent bypass of the right heart. Surgery 59: 1128–1132

    PubMed  Google Scholar 

  7. Fontan F et al. (1971) “Correction” of tricuspid atresia: 2 cases “corrected” using a new surgical technique. Ann Chir Thorac Cardiovasc 10: 39–47

    CAS  PubMed  Google Scholar 

  8. Kreutzer G et al. (1973) An operation for the correction of tricuspid atresia. J Thorac Cardiovasc Surg 66: 613–621

    CAS  PubMed  Google Scholar 

  9. Puga FJ et al. (1987) Modifications of the Fontan operation applicable to patients with left atrioventricular valve atresia or single atrioventricular valve. Circulation 76: III53–60

    CAS  PubMed  Google Scholar 

  10. Jonas RA and Castaneda AR (1988) Modified Fontan procedure: atrial baffle and systemic venous to pulmonary artery anastomotic techniques. J Card Surg 3: 91–96

    Article  CAS  Google Scholar 

  11. de Leval MR et al. (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: 682–695

    CAS  PubMed  Google Scholar 

  12. Stewart JM et al. (1987) Elevated atrial natriuretic peptide after the Fontan procedure. Circulation 76: III77–82

    Article  CAS  Google Scholar 

  13. Marcelletti C et al. (1990) Inferior vena cava–pulmonary artery extracardiac conduit. A new form of right heart bypass. J Thorac Cardiovasc Surg 100: 228–232

    CAS  PubMed  Google Scholar 

  14. de Leval MR et al. (1996) Use of computational fluid dynamics in the design of surgical procedures. Application to the study of competitive flows in cavo-pulmonary connections. J Thorac Cardiovasc Surg 111: 502–513

    Article  CAS  Google Scholar 

  15. Bove EL et al. (2003) Computational fluid dynamics in the evaluation of hemodynamic performance of cavopulmonary connections after the Norwood procedure for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 126: 1040–1047

    Article  Google Scholar 

  16. Fontan F et al. (1990) Outcome after a “perfect” Fontan operation. Circulation 81: 1520–1536

    Article  CAS  Google Scholar 

  17. Cheung YF et al. (2000) Serial assessment of left ventricular diastolic function after Fontan procedure. Heart 83: 420–424

    Article  CAS  Google Scholar 

  18. Nogaki M et al. (2000) Ventricular energetics in Fontan circulation: evaluation with a theoretical model. Pediatr Int 42: 651–657

    Article  CAS  Google Scholar 

  19. Senzaki H et al. (2002) Ventricular afterload and ventricular work in Fontan circulation: Comparison with normal two-ventricle circulation and single-ventricle circulation with Blalock-Taussig shunts. Circulation 105: 2885–2892

    Article  Google Scholar 

  20. Szabo G et al. (2003) Ventricular energetics after the Fontan operation: contractility-afterload mismatch. J Thorac Cardiovasc Surg 125: 1061–1069

    Article  Google Scholar 

  21. Kussmaul WG et al. (1992) Right ventricular-pulmonary arterial interactions. Ann Biomed Eng 20: 63–80

    Article  CAS  Google Scholar 

  22. Milnor NR (1972) Pulmonary hemodynamics. In: Cardiovascular fluid-dynamics, Vol 2, 299–340 (Ed Bergel DH) London: Academic Press

    Google Scholar 

  23. West JB et al. (1964) Distribution of blood flow in isolated lung; relation to vascular and alveolar pressures. J Appl Physiol 19: 713–724

    Article  CAS  Google Scholar 

  24. Hsia TY et al. (2000) Effects of respiration and gravity on infra-diaphragmatic venous flow in normal and Fontan patients. Circulation 102: III148–153

    CAS  PubMed  Google Scholar 

  25. Hsia TY et al. (2001) Subdiaphragmatic venous hemodynamics in the Fontan circulation. J Thorac Cardiovasc Surg 121: 436–447

    Article  CAS  Google Scholar 

  26. Feldt RH et al. (1996) Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg 112: 672–680

    Article  CAS  Google Scholar 

  27. Gentles TL et al. (1997) Functional outcome after the Fontan operation: factors influencing late morbidity. J Thorac Cardiovasc Surg 114: 392–403

    Article  CAS  Google Scholar 

  28. Hug MI et al. (2001) Chylous bronchial casts after Fontan operation. Circulation 103: 1031–1033

    Article  CAS  Google Scholar 

  29. Heimburg P (1964) Bronchial collateral circulation in experimental stenosis of the pulmonary artery. Thorax 19: 306–310

    Article  CAS  Google Scholar 

  30. Triedman JK et al. (1993) Prevalence and risk factors for aortopulmonary collateral vessels after Fontan and bidirectional Glenn procedures. J Am Coll Cardiol 22: 207–215

    Article  CAS  Google Scholar 

  31. Srivastava D et al. (1995) Hepatic venous blood and the development of pulmonary arteriovenous malformations in congenital heart disease. Circulation 92: 1217–1222

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Professor of Cardiothoracic Surgery, Great Ormond Street Hospital for Children NHS Trust, London, UK

    Marc R de Leval

Authors
  1. Marc R de Leval
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Marc R de Leval.

Ethics declarations

Competing interests

The author declares no competing financial interests.

Rights and permissions

Reprints and permissions

About this article

Cite this article

de Leval, M. The Fontan circulation: a challenge to William Harvey?. Nat Rev Cardiol 2, 202–208 (2005). https://doi.org/10.1038/ncpcardio0157

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1038/ncpcardio0157

This article is cited by

Search

Advanced search

Quick links

Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing