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.

  • Viewpoint
  • Published:

The Fontan operation in clinical practice: indications and controversies

Nature Clinical Practice Cardiovascular Medicine volume 2, pages 116–117 (2005)Cite this article

In 1968, Fontan and his colleagues successfully separated the pulmonary and systemic blood flow in a patient with tricuspid atresia by placing the circulations in series, with the single ventricle connected to the systemic circulation.1 The Fontan principle has since become well established for the management of single-ventricle circulations; however, many questions remain about the selection criteria for the procedure, the surgical technique and management of the Fontan state, all of which continue to evolve. This viewpoint will highlight some of these issues.

Potential candidates for the Fontan operation can have a large variety of complex congenital cardiac defects, but only one well-developed ventricle. This increasingly includes patients with hypoplastic left-heart syndrome who, with recent improvements in the results of the Norwood operation, now undergo the Fontan procedure as the last step of a staged palliation. In patients with moderate hypoplasia of one ventricle, the decision between biventricular repair and a single-ventricle strategy can be difficult. This group of patients includes those with an atrioventricular septal defect and moderate hypoplasia of one ventricular chamber, and those with pulmonary atresia and a moderately small right ventricle. Some patients with two adequate ventricles and complex intracardiac defects could also be considered for a single-ventricle strategy if biventricular repair is technically not possible or can only be achieved at high risk to the patient, such as those with major straddling of the atrioventricular valves and some forms of double outlet ventricle.

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

References

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

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Sano S et al. (2003) Right ventricle–pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 126: 504–510

    Article  PubMed  Google Scholar 

  3. Penny DJ et al. (1992) The early response of the systemic ventricle during transition to the Fontan circulation–an acute hypertrophic cardiomyopathy? Cardiol Young 66: 456–459

    Google Scholar 

  4. 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  PubMed  Google Scholar 

  5. Bridges ND et al. (1990) Bidirectional cavopulmonary anastomosis as interim palliation for high-risk Fontan candidates. Early results. Circulation 82 (Suppl 5): 170–176

    Google Scholar 

  6. Gatzoulis MA et al. (2000). Definitive palliation with cavopulmonary or aortopulmonary shunts for adults with single ventricle physiology. Heart 83: 51–57

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. 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 

  8. Azakie A et al. (2001) Extracardiac conduit versus lateral tunnel cavopulmonary connections at a single institution: impact on outcomes. J Thorac Cardiovasc Surg 122: 1219–1228

    Article  CAS  PubMed  Google Scholar 

  9. Bridges ND et al. (1990) Baffle fenestration with subsequent transcatheter closure. Modification of the Fontan operation for patients at increased risk. Circulation 82: 1681–1689

    Article  CAS  PubMed  Google Scholar 

  10. Mavroudis C et al. (1998) Fontan conversion to cavopulmonary connection and arrhythmia circuit cryoblation. J Thorac Cardiovasc Surg 115: 547–556

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. University College London,

    Carin A van Doorn

  2. Great Ormond Street Hospital for Children and the Heart Hospital, London, UK

    Carin A van Doorn

  3. University of London,

    Marc R de Leval

  4. Great Ormond Street Hospital, London, UK

    Marc R de Leval

Authors
  1. Carin A van Doorn
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. 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 authors declare no competing financial interests.

Rights and permissions

Reprints and permissions

About this article

Cite this article

van Doorn, C., de Leval, M. The Fontan operation in clinical practice: indications and controversies. Nat Rev Cardiol 2, 116–117 (2005). https://doi.org/10.1038/ncpcardio0133

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1038/ncpcardio0133

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