Skip to main content
SpringerLink
Log in
Book cover

Right Ventricular Physiology, Adaptation and Failure in Congenital and Acquired Heart Disease pp 185–192Cite as

Single Right Ventricular Function and Failure in the Fontan Circulation

  • Chapter
  • First Online:

  • 1134 Accesses

Abstract

The Fontan procedure generates a unique human circulation, relying on a functionally single ventricle to pump blood around the systemic and pulmonary circulations, in series. If ever the frailty of a particular ventricular morphology were to be exposed, one might imagine that it would be the case for the Fontan circulation. In this chapter we will review the evidence for and against the right ventricle being more susceptible when acting as a functionally singular ventricle.

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

References

  1. Hansen JH, Petko C, Bauer G, Voges I, Kramer HH, Scheewe J. Fifteen-year single-center experience with the Norwood operation for complex lesions with single-ventricle physiology compared with hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2012;144(1):166–72.

    Article  PubMed  Google Scholar 

  2. Gentles TL, Mayer JE Jr, Gauvreau K, Newburger JW, Lock JE, Kupferschmid JP, Burnett J, Jonas RA, Castañeda AR, Wernovsky G. Fontan operation in five hundred consecutive patients: factors influencing early and late outcome. J Thorac Cardiovasc Surg. 1997;114(3):376–91.

    Article  CAS  PubMed  Google Scholar 

  3. Gaynor JW, Bridges ND, Cohen MI, Mahle WT, Decampli WM, Steven JM, Nicolson SC, Spray TL. Predictors of outcome after the Fontan operation: is hypoplastic left heart syndrome still a risk factor? J Thorac Cardiovasc Surg. 2002;123(2):237–45.

    Article  PubMed  Google Scholar 

  4. Ono M, Kasnar-Samprec J, Hager A, Cleuziou J, Burri M, Langenbach C, Callegari A, Strbad M, Vogt M, Hörer J, Schreiber C, Lange R. Clinical outcome following total cavopulmonary connection: a 20-year single-centre experience. Eur J Cardiothorac Surg. 2016;50(4):632–41.

    Article  PubMed  Google Scholar 

  5. Becker Rencoret P, Besa Bandeira S, Riveros González S, Frangini Sanhueza P, Springmüller Pinto D, González Foretic R, Urcelay Montecinos G. Comparative results of Fontan surgery in patients with and without hypoplastic left heart syndrome. Rev Chil Pediatr. 2016;87(5):366–72.

    Article  PubMed  Google Scholar 

  6. Pundi KN, Johnson JN, Dearani JA, Pundi KN, Li Z, Hinck CA, Dahl SH, Cannon BC, O’Leary PW, Driscoll DJ, Cetta F. 40-year follow-up after the Fontan operation: long-term outcomes of 1,052 patients. J Am Coll Cardiol. 2015;66(15):1700–10.

    Article  PubMed  Google Scholar 

  7. d’Udekem Y, Iyengar AJ, Galati JC, Forsdick V, Weintraub RG, Wheaton GR, Bullock A, Justo RN, Grigg LE, Sholler GF, Hope S, Radford DJ, Gentles TL, Celermajer DS, Winlaw DS. Redefining expectations of long-term survival after the Fontan procedure: twenty-five years of follow-up from the entire population of Australia and New Zealand. Circulation. 2014;130(11 Suppl 1):S32–8.

    Article  PubMed  Google Scholar 

  8. Tanoue Y, Sese A, Ueno Y, Joh K, Hijii T. Bidirectional Glenn procedure improves the mechanical efficiency of a total cavopulmonary connection in high-risk Fontan candidates. Circulation. 2001;103(17):2176–80.

    Article  CAS  PubMed  Google Scholar 

  9. Akagi T, Benson LN, Gilday DL, Ash J, Green M, Williams WG, Freedom RM. Influence of ventricular morphology on diastolic filling performance in double-inlet ventricle after the Fontan procedure. J Am Coll Cardiol. 1993;22(7):1948–52.

    Article  CAS  PubMed  Google Scholar 

  10. Myers KA, Leung MT, Terri Potts M, Potts JE, Sandor GG. Noninvasive assessment of vascular function and hydraulic power and efficiency in pediatric Fontan patients. J Am Soc Echocardiogr. 2013;26(10):1221–7.

    Article  PubMed  Google Scholar 

  11. Cheung MM, Smallhorn JF, McCrindle BW, Van Arsdell GS, Redington AN. Non-invasive assessment of ventricular force-frequency relations in the univentricular circulation by tissue Doppler echocardiography: a novel method of assessing myocardial performance in congenital heart disease. Heart. 2005;91(10):1338–42.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. Senzaki H, Masutani S, Kobayashi J, Kobayashi T, Sasaki N, Asano H, Kyo S, Yokote Y, Ishizawa A. Ventricular afterload and ventricular work in fontan circulation: comparison with normal two-ventricle circulation and single-ventricle circulation with blalock-taussig shunts. Circulation. 2002;105(24):2885–92.

    Article  PubMed  Google Scholar 

  13. Kouatli AA, Garcia JA, Zellers TM, Weinstein EM, Mahony L. Enalapril does not enhance exercise capacity in patients after Fontan procedure. Circulation. 1997;96(5):1507–12.

    Article  CAS  PubMed  Google Scholar 

  14. Penny DJ, Lincoln C, Shore DF, Rigby ML, Redington AN. the early response of the systemic ventricle during transition to the Fontan circulation: an acute hypertrophic cardiomyopathy? Cardiol Young. 1992;2:78–84.

    Google Scholar 

  15. Rychik J, Jacobs ML, Norwood WI Jr. Acute changes in left ventricular geometry after volume reduction operation. Ann Thorac Surg. 1995;60(5):1267–73.

    Article  CAS  PubMed  Google Scholar 

  16. Fogel MA, Weinberg PM, Chin AJ, Fellows KE, Hoffman EA. Late ventricular geometry and performance changes of functional single ventricle throughout staged Fontan reconstruction assessed by magnetic resonance imaging. J Am Coll Cardiol. 1996;28(1):212–21.

    Article  CAS  PubMed  Google Scholar 

  17. Rathod RH, Prakash A, Kim YY, Germanakis IE, Powell AJ, Gauvreau K, Geva T. Cardiac magnetic resonance parameters predict transplantation-free survival in patients with fontan circulation. Circ Cardiovasc Imaging. 2014;7(3):502–9.

    Article  PubMed  PubMed Central  Google Scholar 

  18. Penny DJ, Redington AN. Angiographic demonstration of incoordinate motion of the ventricular wall after the Fontan operation. Br Heart J. 1991;66(6):456–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  19. Penny DJ, Rigby ML, Redington AN. Abnormal patterns of intraventricular flow and diastolic filling after the Fontan operation: evidence for incoordinate ventricular wall motion. Br Heart J. 1991;66(5):375–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Penny DJ, Redington AN. Diastolic ventricular function after the Fontan operation. Am J Cardiol. 1992;69(9):974–5.

    Article  CAS  PubMed  Google Scholar 

  21. Fogel MA, Gupta KB, Weinberg PM, Hoffman EA. Regional wall motion and strain analysis across stages of Fontan reconstruction by magnetic resonance tagging. Am J Phys. 1995;269(3 Pt 2):H1132–52.

    CAS  Google Scholar 

  22. Border WL, Syed AU, Michelfelder EC, Khoury P, Uzark KC, Manning PB, Pearl JM. Impaired systemic ventricular relaxation affects postoperative short-term outcome in Fontan patients. J Thorac Cardiovasc Surg. 2003;126(6):1760–4.

    Article  PubMed  Google Scholar 

  23. Bacha EA, Zimmerman FJ, Mor-Avi V, Weinert L, Starr JP, Sugeng L, Lang RM. Ventricular resynchronization by multisite pacing improves myocardial performance in the postoperative single-ventricle patient. Ann Thorac Surg. 2004;78(5):1678–83.

    Article  PubMed  Google Scholar 

  24. Cheung YF, Penny DJ, Redington AN. Serial assessment of left ventricular diastolic function after Fontan procedure. Heart. 2000;83(4):420–4.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. Garofalo CA, Cabreriza SE, Quinn TA, Weinberg AD, Printz BF, Hsu DT, Quaegebeur JM, Mosca RS, Spotnitz HM. Ventricular diastolic stiffness predicts perioperative morbidity and duration of pleural effusions after the Fontan operation. Circulation. 2006;114(1 Suppl):I56–61.

    PubMed  Google Scholar 

  26. Anderson PA, Sleeper LA, Mahony L, Colan SD, Atz AM, Breitbart RE, Gersony WM, Gallagher D, Geva T, Margossian R, McCrindle BW, Paridon S, Schwartz M, Stylianou M, Williams RV, Clark BJ 3rd. Pediatric Heart Network Investigators. Contemporary outcomes after the Fontan procedure: a Pediatric Heart Network multicenter study. J Am Coll Cardiol. 2008;52(2):85–98.

    Article  PubMed  PubMed Central  Google Scholar 

  27. Kaneko S, Khoo NS, Smallhorn JF, Tham EB. Single right ventricles have impaired systolic and diastolic function compared to those of left ventricular morphology. J Am Soc Echocardiogr. 2012;25(11):1222–30.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Heart Institute, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH, 45229, USA

    Andrew N. Redington

Authors
  1. Andrew N. Redington
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Andrew N. Redington .

Editor information

Editors and Affiliations

  1. Paediatric Cardiology, Labatt Family Heart Center Hospital for Sick Children, Toronto, Ontario, Canada

    Mark K. Friedberg

  2. Heart Institute, Cincinnati Children’s Hosp Med Center, Cincinnati, Ohio, USA

    Andrew N. Redington

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer International Publishing AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Redington, A.N. (2018). Single Right Ventricular Function and Failure in the Fontan Circulation. In: Friedberg, M., Redington, A. (eds) Right Ventricular Physiology, Adaptation and Failure in Congenital and Acquired Heart Disease. Springer, Cham. https://doi.org/10.1007/978-3-319-67096-6_14

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-67096-6_14

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-67094-2

  • Online ISBN: 978-3-319-67096-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation