Skip to main content
SpringerLink
Log in

Functional Single Ventricle (FSV)

  • Chapter
  • First Online:
An Illustrated Guide to Congenital Heart Disease

Abstract

This chapter includes classification, morphology, clinical manifestations, diagnosis, natural and unnatural course, treatment, staged Fontan operation, and fetal diagnosis of a functional single ventricle. Particularly, the chapter includes a unique and novel classification of a functional single ventricle, which can be applied to every heart that requires Fontan-type operation. Images of real patients of each type are presented. Also various postoperative problems or sequelae after bidirectional cavopulmonary shunt and Fontan operation and their treatment are presented including images of each problem.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 189.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 249.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 329.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Suggested Reading

  1. Anderson RH, Ho SY. Which hearts are unsuitable for biventricular correction? Ann Thorac Surg. 1998;66:621–6.

    Article  CAS  Google Scholar 

  2. Anne P, Du W, Mattoo TK, Zilbermann MV. Nephropathy in patients after Fontan palliation. Int J Cardiol. 2009;132:244–7.

    Article  Google Scholar 

  3. Atz AM, Zak V, Mahony L, Uzark K, et al. Pediatric heart network investigators. Survival data and predictors of functional outcome an average of 15 years after the Fontan procedure: the pediatric heart network Fontan cohort. Congenit Heart Dis. 2015;10:30–42.

    Article  Google Scholar 

  4. Barker PCA, Nowak C, King K, et al. Risk factors for cerebrovascular events following Fontan palliation in patients with a functional single ventricle. Am J Cardiol. 2005;96:587–91.

    Article  Google Scholar 

  5. Bernstein D, Naftel D, Chin C, et al. Outcome of listing for cardiac transplantation for failed Fontan: A multi-institutional study. Circulation. 2006;114:273–80.

    Article  CAS  Google Scholar 

  6. Betts TR, Roberts PR, Allern SA, et al. Electrophysiological mapping and ablation of intraatrial reentry tachycardia after Fontan surgery with the use of a noncontact mapping system. Circulation. 2000;102:419–25.

    Article  CAS  Google Scholar 

  7. Chan SY, Lau W, Wong WHS, et al. Chylothorax in children after congenital heart surgery. Ann Thorac Surg. 2006;82:1650–7.

    Article  Google Scholar 

  8. Cheung YF, Chay GW, Chiu CSW, Cheng LC. Long-term anticoagulation therapy and the thromboembolic complications after the Fontan procedure. Int J Cardiol. 2005;102:509–13.

    Article  CAS  Google Scholar 

  9. Cook AC, Anderson RH. The anatomy of hearts with double inlet ventricle. Cardiol Young. 2006;16:22–6.

    Article  Google Scholar 

  10. Cook AC, Anderson RH. The functionally univentricular circulation: Anatomic substrates as related to function. Cardiol Young. 2005;15:7–16.

    Article  Google Scholar 

  11. Corno AF, Kocica MJ. Potential implications of the helical heart in congenital heart defects. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2007;10:61–7.

    Article  Google Scholar 

  12. Drenthen W, Pieper PG, Roos-Hesselink JW, et al. Pregnancy and delivery in women after Fontan palliation. Heart. 2006;92:1290–4.

    Article  CAS  Google Scholar 

  13. Elder RW, McCabe NM, Veledar E, et al. Risk factors for major adverse events late after Fontan palliation. Congenit Heart Dis. 2015;10:159–68.

    Article  Google Scholar 

  14. Fiore AC, Turrentine M, Rodefeld M, et al. Fontan operation: A comparison of lateral tunnel with extracardiac conduit. Ann Thorac Surg. 2007;83:622–30.

    Article  Google Scholar 

  15. Gewillig M, Brown SC. The Fontan circulation after 45 years: update in physiology. Heart. 2016;102:1081–6.

    Article  Google Scholar 

  16. Gewillig M, Goldberg DJ. Failure of the Fontan circulation. Heart Fail Clin. 2014;10:105–16.

    Article  Google Scholar 

  17. Giardini A, Napoleone CP, Specchia S, et al. Conversion of atriopulmonary Fontan to extracardiac total cavopulmonary connection improves cardiopulmonary function. Int J Cardiol. 2006;113:341–4.

    Article  Google Scholar 

  18. Goo HW, Jhang WK, Kim YH, Ko JK, Park IS, Park JJ, Yun TJ, Seo DM. CT findings of plastic bronchitis in children after a Fontan operation. Pediatr Radiol. 2008;38:989–93.

    Article  Google Scholar 

  19. Grewal J, Al Hussein M, Feldstein J, et al. Evaluation of silent thrombus after the Fontan operation. Congenit Heart Dis. 2013;8:40–7.

    Article  Google Scholar 

  20. Halaweish I, Ohye RG, Si MS. Berlin heart ventricular assist device as a long-term bridge to transplantation in a Fontan patient with failing single ventricle. Pediatr Transplant. 2015;19:193–5.

    Article  Google Scholar 

  21. Imoto Y, Sese A, Joh K. Redirection of the hepatic venous flow for the treatment of pulmonary arteriovenous malformations after Fontan operation. Pediatr Cardiol. 2006;27:490–2.

    Article  Google Scholar 

  22. Jacobs JP, Franklin RCG, Jacobs ML, et al. Classification of the functionally univentricular heart: Unity from mapped codes. Cardiol Young. 2006;16:9–21.

    Article  Google Scholar 

  23. Jaffe A, Bush A. Anti-inflammatory effects of macrolides in lung disease. Pediatr Pulmonol. 2001;31:464–73.

    Article  CAS  Google Scholar 

  24. Kiesewetter CH, Sheron N, Vettukattill JJ, et al. Hepatic changes in the failing Fontan circulation. Heart. 2007;93:579–84.

    Article  Google Scholar 

  25. Kim SJ, Kim WH, Lim HG, Lee JY. Outcome of 200 patients after an extracardiac Fontan procedure. J Thorac Cardiovasc Surg. 2008;136:108–16.

    Article  Google Scholar 

  26. Landvoigt MT, Mullett CJ. Octreotide efficacy in the treatment of chylothoraces following cardiac surgery in infants and children. Pediatr Crit Care Med. 2006;7:245–8.

    Article  Google Scholar 

  27. Lee C, Lee CH, Hwang SW, et al. Midterm follow-up of the status of Gore-Tex graft after extracardiac conduit Fontan procedure. Eur J Cardiothorac Surg. 2007;31:1008–12.

    Article  Google Scholar 

  28. Mahle WT, Wernovsky G, Bridges ND, et al. Impact of early ventricular unloading on exercise performance in preadolescents with single ventricle Fontan Physiology. J Am Coll Cardiol. 1999;34:1637–43.

    Article  CAS  Google Scholar 

  29. Mahnke CB, Boyle GJ, Janosky JE, et al. Anticoagulation and incidence of late cerebrovascular accidents following the Fontan procedure. Pediatr Cardiol. 2005;26:56–61.

    Article  CAS  Google Scholar 

  30. Martinez RM, Anderson RH. Echo-morphological correlates in atrioventricular valvar atresia. Cardiol Young. 2006;16:27–34.

    Article  Google Scholar 

  31. Mavroudis C, Deal BJ, Backer CL. Surgery for arrhythmias in children. Int J Cardiol. 2004;97:39–51.

    Article  Google Scholar 

  32. Murakami H, Yoshimura N, Kitahara J, et al. Collision of the caval flows caused early failure of the Fontan circulation. J Thorac Cardiovasc Surg. 2006;132:1235–6.

    Article  Google Scholar 

  33. Nayar S, Parmar R, Kulkarni S, Cherian KM. Treatment of Plastic Bronchitis. Ann Thorac Surg. 2007;83:1884–6.

    Article  Google Scholar 

  34. Park CS, Park JJ, Park IS, Goo HW. Ligation of the left hepatic vein for dilated intrahepatic collaterals late after Fontan operation. J Card Surg. 2014;29:564–6.

    Article  Google Scholar 

  35. Pflaumer A, Deisenhofer I, Hausleiter J, Zrenner B. Mapping and ablation of atypical flutter in congenital heart disease with a novel three-dimensional mapping system. Europace. 2006;8:138–9.

    Article  Google Scholar 

  36. Schultz KD, Oermann CM. Treatment of cast bronchitis with low dose oral azithromycin. Pediatr Pulmonol. 2003;35:139–43.

    Article  Google Scholar 

  37. Tarnok A, Bocsi J, Lenz D, Janousek J. Protein losing enteropathy after Fontan surgery - Clinical and diagnostical aspects. Transfus Med Hemother. 2007;34:164–7.

    Article  Google Scholar 

  38. Uzun O, Wong JK, Bhole V, Stumper O. Resolution of protein-losing enteropathy and normalization of mesenteric doppler flow with sildenafil after Fontan. Ann Thorac Surg. 2006;82:39–40.

    Article  Google Scholar 

  39. Veldtman GR, Webb GD. Improved survival in Fontan-associated protein-losing enteropathy. J Am Coll Cardiol. 2014;64(1):63–5.

    Article  Google Scholar 

  40. Viswanathan S. Thromboembolism and anticoagulation after Fontan surgery. Ann Pediatr Cardiol. 2016;9:236–40.

    Article  Google Scholar 

  41. Walker F. Pregnancy and the various forms of the Fontan circulation. Heart. 2007;93:152–4.

    Article  Google Scholar 

  42. Wilson J, Russell J, Williams W, Benson L. Fenestraion of the Fontan circuit as treatment for plastic bronchitis. Pediatr Cardiol. 2005;26:717–9.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pediatric Cardiology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea

    In Sook Park

  2. Department of Radiology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea

    Hyun Woo Goo

Authors
  1. In Sook Park
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Hyun Woo Goo
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea (Republic of)

    In Sook Park

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Singapore Pte Ltd.

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Park, I.S., Goo, H.W. (2019). Functional Single Ventricle (FSV). In: Park, I. (eds) An Illustrated Guide to Congenital Heart Disease. Springer, Singapore. https://doi.org/10.1007/978-981-13-6978-0_23

Download citation

  • DOI: https://doi.org/10.1007/978-981-13-6978-0_23

  • Published:

  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-13-6977-3

  • Online ISBN: 978-981-13-6978-0

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation