The Failing Fontan

Overview

Journal Indian J Thorac Cardiovasc Surg

Publisher Springer

Date 2021 Feb 19

PMID 33603286

Citations 1

Authors

T K Susheel Kumar

Affiliations

Soon will be listed here.

Abstract

Nearly 50 years back, Francis Fontan pioneered an operation for tricuspid atresia that bears his name today. The operation has since undergone numerous modifications and continues to be widely applied to an array of single ventricles. Despite restoring normal oxygen levels in the body, the operation creates a neoportal system where adequate cardiac output can be generated only at the expense of increased systemic venous congestion. This results in slow but relentless damage to the end organ systems especially the liver. Continuous surveillance of the patient to monitor this circulation, that will ultimately fail, is of paramount importance. Timely medical and cardiac catheterization and surgical intervention can extend the life span of Fontan patients. Ultimately a change of the hemodynamic circuit in the form of heart transplantation or ventricular assist device will be required to salvage the failing Fontan circuit.

Citing Articles

Blood Biomarkers as a Non-Invasive Method for the Assessment of the State of the Fontan Circulation.

Wittczak A, Mazurek-Kula A, Banach M, Piotrowski G, Bielecka-Dabrowa A J Clin Med. 2025; 14(2).

PMID: 39860501 PMC: 11765985. DOI: 10.3390/jcm14020496.

Deterioration in Renal Function in Patients With a Fontan Circulation and Association With Mortality.

van Hassel G, Groothof D, Douwes J, Hoendermis E, Liem E, Willems T JACC Adv. 2024; 3(12):101399.

PMID: 39629062 PMC: 11612357. DOI: 10.1016/j.jacadv.2024.101399.

References

FELDT R, Driscoll D, Offord K, Cha R, Perrault J, Schaff H . Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg. 1996; 112(3):672-80. DOI: 10.1016/S0022-5223(96)70051-X. View

Rupp S, Schieke C, Kerst G, Mazhari N, Moysich A, Latus H . Creation of a transcatheter fenestration in children with failure of Fontan circulation: Focus on extracardiac conduit connection. Catheter Cardiovasc Interv. 2015; 86(7):1189-94. DOI: 10.1002/ccd.26042. View

Gewillig M, Goldberg D . Failure of the fontan circulation. Heart Fail Clin. 2013; 10(1):105-16. DOI: 10.1016/j.hfc.2013.09.010. View

Tokunaga S, Kado H, Imoto Y, Masuda M, Shiokawa Y, Fukae K . Total cavopulmonary connection with an extracardiac conduit: experience with 100 patients. Ann Thorac Surg. 2002; 73(1):76-80. DOI: 10.1016/s0003-4975(01)03302-1. View

Miura T, Hiramatsu T, Forbess J, Mayer Jr J . Effects of elevated coronary sinus pressure on coronary blood flow and left ventricular function. Implications after the Fontan operation. Circulation. 1995; 92(9 Suppl):II298-303. DOI: 10.1161/01.cir.92.9.298. View

Huddleston C . The failing Fontan: options for surgical therapy. Pediatr Cardiol. 2007; 28(6):472-6. DOI: 10.1007/s00246-007-9008-z. View

dUdekem Y, Xu M, Galati J, Lu S, Iyengar A, Konstantinov I . Predictors of survival after single-ventricle palliation: the impact of right ventricular dominance. J Am Coll Cardiol. 2012; 59(13):1178-85. DOI: 10.1016/j.jacc.2011.11.049. View

FONTAN F, KIRKLIN J, Fernandez G, Costa F, Naftel D, TRITTO F . Outcome after a "perfect" Fontan operation. Circulation. 1990; 81(5):1520-36. DOI: 10.1161/01.cir.81.5.1520. View

Elder R, Wu F . Clinical Approaches to the Patient with a Failing Fontan Procedure. Curr Cardiol Rep. 2016; 18(5):44. DOI: 10.1007/s11886-016-0716-y. View

10.

Kreutzer J, Keane J, Lock J, Walsh E, Jonas R, Castaneda A . Conversion of modified Fontan procedure to lateral atrial tunnel cavopulmonary anastomosis. J Thorac Cardiovasc Surg. 1996; 111(6):1169-76. DOI: 10.1016/s0022-5223(96)70218-0. View

11.

McElhinney D, Marx G, Marshall A, Mayer J, Del Nido P . Cavopulmonary pathway modification in patients with heterotaxy and newly diagnosed or persistent pulmonary arteriovenous malformations after a modified Fontan operation. J Thorac Cardiovasc Surg. 2010; 141(6):1362-70.e1. DOI: 10.1016/j.jtcvs.2010.08.088. View

12.

Garcia Ropero A, Baskar S, Roos Hesselink J, Girnius A, Zentner D, Swan L . Pregnancy in Women With a Fontan Circulation: A Systematic Review of the Literature. Circ Cardiovasc Qual Outcomes. 2018; 11(5):e004575. DOI: 10.1161/CIRCOUTCOMES.117.004575. View

13.

Do T, Chu J, Berdjis F, Anas N . Fontan patient with plastic bronchitis treated successfully using aerosolized tissue plasminogen activator: a case report and review of the literature. Pediatr Cardiol. 2008; 30(3):352-5. DOI: 10.1007/s00246-008-9312-2. View

14.

Zellers T, Brown K . Protein-losing enteropathy after the modified fontan operation: oral prednisone treatment with biopsy and laboratory proved improvement. Pediatr Cardiol. 1996; 17(2):115-7. DOI: 10.1007/BF02505095. View

15.

Stewart J, Gewitz M, Clark B, Seligman K, Romano A, Zeballos G . The role of vasopressin and atrial natriuretic factor in postoperative fluid retention after the Fontan procedure. J Thorac Cardiovasc Surg. 1991; 102(6):821-9. View

16.

Kovach J, Naftel D, Pearce F, Tresler M, Edens R, Shuhaiber J . Comparison of risk factors and outcomes for pediatric patients listed for heart transplantation after bidirectional Glenn and after Fontan: an analysis from the Pediatric Heart Transplant Study. J Heart Lung Transplant. 2011; 31(2):133-9. DOI: 10.1016/j.healun.2011.11.004. View

17.

Khambadkone S, Li J, de Leval M, Cullen S, Deanfield J, Redington A . Basal pulmonary vascular resistance and nitric oxide responsiveness late after Fontan-type operation. Circulation. 2003; 107(25):3204-8. DOI: 10.1161/01.CIR.0000074210.49434.40. View

18.

Zafar F, Castleberry C, Khan M, Mehta V, Bryant 3rd R, Lorts A . Pediatric heart transplant waiting list mortality in the era of ventricular assist devices. J Heart Lung Transplant. 2014; 34(1):82-88. DOI: 10.1016/j.healun.2014.09.018. View

19.

Hraska V . Decompression of thoracic duct: new approach for the treatment of failing Fontan. Ann Thorac Surg. 2013; 96(2):709-11. DOI: 10.1016/j.athoracsur.2013.02.046. View

20.

Griffiths E, Kaza A, Wyler von Ballmoos M, Loyola H, Valente A, Blume E . Evaluating failing Fontans for heart transplantation: predictors of death. Ann Thorac Surg. 2009; 88(2):558-63. PMC: 2844259. DOI: 10.1016/j.athoracsur.2009.03.085. View