Skip to main content Accessibility help

Is the “Perfect Fontan” operation routinely achievable in the modern era?*

  • James K. Kirklin (a1), Robert N. Brown (a1), Ayesha S. Bryant (a1), David C. Naftel (a1), Edward V. Colvin (a2), F. Bennett Pearce (a2), Robb L. Romp (a2), Walter H. Johnson (a2), Yung R. Lau (a2), William S. McMahon (a2), Christopher J. Knott-Craig (a1) and Albert D. Pacifico (a1)...



In 1990, Fontan, Kirklin, and colleagues published equations for survival after the so-called “Perfect Fontan” operation. After 1988, we evolved a protocol using an internal or external polytetraflouroethylene tube of 16 to 19 millimetres diameter placed from the inferior caval vein to either the right or left pulmonary artery along with a bidirectional cava-pulmonary connection. The objective of this study was to test the hypothesis that a “perfect” outcome is routinely achievable in the current era when using a standardized surgical procedure.


Between 1 January, 1988, and 12 December, 2005, 112 patients underwent the Fontan procedure using an internal or external polytetraflouroethylene tube plus a bidirectional cava-pulmonary connection, the latter usually having been constructed as a previous procedure. This constituted 45% of our overall experience in constructing the Fontan circulation between 1988 and 1996, and 96% of the experience between 1996 and 2005. Among all surviving patients, the median follow-up was 7.3 years. We calculated the expected survival for an optimal candidate, given from the initial equations, and compared this to our entire experience in constructing the Fontan circulation.


An internal tube was utilized in 61 patients, 97% of whom were operated prior to 1998, and an external tube in 51 patients, the latter accounting for 95% of all operations since 1999. At 1, 5, 10 and 15 years, survival of the entire cohort receiving polytetraflouroethylene tubes is superimposable on the curve calculated for a “perfect” outcome. Freedom from replacement or revision of the tube was 97% at 10 years.


Using a standardized operative procedure, combining a bidirectional cavopulmonary connection with a polytetraflouroethylene tube placed from the inferior caval vein to the pulmonary arteries for nearly all patients with functionally univentricular hearts, early and late survival within the “perfect” outcome as predicted by the initial equations of Fontan and Kirklin is routinely achievable in the current era. The need for late revision or replacement of the tube is rare.


Corresponding author

Correspondence to: James K. Kirklin, MD, University of Alabama at Birmingham, Professor & Director, Division of Cardiothoracic Surgery, 760 THT, 1900 University Blvd., Birmingham, AL 35294, United States of America. Tel: 205-934-5486; Fax: 205-975-2553; E-mail:


Hide All

The presentation on which this work is based was given at the Inaugural Meeting of the World Society for Pediatric and Congenital Heart Surgery, held in Washington, District of Columbia, May 3 and 4, 2007.



Hide All
1.Fontan, F, Baudet, E. Surgical repair of tricuspid atresia. Thorax 1971; 26: 240248.
2.Fontan, F, Kirklin, JW, Fernandez, G, et al. Outcome after a “Perfect” Fontan operation. Circulation 1990; 81: 15201536.
3.Jacobs, ML. The Fontan operation, thromboembolism, and anticoagulation: a reappraisal of the single bullet theory. J Thorac Cardiovasc Surg 2005; 129: 491495.
4.Rosenthal, DN, Friedman, AH, Kleinman, CS, Kopf, GS, Rosenfeld, LE, Hellenbrand, WE. Thromboembolic complications after Fontan operations. Circulation 1995; 92 (Suppl II): II-287293.
5.Coon, PD, Rychik, J, Novello, RT, Ro, PS, Gaynor, JW, Spray, TL. Thrombus formation after the Fontan operation. Ann Thorac Surg 2001; 71: 19901994.
6.Chowdhury, UK, Airan, B, Kothari, SS, et al. Specific issues after extracardiac Fontan operation: ventricular function, growth potential, arrhythmia, and thromboembolism. Ann Thorac Surg 2005; 80: 665672.
7.Nurnberg, JH, Ovroutski, S, Alexi-Meskishvili, V, Ewert, P, Hetzer, R, Lange, PE. New onset arrhythmias after the extracardiac conduit Fontan operation compared with the intraatrial lateral tunnel procedure: early and midterm results. Ann Thorac Surg 2004; 78: 19791988.
8.Petrossian, E, Reddy, M, Collins, KK, et al. The extracardiac conduit Fontan operation using minimal approach extracorporeal circulation: early and midterm outcomes. J Thorac Cardiovasc Surg 2006; 132: 10541063.
9.Konstantinov, IE, Puga, FJ, Alex-Meskishvili, VV. Thrombosis of intracardiac or extracardiac conduits after modified Fontan operation in patients with azygous continuation of the inferior vena cava. Ann Thorac Surg 2001; 72: 16411644.
10.Monagle, P, Cochrane, A, McCrindle, B, Benson, L, Williams, W, Andrew, M. Editorial: Thromboembolic complications after Fontan procedures – The role of prophylactic antiocoagulation. J Thorac Cardiovasc Surg 1998; 115: 493498.
11.Ono, M, Boethig, D, Goerler, H, Lange, M, Westhoff-Bleck, M, Breymann, T. Clinical outcome of patients 20 Years after Fontan operation – Effect of fenestration on late morbidity. Eur J Cardiothorac Surg 2006; 30: 923929.



Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed