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

Published online by Cambridge University Press:  01 June 2008

James K. Kirklin ,
Robert N. Brown ,
Ayesha S. Bryant ,
David C. Naftel ,
Edward V. Colvin ,
F. Bennett Pearce ,
Robb L. Romp ,
Walter H. Johnson Jr ,
Yung R. Lau  and
William S. McMahon
...Show all authors
James K. Kirklin*
Affiliation:
Division of Cardiothoracic Surgery, University of Alabama at Birmingham, Birmingham, Alabama, United States of America
Robert N. Brown
Affiliation:
Division of Cardiothoracic Surgery, University of Alabama at Birmingham, Birmingham, Alabama, United States of America
Ayesha S. Bryant
Affiliation:
Division of Cardiothoracic Surgery, University of Alabama at Birmingham, Birmingham, Alabama, United States of America
David C. Naftel
Affiliation:
Division of Cardiothoracic Surgery, University of Alabama at Birmingham, Birmingham, Alabama, United States of America
Edward V. Colvin
Affiliation:
Division of Paediatric Cardiology, University of Alabama at Birmingham, Birmingham, Alabama, United States of America
F. Bennett Pearce
Affiliation:
Division of Paediatric Cardiology, University of Alabama at Birmingham, Birmingham, Alabama, United States of America
Robb L. Romp
Affiliation:
Division of Paediatric Cardiology, University of Alabama at Birmingham, Birmingham, Alabama, United States of America
Walter H. Johnson Jr
Affiliation:
Division of Paediatric Cardiology, University of Alabama at Birmingham, Birmingham, Alabama, United States of America
Yung R. Lau
Affiliation:
Division of Paediatric Cardiology, University of Alabama at Birmingham, Birmingham, Alabama, United States of America
William S. McMahon
Affiliation:
Division of Paediatric Cardiology, University of Alabama at Birmingham, Birmingham, Alabama, United States of America
Christopher J. Knott-Craig
Affiliation:
Division of Cardiothoracic Surgery, University of Alabama at Birmingham, Birmingham, Alabama, United States of America
Albert D. Pacifico
Affiliation:
Division of Cardiothoracic Surgery, University of Alabama at Birmingham, Birmingham, Alabama, United States of America
*
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: jkirklin@uab.edu
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Abstract

Objective

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.

Methods

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.

Results

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.

Conclusion

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.

Keywords

Congenital heart diseasefunctionally single ventricleextra-cardiac connections
Type
Original Article
Information
Cardiology in the Young , Volume 18 , Issue 3 , June 2008 , pp. 328 - 336
Copyright
Copyright © Cambridge University Press 2008

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Footnotes

*

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.

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