The Fontan Procedure: Three Decades of Progress

R. G. Ohye Æ E. L. Bove

Published online: 3 August 2007

� Springer Science+Business Media, LLC 2007

The Fontan procedure has undergone a remarkable journey

covering more than three decades and five continents, a

continuum whose beginnings predate even the first

description of ‘‘a corrective procedure for tricuspid atresia,

which completely suppresses blood mixing,’’ by Fontan

and Baudet in 1971. The pioneering work in the 1950s and

1960s on the cavopulmonary connection by many investi-

gators, including Carlon, Shoemacker, Meshalkin,

Robicsek, Rasmussen, Santy, and Glenn, provided the

groundwork for the operation that would eventually be

known as the Fontan procedure. Although the operation

bears the name of Francis Fontan, important contributions

by Kreutzer, Bjork, and many others since have resulted in

a metamorphosis from valved, unidirectional left and right

cavopulmonary and atriopulmonary connections to not one,

but two distinct ‘‘Fontan procedures’’—the lateral tunnel

and the extracardiac conduit. These refinements have re-

sulted in a current hospital survival of greater than 95% for

even the most challenging single ventricle malformations.

In concert with the improvement in survival, steady inroads

have been made in decreasing long-term morbidity,

including thromboembolism and arrhythmia, associated

with the Fontan procedure. The future will continue to

reveal exciting advancements in surgical technique, such as

defining the roles of the lateral tunnel and the extracardiac

conduit procedures, and in medical management, including

the anticipated results from the Pediatric Heart Clinical

Research Network’s multi-institutional ACE inhibitor trial

for single ventricle patients in the next few years. However,

many obstacles remain. Improved understanding and

management of protein-losing enteropathy, arteriovenous

malformations, and ventricular dysfunction, including the

development of implantable assist devices for the right

heart, all promise to be areas of great interest as many

patients reach adulthood.

It has been a pleasure and a privilege to serve as

editors for the notable authors contributing manuscripts

for this special issue of Pediatric Cardiology celebrating

the more than three decades of progress of the Fontan

procedure. Reviewing these contributions from our col-

leagues in congenital heart disease reminds us of the

remarkable history, progressively improving results, and

ongoing future challenges in the management of the

Fontan patient.

R. G. Ohye (&) � E. L. Bove

Section of Cardiac Surgery, Division of Pediatric Cardiovascular

Surgery, University of Michigan Medical School,

F7830 Mott/0223, 1500 East Medical Center Drive, Ann Arbor,

MI 48109, USA

e-mail: ohye@umich.edu

123

Pediatr Cardiol (2007) 28:421

DOI 10.1007/s00246-007-9000-7