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Management of patients post-Fontan procedure

Authors
Jonathan Johnson, MD
Heidi M Connolly, MD, FASE
Section Editor
Gruschen Veldtman, MBChB, FRCP, Dip Obst
Deputy Editor
Susan B Yeon, MD, JD, FACC

INTRODUCTION

The Fontan operation is a palliative surgical procedure performed in patients with a functional or anatomic single ventricle (also known as univentricular heart) [1]. The Fontan operation originally was described for patients with tricuspid atresia in 1971 [2]. In the past 40 years, the types of malformations for which the Fontan operation is utilized has expanded considerably, and it has become the most common operation performed for patients with any type of single ventricle [3]. Following the Fontan procedure, patients face substantial morbidity and mortality risk and require lifelong follow-up with a cardiologist experienced in the care of patients with complex congenital heart disease (pediatric cardiologist or adult congenital heart disease specialist).

This topic will discuss management of patients who have undergone a Fontan procedure. Lesions for which a Fontan may be performed are discussed separately.

LESIONS TREATED BY THE FONTAN PROCEDURE

The Fontan procedure is performed in patients with functional or anatomic single ventricle, defined as presence of only one well-developed ventricle with the rudimentary ventricle (if present) less than 30 percent of its normal volume [1]. Lesions for which a Fontan may be performed include:

Hypoplastic left heart syndrome (see "Hypoplastic left heart syndrome")

Tricuspid atresia (see "Tricuspid valve (TV) atresia")

                                    

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Literature review current through: Nov 2016. | This topic last updated: Mon Nov 07 00:00:00 GMT+00:00 2016.
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