Fontan Operation and the Single Ventricle

Abstract

The Fontan operation has gone through multiple incarnations since Fontan and Baudet's initial description in 1971. Through the medical dossier of a patient with a single ventricle, we plot the history of medical, surgical, and percutaneous interventions over the past 40 years, specifically focusing on the Fontan procedure, its development, indications, sequelae, and complications. Cardiac computed tomography with angiography is highlighted as a noninvasive imaging tool for the evaluation of the complex Fontan circulation.

A functional understanding of moderate to complex congenital heart disease and the impact of pregnancy. Part II: Tetralogy of Fallot, Eisenmenger’s syndrome and the Fontan operation

“…41 This procedure separates pulmonary and systemic circulations, therefore increasing the oxygen content of systemic blood. It involves either a direct connection between the systemic venous return and the pulmonary artery (the so-called cavo-pulmonary connection) or via the right atrium (atriopulmonary connection or classic Fontan circulation), bypassing the right ventricle.…”

Successful epidural anesthesia for cesarean delivery in a woman with Fontan repair

“…The Fontan procedure was first described in 1971 as a treatment for tricuspid atresia [3] with its use now expanded to all patients with a single functional ventricle, including complete atrioventricular septal defects and hypoplastic left heart syndrome [4]. Currently, encountering a parturient that has undergone a Fontan procedure is an uncommon occurrence.…”

Transconduit puncture for catheter ablation of atrial tachycardia in a patient with extracardiac Fontan palliation

“…However, this frequently caused RA dilation, sluggish RA flow, atrial fibrosis, and was associated with a high incidence of atrial arrhythmias2,3. Exclusion of most or all of the RA decreases the incidence of atrial arrhythmias; therefore, the Fontan procedure evolved by establishing a total cavopulmonary connection (TCPC) by way of either the lateral tunnel (an intra-atrial tunnel) or the extra-cardiac Fontan4,5. Patients who underwent first generation RA-PA Fontan surgery with subsequent development of atrial arrhythmias may benefit from surgical conversion to TCPC with concomitant arrhythmia surgery6,7.…”