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This article was prepared to summarize the points made in a debate that the first author (C.L.B.) had with Dr. Richard Jonas at the American Association for Thoracic Surgery 90th Annual Meeting. The topic of the debate was the optimal surgical approach for functional single-ventricle patients: extracardiac versus intra-atrial lateral tunnel Fontan. My role was to take the viewpoint that the extracardiac Fontan is better. This review summarizes our results at Children''s Memorial Hospital (Chicago, IL) with 180 patients undergoing a primary Fontan procedure and 126 patients undergoing an extracardiac Fontan as part of a Fontan conversion. The world literature was reviewed on outcomes following the Fontan procedure, focusing on six main areas supporting the superiority of the extracardiac Fontan: hemodynamics, arrhythmias, applicability to complex anatomy, use of cardiopulmonary bypass, complications of fenestration and thromboembolism, and operative mortality. Based on this review, it is our conclusion that the extracardiac Fontan is the procedure of choice for patients with a functional single ventricle based on a very low operative mortality, a lower incidence of early and late arrhythmias, improved hemodynamics, fewer postoperative complications, and applicability to a wide variety of complex cardiac anatomy. [Copyright &y& Elsevier]

Summary: Conversion of the atriopulmonary Fontan to a total cavopulmonary extracardiac connection with concomitant arrhythmia surgery and pacemaker placement is a safe and efficacious procedure for this patient population. From 1994 to 2007 a total of 118 patients have undergone this procedure with one (0.8%) early and nine (7.6%) late deaths. During the course of our experience with Fontan conversion our surgical strategy has evolved to include various ablative techniques to treat macro re-entrant atrial tachycardia, focal (automatic) atrial tachycardia, atrioventricular nodal reentry tachycardia, atrial tachycardia due to accessory connections, atrial fibrillation, and ventricular tachycardia. The various mechanisms that we use to treat the underlying atrial arrhythmias are described in this review. We have also encountered patients with variations of the Fontan and other complex anatomic and pathophysiologic aberrations who were not amenable to standard takedown and ablative procedures. We describe those circumstances and the solutions we found to treat those patients. [Copyright &y& Elsevier]

As of September 2007, 120 patients have undergone a Fontan conversion procedure at Children''s Memorial Hospital (Chicago, IL). One of the primary indications for surgery in these patients has been arrhythmias, either atrial reentry tachycardia or atrial fibrillation. The surgical treatment of those two lesions has been with the modified right atrial maze and the Cox-maze III. The purpose of this review is to describe our strategy for performing the maze procedure in these single-ventricle patients. The primary tool for performing the maze in this series has been with the cryocatheter with cryoablation at −160°C for 1 minute. These cryoablation lesions have been combined with standard surgical incisions in the right and left atria. The early mortality in this series is 1%, late mortality is 5%. Freedom from atrial reentry tachycardia recurrence at 5 years is 86%. Freedom from atrial fibrillation recurrence is 98% at 5 years. [Copyright &y& Elsevier]

The principles of Fontan conversion with arrhythmia surgery are to restore the cardiac anatomy by converting the original atriopulmonary connection to a total cavopulmonary artery extracardiac connection and treat the underlying atrial arrhythmias. Successful outcomes of this procedure are dependent on a thorough understanding of several factors: the patient’s fundamental diagnosis of single-ventricle anatomy, the resultant cardiac configuration from the original atriopulmonary Fontan connection, right atrial dilatation that leads to atrial flutter or fibrillation, and associated congenital cardiac anomalies. The purpose of this article is to present some of the more challenging anatomic and electrophysiologic problems we have encountered with Fontan conversion and arrhythmia surgery and the innovative solutions we have used to treat them. The cases reviewed herein include: takedown of a Bjork-Fontan modification, right ventricular hypertension and tricuspid regurgitation after atriopulmonary Fontan for pulmonary atresia and intact ventricular septum, takedown of atrioventricular valve isolation patch for right-sided maze procedure, resultant hemodynamic considerations leading to intraoperative pulmonary vein stenosis after Fontan conversion, unwanted inferior vena cava retraction during the extracardiac connection, right atrial cannulation in the presence of a right atrial clot, distended left superior vena cava causing left pulmonary vein stenosis, dropped atrial septum, and the modified right-sided maze procedure for various single-ventricle pathology. Since 1994 we have performed Fontan conversion with arrhythmia surgery on 109 patients with a 0.9% mortality rate. We attribute our program’s success in no small measure to the strong collaborative efforts of the cardiothoracic surgery and cardiology teams. [Copyright &y& Elsevier]