Total cavopulmonary conversion and maze procedure for patients with failure of the Fontan operation

Abstract

Fontan conversion to total cavopulmonary connection with concomitant arrhythmia surgery is excellent therapy for patients whose Fontan repair has failed. Fontan conversion is safe, improves New York Heart Association class, improves exercise tolerance, and has a low incidence of recurrent arrhythmias.

Atrial Fibrillation in Patients with Congenital Heart Disease

“…[50] The surgical approach has since progressed from isthmus ablation, to a modified right-sided maze procedure, and eventually to the MAZE-Cox III (right and leftsided maze) procedure for AF. [51], [52] Referral for surgical ablation includes patients for whom catheter ablation has failed, patients with concomitant CHD and arrhythmias, and patients with low body mass or MRI with 3D reconstruction. Any time an open-thoracotomy operation is planned, a discussion of concomitant surgical ablation of the arrhythmogenic substrate and excision or exclusion of residual thrombogenic structures should occur.…”

Management of the Fontan circulation in the adult patient

“…(149) Survival for patients with an atriopulmonary Fontan may improve by conversion to a TCPC type Fontan. (150) The associated improvement in fluid mechanics and reduction in arrhythmia burden are likely to lead to survival improvements, but the operation requires careful consideration. (151) In carefully selected patients, the operation is associated with a low operative mortality.…”

Prophylactic arrhythmia surgery in association with congenital heart disease

“…Based on the historic data regarding populations with the highest incidence of atrial arrhythmia development, targeted populations for prophylactic arrhythmia surgery in the right atrium include patients with unrepaired ASDs presenting over 40 years of age (1,5), patients with Ebstein anomaly (1), tetralogy patients presenting for pulmonary valve insertion (1,68,69), and single-ventricle patients who present for Fontan operations (1,16,32,(70)(71)(72). Prophylactic surgery for AF would be considered for patients with significant leftsided atrioventricular disease and severe left atrial dilatation undergoing planned surgery, with lesions including left atrial maze and right-sided cavotricuspid isthmus ablation (16).…”