Beyond fontan conversion: surgical therapy of arrhythmias including patients with associated complex congenital heart disease

Background Arrhythmia operations may be extended to patients with failed ablation procedures or associated structural defects requiring surgical intervention. The purpose of this study is to review our experience with arrhythmia operations in 29 patients who did not have Fontan conversions after the introduction of catheter ablation. Methods Between July 1992 and January 2002, 29 patients had operations for refractory atrial (n = 24) or ventricular (n = 5) arrhythmias. Mechanisms of arrhythmia included atrial reentry (n = 11), atrial fibrillation (n = 5), automatic atrial (n = 3), accessory connections (n = 6), atrioventricular nodal reentry (n = 2), and ventricular tachycardia (n = 5). Median age at operation was 12.3 years (range, 6 days to 45 years). Two patients had structurally normal hearts; the remaining 27 patients underwent concomitant repair of structural heart disease, including atrioventricular valve replacement or repair (n = 8), anatomy-specific repair of Ebstein’s anomaly (n = 4), tetralogy of Fallot repair or revision (n = 4), atrial septal defect closure (n = 3), ventricular septal defect repair (n = 2), Mustard takedown with arterial switch (n = 2), initial Fontan (n = 2), right ventricle-to-pulmonary artery conduit revision (n = 2), Norwood procedure (n = 1), 1 ventricular repair for Uhl’s anomaly (n = 1), Mustard baffle revision (n = 1), pulmonary valve replacement with aneurysm resection (n = 1), and aortic valve replacement with complex repair (n = 1). Results No patient developed heart block, and the surgical mortality rate was 7%. One patient died after Mustard takedown and arterial switch operation, and 1 neonate died after repair of severe Ebstein’s anomaly. There was one late death after arterial switch conversion at another institution. Recurrent clinical supraventricular tachycardia was present in 2 patients (2 of 27, 7.4%) and 2 patients had new-onset tachycardias with different underlying mechanisms of arrhythmia at late follow-up (median follow-up 47 months). Conclusions Successful surgical therapy of arrhythmias can be performed safely at the time of repair of complex congenital heart disease or in patients with failed catheter ablation procedures. Early consideration for single-stage therapy of arrhythmia and structural heart disease is indicated.