Prevalence of atrial tachyarrhythmia in adults after Fontan operation.
OBJECTIVE
The Fontan procedure provides palliation for patients with complex congenital heart disease. A late complication is the development of a pro-arrhythmogenic environment. Modifications to the surgical technique try and reduce the incidence of late arrhythmia. This study aims to establish incidence and outcome of arrhythmias in Fontan patients with long-term follow-up.


METHODS
Retrospective analysis of adult patients with a Fontan circulation cared for by University Hospitals Birmingham between 2001 and 2013.


RESULTS
166 patients, mean age 29.1 years, were identified (63% atriopulmonary (AP) Fontan, 13% lateral tunnel (LT), 24% extracardiac total cavopulmonary connection (TCPC)). The mean follow-up since Fontan surgery was 18.6 years. 42% (70 patients) had suffered at least one tachyarrhythmia, with 100% incidence of arrhythmia in AP Fontan patients surviving at least 26 years following surgery. The most common arrhythmia was intra-atrial re-entrant tachycardia (66%). There was also a significant incidence of arrhythmia in the LT and TCPC groups (23% at mean follow-up of 19.6 years and 13.6 years, respectively). 44 patients had undergone electrical cardioversion, 30 had ablations and 10 had undergone Fontan conversion surgery. Survival analysis showed only age at the time of Fontan as a significant predictor for arrhythmia onset (p<0.001) irrespective of surgical approach.


CONCLUSIONS
There is a significant, increasing arrhythmia burden in adult patients with a Fontan circulation. Arrhythmia development could be regarded as an inevitable consequence of an AP Fontan. There remains a high incidence of arrhythmia with more modern surgical approaches. The long-term future of these patients is unclear and their care poses significant challenges.
Congenital Electrophysiology Group, Department of Cardiology, University Hospitals Birmingham, Birmingham, UK.
Emily
E
Quinton
University Hospitals Birmingham, Birmingham, UK.
Peter
P
Nightingale
Adult Congenital Heart Disease Unit, Department of Cardiology, University Hospitals Birmingham, Birmingham, UK.
Lucy
L
Hudsmith
Adult Congenital Heart Disease Unit, Department of Cardiology, University Hospitals Birmingham, Birmingham, UK.
Sara
S
Thorne
Congenital Electrophysiology Group, Department of Cardiology, University Hospitals Birmingham, Birmingham, UK.
Howard
H
Marshall
Adult Congenital Heart Disease Unit, Department of Cardiology, University Hospitals Birmingham, Birmingham, UK.
Paul
P
Clift
Congenital Electrophysiology Group, Department of Cardiology, University Hospitals Birmingham, Birmingham, UK.
Joseph
J
de Bono
D000293:Adolescent; D000328:Adult; D000368:Aged; D004562:Electrocardiography; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D006325:Heart Atria / Q000503:physiopathology*; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications; D015995:Prevalence; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D013610:Tachycardia / Q000453:epidemiology / Q000209:etiology / Q000503:physiopathology; D006113:United Kingdom / Q000453:epidemiology; D055815:Young Adult