Arrhythmia Burden in Adults With Surgically Repaired Tetralogy of Fallot: A Multi-Institutional Study
Abstract
BACKGROUND: The arrhythmia burden in tetralogy of Fallot, types of arrhythmias encountered, and risk profile may change as the population ages. METHODS AND RESULTS: The Alliance for Adult Research in Congenital Cardiology (AARCC) conducted a multicenter cross-sectional study to quantify the arrhythmia burden in tetralogy of Fallot, to characterize age-related trends, and to identify associated factors. A total of 556 patients, 54.0% female, 36.8+/-12.0 years of age were recruited from 11 centers. Overall, 43.3% had a sustained arrhythmia or arrhythmia intervention. Prevalence of atrial tachyarrhythmias was 20.1%. Factors associated with intraatrial reentrant tachycardia in multivariable analyses were right atrial enlargement (odds ratio [OR], 6.2; 95% confidence interval [CI], 2.8 to 13.6), hypertension (OR, 2.3; 95% CI, 1.1 to 4.6), and number of cardiac surgeries (OR, 1.4; 95% CI, 1.2 to 1.6). Older age (OR, 1.09 per year; 95% CI, 1.05 to 1.12), lower left ventricular ejection fraction (OR, 0.93 per unit; 95% CI, 0.89 to 0.96), left atrial dilation (OR, 3.2; 95% CI, 1.5 to 6.8), and number of cardiac surgeries (OR, 1.5; 95% CI, 1.2 to 1.9) were jointly associated with atrial fibrillation. Ventricular arrhythmias were prevalent in 14.6% and jointly associated with number of cardiac surgeries (OR, 1.3; 95% CI, 1.1 to 1.6), QRS duration (OR, 1.02 per 1 ms; 95% CI, 1.01 to 1.03), and left ventricular diastolic dysfunction (OR, 3.3; 95% CI, 1.5 to 7.1). Prevalence of atrial fibrillation and ventricular arrhythmias markedly increased after 45 years of age. CONCLUSIONS: The arrhythmia burden in adults with tetralogy of Fallot is considerable, with various subtypes characterized by different profiles. Atrial fibrillation and ventricular arrhythmias appear to be influenced more by left- than right-sided heart disease.
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Incisional-related AFL or IART are the most common type of SVT (75%) in ACHD patients, and occur most frequently in patients with Ebstein anomaly, Tetralogy of Fallot, single-ventricle, Fontan procedures, TGA, and atrial septal defects.
“These incisional-related AFL or intra-atrial re-entrant tachycardia (IART) are the most common type of SVT (75%) in adult congenital heart disease (ACHD) patients, and occur most frequently in patients with Ebstein anomaly, Tetralogy of Fallot, single-ventricle, Fontan procedures, transposition of the great arteries (TGA), and atrial septal defects.349,350”
From European Heart Rhythm Association (EHRA) consensus document on the management of supraventricular arrhythmias, endorsed by Heart Rhythm Society (HRS), Asia-Pacific Heart Rhythm Society (APHRS), and Sociedad Latinoamericana de Estimulacion Cardiaca y Electrofisiologia (SOLAECE) (2017) · cites this paper as [350]
Approximately one-third of patients after repair of tetralogy of Fallot develop atrial arrhythmias, including intraatrial reentrant tachycardia, focal atrial tachycardia, and AF.
“Approximately one-third of patients after repair of tetralogy of Fallot develop atrial arrhythmias, including intraatrial reentrant tachycardia, focal atrial tachycardia, and AF.987”
From 2016 ESC Guidelines for the management of atrial fibrillation developed in collaboration with EACTS (2016) · cites this paper as [987]
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- Authors
- Khairy, P., Aboulhosn, J., Gurvitz, M. Z., Opotowsky, A. R., Mongeon, F.-P., Kay, J., Valente, A. M., Earing, M. G., Lui, G., Gersony, D. R., Cook, S., Ting, J. G., Nickolaus, M. J., Webb, G., Landzberg, M. J., & Broberg, C. S.
- Affiliations
- Boston Children's Hospital (US)
- From the Montreal Heart Institute, University of Montreal, Montreal, Quebec, Canada (P.K., F.P.M.); University of California, Los Angeles (J.A.); University of Washington, Seattle (M.Z.G.); Boston Adult Congenital Heart Service, Children’s Hospital Boston, Boston, Mass (P.K., A.R.O., A.M.V., M.J.L.); University of Colorado, Denver (J.K.); Medical College of Wisconsin, Milwaukee (M.G.E.); Columbia University Medical Center, New York, NY (G.L., D.R.G.); Ohio State University, Columbus (S.C.); Hershey...
- ORCID
- Venue
- Circulation
- Published
- 2010-08-31
- Volume
- 122
- Issue
- 9
- Pages
- 868-875
- ISSN
- 0009-7322 (ISSN-L)
- 1524-4539
- OpenAlex
- W1982483328
- Cited by
- 531
- References
- 28
- Citations by year
- 2026: 21 · 2025: 27 · 2024: 35 · 2023: 26 · 2022: 24 · 2021: 35 · 2020: 36 · 2019: 40 · 2018: 53 · 2017: 49 · 2016: 39 · 2015: 32 · 2014: 35 · 2013: 35 · 2012: 25
- FWCI
- 18.39
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Circulation (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac Arrhythmias and Treatments
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cardiovascular and Diving-Related Complications
- Keywords
- tetralogy of Fallot, arrhythmia burden, adult congenital heart disease, atrial fibrillation, ventricular arrhythmias, atrial tachyarrhythmia, right atrial enlargement, left ventricular diastolic dysfunction, QRS duration, left atrial enlargement
- MeSH terms
- Adult, Arrhythmias, Cardiac, Atrial Fibrillation, Atrial Flutter, Cross-Sectional Studies, Female, Humans, Male, Middle Aged, Pacemaker, Artificial, Retrospective Studies, Risk Factors, Tachycardia, Atrioventricular Nodal Reentry, Tetralogy of Fallot, Prevalence, Multivariate Analysis, Tachycardia, Ventricular, Pulmonary Atresia, Young Adult
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