Atrial Arrhythmias in Adults With Congenital Heart Disease
Abstract
Background Atrial arrhythmias increase disease burden in the general adult population. Adults with congenital heart lesions constitute a rapidly growing group of patients with cardiovascular disease. We hypothesized that atrial arrhythmias increase with age and impair health outcomes in this population. Methods and results We conducted a population-based analysis of prevalence, lifetime risk, mortality, and morbidity associated with atrial arrhythmias in adults with congenital heart disease from l983 to 2005. In 38 428 adults with congenital heart disease in 2005, 5812 had atrial arrhythmias. Overall, the 20-year risk of developing atrial arrhythmia was 7% in a 20-year-old subject and 38% in a 50-year-old subject. More than 50% of patients with severe congenital heart disease reaching age 18 years developed atrial arrhythmias by age 65 years. In patients with congenital heart disease, the hazard ratio of any adverse event in those with atrial arrhythmias compared with those without was 2.50 (95% confidence interval, 2.38 to 2.62; P Conclusions Atrial arrhythmias occurred in 15% of adults with congenital heart disease. The lifetime incidence increased steadily with age and was associated with a doubling of the risk of adverse events. An increase in resource allocation should be anticipated to deal with this increasing burden.
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By age 50 years, approximately 38% of patients with ACHD will develop an atrial arrhythmia.
“By age 50 years, approximately 38% of patients with ACHD will develop an atrial arrhythmia, and by age 65 years, >50% of patients with severe CHD will develop atrial arrhythmias (660).”
From 2017 ACC/AHA/HRS Guideline for the Evaluation and Management of Patients With Syncope (2017) · cites this paper as [660]
By age 65 years, >50% of patients with severe CHD will develop atrial arrhythmias.
“By age 50 years, approximately 38% of patients with ACHD will develop an atrial arrhythmia, and by age 65 years, >50% of patients with severe CHD will develop atrial arrhythmias (660).”
From 2017 ACC/AHA/HRS Guideline for the Evaluation and Management of Patients With Syncope (2017) · cites this paper as [660]
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“By age 50 years, approximately 38% of patients with ACHD will develop an atrial arrhythmia, and by age 65 years, >50% of patients with severe CHD will develop atrial arrhythmias (660).”
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“The lifetime risk of atrial fibrillation in a 20-year-old individual with CHD is equivalent to that of a 55-year-old individual without CHD, with >50% of patients with severe CHD developing atrial arrhythmias by 65 years of age.(89) The presence of atrial fibrillation is associated with abnormal brain aging.”
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“Increases in ischemic stroke associated with arrhythmia may also be due to atrial fibrillation being a common complication in adults with congenital heart disease.(20) The higher prevalence of migraine in females is consistent with other reports.”
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“50% of patients with severe CHD will develop atrial arrhythmias.(660) The prevalence of VT after tetralogy of Fallot repair is 3% to 14%.”
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“Indeed, atrial arrhythmias are the leading cause of morbidity and hospital admissions (4,5), and have been implicated as a cause of sudden death (6).”
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“The prevalence of SVT among patients with Ebstein anomaly was 33% in 1 large series, the highest noted among ACHD patients (440), and increased with age.”
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Self-citationAtrial fibrillation in adults with congenital heart disease
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“Consideration should be made for ASD closure or repair as part of reducing risk of tachyarrhythmia recurrence and improving symptoms (2,3,11,14).”
BackgroundCommon long-term complications of adult congenital heart disease: avoid falling in a H.E.A.P.
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“[64] The lifetime incidence increased steadily with”
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“SVT is observed in 10-20% of these patients and is associated with increased risk of stroke and sudden cardiac death.(29) The most common SVTs are atrial arrhythmias in ASD and atrial flutter in operated patients with Fontan and Fallot.”
BackgroundClinical Profile and Prognostic Significance of Atrial Fibrillation in Hypertrophic Cardiomyopathy
Tian, T., Wang, Y.-L., Sun, K., Wang, J.-Z., Zou, Y.-B., Zhang, W.-L., Bao, J.-R., Zhu, L., Shen, H., Hui, R., et al. (2013) · The Cardiology · doi:10.1159/000354953 · Cited by 41
Radiofrequency Catheter Ablation of Atrial Tachycardias in Congenital Heart Disease
Klehs, S., Schneider, H. E., Backhoff, D., Paul, T., & Krause, U. (2017) · Circulation Arrhythmia and Electrophysiology · doi:10.1161/circep.117.005451 · Cited by 46 · see citing works
“Kaplan–Meier analysis of freedom from AT recurrence did not show any difference between the 3 groups (simple CHD, moderate CHD, complex CHD; log-rank nonsignificant; Figure 3).”
ResultGiang, K. W., Fedchenko, M., Dellborg, M., Eriksson, P., & Mandalenakis, Z. (2021) · Journal of the American Heart Association · doi:10.1161/jaha.120.020939 · Cited by 46 · see citing works
“7,8 However, with increasing age, patients with CHD are at risk of developing acquired cardiovascular diseases including myocardial infarction 9– 11 and ischemic stroke (IS).”
Grubb, C. S., Lewis, M. J., Whang, W., Biviano, A. B., Hickey, K., Rosenbaum, M., & Garan, H. (2019) · JACC Clinical Electrophysiology · doi:10.1016/j.jacep.2018.10.011 · Cited by 39 · see citing works
“Latent atrial tachyarrhythmias (AT) in older survivors have become a major clinical problem causing morbidity and even affecting mortality in more than one-half of these patients (3).”
BackgroundHeart Failure in Adult Congenital Heart Disease: From Advanced Therapies to End-of-Life Care
Crossland, D. S., Bruaene, A. V. D., Silversides, C. K., Hickey, E. J., & Roche, S. L. (2019) · Canadian Journal of Cardiology · doi:10.1016/j.cjca.2019.07.626 · Cited by 37 · see citing works
Risk of thromboembolic complications in adult congenital heart disease: A literature review
Karsenty, C., Zhao, A., Marijon, É., & Ladouceur, M. (2018) · Archives of cardiovascular diseases · doi:10.1016/j.acvd.2018.04.003 · Cited by 42 · see citing works
Stan, M., Ammash, N., Warnes, C., Brennan, M. D., Thapa, P., Nannenga, M. R., & Bahn, R. (2013) · International Journal of Cardiology · doi:10.1016/j.ijcard.2012.02.015 · Cited by 38 · see citing works
“Adults with CHD are at an increased risk of cardiac arrhythmias combined with increased risk of adverse events [6].”
BackgroundMarelli, A., & Gurvitz, M. (2011) · Progress in cardiovascular diseases · doi:10.1016/j.pcad.2010.10.001 · Cited by 38 · see citing works
Self-citationIschemic Stroke in Adults With Congenital Heart Disease: A Population‐Based Cohort Study
Pedersen, M. G., Olsen, M., Schmidt, M., Johnsen, S., Learn, C. P., Laursen, H., & Madsen, N. (2019) · Journal of the American Heart Association : Cardiovascular and Cerebrovascular Disease · doi:10.1161/jaha.118.011870 · Cited by 37 · see citing works
“The same applies for atrial arrhythmias and mechanical heart valves, which are also well-known stroke risk factors.(2,7) Previous attempts to use the CHA2DS2VASc score to predict thromboembolic risk in CHD patients suffering from AFib have shown conflicting results.”
Ávila, P., Oliver, J., Gallego, P., González-García, A., Rodríguez-Puras, M., Cambronero, E., Ruiz-Cantador, J., Campos, A., Peinado, R., Prieto, R., et al. (2017) · Circulation: Arrhythmia and Electrophysiology · doi:10.1161/circep.117.005396 · Cited by 37 · see citing works
CT evaluation of congenital heart disease in adults.
Wiant, A., Nyberg, E., & Gilkeson, R. (2009) · AJR. American journal of roentgenology · doi:10.2214/ajr.08.2192 · Cited by 37 · see citing works
Arrhythmias in Adult Congenital Heart Disease
Kumar, S., Tedrow, U. B., & Triedman, J. K. (2015) · Cardiology Clinics · doi:10.1016/j.ccl.2015.07.007 · Cited by 37 · see citing works
Diagnosis and Management of Paroxysmal Supraventricular Tachycardia
Peng, G., & Zei, P. C. (2024) · JAMA · doi:10.1001/jama.2024.0076 · Cited by 40 · see citing works
Cardiac outcomes in adults with supravalvar aortic stenosis
Greutmann, M., Tobler, D., Sharma, N. C., Muhll, I. V., Mebus, S., Kaemmerer, H., Schuler, P. K., Deanfield, J. E., Beauchesne, L., Salehian, O., et al. (2012) · European Heart Journal · doi:10.1093/eurheartj/ehs206 · Cited by 40 · see citing works
“Diffuse form of supravalvar aortic stenosis 16 (14)”
BackgroundCongenital heart defects in adulthood.
Diller, G., Breithardt, G., & Baumgartner, H. (2011) · Deutsches Ärzteblatt International · doi:10.3238/arztebl.2011.0452 · Cited by 36 · see citing works
Atrial Arrhythmias in Adults With Congenital Heart Disease
Graham, J. (2010) · doi:10.1016/s0145-4145(09)79757-6 · Cited by 36
Emergence and robustness of target waves in a neuronal network
Xu, Y., Jin, W.-Y., & Ma, J. (2015) · doi:10.1142/s0217979215501647 · Cited by 35
Masuda, K., Ishizu, T., Niwa, K., Takechi, F., Tateno, S., Horigome, H., & Aonuma, K. (2017) · International Journal of Cardiology · doi:10.1016/j.ijcard.2017.02.004 · Cited by 33 · see citing works
Mavroudis, C. D., Stulak, J. M., Ad, N., Siegel, A., Giamberti, A., Harris, L., Backer, C. L., Tsao, S. S. L., Dearani, J. A., Weerasena, N. A., et al. (2014) · The Annals of Thoracic Surgery · doi:10.1016/j.athoracsur.2014.07.026 · Cited by 39 · see citing works
Born to Age: When Adult Congenital Heart Disease Converges With Geroscience
Moons, P., & Marelli, A. J. (2022) · JACC Advances · doi:10.1016/j.jacadv.2022.100012 · Cited by 41 · see citing works
Self-citationRaissadati, A., Haukka, J., Pätilä, T., Nieminen, H., & Jokinen, E. (2020) · Journal of the American Heart Association : Cardiovascular and Cerebrovascular Disease · doi:10.1161/jaha.119.015354 · Cited by 31 · see citing works
“1579 (100) Sex, n (%) Women 3065 (55) 547 (38) 752 (48) Men 2539 (45) 893 (62) 827 (52) Decade of first operation, n (%) 1966–1969 489 (9) 69 (5) 54 (3) 1970–1979 1325 (23) 231 (16) 208 (13) 1980–1989 1378 (25) 334 (23) 346 (22) 1990–1999 1610 (29) 404 (28) 520 (33) 2000–2009 802 (14) 402 (28) 451 (29) Age at first op…”
Heidendael, J. F., Bokma, J., de Groot, J. D., Koolbergen, D., Mulder, B., & Bouma, B. (2015) · International Journal of Cardiology · doi:10.1016/j.ijcard.2015.02.024 · Cited by 31 · see citing works
“Atrial arrhythmias occur in 15% of adult patients with congenital heart disease (CHD) [1,2].”
BackgroundTrajectories of care in congenital heart disease – the long arm of disease in the womb
Marelli, A. J. (2020) · Journal of Internal Medicine · doi:10.1111/joim.13048 · Cited by 35 · see citing works
Self-citationNeurocognition in Adult Congenital Heart Disease: How to Monitor and Prevent Progressive Decline
Keir, M., Ebert, P. L., Kovacs, A. H., Smith, J., Kwan, E., Field, T. S., Brossard‐Racine, M., & Marelli, A. J. (2019) · Canadian Journal of Cardiology · doi:10.1016/j.cjca.2019.06.020 · Cited by 28 · see citing works
Self-citationEfficacy of catheter ablation for atrial fibrillation in patients with congenital heart disease.
Guarguagli, S., Kempny, A., Cazzoli, I., Barracano, R., Gatzoulis, M., Dimopoulos, K., & Ernst, S. (2019) · Europace · doi:10.1093/europace/euz157 · Cited by 30 · see citing works
El-Assaad, I., Al-Kindi, S., Abraham, J. M., Sanatani, S., Bradley, D., Halsey, C., Law, I., Balaji, S., Shetty, I., & Aziz, P. (2016) · Heart Rhythm · doi:10.1016/j.hrthm.2016.07.016 · Cited by 30 · see citing works
Congenital Heart Disease Hospitalizations in Canada: A 10-Year Experience.
Islam, S., Yasui, Y., Kaul, P., Marelli, A., & Mackie, A. (2015) · Canadian Journal of Cardiology · doi:10.1016/j.cjca.2015.05.022 · Cited by 30
Self-citationSevere Fontan‐Associated Liver Disease and Its Association With Mortality
Guerrero-Chalela, C., Therrien, J., Grossman, Y., Guo, L.-M., Liu, A.-H., & Marelli, A. (2023) · Journal of the American Heart Association : Cardiovascular and Cerebrovascular Disease · doi:10.1161/jaha.121.024034 · Cited by 29
Self-citationImpact of atrial arrhythmias on outcome in adults with congenital heart disease.
Yang, H., Kuijpers, J. M., de Groot, J. D., Konings, T., van Dijk, A. V., Sieswerda, G., Post, M. C., Mulder, B., & Bouma, B. (2017) · International Journal of Cardiology · doi:10.1016/j.ijcard.2017.06.073 · Cited by 29
Is Initiating NOACs for Atrial Arrhythmias Safe in Adults with Congenital Heart Disease?
Yang, H.-Y., Bouma, B., Mulder, B., & O. B. O. C. Investigators (2017) · Cardiovascular Drugs and Therapy · doi:10.1007/s10557-017-6745-y · Cited by 29 · see citing works
“Atrial arrhythmias are prevalent in 15% of adults with congenital heart defects (CHD) and are associated with increased risk for thromboembolism [1].”
BackgroundAtrial Septal Defects and Cardioembolic Strokes.
Leppert, M. H., Poisson, S., & Carroll, J. (2016) · Cardiology Clinics · doi:10.1016/j.ccl.2015.12.004 · Cited by 29 · see citing works
“a large cohort of adult patients with ASDs, which is 4-fold higher than the general population.(13) The development of atrial fibrillation or flutter is an age-related reflection of atrial dilation, which in patients with ASDs older than 60 years can be as high as 50%.”
BackgroundMartin, C. A., Yue, A., Martin, R., Claridge, S., Sawhney, V., Maury, P., Lowe, M., Combes, N., Heck, P., Begley, D., et al. (2019) · JACC Clinical Electrophysiology · doi:10.1016/j.jacep.2019.08.001 · Cited by 28 · see citing works
Clinical Outcomes During Admissions for Heart Failure Among Adults With Congenital Heart Disease
Agarwal, A., Dudley, C. W., Nah, G., Hayward, R. M., & Tseng, Z. H. (2019) · Journal of the American Heart Association : Cardiovascular and Cerebrovascular Disease · doi:10.1161/jaha.119.012595 · Cited by 28 · see citing works
“We compared in-hospital and readmission events among adults with and without CHD admitted for HF. Methods and Results-—”
ResultInfluentialRecommendations for the use of electrophysiological study: Update 2018
Mureşan, L., Cismaru, G. L., Martins, R. P., Bataglia, A., Roşu, R., Puiu, M., Guşetu, G., Mada, R. O., Muresan, C., Ispas, D. R., et al. (2018) · Hellenic Journal of Cardiology · doi:10.1016/j.hjc.2018.09.002 · Cited by 37 · see citing works
Takeuchi, D., Honda, K., Shinohara, T., Inai, K., Toyohara, K., & Nakanishi, T. (2015) · Circulation Journal · doi:10.1253/circj.cj-15-0042 · Cited by 31 · see citing works
Broberg, C. S., & Burchill, L. J. (2015) · International Journal of Cardiology · doi:10.1016/j.ijcard.2015.04.064 · Cited by 37 · see citing works
Griffiths, J. R., Nussinovitch, U., Liang, J., Sims, R. A., Yoneda, Z., Bernstein, H. M., Viswanathan, M., Khairy, P., Srivatsa, U., Frankel, D., et al. (2022) · Circulation: Arrhythmia and Electrophysiology · doi:10.1161/circep.122.010954 · Cited by 27
Management of Heart Failure in Adult Congenital Heart Disease
Sabanayagam, A., Cavus, O., Williams, J., & Bradley, E. (2018) · Heart Failure Clinics · doi:10.1016/j.hfc.2018.06.005 · Cited by 27 · see citing works
Roca-Luque, I., Rivas-Gándara, N., Subirà, L. D., Pascual, J. F., Pijuan-Domenech, A., Pérez-Rodon, J., Subirana-Domènech, M., Santos-Ortega, A., Rosés-Noguer, F., Miranda-Barrio, B., et al. (2018) · JACC Clinical Electrophysiology · doi:10.1016/j.jacep.2018.04.011 · Cited by 27 · see citing works
Thrombosis in congenital heart disease
Khairy, P. (2013) · Expert Review of Cardiovascular Therapy · doi:10.1586/14779072.2013.854703 · Cited by 27 · see citing works
- Authors
- Bouchardy, J., Therrien, J., Pilote, L., Ionescu-Ittu, R., Martucci, G., Bottega, N., & Marelli, A. J.
- Affiliations
- McGill University Health Centre (CA)
- From the McGill Adult Unit for Congenital Heart Disease Excellence, McGill University Health Center and the Jewish General Hospital (J.B., J.T., R.I.-I., G.M., N.B., A.J.M.) and Division of Internal Medicine, McGill University Health Centre Research Institute (L.P., R.I.-I.), Montreal, Quebec, Canada.
- Venue
- Circulation
- Published
- 2009-10-27
- Volume
- 120
- Issue
- 17
- Pages
- 1679-1686
- ISSN
- 0009-7322 (ISSN-L)
- 1524-4539
- OpenAlex
- W2075290555
- Cited by
- 476
- References
- 0
- Citations by year
- 2026: 23 · 2025: 26 · 2024: 33 · 2023: 36 · 2022: 22 · 2021: 30 · 2020: 32 · 2019: 34 · 2018: 44 · 2017: 47 · 2016: 25 · 2015: 34 · 2014: 18 · 2013: 24 · 2012: 20
- FWCI
- 10.52
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Circulation (publishedVersion)
- PubMed (publishedVersion)
- IRIS (submittedVersion)
- SERVAL (Université de Lausanne) (submittedVersion)
- Topics
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cardiovascular and Diving-Related Complications
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac Arrhythmias and Treatments
- Keywords
- atrial arrhythmias, adult congenital heart disease, atrial fibrillation, lifetime risk, prevalence, mortality, stroke, heart failure, cardiac interventions, health burden, aging
- MeSH terms
- Adult, Age Factors, Aged, Atrial Fibrillation, Female, Follow-Up Studies, Heart Defects, Congenital, Humans, Male, Middle Aged, Population Surveillance, Retrospective Studies, Cohort Studies, Young Adult
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