Comparison of anatomic isthmus block with the modified right atrial maze procedure for late atrial tachycardia in Fontan patients
Abstract
BACKGROUND: Late atrial reentry tachycardia (AT) after Fontan repair is common, with limited efficacy of medical therapy in preventing AT recurrence. In this study, two approaches to surgical arrhythmia ablation in patients with refractory AT undergoing Fontan revision are compared: cryoablation of the inferomedial right atrium (RA), and a more extensive modified RA maze procedure designed to eliminate all potential RA reentrant circuits. METHODS AND RESULTS: Fontan revision was performed in 23 patients with AT, using inferomedial RA cryoablation (Group 1, n=8) and modified RA maze procedure (Group 2, n=15). There was no difference in age at initial Fontan, age at Fontan revision, age at onset of AT, or number of failed antiarrhythmic medications. Patients underwent preoperative, intraoperative, and postoperative electrophysiological studies. Thirty-eight different tachycardia circuits were induced in preoperative studies with 3 major areas of RA involvement: the lower lateral RA, the atrial septum, and the inferomedial RA. At postoperative electrophysiological study, AT was inducible in 62% of Group 1 patients but only 7% of Group 2 patients (P<0.02). With mean follow-up of 43 months, 5 of 8 patients in Group 1 experienced AT recurrence compared with none in Group 2 (P<0.001). There was no significant difference in length of hospital stay or complication rate comparing the two groups. CONCLUSION: Modified RA maze procedure is superior to anatomic isthmus block in treating reentrant AT in postoperative Fontan patients. The modified RA maze has eliminated AT recurrence at mid-term follow-up with low morbidity and mortality.
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A modified right atrial maze procedure versus CTI ablation was associated with lower recurrence rates.
“A modified right atrial maze procedure vs. CTI ablation was associated with lower recurrence rates, 373 but selection of suitable patients is important.394”
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 [373]
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Late Arrhythmias Following Fontan Surgery
Deal, B. J. (2012) · World Journal for Pediatric and Congenital Heart Surgery · doi:10.1177/2150135111436314 · Cited by 24 · see citing works
“In addition to right atrial reduction, a modified right atrial maze procedure is performed, intended to address the most common right atrial macro-reentrant circuits identified in the right atrial isthmus, low lateral right atrial wall, and between the superior rim of the atrial septal patch and the superior vena cava…”
MethodManagement of late atrial tachyarrhythmia long after Fontan operation
Fujita, S., Takahashi, K., Takeuchi, D., Manaka, T., Shoda, M., Hagiwara, N., Kurosawa, H., & Nakanishi, T. (2009) · Journal of Cardiology · doi:10.1016/j.jjcc.2009.01.009 · Cited by 23 · see citing works
2020 Clinical practice guidelines for Supraventricular tachycardia in adults
Bokeria, L. A., Golukhova, E. Z., Popov, S. V., Artyukhina, Е. А., Базаев, В. А., Batalov, R. E., Bokeria, O. L., Vygovsky, A. B., Gilyarov, M. Y., Sp, G., et al. (2021) · Russian Journal of Cardiology · doi:10.15829/1560-4071-2021-4484 · Cited by 23 · see citing works
Supraventricular Arrhythmias in Patients with Adult Congenital Heart Disease
Lundqvist, C. B., Potpara, T., & Malmborg, H. (2017) · Arrhythmia & Electrophysiology Review · doi:10.15420/aer.2016:29:3 · Cited by 20 · see citing works
Long-term results of atrial maze surgery in patients with congenital heart disease
Corcia, M. C. G., Walsh, E. P., & Emani, S. M. (2019) · EP Europace · doi:10.1093/europace/euz056 · Cited by 20 · see citing works
The Adult With a Fontan: A Panacea Without a Cure?
Lastinger, L. T., & Zaidi, A. N. (2013) · Circulation Journal · doi:10.1253/circj.cj-13-1105 · Cited by 18 · see citing works
Pedra, S. R. F. F., Hornberger, L. K., Leal, S., Taylor, G. P., & Smallhorn, J. F. (2005) · Pediatric Cardiology · doi:10.1007/s00246-004-0688-3 · Cited by 18 · see citing works
Koh, M., Yagihara, T., Uemura, H., Kagisaki, K., Hagino, I., Ishizaka, T., & Kitamura, S. (2007) · Journal of Thoracic and Cardiovascular Surgery · doi:10.1016/j.jtcvs.2006.12.008 · Cited by 18 · see citing works
Surgical outcome of Fontan conversion and arrhythmia surgery: Need a pacemaker?
Terada, T., Sakurai, H., Nonaka, T., Sakurai, T., Sugiura, J., Taneichi, T., & Ohtsuka, R. (2013) · Asian Cardiovascular and Thoracic Annals · doi:10.1177/0218492313504939 · Cited by 16 · see citing works
“The ablation procedure was similar to that described by Deal and colleagues.(12) In 2 patients with a diagnosis of corrected transposition of the great arteries with paradoxical ventricular contraction or poor ventricular function, biventricular pacing was applied during the conversion procedure.”
MethodResultAdvances in the approach to treatment of supraventricular tachycardia in the pediatric population
Chun, T., & Hare, G. F. V. (2004) · Current Cardiology Reports · doi:10.1007/s11886-004-0033-8 · Cited by 15 · see citing works
“The results of these combined procedures have generally been good, with low morbidity or mortality in addition to that associated with the surgical revision [33–35], although follow-up of this group of patients has suggested that a modified maze procedure is superior to anatomic isthmus block in patients with IART and…”
ResultOhuchi, H., Miyazaki, A., Watanabe, T., Yamada, O., Yagihara, T., & Echigo, S. (2006) · International Journal of Cardiology · doi:10.1016/j.ijcard.2006.05.024 · Cited by 15 · see citing works
Atypical atrial tachycardias in patients with congenital heart disease
Triedman, J. K. (2007) · Heart Rhythm · doi:10.1016/j.hrthm.2007.09.001 · Cited by 13 · see citing works
2025 Clinical practice guidelines for Supraventricular tachycardias
Popov, S. V., Davtyan, K. V., Shubik, Y. V., Artyukhina, Е. А., Базаев, В. А., Batalov, R. E., Bokeria, L. A., Bubnova, M. G., Vygovsky, A. B., Sp, G., et al. (2025) · Russian Journal of Cardiology · doi:10.15829/1560-4071-2025-6448 · Cited by 12 · see citing works
Prophylactic arrhythmia surgery in association with congenital heart disease
Mavroudis, C. D., & Deal, B. J. (2016) · Translational Pediatrics · doi:10.21037/tp.2016.06.04 · Cited by 12 · see citing works
“In the congenital heart disease population, additional right atrial macroreentrant circuits have been identified commonly referred to as “non-isthmus” dependent tachycardia (1,62).”
BackgroundSurgical Therapy of Arrhythmias in Single‐Ventricle Patients Undergoing Fontan or Fontan Conversion
Kwak, J. G., Kim, W., Lee, J. R., & Kim, Y. (2009) · Journal of Cardiac Surgery · doi:10.1111/j.1540-8191.2009.00914.x · Cited by 11 · see citing works
Surgical aspects of atrial arrhythmia
Uemura, H. (2016) · Herzschrittmachertherapie + Elektrophysiologie · doi:10.1007/s00399-016-0434-6 · Cited by 10 · see citing works
Jacobs, J. P. (2010) · Cardiology in the Young · doi:10.1017/s1047951110001034 · Cited by 10 · see citing works
Management of intra-atrial reentrant tachycardia
Kannankeril, P. J., & Fish, F. A. (2005) · Current Opinion in Cardiology · doi:10.1097/01.hco.0000153454.91083.70 · Cited by 10 · see citing works
Arrhythmia Surgery for Adults with Congenital Heart Disease.
Deal, B., & Mavroudis, C. (2017) · Cardiac Electrophysiology Clinics · doi:10.1016/j.ccep.2017.02.014 · Cited by 6 · see citing works
Katritsis, D. G., Boriani, G., Cosío, F. G., Jaı̈s, P., Hindricks, G., Josephson, M. E., Keegan, R., Knight, B. P., Kück, K., Lane, D. A., et al. (2016) · Arrhythmia & Electrophysiology Review · doi:10.15420/aer.2016:5.3.gl1 · Cited by 7 · see citing works
Bhaskaran, A. P., Nayyar, S., Charla, P., Saeed, Y., Haldar, S., Porta‐Sánchez, A., Kugamoorthy, P., Hans, A. K., Jøns, C., Oechslin, E. N., et al. (2019) · Journal of Interventional Cardiac Electrophysiology · doi:10.1007/s10840-019-00580-5 · Cited by 6 · see citing works
Surgical Therapy of Cardiac Arrhythmias
Mavroudis, C. D., Deal, B. J., & Backer, C. L. (2012) · Wiley eBooks · doi:10.1002/9781118320754.ch41 · Cited by 6 · see citing works
Atrial arrhythmia surgery in congenital heart disease
Gandhi, S. K. (2007) · Journal of Interventional Cardiac Electrophysiology · doi:10.1007/s10840-007-9181-2 · Cited by 5 · see citing works
“Simple “isthmus” blocks were found not to be adequate and, of course, did not account for the myriad of potential reentrant circuits [ 14 ].”
BackgroundAtrial arrhythmias and special circumstances.
Harris, L., Gow, R. M., Kirsh, J. A., Klein, G. J., & Siu, S. C. (2005) · PubMed · Cited by 4 · see citing works
Poretti, G., Hoxha, S., Segreto, A., Gardellini, J., Sandrini, C., Faggian, G. G., Varrica, A., Chessa, M., Giamberti, A., & Luciani, G. B. (2021) · Frontiers in Cardiovascular Medicine · doi:10.3389/fcvm.2021.770221 · Cited by 4 · see citing works
“Atrial arrhythmias in Fontan patients are very poorly tolerated as they can lead to severe heart failure with low cardiac output and to a significant reduction in exercise tolerance (11).”
BackgroundSurgical ablation of atrial arrhythmias: The electrophysiologist's point of view
Sarubbi, B. (2015) · Progress in Pediatric Cardiology · doi:10.1016/j.ppedcard.2015.10.014 · Cited by 2 · see citing works
An Integrated Approach to the Care of Adult Patients with Prior Atriopulmonary Fontan Surgery
Dechert, B. E., & Deal, B. J. (2008) · Journal of Pediatric Health Care · doi:10.1016/j.pedhc.2007.08.016 · Cited by 3 · see citing works
Uchita, S., Harada, Y., Yasukochi, S., & Satomi, G. (2008) · General Thoracic and Cardiovascular Surgery · doi:10.1007/s11748-008-0224-0 · Cited by 2 · see citing works
Systematic review of intraoperative ablation for the treatment of atrial fibrillation
Hazel, S. (2004) · Cited by 2 · see citing works
Surgical Therapy of Arrhythmias and Conductive Disorders
Mavroudis, C. D., Deal, B. J., & Backer, C. L. (2013) · Springer eBooks · doi:10.1007/978-1-4471-4619-3_143 · Cited by 1 · see citing works
Atrial Arrhythmias in Congenital Heart Disease
Triedman, J. K. (2004) · Elsevier eBooks · doi:10.1016/b0-7216-0323-8/50063-4 · Cited by 1 · see citing works
Cardiac Surgery in Adults with Congenital Heart Disease: Operation and Reoperation
Marelli, D., PLUNKETT, M. D., Perloff, J. K., & Laks, H. (2009) · Elsevier eBooks · doi:10.1016/b978-1-4160-5894-6.50019-8 · Cited by 1 · see citing works
Radiofrequency ablation in congenital heart disease: a call to arms
Dubin, A. M. (2004) · Heart Rhythm · doi:10.1016/j.hrthm.2004.04.001 · Cited by 1 · see citing works
Surgical and Transcatheter Management of Arrhythmias
Deal, B. J., & Mavroudis, C. D. (2023) · doi:10.1002/9781119282327.ch42 · Cited by 1 · see citing works
Surgical Ablation of Arrhythmias Associated with Congenital Cardiopathies
Sandoval, N. F., Rosas, F., & Velasco, V. M. (2008) · Wiley eBooks · doi:10.1002/9780470696354.ch19
Catheter Ablation for Atrial Arrhythmia
Abrams, D. J., Earley, M. J., & SCHILLING, R. J. (2010) · Elsevier eBooks · doi:10.1016/b978-0-443-06712-9.00064-7
Arrhythmias and Adult Congenital Heart Disease
Robinson, M., & Gerstenfeld, E. P. (2012) · Elsevier eBooks · doi:10.1016/b978-1-4377-0285-9.00081-8
Arrhythmia and Pacemaker Surgery in Congenital Heart Disease
Fynn‐Thompson, F., & Cecchin, F. (2010) · Elsevier eBooks · doi:10.1016/b978-1-4160-5225-8.00132-x
Mavroudis, C. D., Deal, B. J., & Backer, C. L. (2023) · Wiley eBooks · doi:10.1002/9781119282327.ch28
Arrhythmia and Pacemaker Surgery in Congenital Heart Disease
Fynn‐Thompson, F., & Cecchin, F. (2016) · Elsevier eBooks · doi:10.1016/b978-0-323-24126-7.00132-0
Surgical Ablation in Congenital Heart Disease: Advances in Techniques and Clinical Outcomes
van den Bosch, A. E., van Schie, M. S., Yildirim, V., Linderhof, M. H. C., de Groot, N. M. S., Nguyen, H. H., & Taverne, Y. J. H. J. (2024) · Congenital Heart Disease · doi:10.32604/chd.2025.062129
“3) [18].”
Surgical strategies for the failing single ventricle
Kulshrestha, K., Morales, D. L. S., & Maeda, K. (2025) · Elsevier eBooks · doi:10.1016/b978-0-443-13279-7.00037-7
Complications Associated with Bidirectional Glenn, Fontan Operations, and Arrhythmia Surgery
Mavroudis, C. D., & Backer, C. L. (2025) · Springer eBooks · doi:10.1007/978-3-032-02661-3_17
Kinjo, T., Takeuchi, D., Toyohara, K., Inai, K., & Shoda, M. (2026) · Journal of Arrhythmia · doi:10.1002/joa3.70387
“Several studies have shown that TCPC conversion [1] or combined TCPC conversion and modified Maze procedure [3] inhibits atrial tachyarrhythmia.”
Principles of the Fontan Conversion Operation
Mavroudis, C. D., Deal, B. J., & Backer, C. L. (2013) · Springer eBooks · doi:10.1007/978-1-4471-4619-3_187
- Authors
- Deal, B. J., Mavroudis, C., Backer, C. L., Buck, S. H., & Johnsrude, C.
- Affiliations
- Northwestern University (US)
- Venue
- Circulation
- Published
- 2002-07-30
- Volume
- 106
- Issue
- 5
- Pages
- 575-579
- ISSN
- 0009-7322 (ISSN-L)
- 1524-4539
- OpenAlex
- W2082791517
- Cited by
- 87
- References
- 35
- Citations by year
- 2026: 1 · 2025: 5 · 2024: 1 · 2023: 2 · 2022: 1 · 2021: 2 · 2019: 4 · 2018: 2 · 2017: 2 · 2016: 5 · 2015: 9 · 2014: 4 · 2013: 4 · 2012: 3
- FWCI
- 3.27
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Circulation (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac Arrhythmias and Treatments
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Atrial Fibrillation Management and Outcomes
- Keywords
- Fontan circulation, cryoablation, atrial reentry, tachycardia recurrence
- MeSH terms
- Adolescent, Child, Cryosurgery, Follow-Up Studies, Heart Atria, Cardiac Surgical Procedures, Humans, Pacemaker, Artificial, Recurrence, Reoperation, Retrospective Studies, Tachycardia, Treatment Outcome, Defibrillators, Implantable, Fontan Procedure, Body Surface Potential Mapping, Electrophysiologic Techniques, Cardiac
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