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A retrospective cohort of 341 patients with LT and EC Fontan operations was identified in a single adult congenital heart disease (ACHD) center. Arrhythmias and related procedures were recorded. Logistic regression was used to determine factors affecting arrhythmia prevalence. Arrhythmia incidence was compared using interval-censored Cox regression. Of 341 patients, 254 (74.5%) had LT Fontan and 87 (25.5%) had EC Fontan. Patients with LT Fontan were older at their last ACHD follow-up (29 versus 25\u00a0years). Arrhythmia occurred in 52.2% of patients, 57.9% of the LT and 35.6% of the EC Fontan group. Compared to EC Fontan patients, patients with LT were both more likely to have an arrhythmia (OR 1.84, 95% CI 1.04\u20133.24) and at a higher risk of developing a new arrhythmia (HR 1.67, 95% CI 1.10\u20132.54). Patients with LT had more cardioversions, ablations, and revisions compared with EC Fontan patients. However, a similar proportion in each group were treated with antiarrhythmics and had a pacemaker. We summarize arrhythmia burden in a large single-center adult cohort of patients with TCPC Fontan, demonstrating increased arrhythmia in patients with LT. This work can inform screening protocols and risk assessment for this growing population.<\/jats:p>","DOI":"10.1007\/s00246-025-03950-1","type":"journal-article","created":{"date-parts":[[2025,8,20]],"date-time":"2025-08-20T10:17:43Z","timestamp":1755685063000},"page":"1658-1665","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Comparison of Arrhythmia Prevalence and Incidence in Adult Patients with Lateral Tunnel and Extra-Cardiac Fontan Circulation"],"prefix":"10.1007","volume":"47","author":[{"given":"Andrew M.","family":"Freddo","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antara","family":"Mondal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexis Z.","family":"Tomlinson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Molly","family":"Eron","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Srinivas","family":"Denduluri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabella","family":"Farkas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Partington","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily","family":"Ruckdeschel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Allison L.","family":"Tsao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Constantine D.","family":"Mavroudis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muhammad","family":"Nuri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephanie","family":"Fuller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuli Y.","family":"Kim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sumeet","family":"Vaikunth","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,8,20]]},"reference":[{"key":"3950_CR1","doi-asserted-by":"publisher","first-page":"682","DOI":"10.1016\/s0022-5223(19)35174-8","volume":"96","author":"MR de Leval","year":"1988","unstructured":"de Leval MR, Kilner P, Gewillig M et al (1988) Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations experimental studies and early clinical experience. 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