{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,13]],"date-time":"2025-05-13T14:22:06Z","timestamp":1747146126850,"version":"3.40.5"},"reference-count":14,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2023,6,12]],"date-time":"2023-06-12T00:00:00Z","timestamp":1686528000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J of Med Genetics Pt A"],"published-print":{"date-parts":[[2023,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Patients with Trisomy 18 have a high incidence of cardiac anomalies and are associated with early death. Because of early mortality, electrical system disease and arrhythmia has been difficult to delineate and the incidence remain unknown. We sought to describe the association and clinical outcomes of electrical system disease and cardiac tachy\u2010arrhythmias in patients with Trisomy 18. This was a retrospective, single institutional study. All patients with Trisomy 18 were included in the study. Patient characteristics, congenital heart disease (CHD), conduction system and clinical tachy\u2010arrhythmia data were collected on all patients. Outcomes including cardiac surgical interventions, electrical system interventions and death were collected until the time of study. Patients with tachy\u2010arrhythmias\/electrical system involvement were compared to those without to identify potential associated variables. A total of 54 patients with Trisomy 18 were included in analysis. The majority of patients was female and had associated CHD. AV nodal conduction system abnormalities with either first or second degree AV block were common (15%) as was QTc prolongation (37%). Tachy\u2010arrhythmias were common with 22% of patients having at least one form of tachy\u2010arrhythmia and associated with concomitant conduction system disease (<jats:italic>p<\/jats:italic>\u2009=\u20090.002). Tachy\u2010arrhythmias were typically treatable with monitoring or medication with eventual resolution without need for procedural intervention. Although early death was common, there were no causes of death associated with tachy\u2010arrhythmia or conduction system disease. In conclusion, patients with Trisomy 18 have a high incidence of conduction system abnormalities and burden of clinical tachy\u2010arrhythmias. Although frequent, electrical system disease did not affect patient outcome or difficultly of care delivery.<\/jats:p>","DOI":"10.1002\/ajmg.a.63324","type":"journal-article","created":{"date-parts":[[2023,6,12]],"date-time":"2023-06-12T07:35:01Z","timestamp":1686555301000},"page":"2518-2523","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Incidence and outcome of arrhythmias and electrical disease in patients with Trisomy 18"],"prefix":"10.1002","volume":"191","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7497-9555","authenticated-orcid":false,"given":"Richard J.","family":"Czosek","sequence":"first","affiliation":[{"name":"Department of Pediatrics Cincinnati Children's Hospital Medical Center, The Heart Institute  Cincinnati Ohio USA"},{"name":"Department of Pediatrics University of Cincinnati Medical Center  Cincinnati Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shankar","family":"Baskar","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Cincinnati Children's Hospital Medical Center, The Heart Institute  Cincinnati Ohio USA"},{"name":"Department of Pediatrics University of Cincinnati Medical Center  Cincinnati Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shaun","family":"Mohan","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Cincinnati Children's Hospital Medical Center, The Heart Institute  Cincinnati Ohio USA"},{"name":"Department of Pediatrics University of Kentucky Albert B. Chandler Hospital  Lexington Kentucky USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeffrey B.","family":"Anderson","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Cincinnati Children's Hospital Medical Center, The Heart Institute  Cincinnati Ohio USA"},{"name":"Department of Pediatrics University of Cincinnati Medical Center  Cincinnati Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David S.","family":"Spar","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Cincinnati Children's Hospital Medical Center, The Heart Institute  Cincinnati Ohio USA"},{"name":"Department of Pediatrics University of Cincinnati Medical Center  Cincinnati Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,6,12]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-020-02444-6"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1161\/jaha.119.012349"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.38191"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-018-2001-x"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.80.5.470"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.hrthm.2022.04.022"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.semperi.2015.12.015"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1093\/europace\/euaa256"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2017-0772"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-018-2032-3"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/s0022-0736(84)80018-7"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1253\/circj.67.992"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/ket377"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/pace.14607"}],"container-title":["American Journal of Medical Genetics Part A"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajmg.a.63324","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,20]],"date-time":"2023-09-20T06:24:44Z","timestamp":1695191084000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajmg.a.63324"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,6,12]]},"references-count":14,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2023,10]]}},"alternative-id":["10.1002\/ajmg.a.63324"],"URL":"https:\/\/doi.org\/10.1002\/ajmg.a.63324","archive":["Portico"],"relation":{},"ISSN":["1552-4825","1552-4833"],"issn-type":[{"type":"print","value":"1552-4825"},{"type":"electronic","value":"1552-4833"}],"subject":[],"published":{"date-parts":[[2023,6,12]]},"assertion":[{"value":"2022-11-25","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-05-26","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-06-12","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}