{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T06:59:42Z","timestamp":1785394782675,"version":"3.55.0"},"reference-count":23,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2022,10,27]],"date-time":"2022-10-27T00:00:00Z","timestamp":1666828800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pacing Clinical Electrophis"],"published-print":{"date-parts":[[2022,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>BACKGROUND<\/jats:title><jats:p>Degeneration of the cardiac conduction system resulting in complete heart block (CHB), ventricular arrhythmias (VA), and sudden cardiac death (SCD) is recognized in patients with Kearns\u2013Sayre syndrome (KSS) and is potentially preventable with permanent pacemaker (PPM) implantation. However, other mechanisms for SCD have been proposed, and the efficacy of implanting a defibrillator instead of PPM remains to be investigated.<\/jats:p><\/jats:sec><jats:sec><jats:title>METHODS<\/jats:title><jats:p>We utilized the National Inpatient Sample (NIS) database 2016\u20132019 to investigate the risk of VA or dysrhythmic cardiac arrest (dCA) in KSS patients. We compared the outcomes of KSS to myotonic dystrophy (MD), a more common genetic disorder with similar clinical cardiac features and course.<\/jats:p><\/jats:sec><jats:sec><jats:title>RESULTS<\/jats:title><jats:p>We identified 640 admissions for KSS. VA or dCA were lower in admissions for KSS than MD patients (2.3%\u00a0vs. 4.5%, <jats:italic>p<\/jats:italic>\u00a0=\u00a0.009). Device implantation differed between study groups. Approximately, 70% of cases with KSS and conduction abnormalities had pacemaker\u00a0(\u00b1\u00a0defibrillator) on hospital discharge, compared to 35% in MD. Conduction abnormalities were associated with higher rates of VA or dCA in both study groups. None of the admissions for KSS patients who developed VA or dCA had a pacemaker, and all of them had conduction abnormalities. One\u2010third of admissions for MD patients who developed VA or dCA had a device already implanted prior to the event.<\/jats:p><\/jats:sec><jats:sec><jats:title>CONCLUSION<\/jats:title><jats:p>Despite its effectiveness in preventing VA, PPM remains underutilized in patients with KSS or MD who have conduction abnormalities. PPM alone do not fully prevent VA in MD patients; therefore, addition of defibrillator capacity might be necessary.<\/jats:p><\/jats:sec>","DOI":"10.1111\/pace.14607","type":"journal-article","created":{"date-parts":[[2022,10,8]],"date-time":"2022-10-08T07:40:04Z","timestamp":1665214804000},"page":"1357-1363","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Ventricular arrhythmias in Kearns\u2013Sayre syndrome: A cohort study using the National Inpatient Sample database 2016\u20132019"],"prefix":"10.1111","volume":"45","author":[{"given":"Kyle","family":"Wiseman","sequence":"first","affiliation":[{"name":"Department of Medicine Hackensack Meridian Jersey Shore University Medical Center  Neptune New Jersey USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dhairya","family":"Gor","sequence":"additional","affiliation":[{"name":"Department of Medicine Hackensack Meridian Jersey Shore University Medical Center  Neptune New Jersey USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ndausung","family":"Udongwo","sequence":"additional","affiliation":[{"name":"Department of Medicine Hackensack Meridian Jersey Shore University Medical Center  Neptune New Jersey USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0726-3498","authenticated-orcid":false,"given":"Abbas","family":"Alshami","sequence":"additional","affiliation":[{"name":"Division of Cardiology Hackensack Meridian Jersey Shore University Medical Center  Neptune New Jersey USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vandan","family":"Upadhaya","sequence":"additional","affiliation":[{"name":"Division of Cardiology Hackensack Meridian Jersey Shore University Medical Center  Neptune New Jersey USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven J.","family":"Daniels","sequence":"additional","affiliation":[{"name":"Division of Cardiology Hackensack Meridian Jersey Shore University Medical Center  Neptune New Jersey USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wendy K.","family":"Chung","sequence":"additional","affiliation":[{"name":"Department of Medicine Columbia University Irving Medical Center  New York USA"},{"name":"Department of Pediatrics Columbia University Irving Medical Center  New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charles H.","family":"Koo","sequence":"additional","affiliation":[{"name":"Division of Cardiology Hackensack Meridian Jersey Shore University Medical Center  Neptune New Jersey USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,10,27]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11910-010-0096-4"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2014.12.038"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-012-0365-x"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/8904_2011_32"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1540-8159.2005.40049.x"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ihj.2012.07.010"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/heart.88.6.665"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2018.10.043"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.hrthm.2021.07.051"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.hrthm.2017.10.035"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2018.12.064"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ipej.2017.03.003"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1540-8159.2007.00590.x"},{"key":"e_1_2_10_15_1","unstructured":"HouchensRL RossDEA.Using the HCUP National Inpatient Sample to Estimate Trends. 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