{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T12:30:03Z","timestamp":1781872203241,"version":"3.54.5"},"reference-count":25,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2022,3,18]],"date-time":"2022-03-18T00:00:00Z","timestamp":1647561600000},"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":[[2022,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>As targeted treatments for amyloid transthyretin cardiomyopathy (ATTR\u2010CM) are becoming available, we aim to characterize the rates of ventricular arrhythmias (VAs), implantable cardioverter\u2010defibrillator (ICD) utilization, and their impact on survival.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This is a retrospective cohort study of 130 patients with ATTR\u2010CM diagnosed at Emory University's Cardiac Amyloidosis Center between April 2012 and September 2020. VAs were defined as nonsustained or sustained ventricular tachycardia and ventricular fibrillation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of 130 patients, 42 had wild\u2010type disease (wtATTR) and 88 had hereditary variants (hATTR), most commonly Val122Ile (89%). At ATTR\u2010CM diagnosis, 80 (62%) patients had EF \u2264 40% consistent with systolic heart failure. Of the 69 (53%) patients with documented VAs significantly higher rates occurred among those with EF \u2264 40% compared with EF\u00a0&gt;\u00a040% (67%\u00a0vs. 28%, <jats:italic>p<\/jats:italic>\u00a0=\u00a0.001). Thirty\u2010two patients (25 hATTR, 7 wtATTR) had primary prevention ICDs implanted. Eight (25%) of these patients received appropriate ICD therapy while two (6%) experienced inappropriate therapy. Comparing patients with EF \u2264 35% with and without ICDs did not reveal any survival difference (3.3 \u00b1 0.5\u00a0vs. 2.8 \u00b1 0.4 years, <jats:italic>p<\/jats:italic>\u00a0=\u00a0.699).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>High rates of VAs and appropriate ICD therapy were found among a unique cohort of largely hereditary ATTR\u2010CM patients with a high rate of systolic heart failure.<\/jats:p><\/jats:sec>","DOI":"10.1111\/pace.14458","type":"journal-article","created":{"date-parts":[[2022,3,8]],"date-time":"2022-03-08T06:42:56Z","timestamp":1646721776000},"page":"443-451","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["Ventricular arrhythmia burden and implantable cardioverter\u2010defibrillator outcomes in transthyretin cardiac amyloidosis"],"prefix":"10.1111","volume":"45","author":[{"given":"Matthew T.","family":"Brown","sequence":"first","affiliation":[{"name":"Division of Cardiology, Department of Medicine Emory University  Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sreeram","family":"Yalamanchili","sequence":"additional","affiliation":[{"name":"Department of Medicine East Carolina University  Greenville North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sean T.","family":"Evans","sequence":"additional","affiliation":[{"name":"Department of Medicine Emory University  Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pradhum","family":"Ram","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine Emory University  Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evan A.","family":"Blank","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine Emory University  Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melissa A.","family":"Lyle","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine Mayo Clinic  Jacksonville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Faisal M.","family":"Merchant","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine Emory University  Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kunal N.","family":"Bhatt","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine Emory University  Atlanta Georgia 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