{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,22]],"date-time":"2026-02-22T01:36:56Z","timestamp":1771724216518,"version":"3.50.1"},"reference-count":17,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2021,10,12]],"date-time":"2021-10-12T00:00:00Z","timestamp":1633996800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,11,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Hereditary or variant transthyretin amyloidosis (ATTRv) is a progressive disease manifesting with neuropathy and\/or cardiomyopathy. An early and accurate diagnosis of cardiac amyloidosis is a pre-requisite for timely and appropriate patient management, including anti-amyloid therapies, as it is associated with heart failure, conduction disease, and arrhythmias, leading to reduced quality of life and early death.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Case Summary<\/jats:title>\n                  <jats:p>We present the case of an ATTRv male patient presenting with a mixed amyloidosis phenotype (neuropathy and cardiomyopathy). Cardiac disease manifestation comprised tachyarrhythmias (atrial fibrillation) and conduction abnormalities (atrio-ventricular block) in addition to segmental left ventricular (LV) hypertrophy (septal wall) due to regionally pronounced amyloid deposits in the basal LV myocardium. Interestingly, by means of serial cardiovascular magnetic resonance (CMR) studies, we were able to demonstrate an impressive and unexpected improvement of cardiomyopathy findings within a relatively short period-of-time after the implementation of genome-silencer therapies.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Discussion<\/jats:title>\n                  <jats:p>This is our second case report that showed ATTRv cardiomyopathy reversal under anti-amyloid therapy\u2014documented by multi-parametric CMR. Our findings support the hypothesis that amyloid infiltration leading to cardiomyopathy is not an irreversible pathological process\u2014but rather a dynamic one, that cannot only be stopped but even reversed (to a certain degree) by currently emerging anti-amyloid therapies. Moreover, the role of serial multi-parametric CMR imaging for surveillance of cardiomyopathy dynamics under these therapies is nicely illustrated.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ehjcr\/ytab415","type":"journal-article","created":{"date-parts":[[2021,10,17]],"date-time":"2021-10-17T20:21:30Z","timestamp":1634502090000},"source":"Crossref","is-referenced-by-count":3,"title":["Genome silencer therapy leading to \u2018regression\u2019 of cardiac amyloid load on cardiovascular magnetic resonance: a case report"],"prefix":"10.1093","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5142-7686","authenticated-orcid":false,"given":"Anca","family":"Florian","sequence":"first","affiliation":[{"name":"Department of Cardiology I, Division of Cardiovascular Imaging, University Hospital M\u00fcnster, Albert-Schweitzer-Campus 1, Building A1, 48149 M\u00fcnster, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael","family":"Bietenbeck","sequence":"additional","affiliation":[{"name":"Department of Cardiology I, Division of Cardiovascular Imaging, University Hospital M\u00fcnster, Albert-Schweitzer-Campus 1, Building A1, 48149 M\u00fcnster, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna","family":"H\u00fcsing-Kabar","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology\/Hepatology, University Hospital M\u00fcnster, M\u00fcnster, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matthias","family":"Schilling","sequence":"additional","affiliation":[{"name":"Department of Neurology with Institute of Translational Neurology, University Hospital M\u00fcnster, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hartmut H","family":"Schmidt","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology\/Hepatology, University Hospital M\u00fcnster, M\u00fcnster, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ali","family":"Yilmaz","sequence":"additional","affiliation":[{"name":"Department of Cardiology I, Division of Cardiovascular Imaging, University Hospital M\u00fcnster, Albert-Schweitzer-Campus 1, Building A1, 48149 M\u00fcnster, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2021,10,12]]},"reference":[{"key":"2021110806004620300_ytab415-B1","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1038\/s41582-019-0210-4","article-title":"Hereditary transthyretin amyloidosis: a model of medical progress for a fatal disease","volume":"15","author":"Adams","year":"2019","journal-title":"Nat Rev Neurol"},{"key":"2021110806004620300_ytab415-B2","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1161\/CIRCULATIONAHA.118.038169","article-title":"Natural history, quality 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