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His electrocardiogram demonstrated the presence of premature ventricular contraction with a QRS complex of the right bundle branch block type and superior axis. His echocardiography showed systolic dysfunction of the LV (LV ejection fraction, 44.6%). Cardiac computed tomography imaging revealed banded and patchy densities observed frequently from the middle to epicardial layer of the LV wall. Cardiac magnetic resonance imaging showed fat signals on fat-selective images and late gadolinium enhancement in the mid-wall to subepicardial layers in the LV myocardium. Endomyocardial biopsy revealed the histological presence of fibrofatty replacement. A genetic analysis revealed a nonsense mutation in the desmoplakin gene. Thus, he was diagnosed with left-dominant arrhythmogenic cardiomyopathy. To prevent fatal ventricular arrhythmias, an implantable cardioverter defibrillator was successfully implanted.<\/jats:p>","DOI":"10.1002\/ehf2.12790","type":"journal-article","created":{"date-parts":[[2020,6,27]],"date-time":"2020-06-27T08:50:11Z","timestamp":1593247811000},"page":"3174-3178","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Left-Dominant Arrhythmogenic Cardiomyopathy with a Nonsense Mutation in\n                    <i>DSP<\/i>"],"prefix":"10.1093","volume":"7","author":[{"given":"Yuichiro","family":"Tsuruta","sequence":"first","affiliation":[{"name":"Department of Cardiovascular Medicine and Diagnostic Radiology, Faculty of Life Sciences, Graduate School of Medical Sciences, Kumamoto University , 1-1-1, Honjo, Chuo-ku, Kumamoto, 860-8556 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