PMID- 31953454 OWN - NLM STAT- MEDLINE DCOM- 20201111 LR - 20210116 IS - 2045-2322 (Electronic) IS - 2045-2322 (Linking) VI - 10 IP - 1 DP - 2020 Jan 17 TI - Arrhythmogenic Left Ventricular Cardiomyopathy: A Clinical and CMR Study. PG - 533 LID - 10.1038/s41598-019-57203-2 [doi] LID - 533 AB - The clinical features, CMR characteristics and outcomes of arrhythmogenic left ventricular cardiomyopathy (ALVC), which is a very rare nonischemic cardiomyopathy, are currently not well studied. The purpose of the study is to investigate the clinical and cardiovascular magnetic resonance (CMR) imaging characteristics of arrhythmogenic left ventricular cardiomyopathy (ALVC). Fifty-three consecutive patients with ALVC were divided into two groups: ALVC patients without right ventricular (RV) involvement (n = 36, group 1) and those with RV involvement (n = 17, group 2). Clinical symptoms, cardiac electrophysiological findings, and CMR parameters (morphology, ventricular function, and myocardial fibrosis and fatty infiltration) were evaluated in both groups. The two groups showed no significant difference in age, gender, or presenting symptoms (P > 0.05). Right bundle branch block ventricular arrhythmia was less common in patients without RV involvement (50.0% vs.64.7%, P = 0.031). There were no significant differences in left ventricular function between the two groups, however right ventricular ejection fraction was significantly lower in group 2 (40.1 +/- 4.0% vs. 48.7 +/- 3.9%, P < 0.001). Inverse correlations of left ventricular ejection fraction with fat volume (r = -0.883, p = 0.001), late gadolinium enhancement (LGE) volume (r = -0.892, 0.013), ratio of fat/LGE (r = -0.848, p < 0.001), indexed left ventricular end diastolic volume (r = -0.877, p < 0.001) and indexed left ventricular end systolic volume (r = -0.943, p < 0.001) were all significant. ALVC is a rare disease with fibro-fatty replacement predominantly in the left ventricle, impaired left ventricular systolic function, and ventricular arrhythmias originating from the left ventricle. ALVC with right ventricular involvement may have a worse prognosis. FAU - He, Jian AU - He J AD - Department of Magnetic Resonance Imaging, Fuwai Hospital, National Center for Cardiovascular Diseases, State Key Laboratory of Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. FAU - Xu, Jing AU - Xu J AD - Department of Magnetic Resonance Imaging, Fuwai Hospital, National Center for Cardiovascular Diseases, State Key Laboratory of Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. FAU - Li, Guozhong AU - Li G AD - Department of Radiology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei, China. FAU - Zhou, Di AU - Zhou D AD - Department of Magnetic Resonance Imaging, Fuwai Hospital, National Center for Cardiovascular Diseases, State Key Laboratory of Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. FAU - Li, Shuang AU - Li S AD - Department of Magnetic Resonance Imaging, Fuwai Hospital, National Center for Cardiovascular Diseases, State Key Laboratory of Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. FAU - Zhuang, Baiyan AU - Zhuang B AD - Department of Magnetic Resonance Imaging, Fuwai Hospital, National Center for Cardiovascular Diseases, State Key Laboratory of Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. FAU - Chen, Xiuyu AU - Chen X AD - Department of Magnetic Resonance Imaging, Fuwai Hospital, National Center for Cardiovascular Diseases, State Key Laboratory of Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. AD - Key Laboratory of Cardiovascular Imaging, Chinese Academy of Medical Sciences, Beijing, China. FAU - Duan, Xuejin AU - Duan X AD - Department of Pathology, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. FAU - Li, Li AU - Li L AD - Department of Pathology, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. FAU - Fan, Xiaohan AU - Fan X AD - Department of Cardiology, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. FAU - Huang, Jinghan AU - Huang J AD - The Heart-lung Testing Center, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. FAU - Yin, Gang AU - Yin G AD - Department of Magnetic Resonance Imaging, Fuwai Hospital, National Center for Cardiovascular Diseases, State Key Laboratory of Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. AD - Key Laboratory of Cardiovascular Imaging, Chinese Academy of Medical Sciences, Beijing, China. FAU - Jiang, Yong AU - Jiang Y AD - Key Laboratory of Cardiovascular Imaging, Chinese Academy of Medical Sciences, Beijing, China. AD - Department of Echocardiography, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. FAU - Wang, Yang AU - Wang Y AD - Medical Research & Biometrics Center, Cardiovascular Institute and Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. FAU - Zhao, Shihua AU - Zhao S AD - Department of Magnetic Resonance Imaging, Fuwai Hospital, National Center for Cardiovascular Diseases, State Key Laboratory of Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. cjr_zhaoshihua@163.com. FAU - Lu, Minjie AU - Lu M AD - Department of Magnetic Resonance Imaging, Fuwai Hospital, National Center for Cardiovascular Diseases, State Key Laboratory of Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. coolkan@163.com. AD - Key Laboratory of Cardiovascular Imaging, Chinese Academy of Medical Sciences, Beijing, China. coolkan@163.com. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20200117 PL - England TA - Sci Rep JT - Scientific reports JID - 101563288 SB - IM MH - Aged MH - Arrhythmias, Cardiac/*diagnostic imaging MH - Female MH - Heart Ventricles/*diagnostic imaging MH - Humans MH - Image Processing, Computer-Assisted MH - *Magnetic Resonance Imaging MH - Male MH - Middle Aged MH - Observer Variation MH - Prognosis PMC - PMC6969116 COIS- The authors declare no competing interests. EDAT- 2020/01/19 06:00 MHDA- 2020/11/12 06:00 PMCR- 2020/01/17 CRDT- 2020/01/19 06:00 PHST- 2019/08/29 00:00 [received] PHST- 2019/12/23 00:00 [accepted] PHST- 2020/01/19 06:00 [entrez] PHST- 2020/01/19 06:00 [pubmed] PHST- 2020/11/12 06:00 [medline] PHST- 2020/01/17 00:00 [pmc-release] AID - 10.1038/s41598-019-57203-2 [pii] AID - 57203 [pii] AID - 10.1038/s41598-019-57203-2 [doi] PST - epublish SO - Sci Rep. 2020 Jan 17;10(1):533. doi: 10.1038/s41598-019-57203-2.