Arrhythmogenic left ventricular cardiomyopathy

被引:4
作者
Mirmomen, Seyedeh Mojdeh [1 ]
Bradley, Andrew Jay [1 ]
Arai, Andrew Ernest [1 ]
Sirajuddin, Arlene [1 ]
机构
[1] NHLBI, NIH, Bldg 10, Bethesda, MD 20892 USA
来源
BJR CASE REPORTS | 2020年 / 6卷 / 01期
关键词
DILATED CARDIOMYOPATHY; MYOCARDIAL FAT; MUTATION;
D O I
10.1259/bjrcr.20190079
中图分类号
R8 [特种医学]; R445 [影像诊断学];
学科分类号
1002 ; 100207 ; 1009 ;
摘要
Arrhythmogenic ventricular cardiomyopathy AVC is a heritable heart muscle disorder characterized by fibrofatty infiltration of the myocardium. Intramyocardial fat deposition is considered arrhythmogenic and predisposes patients to life-threatening arrhythmias and sudden cardiac death. The classic subtype of AVC is characterized by fibrofatty replacement of the right ventricular myocardium (i.e. arrhythmogenic right ventricular cardiomyopathy). In advanced cases of arrhythmogenic right ventricular cardiomyopathy, the Ieft ventricle may be involved as well. Predominantly left ventricular involvement by AVC is exceedingly rare and lack of specific diagnostic criteria as well as its potential cardiotoxic effect make its diagnosis challenging and of high importance.
引用
收藏
页数:4
相关论文
共 18 条
[11]   Desmoplakin truncations and arrhythmogenic left ventricular cardiomyopathy: characterizing a phenotype [J].
Maria Lopez-Ayala, Jose ;
Gomez-Milanes, Ivan ;
Sanchez Munoz, Juan Jose ;
Ruiz-Espejo, Francisco ;
Ortiz, Martin ;
Gonzalez-Carrillo, Josefa ;
Lopez-Cuenca, David ;
Oliva-Sandoval, M. J. ;
Monserrat, Lorenzo ;
Valdes, Mariano ;
Gimeno, Juan R. .
EUROPACE, 2014, 16 (12) :1838-1846
[12]   Cardiac involvement in Duchenne and Becker muscular dystrophy [J].
Mavrogeni, Sophie ;
Markousis-Mavrogenis, George ;
Papavasiliou, Antigoni ;
Kolovou, Genovefa .
WORLD JOURNAL OF CARDIOLOGY, 2015, 7 (07) :410-414
[13]   Novel mutation in desmoplakin causes arrhythmogenic left ventricular cardiomyopathy [J].
Norman, M ;
Simpson, M ;
Mogensen, J ;
Shaw, A ;
Hughes, S ;
Syrris, P ;
Sen-Chowdhry, S ;
Rowland, E ;
Crosby, A ;
McKenna, WJ .
CIRCULATION, 2005, 112 (05) :636-642
[14]   Arrhythmogenic cardiomyopathy [J].
Pilichou, Kalliopi ;
Thiene, Gaetano ;
Bauce, Barbara ;
Rigato, Ilaria ;
Lazzarini, Elisabetta ;
Migliore, Federico ;
Marra, Martina Perazzolo ;
Rizzo, Stefania ;
Zorzi, Alessandro ;
Daliento, Luciano ;
Corrado, Domenico ;
Basso, Cristina .
ORPHANET JOURNAL OF RARE DISEASES, 2016, 11
[15]   Arrhythmogenic Left Ventricular Cardiomyopathy Suspected by Cardiac Magnetic Resonance Imaging, Confirmed by Identification of a Novel Plakophilin-2 Variant [J].
Saguner, Ardan M. ;
Buchmann, Beate ;
Wyler, Daniel ;
Manka, Robert ;
Gotschy, Alexander ;
Medeiros-Domingo, Argelia ;
Brunckhorst, Corinna ;
Duru, Firat ;
Mayer, Kurt A. .
CIRCULATION, 2015, 132 (06) :E38-E40
[16]   Left-Dominant Arrhythmogenic Cardiomyopathy [J].
Sen-Chowdhry, Srijita ;
Syrris, Petros ;
Prasad, Sanjay K. ;
Hughes, Sian E. ;
Merrifield, Robert ;
Ward, Deirdre ;
Pennell, Dudley J. ;
McKenna, William J. .
JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, 2008, 52 (25) :2175-2187
[17]   Phospholamban R14del mutation in patients diagnosed with dilated cardiomyopathy or arrhythmogenic right ventricular cardiomyopathy: evidence supporting the concept of arrhythmogenic cardiomyopathy [J].
van der Zwaag, Paul A. ;
van Rijsingen, Ingrid A. W. ;
Asimaki, Angeliki ;
Jongbloed, Jan D. H. ;
van Veldhuisen, Dirk J. ;
Wiesfeld, Ans C. P. ;
Cox, Moniek G. P. J. ;
van Lochem, Laura T. ;
de Boer, Rudolf A. ;
Hofstra, Robert M. W. ;
Christiaans, Imke ;
van Spaendonck-Zwarts, Karin Y. ;
Deprez, Ronald H. Lekanne dit ;
Judge, Daniel P. ;
Calkins, Hugh ;
Suurmeijer, Albert J. H. ;
Hauer, Richard N. W. ;
Saffitz, Jeffrey E. ;
Wilde, Arthur A. M. ;
van den Berg, Maarten P. ;
van Tintelen, J. Peter .
EUROPEAN JOURNAL OF HEART FAILURE, 2012, 14 (11) :1199-1207
[18]   CT imaging features and frequency of left ventricular myocardial fat in patients with CT findings of chronic left ventricular myocardial infarction [J].
Zafar, H. M. ;
Litt, H. I. ;
Torigian, D. A. .
CLINICAL RADIOLOGY, 2008, 63 (03) :256-262