Incremental Value of Cardiac Magnetic Resonance Imaging in Arrhythmic Risk Stratification of Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy-Associated Desmosomal Mutation Carriers

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

卷:62

Article

te Riele, Anneline S. J. M.¹  Bhonsale, Aditya²  James, Cynthia A.²  Rastegar, Neda³  Murray, Brittney²  Burt, Jeremy R.³  Tichnell, Crystal²  Madhavan, Srinivasa²  Judge, Daniel P.²  Bluemke, David A.^3,4  Zimmerman, Stefan L.³  Kamel, Ihab R.³  Calkins, Hugh²  Tandri, Harikrishna² 

[1] Univ Med Ctr Utrecht, Dept Med, Div Cardiol, Utrecht, Netherlands

[2] Johns Hopkins Univ, Sch Med, Dept Med, Div Cardiol, Baltimore, MD 21205 USA

[3] Johns Hopkins Univ, Sch Med, Dept Radiol, Baltimore, MD 21205 USA

[4] NIH, Ctr Clin, Bethesda, MD 20892 USA

关键词: cardiomyopathy;    electrocardiography;    magnetic resonance imaging;    risk stratification;    tachyarrhythmias;   

DOI  :  10.1016/j.jacc.2012.11.087

来源: Elsevier

【 摘 要 】

Objectives The aim of this study was to identify the incremental value and optimal role of cardiac magnetic resonance (CMR) imaging in arrhythmic risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C)associated desmosomal mutation carriers without histories of sustained ventricular arrhythmia. Background Risk stratification of ARVD/C mutation carriers is challenging. Methods Sixty-nine patients (mean age 27.0 +/- 15.3 years, 42% men) harboring ARVD/C-associated pathogenic mutations (83% plakophilin 2) without prior sustained ventricular arrhythmias were included. Electrocardiographic and 24-h Holter monitoring findings closest to presentation were analyzed for electrical abnormalities per revised task force criteria. CMR studies were done to identify abnormal cardiac structure and function according to the revised task force criteria. Results Overall, 42 patients (61%) presented with electrical abnormalities on the basis of electrocardiography and Holter monitoring, of whom 20 (48%) had abnormal results on CMR. Only 1 of 27 patients (4%) without electrical abnormalities at initial evaluation had abnormal CMR results. Over a mean follow-up period of 5.8 +/- 4.4 years, 11 patients (16%) experienced sustained ventricular arrhythmias, exclusively in patients with both electrical abnormalities (electrocardiography and/or Holter monitoring) and abnormal CMR results. Conclusions These results suggest that electrical abnormalities on electrocardiography and Holter monitoring precede detectable structural abnormalities in ARVD/C mutation carriers. Therefore, evaluation of cardiac structure and function using CMR is probably not necessary in the absence of baseline electrical abnormalities. Among ARVD/C mutation carriers, the presence of both electrical and CMR abnormalities identifies patients at high risk for events and thus patients who might benefit from prophylactic implantable cardioverter-defibrillator placement. (C) 2013 by the American College of Cardiology Foundation

【 授权许可】

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