致心律失常性右室心肌病/发育异常：一种更新的影像学检查方法

Arrhythmogenic right ventricular cardiomyopathy/dysplasia: an updated imaging approach.

作者信息

Zimmerman Stefan L

机构信息

The Russell H. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins University School of Medicine, 601 North Caroline Street, JHOC 3142, Box 0818, Baltimore, MD 21287, USA.

出版信息

Magn Reson Imaging Clin N Am. 2015 Feb;23(1):69-79. doi: 10.1016/j.mric.2014.09.001. Epub 2014 Oct 7.

DOI:10.1016/j.mric.2014.09.001

PMID:25476675

Abstract

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a rare inherited cardiomyopathy characterized by fibrofatty replacement of the right ventricular myocardium and risk of sudden death from ventricular tachyarrhythmias. Cardiac magnetic resonance (MR) imaging plays an important role in the diagnostic evaluation of patients and family members suspected of having ARVC/D. This article discusses the epidemiology and pathophysiology of ARVC/D, reviews typical MR imaging findings and diagnostic criteria, and summarizes potential pitfalls in the MR imaging evaluation of patients suspected of having ARVC/D.

摘要

致心律失常性右室心肌病/发育异常（ARVC/D）是一种罕见的遗传性心肌病，其特征为右心室心肌被纤维脂肪组织替代，并存在因室性快速心律失常导致猝死的风险。心脏磁共振（MR）成像在疑似患有ARVC/D的患者及其家庭成员的诊断评估中起着重要作用。本文讨论了ARVC/D的流行病学和病理生理学，回顾了典型的MR成像表现和诊断标准，并总结了疑似患有ARVC/D患者的MR成像评估中可能存在的陷阱。