Non-ischemic scar underlines ventricular arrhythmias in Kearns-Sayre Syndrome.

IF 0.5 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS
S. Figliozzi, A. Zorzi, M. P. Marra, A. Ruocco, S. Iliceto, D. Corrado, C. Calore
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引用次数: 0

Abstract

Kearns-Sayre Syndrome (KSS) is a rare, mitochondrial disease in which cardiac involvement has been associated with poor prognosis. Although the most common clinical manifestation is progressive conduction system impairment, patients can suffer from ventricular arrhythmias. Yet, they show a high prevalence of sudden cardiac death, whose etiopathological mechanism is not completely understood. Cardiac magnetic resonance (CMR) is a rising tool to detect subclinical heart involvement in many heart diseases and was recently able to detect non-ischemic scar in patients affected by KSS.   We present a case of a 44 years old patient affected by KSS with a long-standing history of ventricular arrhythmias. Despite electrocardiogram and echocardiogram were normal, CMR showed non-ischemic myocardial scar in the basal-mid lateral wall. Ventricular ectopic beats were assumed to origin from this site, according to their morphology. Our case highlights non-ischemic myocardial scar as a possible etiopathological mechanism of ventricular arrhythmias in patients with KSS. The added value of CMR to provide cardiac involvement identification and risk stratification of these patients is also shown.
Kearns-Sayre综合征的非缺血性瘢痕突出了室性心律失常。
Kearns-Sayre综合征(KSS)是一种罕见的线粒体疾病,心脏受累与预后不良有关。尽管最常见的临床表现是进行性传导系统损伤,但患者也可能出现室性心律失常。然而,他们显示心脏性猝死的发病率很高,其发病机制尚不完全清楚。心脏磁共振(CMR)是检测许多心脏病亚临床心脏受累的一种新兴工具,最近能够检测KSS患者的非缺血性瘢痕。我们报告了一例44岁的KSS患者,该患者长期有室性心律失常病史。尽管心电图和超声心动图正常,CMR显示基底中外侧壁有非缺血性心肌瘢痕。根据其形态,假定心室异位搏动起源于该部位。我们的病例强调非缺血性心肌瘢痕可能是KSS患者室性心律失常的病理机制。还显示了CMR对这些患者的心脏受累识别和风险分层的附加价值。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Cardiogenetics
Cardiogenetics CARDIAC & CARDIOVASCULAR SYSTEMS-
自引率
0.00%
发文量
26
审稿时长
11 weeks
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