The role of cardiac magnetic resonance imaging in the evaluation of malignant ventricular arrhythmias in Brugada syndrome.
The role of cardiac magnetic resonance imaging in the evaluation of malignant ventricular arrhythmias in Brugada syndrome.
复制标题
DOI：
10.1016/j.hrcr.2023.10.014
复制
发表时间：
2024-01
影响因子：
--
通讯作者：
Ashwath, Mahi L
中科院分区：
文献类型：
--
作者：
Frederiksen, Hunter;Prathivadhi-Bhayankaram, Sruti;London, Barry;Ashwath, Mahi L
布鲁格达综合征是一种遗传性疾病，与室性心律失常相关的心源性猝死风险增加有关。 1, 2, 3 初步研究报告晕厥或心源性猝死的发生率为 17% 至 42%。尽管通常被认为是通道病，但在这种疾病的组织病理学和磁共振成像中已报道了微妙的结构变化。这包括纤维化、脂肪浸润和伴有胶原沉积的炎症浸润。 2, 4我们介绍了一名年轻患者的病例，该患者已知因 SCN5A 突变而患有 Brugada 综合征，该患者在运动时出现心脏骤停和心律失常，并被发现患有非缺血性心肌病。该病例明确讨论了 Brugada 综合征的病理生理学和诊断、诊断为 Brugada 综合征的患者的推荐护理和管理，以及心脏磁共振成像 (cMRI) 在这些患者中的作用。
Brugada syndrome is a genetic disorder associated with an increased risk of sudden cardiac death related to ventricular arrhythmia. 1, 2, 3 Initial studies reported rates of syncope or sudden cardiac death ranging from 17% to 42%. Although typically considered channelopathy, subtle structural changes have been reported in this disease’s histopathology and magnetic resonance imaging. This includes fibrosis, fatty infiltration, and inflammatory infiltrates with collagen deposition. 2, 4We present a case of a young patient with a known history of Brugada syndrome from an SCN5A mutation who suffered a cardiac arrest and arrhythmias with exercise and was found to have a nonischemic cardiomyopathy. The case discusses explicitly the pathophysiology and diagnosis of Brugada syndrome, recommended care and management in patients diagnosed with Brugada syndrome, and the role of cardiac magnetic resonance imaging (cMRI) in these patients.