关键词: CARDIOMYOPATHY Case report Hereditary Transthyretin amyloidosis Ventricular tachycardia

来  源:   DOI:10.1093/ehjcr/ytae273   PDF(Pubmed)

Abstract:
UNASSIGNED: Previous literature suggests that patients with transthyretin amyloidosis (ATTR) experience a high burden of ventricular arrhythmias. Despite this evidence, optimal strategies for arrhythmia prevention and treatment remain subject to debate.
UNASSIGNED: We report the case of a patient with hereditary ATTR cardiomyopathy who developed recurrent ventricular tachycardia prior to a decline in his left ventricular ejection fraction (LVEF). Although he ultimately received an intracardiac device (ICD) for secondary prevention of ventricular tachycardia, his clinical course begets the question of whether more aggressive arrhythmia prevention upfront could have prevented his global functional decline.
UNASSIGNED: Given the advent of new disease-modifying therapies for ATTR, it is imperative to reconsider antiarrhythmic strategies in these patients. New decision tools are needed to decide what additional parameters (beyond LVEF ≤ 35%) may warrant ICD placement for primary prevention of ventricular arrhythmias in these patients.
摘要:
先前的文献表明,甲状腺素运载蛋白淀粉样变性(ATTR)患者的室性心律失常负担很高。尽管有这些证据,心律失常预防和治疗的最佳策略仍存在争议。
我们报告了一例遗传性ATTR心肌病患者,在左心室射血分数(LVEF)下降之前发生了复发性室性心动过速。尽管他最终接受了用于二级预防室性心动过速的心内装置(ICD),他的临床课程引发了一个问题,即早期更积极的心律失常预防是否可以防止他的全球功能下降。
鉴于ATTR新的疾病修饰疗法的出现,必须重新考虑这些患者的抗心律失常策略.需要新的决策工具来决定哪些其他参数(LVEF≤35%)可能需要ICD放置以一级预防这些患者的室性心律失常。
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