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在患有德斯莫普拉金心肌病症的患者中进行静脉动脉移除

Michael Ghannam¹, Jackson J Liang¹, Jarieke Hoogendoorn²

¹Section of Electrophysiology, Division of Cardiovascular Medicine, Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan, USA.

JACC. Clinical electrophysiology

|January 11, 2024

概括

在患有Desmoplakin (DSP) 心肌病的患者中,心室动脉障碍 (VT) 切除是复杂的,通常需要双心室和上心脏的方法. 虽然出现复发,但大多数患者在重复手术后达到长期无静脉瘤状态.

科学领域:

心脏病学心脏病学
遗传学是一个遗传学.
电子生理学电子生理学

背景情况:

德斯莫普拉金 (DSP) 致病变体导致心律失常性心肌病,经常影响两个心室.
腹腔动脉冲动 (VT) 移除是一种潜在的治疗方法,但程序细节没有得到充分记录.

研究的目的:

报告在患有 DSP 病原变异的患者中进行静脉瘤切除的多中心经验.
在这个特定的患者群体中分析静脉管切除的程序特征和结果.

主要方法:

在3个国家的6个中心对VT废除的回顾性分析.
包括已知DSP致病变体和症状VTs的患者.
详细报告患者特征,程序目标 (内心/表心,RV/LV) 和结果.

主要成果:

20名患者接受了静脉管切除,其中16人需要进行心表手术.
静脉瘤的目标部位是多样化的,包括RV内心,RV上心,LV内心和LV上心.
在11名患者中观察到静脉瘤复发,需要在9名患者中重复切除.
在20名患者中,有19名患者在经过18个月的中位随访后仍然没有静脉瘤,并考虑了多次手术.

结论:

在DSP心肌病症中,静脉管切除通常涉及由于疾病分布的双心室和上心室方法.
静脉动脉基质与冠状动脉的接近程度可能会限制切除成功.
尽管复发,但重复的切除有助于大多数患者的长期静脉瘤控制.

关键词:

这就是VT的废除.节律失常性心肌病变性心脏病变症德斯莫普拉金基因突变

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