A Fasciculoventricular Accessory Pathway Featuring Functional Decremental Conduction and QRS Variability.

IF 3.3 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS
Arrhythmia & Electrophysiology Review Pub Date : 2025-07-21 eCollection Date: 2025-01-01 DOI:10.15420/aer.2025.12
Pin Wang, Yanwei Wang, Chenglong Miao, Lu Xu
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引用次数: 0

Abstract

Fasciculoventricular accessory pathways (FVAPs), once considered rare variants of pre-excitation syndrome, are now recognised as ubiquitous in both humans and murine. Nonetheless, most FVAPs are likely electrically silent. However, they can become evident, as reported for some glycogen storage diseases (such as Danon disease and PRKAG2) and during high-voltage septal pacing. Typically, FVAPs only exhibit antegrade and non-decremental conducting properties. A block at the FVAP results in a normal His-to-ventricle interval and a narrow QRS complex without signs of pre-excitation. Decremental conduction over FVAP was once reported in a setting of PRKAG2 mutation. However, in the present case, incremental atrial pacing revealed varying His-to-ventricle intervals, with functional decremental conduction that was accompanied by varied QRS morphologies. These findings underscore the diagnostic challenges posed by FVAPs, and highlight the need for meticulous electrophysiological assessment to accurately distinguish them from other pre-excitation syndromes. This case exemplifies the nuanced behaviour of FVAPs, emphasising their clinical and diagnostic complexity in electrophysiological practice.

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以功能性递减传导和QRS变异性为特征的束状室副通路。
束状室副通路(FVAPs),曾经被认为是预兴奋综合征的罕见变体,现在被认为在人类和小鼠中普遍存在。尽管如此,大多数fvap可能是无声的。然而,在一些糖原储存疾病(如Danon病和PRKAG2)和高压间隔起搏期间,它们可能变得明显。通常,FVAPs仅表现出顺行和非递减的导电特性。FVAP阻滞导致心室间隔正常,QRS复合体窄,无预兴奋迹象。在PRKAG2突变的情况下,曾报道过FVAP上的递减传导。然而,在本病例中,增量心房起搏显示了不同的心室间隔,伴随着不同的QRS形态的功能性递减传导。这些发现强调了fvap带来的诊断挑战,并强调了细致的电生理评估以准确区分它们与其他预兴奋综合征的必要性。本病例体现了fvap的微妙行为,强调了其在电生理实践中的临床和诊断复杂性。
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来源期刊
Arrhythmia & Electrophysiology Review
Arrhythmia & Electrophysiology Review CARDIAC & CARDIOVASCULAR SYSTEMS-
CiteScore
5.10
自引率
6.70%
发文量
22
审稿时长
7 weeks
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