在年轻人中增高性心肌病和心室预兴奋:原因和辅助途径特征
Robert Przybylski1, Sakethram Saravu Vijayashankar1, Edward T O'Leary1
1Department of Cardiology, Boston Children's Hospital, Harvard Medical School, MA.
Circulation. Arrhythmia and electrophysiology
|October 25, 2023
概括
患有多变性心肌病 (HCM) 和预兴奋的年轻患者往往有潜在的储存或代谢疾病. 标准的心电图无法可靠地区分危险的辅助路径和良性纤维.
科学领域:
- 心脏病学 心脏病学
- 电子生理学 电子生理学
- 儿童心脏病学 儿童心脏病学
背景情况:
- 在年轻人中,多变性心肌病 (HCM) 有多种不同的原因.
- 在HCM中已知心室预激发,但其与特定原因和辅助通路 (AP) 特征的联系尚不清楚.
研究的目的:
- 调查HCM年轻患者预兴奋的原因和电生理学特征.
- 在这个群体中,要区分真正的辅助通路 (AP) 和带心纤维 (FVF).
主要方法:
- 对21岁以下患有HCM和预兴奋症的患者进行回顾性队列研究 (2000-2022年).
- HCM的原因分类:孤立的,储存障碍,代谢疾病,遗传综合征.
- 侵入性电生理学研究区分了真实AP和FVF,根据导电特性评估AP风险.
主要成果:
- 在345名HCM患者中,28%的患者出现了预兴奋,经常与储存或代谢障碍有关.
- 电生理学确定了23个真正的AP和16个FVF;36%的预兴奋病例是FVF介导的.
- 5名患者有高风险的AP;心电图无法区分AP和FVF.
结论:
- 患有HCM和预兴奋的年轻患者经常有潜在的储存或代谢疾病.
- 具有较大的QRS/delta波的非隔离性HCM表明这些病因.
- 表面心电图不足以区分良性FVF和可能危及生命的AP.
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