在儿童中识别致病性PVC:多焦点外宫皮肤炎相关的过早收缩病例
Lane McLendon1, Akash Patel1, Peter Aziz1
1Department of Heart, Vascular & Thoracic, Division of Cardiology & Cardiovascular Medicine, Children's Institute, Cleveland Clinic Children's, Cleveland, Ohio, USA.
这一案例突出显示了一名儿科患者的多焦点异位子宫外Purkinje相关过早收缩 (MEPPC). 弗莱凯尼德有效地治疗了心室节律失常,并改善了心脏功能.
科学领域:
- 儿童心脏病学 儿童心脏病学
- 临床电生理学 临床电生理学
- 遗传学 遗传学 是一个
背景情况:
- 过早心室收缩 (PVC) 在儿童中通常是良性的,但可以表明严重的心脏问题.
- 家庭史突发心脏病死亡 (SCD) 和心肌病引起人们对遗传性疾病的担忧.
研究的目的:
- 提出一个儿科病例的多焦点宫外Purkinje相关的过早收缩 (MEPPC).
- 为了说明识别致病性PVC及其与遗传因素的潜在联系的重要性.
- 为了证明 flecainide 在治疗 MEPPC 的有效性.
主要方法:
- 一个13岁的女性病例报告,患有频繁的PVCs,过早的结节收缩和非持续的腹腔动脉冲动.
- 诊断工具包括心电图,24小时霍尔特监测和心声回声图.
- 对一个家庭成员的基因检测显示,SCN5A.中的VUS是SCN5A.
- 治疗涉及启动弗莱卡因尼德.
主要成果:
- 患者经常出现多形PVC和NSVT,荷尔特监测负担为48%.
- 心声图显示左心室功能中度抑郁.
- 弗莱卡尼德治疗导致心室心律失常症在几天内得到解决.
- 重复回声心电图显示心脏功能有所改善.
结论:
- 在患有频繁PVC和令人担忧的家族病史的儿科患者中,应考虑MEPPC.
- 弗莱卡尼德可以作为儿科患者MEPPC的有效治疗方法.
- 早期识别和适当的治疗对于预防心脏不良事件至关重要.
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