节律失常可能隐藏在儿童左心室高血压的遗传性心肌病:一个单一中心的经验
Irma Battipaglia1, Nicoletta Cantarutti1, Marianna Cicenia1
1Pediatric Cardiology and Cardiac Arrhythmias Complex Unit, Bambino Gesù Children's Hospital IRCCS, 00050 Rome, Italy.
Children (Basel, Switzerland)
|October 26, 2024
概括
在儿童中,左心室高血压 (LVHT) 可以在23.6%的病例中引起心律失常,尽管结果通常是好的. 遗传变异与儿科LVHT患者的这些心律失常症状有关.
科学领域:
- 心脏病学 心脏病学
- 儿童心脏病学 儿童心脏病学
- 遗传学 是一个遗传学.
背景情况:
- 左心室高血压 (LVHT) 是一种心肌疾病,临床表现各不相同,从无症状到严重的心力衰竭.
- 区分良性和病态形式的LVHT仍然是一个临床挑战.
- 心律失常,血栓塞栓事件和心脏突然死亡 (SCD) 是LVHT的潜在并发症.
研究的目的:
- 在患有LVHT的大型儿科队列中调查心律失常症状的流行率和类型.
- 在患有LVHT的儿科患者中,将心律失常事件与遗传发现和其他临床标记相关联.
- 评估诊断为LVHT的儿科患者的临床结果.
主要方法:
- 在2013年至2023年期间诊断出LVHT的140名儿科患者的回顾性分析.
- 收集关于家族病史,仪器检查,心脏磁共振成像和遗传检测的数据.
- 对临床结果的评估,包括心律失常的发生情况和对诸如植入式心脏转换器-除器 (ICD) 等干预措施的需要.
主要成果:
- 在33名 (23.6%) 儿科患者中发现了心律失常症,包括脑上脉动节律失常症,心室心律失常症 (VT,VF,PVCs),鼻节功能障碍和心室塞 (AVB).
- 在心律不整和4级或5级遗传变异 (p = 0.037) 的存在之间发现了显著的关联.
- 三名患者需要植入式心脏转换器-除器 (ICD);没有报告突然心脏死亡 (SCD) 的病例.
结论:
- 心律失常是患有LVHT的儿科患者的一个重大问题,在近四分之一的队列中发生.
- 遗传变异可能在儿科LVHT中节律失常的发展中发挥作用.
- 尽管心律不整的发病率很高,但这一儿科队列的整体结果是有利的,没有报告SCD.
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