在第三级转诊中心的儿科左心室非紧缩症中的死亡率和突然死亡率
Samuel T Brescia1, Joseph W Rossano, Ricardo Pignatelli
1Texas Children's Hospital/Section of Pediatric Cardiology, 6621 Fannin, Houston, TX 77030, USA. jjkim@texaschildrens.org
Circulation
|May 2, 2013
概括
儿童的左心室非紧缩 (LVNC) 死亡率很高,通常与心律失常和心脏功能障碍有关. 儿科LVNC患者的正常心脏功能和尺寸表明突然心脏死亡风险低.
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:
- 左心室非紧缩 (LVNC) 是一种罕见的心肌病.
- 它的特点是过度的左心室轨道,心肌功能障碍和高死亡率.
- LVNC的临床表现不同,包括心律失常和心脏突然死亡.
研究的目的:
- 调查诊断为孤立左心室非紧缩的儿童的临床特征和结果.
- 在这个儿科群体中确定与死亡率和突然心脏病死亡相关的因素.
主要方法:
- 在1990年1月至2009年1月期间诊断出左心室孤立非紧缩 (LVNC) 的儿科患者的回顾性评估.
- 排除患有先天性心脏病变的患者.
- 分析临床数据,包括心脏功能,心电图异常,心律失常和死亡率.
主要成果:
- 242名儿童被诊断出患有孤立的LVNC.
- 死亡率为12.8%,5.4%的人接受了移植.
- 心脏功能障碍 (62%) 和心律失常 (33.1%) 与死亡率的增加密切相关.
- 在87%的患者中存在心电图异常,再极化异常与较高的死亡率有关.
- 突然心脏死亡发生在6.2%的患者中,主要是心脏尺寸异常或功能障碍患者.
结论:
- 左心室非紧缩 (LVNC) 在儿童中具有很高的死亡率.
- 心脏功能障碍和心室节律失常是儿科LVNC中死亡率的显著预测因素.
- 儿童心脏尺寸和功能正常,没有先前的心律失常,突然死亡的风险很低.
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