Mortality in myotonic dystrophy patients in the area of prophylactic pacing devices
Mortality in myotonic dystrophy patients in the area of prophylactic pacing devices
复制标题
DOI：
10.1016/j.ijcard.2010.02.029
复制
发表时间：
2011-07-01
影响因子：
3.5
通讯作者：
Babuty, Dominique
中科院分区：
文献类型：
--
作者：
Laurent, Valerie;Pellieux, Sybille;Babuty, Dominique
目的：我们的研究旨在确定在HV间期>= 70 ms的MD患者中植入预防性起搏器是否能降低猝死的风险，猝死可能主要是由于完全性房室传导阻滞。背景：强直性肌营养不良（MD）患者的猝死发生率高于对照人群。研究方法：从1994年至2008年，100例患者连续入组，其中49人植入。结果：在平均随访74 +/- 39个月，10例死亡。9人死于呼吸衰竭。仅发生1例猝死，而根据Groh等人的标准，46%的患者被认为存在猝死风险[5]。猝死发生率仅为0.2/100患者年。1例患者发生阵发性晕厥持续性室性心动过速。结论：根据Groh标准确定为猝死风险的MD患者预防性植入PM可降低猝死的发生率。植入的MD患者中的1例猝死表明，起搏器植入可能无法完全阻止该事件。室性心律失常可能参与MD患者的猝死，在这种情况下，植入心脏除颤器可能是指征。(C)2010爱思唯尔爱尔兰有限公司版权所有。
Objectives: Our study purports to determine whether implantation of a prophylactic pacemaker in MD patients with HV interval >= 70 ms lowers the risk of sudden death, which may be essentially due to complete atrioventricular block.Background: Sudden death occurs more frequently in patients with myotonic dystrophy (MD) than in the control population. Methods: From 1994 to 2008, 100 consecutive patients were enrolled, 49 of whom were implanted.Results: During an average follow-up of 74 +/- 39 months, 10 deaths occurred. Nine were due to respiratory failure. Only one sudden death occurred, whereas 46% of patients were considered at risk of sudden death according to the criteria of Groh et al. [5]. The incidence rate of sudden death was only 0.2 per 100 patient-years. One patient developed a paroxysmal syncopal sustained ventricular tachycardia.Conclusions: The prophylactic implantation of PM in MD patients who are identified as being at risk of sudden death according to Groh's criteria reduced the incidence rate of sudden death. The one sudden death in an implanted MD patient suggests the likelihood that pacemaker implantation did not totally forestall this event. Ventricular arrhythmias may be involved in the sudden deaths in MD patients, in which case the implantation of an implantable cardiac defibrillator could be indicated. (C) 2010 Elsevier Ireland Ltd. All rights reserved.