UNASSIGNED: In this study, we report an 18-year-old woman patient with sudden cardiac arrest due to VF and successful cardiopulmonary resuscitation as the first clinical manifestation of non-obstructive HCM. Subsequently, a subcutaneous implantable cardioverter-defibrillator (ICD) was implanted for secondary VF prophylaxis. However, additional episodes of VF occurred. During these, device interrogation revealed a combined occurrence of VF, bradycardia, and SAN arrest, requiring a device exchange into a dual-chamber ICD. A heterozygous, pathogenic variant of the MYH7 gene (c.2155C>T; p.Arg719Trp) was identified as causative for HCM.
UNASSIGNED: First published in 1994, the particular MYH7 variant (p.Arg719Trp) was described in HCM patients with a high incidence of premature cardiac death and a reduced life expectancy. Electrophysiological studies on HCM patients are mainly performed to treat AF and ventricular tachycardia. Further extraordinary arrhythmic phenotypes were reported only in a few HCM patients. Taken together, the present case with documented co-existing VF and SAN arrest is rare and also emphasizes addressing the presence of SAN arrest (in particular, during VF episodes) in HCM patients when a distinct ICD device is considered for implantation.
■在这项研究中,我们报道了1例18岁女性患者,因VF导致心脏骤停并成功进行心肺复苏为非阻塞性HCM的首发临床表现.随后,植入了皮下植入式心律转复除颤器(ICD),用于二次预防VF.然而,发生了额外的VF发作。在此期间,设备询问显示同时发生了VF,心动过缓,和SAN逮捕,需要将设备交换到双腔ICD中。杂合的,MYH7基因的致病变异(c.2155C>T;p.Arg719Trp)被鉴定为HCM的致病原因。
■于1994年首次发布,特定的MYH7变体(p。在HCM患者中描述了Arg719Trp),其早逝性心脏病死亡的发生率很高,预期寿命缩短。对HCM患者的电生理研究主要用于治疗AF和室性心动过速。仅在少数HCM患者中报告了进一步的异常心律失常表型。一起来看,目前有记录的共存VF和SAN逮捕的案例很少见,并且还强调解决SAN逮捕的存在(特别是,在VF发作期间),当考虑植入不同的ICD装置时,在HCM患者中。