PROGNOSIS AND ELECTROPHYSIOLOGIC FUNDINGS IN HCM SUDDEN CARDIAC DEATH SURVIVORS

肥厚型心肌病（HCM）患者的识别， 需要积极的抗肿瘤治疗（例如，植入 除颤器设备）需要了解 这种异质性疾病的不同亚群。 我们比较了 41例连续HCM突发心脏病存活者的长期结局 66例无室性心动过速的HCM患者死亡（SCD） (VT)24小时动态霍尔特监测室性心动过速17例。 的 SCD患者中，23例因左心室流出道接受手术 梗阻（OBST）和11例患者进行了电生理检查 （EPS）。 5年无事件（进一步SCD或死亡）率 无VT、有VT、SCD（手术）和SCD（非手术）的患者 分别为90%、76%、67%和37%。 在有EPS的SCD中， 房室传导异常8例（73%）， 10例有可诱导的持续性恶性VT。 因此，（a）SCD 预后相对较差，特别是在那些 不是手术候选者，和（B）大多数患有HCM的SCD患者具有 传导异常和诱发性室速

Identification of hypertrophic cardiomyopathy (HCM) patients who require aggressive antiarrhythmic therapy (e.g., implantation of defibrillator device) requires knowledge of the prognosis of various subgroups of this heterogenous disease. We compared the long-term outcome in 41 consecutive HCM survivors of sudden cardiac death (SCD) with 66 HCM patients without ventricular tachycardia (VT) and 17 patients with VT on 24-hour Holter monitoring. Of the SCD patients, 23 had surgery for left ventricular outflow obstruction (OBST) and 11 patients had electrophysiologic studies (EPS). Five-year event-free (further SCD or death) rates for patients with no VT, with VT, SCD (surgical) and SCD (nonsurgical) were 90%, 76%, 67% and 37% respectively. Of the SCD who had EPS, 8 (73%) had atrioventricular conduction abnormalities and 9 (82%) patients had inducible sustained malignant VT. Thus, (a) SCD conveys a relatively poor prognosis especially in patients who were not surgical candidates, and (b) most SCD patients with HCM have both conduction abnormalities and inducible VT.