PROGNOSTIC DETERMINANTS IN HYPERTROPHIC CARDIOMYOPATHY

肥厚型心肌病（HCM）患者经常有 心律失常和血流动力学异常，并易于猝死 还有晕厥 非常需要改进识别 有猝死风险的患者和适当的定义 调查和治疗。 31例临床、霍尔特、心脏 导管插入术和电生理（EP）变量， 对230例猝死高危患者的心脏事件进行了检查 使用多变量分析。 风险评估是心脏骤停， 32例，晕厥80例，晕厥前期52例，室性心动过速（VT） 霍尔特36例，猝死家族史9例， 心悸21例。 82例（36%）诱发持续性VT 患者 17例心脏事件（8例猝死，1例心脏骤停， 8例晕厥伴除颤器放电）在随访期间发生28 + 19个月 1年和5年无事件率分别为99%和79%， 分别 两个变量是显著的独立预测因子， 随后的心脏事件：诱导持续性心室 心律失常和心脏骤停/晕厥病史。 只有两个突然 66名患者死亡，无意识障碍症状： 36例霍尔特显示非持续性VT患者发生1例猝死 30例霍尔特无室速者发生猝死1例。 值得注意的是，51名没有意识障碍症状或 诱发性室性心动过速发生了心脏事件 因此，在HCM中，1）霍尔特上的VT为 良性预后的意义，在没有症状的损害 意识和诱发性室性心动过速; 2）诱发持续性室性心动过速 特别是当与心脏骤停或晕厥相关时， 一个具有后续心脏事件高风险的患者亚组。

Patients with hypertrophic cardiomyopathy (HCM) frequently have arrhythmias and hemodynamic abnormalities, and are prone to sudden death and syncope. There is an important need for improved identification of patients at risk for sudden death and definition of appropriate investigation and therapy. The relation of 31 clinical, Holter, cardiac catheterization and electrophysiologic (EP) variables to subsequent cardiac events in 230 patients at high risk for sudden death was examined using multivariate analysis. Risk evaluation was for cardiac arrest in 32, syncope in 80, presyncope in 52, ventricular tachycardia (VT) on Holter in 36, a strong family history of sudden death in 9, and palpitations in 21 patients. Sustained VT was induced in 82 (36%) patients. Seventeen cardiac events (8 sudden deaths, 1 cardiac arrest and 8 syncope with defibrillator discharges) occurred during a follow-up 28 + 19 months. The 1-year and 5-year event-free rates were 99% and 79%, respectively. Two variables were significant independent predictors of subsequent cardiac events: inducibility of sustained ventricular arrhythmia, and a history of cardiac arrest/syncope. Only two sudden deaths occurred in 66 patients without symptoms of impaired consciousness: one sudden death occurred in 36 patients with non-sustained VT on Holter and one sudden death occurred in 30 patients without VT on Holter. Notably, none of 51 patients without symptoms of impaired consciousness or inducible VT had a cardiac event. Thus, in HCM 1) VT on Holter is of benign prognostic significance in the absence of symptoms of impaired consciousness and inducible VT, and 2) inducibility of sustained VT at EP study specially when associated with cardiac arrest or syncope identifies a subgroup of patients at high risk for subsequent cardiac events.