Gardin L L, Nanton M A, Hanna B D
Faculty of Medicine, Department of Physiology and Biophysics, Dalhousie University.
Can J Cardiol. 1994 Jun;10(5):548-50.
Sudden death is an important cause of mortality in hypertrophic cardiomyopathy. Several mechanisms have been proposed, but ambulatory monitoring of the event has rarely occurred. The case of an adolescent girl with congenital hypertrophic cardiomyopathy and arthrogryposis multiplex congenita is presented. Despite appropriate therapy and severe activity limitation, the patient's condition progressively worsened. The patient died suddenly in her sleep, one month after a myocardial myomectomy for subaortic stenosis. The fortuitous ambulatory electrocardiographic (ECG) monitoring of the event recorded polymorphic ventricular tachycardia degrading into ventricular fibrillation. Retrospective review of previous ambulatory ECG revealed the presence of nonsustained ventricular tachycardia, a predictive determinant for sudden death, and atrioventricular node dysfunction. However, the patient had never reported symptoms during the dysrhythmias. The pathological specimen showed an unexpected old apical infarction scar, suggesting that her preoperative cardiac status was worse than suspected. In summary, the mechanism of sudden death in this patient was revealed by ambulatory ECG monitoring, and the involvement of neural and myocardial factors are proposed.
猝死是肥厚型心肌病的一个重要死亡原因。已经提出了几种机制，但对该事件的动态监测却很少发生。本文介绍了一名患有先天性肥厚型心肌病和先天性多发性关节挛缩症的青春期女孩的病例。尽管进行了适当的治疗且活动受到严重限制，但患者的病情仍逐渐恶化。在因主动脉瓣下狭窄接受心肌肌瘤切除术后一个月，患者在睡眠中突然死亡。对该事件的偶然动态心电图（ECG）监测记录到多形性室性心动过速恶化为心室颤动。回顾性分析之前的动态心电图发现存在非持续性室性心动过速，这是猝死的一个预测决定因素，以及房室结功能障碍。然而，患者在心律失常期间从未报告过症状。病理标本显示有一个意外的陈旧性心尖梗死瘢痕，表明她术前的心脏状况比怀疑的更差。总之，动态心电图监测揭示了该患者猝死的机制，并提出了神经和心肌因素的参与。
