孤立性左心室致密化不全心肌病患者的临床特点及相关因素预后分析

Prognosis and clinical characteristics for ventricular tachycardia in patients with isolated non-compaction cardiomyopathy

目的 分析孤立性致密化不全心肌病的临床特点及预后相关因素.方法 2008年5月至2011年4月在阜外心血管病医院确诊的34例孤立性左心室致密化不全患者分为室性心动过速（室速）组和非室速组,比较两组患者的左心室大小及左心室射血分数（LVEF）,随访患者临床事件.结果 入选时室速组和非室速组在左心室大小及LVEF平均值差异无统计学意义.平均随访2.92年,室速组死亡5例,非室速组死亡2例,死亡患者的平均左心室舒张末期内径（LVEDD）73mm,LVEF0.31.室速组有6例患者植入植入型心律转复除颤器（ICD）,随访期间4例发生持续性室速,其中3例患者ICD正确识别并电除颤治疗分别为2次、2次和8次;1例患者ICD正确识别并电除颤治疗1次,误识别2次.结论 孤立性左心室致密化不全患者预后与LVEDD、LVEF及室速相关.

Objective The aim was to analyze clinical characteristics and prognosis in patients with isolated non-compaction cardiomyopathy.Methods Thirty-four patients with isolated non-compaction cardiomyopathy were collected from May 2008 to April 2011 in Fuwai Hospital.They were divided into ventricular tachycardia （VT） group and non-VT group.The left ventricular end-diasolic diameter（LVEDD） and the left ventricular ejection fraction （LVEF） were compared between groups.The clinical events were recorded through clinic or telephone interview.Results There was no significant difference in LVEDD and LVEF between VT group and non-VT group.The mean follow-up period was 2.92 years.During the study period 7 patients were dead and the mean LVEDD was 73 mm,and the mean LVEF was 0.31.Six patients were implanted with implantable cardioverter defibrillator （ICD） and 4 of them were shocked with appropriate detection.Conclusion LVEDD,LVEF and VT provide additive prognostic value in patients with isolated non-compaction cardiomyopathy.