36例左室心肌致密化不全的临床特征及中期预后分析

目的:分析中国人左室心肌致密化不全的临床特征及中期预后.方法:回顾性分析36例左室心肌致密化不全患者的临床表现、心电图、超声心动图等资料,随访(30±10)个月.进行生存分析,运用Cox风险回归模型分析临床不良事件(死亡或心脏移植)的预测因素.结果:左室心肌致密化不全占同期确诊的各类型原发性心肌病的9.4％,仅次于扩张性心肌病(67.3％)和肥厚性心肌病(20.9％).发病年龄1.5-72岁,男性占66.7％.心尖部及左室中段侧壁和下壁最常受累及.随访期间,25例患者出现心力衰竭,8例患者出现室性心动过速,2例患者出现血栓栓塞,8例患者死亡,1例患者接受心脏移植.临床不良事件(死亡或心脏移植)的独立预测因素包括:心功能NYHAⅢ/Ⅳ级(RR:6.139,95％CI:1.072-35.157,P=0.042)和室性心动过速(RR:1.676,95％CI:1.25022.846,P=0.024).结论:左室心肌致密化不全在原发性心肌病中并不罕见.心力衰竭、心律失常和血栓栓塞是主要并发症.心功能NYHAⅢ/Ⅳ级和室性心动过速是预后不良(死亡或心脏移植)的独立预测因素.

Clinic Features and Prognosis of Patients with Left Ventricular Noncompaction

Objective: To define the clinical features and prognosis of the patients with left ventricular noncompaction(LVNC) in China.Methods: We screened and identified 36 patients who met the diagnostic criteria for LVNC between Match 2002 and May 2011.Their clinical manifestation,electrocardiogram,and echocardiogram data were analyzed.The mean follow-up time was 30±10 months.Multivariate analysis with Cox proportional hazard regression model was used to evaluate the prognostic risk for death.Results: A total of 36 patients fullfilled the diagnostic criteria for LVNC.It was the third most common cause for cardiomyopathy diagnozed in this period following dilated cardiomyopathy and hypertrophic cardiomyopathy.The mean age at diagnosis was(47±22) years old.Apex and mid-ventricular segments of the lateral and inferior wall were involved in more than 80% of patients.Eight patients died and one patient underwent heart transplantation during the follow-up period.Other major adverse events included heart failure(25 cases,69.4%),ventricular tachycardia(8 cases,22.2%),and thromboembolic events(2 cases,5.6%).The independent predictors for death and heart transplantation were cardiac function of NYHA class Ⅲ/Ⅳ(RR=6.139,95%CI:1.072-35.157,P=0.042),and ventricular tachycardia(RR=1.676,95%CI:1.250-22.846,P=0.024).Conclusion: LVNC is not as rare as previously considered.The prognosis of LVNC appears unfavorable.Cardiac function of NYHA Class Ⅲ/Ⅳ and ventricular tachycardia are associated with death.