刘双，王明宇，陈丽萍，托丽斯，高璐，刘培培，朱青，孙健



心肌致密化不全与扩张型心肌病合并过度小梁化的对比分析

刘双*，王明宇，陈丽萍，托丽斯，高璐，刘培培，朱青，孙健

摘要

Comparative Study Between the Patients With Noncompaction of Ventricular Myocardium and Dilated Cardiomyopathy Combining Hypertrabeculation

LIU Shuang**,WANG Ming-Yu,CHEN Li-ping,TUO Li-si,GAO Lu,LIU Pei-pei,ZHU Qing,SUN Jian.
Department of Cardiology,The First Affiliated Hospital of Jilin University,Changchun(130021),Jilin,China.
Corresponding Author:SUN Jian,Email:sunjianemail@126.com

Abstract

Objective:To explore the clinical and echocardiography characteristics between noncompaction of ventricular myocardium(NVM)and dilated cardiomyopathy(DCM)combining hypertrabeculation in order to distinguish NVM from DCM.

Methods:Our research included 2 groups of patients:NVM group,n=31 and DCM combining hypertrabeculation group,n=50.The basic information as gender,age,family history,symptoms,ECG,plasma levels of BNP and echocardiography were recorded and examined in all patients;the size of cardiac chambers,myocardium,endocardium and hemodynamics were particularly focused.The trabeculation was analyzed by 17 segments method.

Results:①Compared with NVM group,the patients in DCM combining hypertrabeculation group had the worse cardiac classification,higher plasma levels of BNP(P<0.05)and more obvious cardiac dilatation.②The patients in NVM group had the most trabeculation segments(9.82 ± 2.02)and the apical(17thsegment)was involved,patients had the higher ratio of noncompacton/compaction(NC/C)as(2.84 ± 0.61),there were(4.12 ± 2.68)segments with NC/C >2.③The patients in DCM combining hypertrabeculation group had the less trabeculation segments(5.56 ± 1.56)and the apical was seldom involved,patients had the lower ration of NC/C as(1.91± 0.42),there was at most 1 segment with NC/C >2.All P<0.05.

Conclusion:Echocardiography is a simple,practical and noninvasive method to distinguish NVM from DCM.NVM could be diagnosed by obvious left ventricular apex involvement with NC/C >2 in at least 2 segments of free ventricular walls.

Key words Cardiomyopathy,dilated;Noncompaction of ventricular myocardium;Echocardiography;Hypertrabeculation

(Chinese Circulation Journal,2016,31:229.)

心肌致密化不全（NVM）是一种罕见的原发性遗传性心肌病。以心室壁内层网状肌小梁及深陷的隐窝为特征[1]。目前心肌致密化不全确诊的最终标准为心脏磁共振成像（MRI），但因价格昂贵且能够开展的医院有限，故超声心动图仍是目前临床上最简便、实用的检查方法。NVM与扩张型心肌病（DCM）在超声心动图上通常容易鉴别，但在临床实践中我们发现部分DCM心室壁也可出现增厚，增厚的室壁上也可见肥大增粗的肌小梁，称为过度小梁化，易与NVM混淆。本文通过对比分析探讨两者的鉴别要点。

1　资料与方法

一般资料：收集本院2012-01至2015-01行心脏超声心动图检查的住院患者81例，NVM患者31例为NVM组，入选标准为心脏超声提示心脏扩大。排除标准为合并其他心肌病或心脏畸形。采用Jenni 等[2]在2001年提出的诊断标准，诊断难以确定的经心脏MRI或左心室造影进一步明确。合并过度小梁化的DCM患者50例为DCM合并过度小梁化组，入选标准为心脏超声可见突出于心室腔的肌小梁。诊断按世界卫生组织和国际心脏病联合会（WHO/ISFC）1995年拟定的标准。排除可以引起心脏扩大的其它心脏疾患。两组患者均结合遗传史、临床表现、彩色超声心动图、心电图、心肌酶学、肌钙蛋白I检查，并行冠状动脉造影或多层螺旋CT冠状动脉成像（CTA）排除缺血性心肌病。所有……