家族史在致心律失常性右心室心肌病危险分层中的地位

The status of family history for the risk stratification in arrhythmogenic right ventricular cardiomyopathy

目的探讨家族史在致心律失常性右心室心肌病（ARVC）危险分层中的地位。方法根据1994年ARVC诊断标准，纳入34例ARVC先证者，男性26例，女性8例，平均年龄（38±15）岁。对其家族成员行临床筛查，项目包括：（1）心电图V1～V3导联QRS≥110ms、V1～V3导联S波升支≥55ms、Epsilon波、T波倒置（V1～V3导联倒置）、（V1＋V2＋V3）／（V4＋V5＋V6）QRS≥1．2、V1～V3导联与V6导联QRS差值≥25ms，QRS离散度／〉40ms，QT离散度／〉65ms；（2）动态心电图记录室性早搏≥2000个／24h或室性心动过速（VT）；（3）超声心动图记录双心房、双心室及右心室流出道、流人道内径大小。比较ARVC家族史和上述各项临床参数的关系。分类变量用Fisher检验，连续变量使用t检验。P≤0．05为差异有统计学意义。结果34例ARVC先证者中55个家族成员接受评估，男性28例（6例诊断ARVC）、女性27例（3例诊断ARVC），平均年龄（35±16）岁。8例先证者有家庭成员受累，其中5例有芹束支阻滞形室性心动过速（LBBB—VT，63％）；26例先证者家庭成员无受累，其中20例有LBBB—VT（77％），P=0．649。家族史和室性心动过速的发生差异无统计学意义。结论家族史并不能反映ARVC的危险程度。

Objective To systematically investigate effect of family history on risk stratification in arrhythmogenic right ventrieular cardiomyopathy（ARVC） probands. Methods The study included 34 probands [ 26 men and 8 women ; mean age, （38 ± 15） years＇ with ARVC meeting the criteria in 1994. Their family members received clinical evaluation. The items include： （1）The value of different electrocardiogram criteria（ QRS duration in V1 - V3 leads ≥ 110 ms,S-wave upstroke duration in V1 - V3 leads 〉155 ms,Epsilon potentials and T-wave inversions in the right precordial leads, （V1 ＋ V2 ＋ V3 ）/（ V4 ＋ V5 ＋ V6 ）≥1.2 ,QRS duration in leads V1 through V3 that exceeds the QRS duration in lead V6 by t〉25 ms,QRS dispersion≥40 ms,QT dispersions〉 65 ms. （2）Ventricular tachycardia and premature ventricular contraction （ ≥ 2000 beats/24 h）during 24 h-Holter monitor. （3）The size of the atrium and ventricle from echocardiography. It is used for Fisher＇ s test and Student＇ s test to comparise the correlation among family history and these clinical parameters. A probability value of ≤0. 05 is considered significant. Results Fifty-five families members of 34 probands were evaluated,28 men（6 were ARVC）and 27 women （3 were ARVC） ;mean age （35 ± 16 ）years. Five had left bundle branch block shape VT （LBBB-VT） （ 63 % ） in 8 probands who had family history; 20 had LBBB-VT （ 77 % ） in 26 probands who had not family history ; P = 0. 649. The family history was not significantly different among the ARVC probands with clinical parameters including ventricular tachycardia. Conclusion valuable parameter for the risk stratification of ARVC probands from the study.