超声诊断致心律失常性右心室心肌病的临床价值再评价

Clinical reevaluation of the echocardiographic value in diagnosing the arrhythmogenic right ventricular cardiomyopathy

目的总结临床确诊为致心律失常性右室心肌病（ARVC）患者的心电生理及影像特点，评价超声对ARVC的诊断价值。方法65例ARVC患者术前经超声和MRI检查评估患者右室大小（TTE-RV、MRI-RV）和右室壁厚度（TTE—RVFW），同时测量患者三尖瓣反流速度（TRmax）及左室射血分数（LVEF）；术中使用Carto三维标测系统重建右室三维电解剖模型，测量右室面积（Area—RV）、瘢痕面积（Area—Scar）。结果超声心动图确诊27例（41．5％），疑诊21例（32．3％），漏诊14例（21．5％），误诊3例（4．6％）。确诊组TTE—RV、TTE—RVFW、TRmax、LVEF、MRI-RV、Area—RV、Area—Scar与疑诊组和漏诊组相比，差异均有统计学意义（P〈0．05）。疑诊组Area-RV、Area—Scar测值与漏诊组相比，差异有统计学意义（P〈0．05）。不同病程阶段ARVc超声表现迥异，但所有患者均在MRI和电生理标测中存在心肌纤维化和低电压瘢痕区。结论超声心动图对典型ARVC可以确诊，但对于早、中期患者的诊断，应结合患者临床资料综合评估。

Objective To investigate the diagnostic value of echocardiography in arrhythmogenic right ventricular cardiomyopathy （ARVC） by summarizing and comparing the electrophysiological and the imaging features. Methods The echocardiography and MRI were performed in the 65 cases of ARVC to measure the right ventricle and the free wall, noted as TTE-RV,MRI-RV and TTE-RVFW. The velocity of tricuspid valve regurgitation（TRmax） and left ventrieular ejection fraction（LVEF） were measured. The three-dimensional electric anatomical model of right ventricular was get by the Carto system, and the right ventricular area （Area-RV）, the scar area （Area-Scar） was calculated. Results Twenty-seven cases （41.5% ） was confirmed by the eehocardiography, 21 cases （32.3 % ） was suspiciously diagnosed, 14 cases （32.3%） was miss diagnosed, and 3 eases （4.6%） was misdiagnosed. Statistically significant difference could be detected among the echocardiography confirmed groups and the other two groups for the parameters TTE-RV, MRI-RV, Area-RV, Area-Scar, and TTE-RVFW （ P 〈 0.05）. Also there was a statistically significant difference of the parameters of Area-RV and Area-Scar between the suspiciously and miss diagnosed groups （ P %0.05）. Different echocardiographic findings was found in ARVC with different stages, but myocardial fibrosis and low voltage scar could be detected in all patients on the MRI imaging and and electrophysiological mapping. Conclusions The diagnosis of typical ARVC can be confirmed by echocardiography,but for the patients with early and middle stages, comprehensive evaluation should be refered to the clinical data.