中文摘要：

目的总结致心律失常性右室心肌病（ARVC）的超声心动图特点，探讨超声心动图漏诊、误诊原因，提高诊断符合率。方法回顾分析21例确诊为ARVC患者的超声心动图结果，临床诊断标准为2010年欧洲心脏病协会ARVC指南。根据指南中超声心动图诊断标准将患者分为超声确诊组、疑诊组、漏诊组及误诊组，分析各组超声表现，包括：右室壁运动、右室流出道内径（RVOTd）、右室面积变化率（RVFAC）、三尖瓣反流程度及肺动脉收缩期压力（PASP）。结果21例患者中，超声心动图确诊组15例（71．4％），疑诊组3例（14．3％），漏诊组2例（9．5％），误诊组1例（4．8％）。确诊组具有ARVC的典型超声心动特点，表现为右室壁局部运动减低、无运动和（或）室壁瘤；伴有RVOTd扩张[平均内径（40±3）mm]；RVFAC均〈33％[平均（21±7）％]；均伴有三尖瓣反流，PASP正常范围[平均（27±9）mmHg，1mmHg=0．133kPa]。疑诊组3例均表现为右室局部运动减低；1例单纯右室流出道增宽，2例右室流出道增宽及右室增大；三尖瓣轻度反流，PASP正常范围，33％〈RVFAC≤40％。漏诊组超声心动图结果正常。误诊组为扩张型心肌病超声表现。结论ARVC的不同阶段超声心动图表现不同，对处于右心衰竭期的患者可以确诊，对早期、晚期患者的诊断需结合临床资料及其他检查以避免漏诊、误诊．

英文摘要：

Objective To evaluate the value of echocardiography on the diagnosis of arrhythmogenic right ventricular cardiomyopathy （ARVC）, and to improve the diagnositic accuracy of ARVC by echocardiography. Methods According to the 2010 European Heart Association guideline, twenty-one patients with ARVC were diagnosed from September 2003 to June 2014. The patients were divided into four groups （confirmed, suspiciously diagnosis, miss diagonisis, misdiagnosis） and the echocardiographic features were retrospectively analyzed including the right ventricular （RV） movement, the diameter of RV outflow tract （RVOTd）, fractional area change of RV （RVFAC）,the severity of tricuspid regurgitation （TR） and peak pulmonary artery systolic pressure （PASP）. Results Of 21 patients, 15 （71.4~） were confirmed by echocardiography, which had the typical ARVC echocardiographic features including the hypokinetic, akinetic or aneurysm of RV,dilation of RVOTd [-mean RVOTd （40 ± 3）mm-], and RV FAC〈33 % [-mean （21 ± 7）%]. TR were noticed in all the 15 patients but the PASP were normal [-mean （27 ± 9）mmHg, l mmHg = 0.133 kPa3. Three （14.3 % ） were suspiciously diagnosed which had the RV wall hypoakinetic, 1 with pure RVOTd dilation and 2 with RV and RVOTd dilation,all 3 patients had mild TR,33%RVFAC ≤40% and PASP were in normal range. Two patients had normal echocardiography which was miss diagnosed,one patient was misdiagnosed as dilated cardiomyopathy. Conclusions The different stages of ARVC patients had different echocardiographic features,the patients were easily diagnosed when the ARVC patients in RV failure stage. But for the early and late stage, the diagnosis should combine the clinical manifestation and other imaging facilities to avoid miss diagnosis and misdiagnosis.