先天性右位心及合并畸形的超声心动图诊断

Echocardiographic diagnosis of congenital dextrocardia and cardiovascular anomaly

目的探讨经胸超声心动图对先天性右位心及合并畸形的诊断价值。方法经胸超声心动图检查，按VanPraagh节段分析法对76例先天性右位心的结构进行诊断，并与手术对照分析。结果76例先天性右位心患者中，镜像右位心47例，27例心室连接一致，大部分合并简单畸形，如房间隔缺损、室间隔缺损，多数伴有肺动脉高压；各节段连接不一致的均伴有复杂畸形，主要为右心室双出口、完全型大动脉转位、单心室伴肺动脉狭窄或肺动脉瓣闭锁。右旋心23例，21例伴有复杂畸形，主要为右心室双出口、单心室、矫正型大动脉转位、完全型大动脉转位合并肺动脉狭窄。左心房异构右位心2例，右心房异构4例，均合并复杂畸形。与手术对照，超声心动图能准确评估先天性右位心及伴随的心血管畸形，但对某些合并畸形的诊断仍有一定的局限性。结论经胸超声心动图可作为先天性右位心诊断的重要方法，能够满足临床需要。

Objective To observe the echocardiographic diagnosis value of congenital dextrocardia and cardiovascular anomaly. Methods Seven-six patients with dextrocardia were classified according to Van Praagh＇s method. The cardiovascular anomalies were diagnosed by echocardiography combined with operation results. Results Fourty-seven cases were mirror-image dextrocardia. 57.4 % of the 47 cases had concordant segmental connection, mostly with simple anomalies, including ASD and VSD with PH. Other patients with diseoncordant segmental connec tion were complicated with complex anomalies, including DORV, TGA, SV ＋ PS or PAA. 23 cases were dextroversion, mostly with disconcordant segmental connection and complex anomalies, including DORV, SV, C-TGA and TGA ＋ PS. 2 cases were left isomerism and 4 were right isomerism and these 6 cases presented with complex anomalies. Compared with the operation results, echocardiography could accurately detect congenital dextrocardia and cardiovascular anomalies, but there is still limitation for diagnosis of some anomalies. Conclusion Echocardiography can be the first choice for diagnosis of congenital dextroeardia.