超声心动图诊断无顶冠状静脉窦综合征及合并畸形的价值  

Value of echocardiography to the diagnosis of unroofed coronary sinus syndrome and its associated cardiac malformations

作　　者：莫莹[1] 丁文虹[1] 季巍[2] 朱耀斌[1] 金梅[1] 刘迎龙[1] 

机构地区：[1]首都医科大学附属北京安贞医院小儿心脏中心,北京100029 [2]首都儿科研究所附属儿童医院心脏外科,北京100020

出　　处：《中华实用诊断与治疗杂志》2018年第1期77-79,共3页Journal of Chinese Practical Diagnosis and Therapy

基　　金：国家自然科学基金(81371443);北京市自然科学基金(7152045)

摘　　要：目的探讨超声心动图在无顶冠状静脉窦综合征(unroofed coronary sinus syndrome,UCSS)及合并畸形诊断中的临床价值。方法回顾性分析48例行外科手术成功矫治心内畸形UCSS患者的临床资料。结果 48例UCSS患者超声心动图诊断Ⅰa型27例,Ⅰb型9例,Ⅱa型、Ⅱb型、Ⅲa型、Ⅲb型各3例,UCSS超声心动图诊断及分型与手术结果相符;超声心动图表现为在常规冠状静脉窦切面无法扫查到完整窦壁,并造成左心房血流经过缺损的窦壁进入右心房从而造成左、右心房之间的分流。33例合并左上腔静脉入左心房,10例合并单心房,16例合并心内膜垫缺损,7例合并右心室双出口,5例合并单心室,21例合并室间隔缺损,超声心动图漏诊1例细小动脉导管未闭。结论超声心动图诊断UCSS及合并的心脏畸形准确率高,并可明确缺损具体部位,为手术方案制定提供重要依据。Objective To analyze the value of echocardiography to the diagnosis of unroofed coronary sinus syndrome （UCSS） and its associated cardiac malformations. Methods The clinical data of 48 UCSS patients undergoing operation for correcting intracardiac malformation were retrospectively analyzed. Results Among 48 UCSS patients, 27 were diagnosed as type I a and 9 were type I b, 3 were type Ⅱ a, 3 were type Ⅱb, 3 were type Ilia and 3 were type Ⅲb. The echocardiographic result was coincident with the surgical findings, showing no complete sinus wall at normal coronary sinus view, resulting in inter-atrial shunt through the defected sinus wall. The complications included left superior vena cava incorporated into the left atrium in 33 patients, single atrium in 10, endocardial cushion defect in 16, double outlet of right ventricle in 7, single ventricle in 5 and ventricular septal defect in 21. One patient was misdiagnosed as patent ductus arteriosus. Conclusion Echocardiography has a high diagnostic rate for UCSS and its associated cardiac malformations. It could define the exact defect site so as to provide important basis for surgery.

关 键 词：无顶冠状静脉窦综合征 心脏畸形 超声心动图 

分 类 号：R540.45[医药卫生—心血管疾病] R654.2[医药卫生—内科学]