无顶冠状静脉窦综合征1例

Unroofed coronary sinus syndrome: A case report

无顶冠状静脉窦综合征(unroofed coronary sinus syndrome,UCSS)也称冠状静脉窦间隔缺损,是房间隔缺损中的一种少见类型,发病率不到房间隔缺损的1%,因胚胎发育过程中左心房静脉皱褶形成不完全造成。为提高临床医生对这一罕见病的认识,现报道中南大学湘雅二医院收治的1例UCSS患者。该患者年龄50岁,主要临床表现为反复活动后乏力、气促。心脏彩超示冠状静脉窦扩张(17 mm×14 mm),提示永存左上腔静脉可能。肺动脉造影可见左心房显影后,左心室、冠状静脉窦同时显影,房间隔完整,随后右心房、右心室相继显影,提示部分肺静脉异位引流(心内型)。心脏CT血管造影示4条肺静脉入左心房,永存左上腔静脉向下汇入冠状静脉窦,冠状静脉窦间隔缺如且与左心房相通,确诊为UCSS。后于我院心血管外科行UCSS矫治术。

Unroofed coronary sinus syndrome(UCSS),also named coronary sinus septal defect,is a rare type of atrial septal defect with the incidence less than 1%of the total number of atrial septal defects.It is caused by incomplete formation of left atrial venous folds during embryonic development.Here we reported a patient with UCSS,who was treated in the Second Xiangya Hospital of Central South University.The patient was 50 years old and the main clinical manifestations were fatigue and shortness of breath after repeated exercise.Color Doppler echocardiography showed coronary sinus dilatation(17 mm×14 mm),indicating the possibility of permanent left superior vena cava.Pulmonary angiography showed that the left ventricle and coronary sinus were developed at the same time while the atrial septum was intact after the development of the left atrium,followed by the right atrium and right ventricle,indicating a partial anomalous pulmonary venous drainage(intracardiac type).Finally,the cardiac computed tomograhic angiography showed that 4 pulmonary veins and permanent left superior vena cava(PLSVC)went into the left atrium and the coronary sinus,respectively,while the coronary sinus septum was absent and the PLSVC was connected with the left atrium.The patient was later treated with the correction of non-parietal sinus syndrome in the Cardiovascular Surgery Department of our hospital.