特殊类型房室间隔缺损的超声心动图诊断  

Echocardiographic diagnosis of special type of atrioventricular septal defect

作　　者：马艳春[1] 李文秀[2] 耿斌[2] 苏俊武 Ma Yanchun;Li Wenxiu;Geng Bin;Su Junwu(Department of Medical Inspection,Children′s Hospital of Shanxi Province,Taiyuan 030013,China;Pediatric Cardiovascular Center,Beijing Anzhen Hospital Affiliated to the Capital Medical University,Beijing 100029,China)

机构地区：[1]山西省儿童医院综合检查科,太原030013 [2]首都医科大学附属北京安贞医院小儿心脏中心,北京100029

出　　处：《中华超声影像学杂志》2022年第11期946-952,共7页Chinese Journal of Ultrasonography

基　　金：北京市自然科学基金项目(7202040)。

摘　　要：目的:探讨特殊类型房室间隔缺损(AVSD)的超声心动图特征,提高对AVSD的认识,减少临床漏诊及误诊。方法:回顾性分析总结2011年11月至2021年11月首都医科大学附属北京安贞医院小儿心脏中心诊断并经手术证实的特殊类型AVSD患者的超声心动图资料,根据是否存在室间隔缺损(VSD)将特殊类型AVSD分为两种,即房间隔(原发间隔)完整的AVSD和房间隔(原发间隔)及室间隔均完整的AVSD,总结该病的超声心动图特征。结果:超声心动图共诊断25例特殊类型AVSD。其中手术确诊13例,包括房间隔(原发间隔)完整的AVSD 12例,房间隔(原发间隔)及室间隔均完整的AVSD 1例;无手术适应证5例;因VSD小继续观察1例;1例合并复杂畸形仅行肺动脉环缩术,未处理心内畸形;其他4例膜周部VSD及1例部分型AVSD超声误诊为特殊类型AVSD。患者术前均存在轻至重度左侧房室瓣反流,左侧房室瓣成形术后多残存不同程度的反流。特殊类型AVSD超声心动图特征表现为:①原发房间隔连续完整,无原发孔缺损;②左右房室瓣叶附着点位于同一水平;③左侧房室瓣三叶化,并附着于室间隔上,即形成所谓的"前叶裂";④左心室两组乳头肌改变了正常的前外侧、后内侧的排列,而呈前后关系(逆钟向转位);⑤存在VSD时,VSD位于膜周部偏流入道肌部;⑥CDFI显示收缩期左侧房室瓣可见源自裂隙不同程度的反流信号;⑦可合并其他复杂畸形。结论:超声心动图可对特殊类型的AVSD作出明确诊断,该畸形预后与左侧房室瓣密切相关,成形效果取决于左侧房室瓣发育及反流情况。Objective To improve the understanding of special type of atrioventricular septal defect(AVSD)and reduce clinical missed diagnosis and misdiagnosis by investigating its echocardiographic features.Methods Echocardiographic images of patients with special type of AVSD diagnosed by echocardiography and confirmed by surgery in Pediatric Cardiovascular Center,Beijing Anzhen Hospital,Capital Medical University from November 2011 to November 2021 were retrospectively analyzed.Depending on the presence of ventricular septal defect(VSD),special type of AVSD were divided into two types,namely AVSD with intact ostium primum and AVSD with intact ostium primum and ventricular septum.The echocardiographic features of special type of AVSD were summarized.Results Twenty-five cases of special type of AVSD were diagnosed by echocardiography and 13 cases were confirmed by surgery,including 12 cases with intact ostium primum and 1 case with intact ostium primum and ventricular septum.There were 5 cases without surgical indications,and 1 case was kept under observation due to small VSD.One patient with complex malformation only underwent pulmonary artery banding operation without treating intracardiac malformation.The other 4 cases of perimembrane VSD and 1 case of partial AVSD were misdiagnosed as special type of AVSD by echocardiography.All patients had mild to severe left atrioventricular regurgitation before surgery,and most patients remained left atrioventricular regurgitation of varying degrees after left atrioventricular valve formation.Characteristic sonographical findings included:①The ostium primum was continuous and there was no ostium primum defect.②The attachment points of the left and right atrioventricular valves were at the same level.③The left atvioventricular valve was a trileaflet left atrioventricular valve and attached to the crest of the ventricular septum,forming the so-called"anterior mitral valve leaflet cleft".④The two groups of papillary muscles of the left ventricle changed the normal anterolateral

关 键 词：超声心动描记术 房室间隔缺损 心内膜垫缺损 室间隔缺损 二尖瓣前叶裂 

分 类 号：R540.45[医药卫生—心血管疾病] R725.4[医药卫生—内科学]