超声诊断胎儿三尖瓣口无挡畸形2例及文献复习  

Ultrasonic diagnosis of fetal congenitally unguarded tricuspid valve orifice:analysis of two cases and literature review

作　　者：刘海兰[1] 宋嫣[1] Liu Hailan;Song Yan(epartment of Ultrasonography,Nanchang First Hospital,Nanchang 330006,China)

机构地区：[1]南昌市第一医院超声科,南昌330006

出　　处：《中华围产医学杂志》2023年第9期741-745,共5页Chinese Journal of Perinatal Medicine

基　　金：江西省卫生健康委员会课题(20204022)。

摘　　要：目的分析胎儿三尖瓣口无挡畸形(congenitally unguarded tricuspid valve orifice,CUTVO)的超声心动图典型特征。方法回顾性分析2019年4月至2021年12月在南昌市第一医院经超声诊断,并经病理检查证实的胎儿CUTVO的2例孕妇的临床资料及超声图像。在万方数据库、维普数据库、中国知网数据库、中华医学期刊数据库、PubMed和Web of Science数据库中检索确诊CUTVO并有超声诊断结果的文献。对数据资料采用描述性统计分析。结果(1)文献检索获得CUTVO病例15例,加上本单位诊断的2例,共17例。17例中,16例为单胎妊娠,1例为单绒毛膜单羊膜囊双胎妊娠。17例胎儿中,三尖瓣完全缺如或发育不全10例,部分缺如7例。9例为合并室间隔完整型肺动脉闭锁;2例合并室间隔缺损;6例文献未描述并发症情况。(2)共6例误诊,其中4例产前误诊为胎儿Ebstein畸形,2例误诊为Uhl病和心肌致密化不全可能,引产后证实为CUVTO。共2例漏诊,其中1例为双胎输血综合征,另1例为肺动脉瓣狭窄。2例均为中孕期,右心增大不显著。(3)9例终止妊娠,其中7例引产后证实为CUTVO。8例继续妊娠至分娩,其中2例由于病情较重死亡,6例存活患儿产后超声检查均发现合并房间隔缺损及动脉导管未闭,均行手术治疗。其中2例行三尖瓣成型术,术后1年内随访,三尖瓣中量返流;2例行三尖瓣机械瓣置换手术,术后三尖瓣少-中量返流;1例行改良Fontan术,术后心功能正常;1例未明确描述手术方式。CUTVO典型的超声心动图特征为房室结构及与大动脉连接正常,三尖瓣瓣叶及乳头肌完全或部分缺失,三尖瓣口呈空洞样,血流来回往返,多普勒呈低速双向频谱。结论CUTVO较典型的超声心动图表现为三尖瓣叶缺失并中量或大量暗淡返流、返流频谱形态呈双向。检查时应注意多个切面动态观察,以减少漏诊和误诊。Objective To review the imaging characteristics and evaluate the diagnostic value of echocardiography for fetal congenitally unguarded tricuspid valve orifice(CUTVO).Methods Clinical data and ultrasonic images of two pregnant women diagnosed with fetal CUTVO through ultrasound and confirmed by pathology in Nanchang First Hospital between April 2019 and December 2021 were retrospectively analyzed.Relevant literature on confirmed CUTVO diagnosis cases with ultrasonic diagnosis results was searched in the Wanfang database,CNKI database,VIP database,Chinese Medical Journal database,PubMed,and Web of Science database.Descriptive statistical analysis was used to analyze the data.Results The study involved 17 patients,15 of whom were retrieved from the databases and two cases from our hospital.Among the 17 cases,16 were singleton pregnancies,while one case involved a monochorionic monoamniotic twin pregnancy.The tricuspid valve was completely absent or hypoplastic in ten cases,and partial absence of the tricuspid leaflet was found in seven cases.Nine cases had pulmonary atresia with intact ventricular septum and two with ventricular septal defect,while complications were not described in the other six cases.Among the six misdiagnosed cases,four were prenatally misdiagnosed as fetal Ebstein malformation,two cases for Uhl's disease and suspected noncompaction of the ventricular myocardium.CUVTO were confirmed after pregnancy termination.Both cases of missed diagnosis were in the second trimester with no significant enlargement in the right heart,including one with twin transfusion syndrome and one with pulmonary valve stenosis.Nine cases terminated their pregnancies and seven of them had confirmed CUTVO by autopsy after an induced labor.Eight fetuses were delivered.Except for two deaths due to severe conditions,six neonates survived and underwent surgical treatment due to atrial septal defect and patent ductus arteriosus on postnatal echocardiography examination.Among them,two cases underwent tricuspid valve plasty and th

关 键 词：心脏缺损 先天性 三尖瓣 超声心动描记术 超声检查 产前 

分 类 号：R445.1[医药卫生—影像医学与核医学] R714.5[医药卫生—诊断学]