产前超声诊断永存左上腔静脉及其合并畸形的价值

正永存左上腔静脉(persistent left superior vena cava,PLSVC)是最常见的体循环静脉畸形,发生率约为0.3%～0.5%,占所有先天性心脏病的2.8%～4.3%~([1-2]),可以单独存在,也可合并其他心内外畸形。本组回顾性分析86例PLSVC胎儿及其合并畸形的相关资料,总结PLSVC的声像图特征、伴发畸形及围产结局,旨在提高产前超声对该病的认识。     

产前超声检查对胎儿永存左上腔静脉的诊断价值

目的研究胎儿永存左上腔静脉(PLSVC)超声声像图,探讨产前超声检查对PLSVC胎儿的诊断价值。方法回顾性分析27例PLSVC胎儿的超声声像图,其中经尸解证实19例,产后随访证实8例。结果超声诊断27例胎儿均为Ⅰ型PLSVC,即经冠状静脉窦引流入右心房。27例中5例为单纯PLSVC,22例合并其他心脏异常及心外畸形,其中2例合并染色体异常。结论产前超声检查可准确诊断胎儿PLSVC,具有重要临床意义。更多还原     

产前超声诊断胎儿永存左上腔静脉并右上腔静脉缺如畸形

目的 探讨永存左上腔静脉并右上腔静脉缺如胎儿的产前超声特点及其诊断价值。方法 回顾性分析产前诊断为永存左上腔静脉并右上腔静脉缺如的8胎胎儿的超声资料,观察其超声表现及合并畸形,并随访其结局。结果 胎儿永存左上腔静脉并右上腔静脉缺如的超声特征为三血管-气管切面于肺动脉左侧见一血管回声,追踪走行见其汇入增宽的冠状静脉窦,同时主动脉右侧未见右上腔静脉显示。时间-空间关联成像（STIC）联合高分辨血流显像（HDF）可立体显示左上腔静脉与主动脉、肺动脉的空间位置关系。8胎均伴冠状静脉窦增宽,5胎合并其他心内畸形,1胎合并心外畸形。结论 超声可在产前准确诊断永存左上腔静脉并右上腔静脉缺如及合并畸形。冠状静脉窦扩张是产前超声诊断的重要线索。     

先心病合并永存左上腔静脉的诊断和处理

永存左上腔静脉畸形(PLSVC)是最常见的一种体静脉畸形,常给临床诊断和治疗带来困难.现将43例先天性心脏病合并永存左上腔静脉的临床资料分析如下.     

心脏超声右心声学造影诊断1例上腔静脉发育异常

永存左上腔静脉（PLSVC）合并右上腔（RSVC）缺如为一种非常罕见的疾病，对合并复杂性畸形的患者，术前可通过彩色多普勒心脏超声右心声学造影技术作出诊断，对心导管介入及外科术中处理具有非常重要的意义。     

胎儿超声心动图在诊断永存左上腔静脉中的应用

目的 总结永存左上腔静脉胎儿超声心动图特征及临床意义.方法 对2008年5月至2011年1月在我院诊断为永存左上腔静脉的33例胎儿超声心动图表现进行总结分析,并与引产后病理或产后超声心动图诊断结果进行对照分析,总结永存左上腔静脉胎儿超声心动图特征及临床价值.结果 33例永存左上腔静脉胎儿中引产后病理或产后超声心动图证实32例,超声表现为:四腔观可见扩张的冠状静脉窦,三血管观在肺动脉左侧可见一圆形血管回声,为永存左上腔静脉,同时可见右上腔静脉变细,追踪走行可见其与冠状静脉窦相连续.其中18例为单发畸形(18/32,56.25%),14例合并其他畸形(7例合并心内畸形,2例合并心外畸形,5例同时合并心内和心外畸形).胎儿超声心动图将内脏反位综合征合并完全型房室间隔缺损误诊为同时合并永存左上腔静脉1例.结论 胎儿超声心动图是诊断永存左上腔静脉的可靠方法.永存左上腔静脉常合并其他畸形,产前超声发现永存左上腔静脉需进一步检查以排除其他合并畸形.     

产前超声诊断胎儿永存左上腔静脉中的价值

目的 探讨胎儿期永存左上腔静脉声像图特征及临床应用价值.方法 应用胎儿超声心动图检查3368例胎儿,检出31例胎儿永存左上腔静脉及相关畸形.结果 胎儿超声心动图显示,31例胎儿中30例合并冠状静脉窦扩张,14例合并其他心血管异常,6例合并心外畸形,4例同时合并心内外畸形.结论 永存左上腔静脉常合并先天性心脏病,胎儿预后取决于有无合并其他先天性心脏病,产前诊断永存左上腔静脉可帮助正确评估胎儿预后.     

单中心儿童永存左上腔静脉合并先天性心脏病的超声心动图分析

目的应用超声心动图分析永存左上腔静脉(PLSVC)患儿合并先天性心脏病种类,提高超声诊断PLSVC及相关心脏畸形的准确率。方法回顾性分析我院2013～2017年行超声心动图检查的212 309例患儿的超声心动图资料及临床资料,其中先天性心脏病患儿28 403例,永存左上腔静脉患儿1676例,分析永存左上腔静脉合并先天性心脏病类型的分布特征及发生率。结果永存左上腔静脉的检出率约0.79%(1676/212 309),其中Ⅰ型永存左上腔静脉最常见约占99.7%(1671/1676)。永存左上腔静脉在先天性心脏病患儿中的检出率约3.0%(866/28 403)。1676例永存左上腔静脉的患儿中,合并先天性心脏心脏病的患儿866例(51.7%),常见的为室间隔缺损202例(23.3%)、房间隔缺损149例(17.2%)、法洛四联症73例(8.4%)、动脉导管未闭58例(6.7%)、主动脉该缩窄及主动脉缩窄复合畸形52例(6.0%)、心内膜垫缺损34例(4.0%)、右室双出口29例(3.3%)。结论永存左上腔静脉常合并先天性心脏大血管畸形;超声心动图检查可准确诊断永存左上腔静脉及其合并畸形,能够为临床制定患儿治疗方案提供可靠依据。     

超声心动图诊断胎儿冠状静脉窦扩张

目的 探讨超声心动图诊断胎儿冠状静脉窦扩张及其相关疾病的临床意义.方法 2010年1月-2012年4月19 914名孕妇于我院接受产前超声心动图检查.回顾性分析其中冠状静脉窦扩张胎儿的超声心动图表现及其合并相关疾病的特点.结果 共检出54胎冠状静脉窦扩张胎儿,均合并永存左上腔静脉;其中12胎合并其他心内畸形,3胎合并心外异常,6胎同时合并心内、心外异常.结论 产前超声对冠状静脉窦扩张及其相关疾病具有重要诊断价值和临床意义.     

产前超声心动图与解剖铸型观察胎儿完全性肺静脉异位引流

目的 采用产前超声心动图与解剖铸型观察胎儿完全性肺静脉异位引流（TAPVC）。方法 纳入9胎经产前超声心动图诊断为TAPVC的胎儿,对引产后3胎标本进行解剖、6胎标本制作胎儿心脏铸型,观察胎儿静脉回流特点及合并心内外畸形。结果 9胎TAPVC中,66.67%（6/9）为心上型,33.33%（3/9）为心下型。胎儿超声四腔心切面见左心房缩小,未见静脉与左心房相连;三血管气管切面见左心房后方赘余血管;6胎心上型TAPVC中,4胎肺静脉汇入右上腔静脉、2胎肺静脉汇入左上腔静脉;3胎心下型TAPVC肺静脉均汇入门静脉。9胎均合并心房异构综合征,8胎（8/9,88.89%）合并单心室,7胎（7/9,77.78%）合并肺动脉狭窄,合并单心房及完全型心内膜垫缺损各4胎（4/9,44.44%）;5胎（5/9,55.56%）合并永存左上腔静脉,合并双下腔静脉及左头臂静脉主动脉弓下走行各1胎（1/9,11.11%）;1胎（1/9,11.11%）合并膈疝。结论 TAPVC胎儿多合并心内外畸形及体静脉异常。产前超声具有重要诊断价值,解剖铸型有助于认识TAPVC胎儿血管走行及静脉引流途径。     

Prenatal diagnosis of persistent left superior vena cava and its associated congenital anomalies.

OBJECTIVE: To evaluate the associated conditions and the outcome of persistent left superior vena cava (PLSVC) detected in fetal life. METHODS: This was a retrospective review of all cases of PLSVC detected prenatally between 1998 and 2004 in two tertiary referral centers in Germany. Patient charts, ultrasound video recordings and still frames of all cases were reviewed for associated conditions and outcome. RESULTS: Eighty-two cases of PLSVC were detected in the study period. Thirty-seven cases (45%) were associated with heterotaxy syndromes, 19 (23%) with isolated cardiac malformations, seven (9%) with aneuploidy, six (7%) with complex malformation syndromes and six (7%) with isolated extracardiac malformations. Seven cases (9%) had no associated condition. Eighty-three percent of the fetuses in this series had associated cardiac malformations; the most frequent cardiac malformations in those with heterotaxy syndromes were complete atrioventricular septal defect (75%) and right outflow tract obstruction (58%). After exclusion of cases with heterotaxy, most congenital heart defects were ventricular septal defects (41%) and coarctation (34%). The outcome of PLSVC was determined solely by the associated conditions. After exclusion of terminated cases, heterotaxy syndromes as well as complete atrioventricular septal defects were associated significantly with perinatal and infant death. In contrast, all cases with isolated PLSVC or associated correctable extracardiac malformations survived and were doing well at the time of writing. CONCLUSIONS: PLSVC detected in fetal life has to be followed by a meticulous inspection of the fetal anatomy as it is frequently associated with heterotaxy syndromes, other cardiac/non-cardiac malformations and aneuploidy that determine the outcome. Isolated PLSVC is a benign vascular anomaly and may not affect the outcome.     

产前超声诊断胎儿食管后左头臂静脉

目的 观察产前超声诊断胎儿食管后左头臂静脉（RELBCV）的价值。方法 回顾性分析10胎产前超声诊断RELBCV胎儿，观察其超声表现、伴发畸形、染色体异常及妊娠结局等；结合产后随访结果分析产前超声诊断RELBCV的效能。结果 产前超声检出单纯RELBCV 5胎，RELBCV伴心内畸形3胎，伴心外畸形2胎。9胎RELBCV超声三血管气管（3VT）切面图像见左头臂静脉（LBCV）走行于食管和气管后方，与扩张的奇静脉汇合后汇入上腔静脉而形成"U"形血管环；1胎RELBCV见LBCV分为2支，前支较纤细，走行于主动脉弓上方，后支走行于主动脉弓平面食管后方，均汇入上腔静脉。6胎接受羊膜腔穿刺染色体核型及基因组疾病检测，1胎致病性染色体微结构异常，1胎染色体缺失，4胎均未见明显异常。4胎接受无创DNA检测，结果均提示非整倍体异常风险较低。7胎正常出生后经超声心动图证实RELBCV。结论 胎儿RELBVC产前超声表现为LBCV走行于食管或气管后方，与奇静脉汇合后流入上腔静脉，形成"U"形血管环；产前超声检出胎儿RELBCV时，应全面筛查是否伴有其他系统异常，必要时行产前基因组疾病检测。     

超声心动图在诊断新生儿期永存左上腔静脉中的价值

目的探讨超声心动图对新生儿期永存左上腔静脉的诊断价值。方法我院产前胎儿超声心动图检查时明确诊断或疑为永存左上腔静脉胎儿共11例,出生后再行超声心动图检查,总结永存左上腔静脉的声像图特征。结果 11例胎儿产后均明确诊断为永存左上腔静脉。超声图像表现为永存左上腔静脉全程可显示,位于主动脉弓左侧,由左颈内静脉与左锁骨下静脉交汇形成,呈"Y"形,近心端与冠状静脉窦相连;永存左上腔静脉血流频谱与右侧上腔静脉相一致。结论超声心动图对新生儿期引流入冠状静脉窦到右心房的永存左上腔静脉有较好的诊断价值。     

胎儿静脉导管异位引流至冠状静脉窦产前超声表现:3例报告及文献复习

目的 结合文献及3例临床所见,观察胎儿静脉导管（DV）异位引流至冠状静脉窦（CS）产前超声表现。方法 回顾性分析3胎DV异位引流至CS胎儿,结合文献复习观察其产前超声特征。结果 产前超声于3胎四腔心后切面均见CS扩张,内径分别为3.6、8.2及4.8 mm;DV异位引流至扩张的CS。于1胎（序号1）见4支肺静脉汇入左心房（LA）,DV经房间隔后方于左心房顶绕行至扩张的CS;1胎（序号2）三血管气管切面肺动脉主干左侧见永存左上腔静脉（PLSVC）,旋转探头追踪观察发现DV与CS相连;1胎（序号3）4支肺静脉均回流入LA,三血管气管切面见主动脉弓位于气管右侧、左锁骨下动脉起自降主动脉起始部。结论 产前超声观察胎儿DV异位引流至CS四腔心后切面可见CS扩张,并可合并其他血管畸形。     

Peripherally inserted central catheter malposition in a persistent left superior vena cava.

Persistent left superior vena cava (PLSVC) is the most common congenital anomaly of the superior vena cava system. Left superior vena cava exists in early embryonic development, but this vessel degenerates as the cardiovascular system matures. Failure of this process to occur results in PLSVC. If not associated with other cardiac malformations, PLSVC has no clinical signs or symptoms. This article discusses a case of PLSVC diagnosed after placement of two peripherally inserted central venous catheters that appeared to malposition outside the central circulation. After unsuccessful bedside placement of the two catheters, the patient was referred to interventional radiology for placement of a Hickman catheter. A contrast injection study showed the presence of a PLSVC with the absence of a normal right superior vena cava. The tip of the peripherally inserted central venous catheter was found to be at a satisfactory location within the PLSVC.     

Clinical significance of persistent left superior vena cava diagnosed in fetal life.

OBJECTIVES: To determine the prevalence and clinical significance of persistent left superior vena cava (PLSVC) in fetuses with and without cardiac and extracardiac anomalies. METHODS: Charts and recorded images were reviewed from high-risk patients who underwent fetal echocardiography between January 2000 and December 2005. This retrospective study included 54 fetuses with confirmed PLSVC who were diagnosed based on the presence of an additional vessel identified to the left of the pulmonary artery in the three-vessel view of the heart. Associated congenital heart defects (CHDs), extracardiac abnormalities, including first trimester nuchal translucency (NT) thickness, and fetal/postnatal outcome were analyzed. RESULTS: Of 5,737 referrals, 5,233 had a normal heart, and PLSVC was observed in 10 of these fetuses (0.2%; Group 1). CHDs were present in 504 and PLSVC was observed in 44 of these cases (9%). In the latter group, 18/44 (41%) fetuses had heterotaxy syndrome (Group 2) where the most common structural heart defects were atrioventricular septal defect and double-outlet right ventricle. Fetuses without heterotaxy syndrome (Group 3) accounted for 26/44 (59%) cases of CHDs associated with PLSVC. In this group of fetuses the most common CHDs were left outflow tract obstructive defects and conotruncal anomalies. Increased NT was observed in 29%, without differences among the three groups. The survival rates among fetuses in Groups 1, 2 and 3 were 100%, 44% and 50%, respectively. After excluding patients who underwent pregnancy termination, there were no significant differences in the survival rates among the groups, probably due to the small size of the samples. CONCLUSIONS PLSVC is associated with CHDs. The identification of PLSVC should prompt a thorough examination of the fetus to identify additional cardiac and extracardiac anomalies. The prognosis of affected fetuses largely depends on whether or not the PLSVC is associated with a CHD.     

三步节段法产前超声诊断胎儿内脏异位综合征

目的探讨产前超声"三步节段法"对诊断胎儿内脏异位综合征的临床价值。方法在40 884胎产前超声检查中采用"三步节段法",胎龄14～38周,平均(27.4±6.5)周;孕妇年龄20～33岁,平均(23.6±4.3)岁。11胎诊断为内脏异位综合征,将其产前超声表现与引产后解剖学所见进行对比分析。结果 11胎内脏异位综合征胎儿中,左异构3胎,右异构8胎;10胎引产后经尸体解剖证实。内脏异位综合征的主要产前超声表现包括内脏心脏异位,下腔静脉离断伴奇静脉异位引流或下腔静脉紧靠腹主动脉和心脏畸形。结论产前超声是诊断胎儿内脏异位综合征有效可行的方法。采用产前超声"三步节段法"全面有序扫查获得主要超声征象可减少漏诊。