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.     

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。     

单中心儿童永存左上腔静脉合并先天性心脏病超声心动图分析

目的 分析儿童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。 方法 对2013年至2017年北京儿童医院1676例永存左上腔静脉患儿的超声心动图资料及临床资料进行回顾性分析。 结果 永存左上腔静脉的检出率约0.79%(1676/212309),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儿童中的检出率约3.0%(866/28403)。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%),常见合并的先天性心脏病依次是室间隔缺损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,应提高对永存左上腔静脉合并先天性心脏病类型分布特征的认识,行超声心动图检查时正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。     

胎儿冠状静脉窦扩张合并心内外畸形的超声表现

目的探讨胎儿冠状静脉窦扩张及其合并心内外畸形的产前超声诊断特点。方法回顾性分析我院经超声诊断的202例冠状静脉窦扩张胎儿,并与出生后随访或引产后病理解剖结果进行对照分析。结果 202例冠状静脉窦扩张胎儿中,合并永存左上腔静脉194例(96.0%),肺静脉异位引流4例,无顶冠状静脉窦1例,同时合并永存左上腔静脉及肺静脉异位引流1例,余2例为单纯冠状静脉窦扩张。其中合并其他畸形78例(38.6%),包括永存左上腔静脉合并其他心内畸形52例,合并心外畸形17例,合并心内外畸形7例;肺静脉异位引流合并其他心内畸形1例;无顶冠状静脉窦合并其他心内畸形1例。结论永存左上腔静脉是胎儿冠状静脉窦扩张的主要原因,其中室间隔缺损、肺动脉狭窄及法洛四联症是其最常见的并发心内畸形。产前超声在胎儿冠状静脉窦扩张及合并心内外畸形鉴别诊断中具有重要意义。     

时间-空间关联成像联合B-flow在胎儿永存左上腔静脉产前超声诊断中的应用价值

目的探讨时间-空间关联成像(STIC)联合B-flow在胎儿永存左上腔静脉产前超声诊断中的应用价值。方法对产前胎儿常规二维超声心动图可疑或明确心脏畸形的132例胎儿进行B-flow显像及STIC数据采集,观察有无左上腔静脉,并将产前超声诊断结果与产后新生儿超声心动图及引产后胎儿尸体解剖病理检查结果进行对比分析。结果 132例胎儿中120例在B-flow显像模式下STIC数据采集成功,采集成功率为90.9%(120/132);120例STIC数据采集成功的胎儿中,常规二维超声心动图诊断永存左上腔静脉15例(15/20)。B-flow-STIC诊断永存左上腔静脉20例。其中5例伴无名静脉缺如,4例合并心脏严重复杂畸形(2例合并完全性房室间隔缺损,1例合并三尖瓣发育不良,1例合并右心室双出口,1例合并右位主动脉弓,5例合并室间隔缺损),6例为单纯性永存左上腔静脉。B-flow-STIC修正常规二维超声心动图诊断5例;其中2例常规二维超声心动图诊断为房间隔缺损,1例常规二维超声心动图诊断为完全性房室间隔缺损,1例常规二维超声心动图诊断为心内型肺静脉异位引流,均经B-flow-STIC修正诊断为永存左上腔静脉;1例常规二维超声心动图漏诊,B-flow-STIC诊断为永存左上腔静脉引流至左心房。结论 STIC联合B-flow可清晰显示胎儿永存左上腔静脉及其空间位置关系,有利于提高永存左上腔静脉产前超声诊断准确性。     

产前超声诊断胎儿永存左上腔静脉

目的探讨胎儿永存左上腔静脉的声像图特征及产前超声诊断胎儿永存左上腔静脉的价值。方法对8例产前超声诊断为永存左上腔静脉胎儿的超声图像特征进行回顾性分析,并结合文献分析总结胎儿永存左上腔静脉各切面声像图特点。结果 8例中1例合并完全性心内膜垫缺损,1例合并Dandy-Walker畸形,2例合并多系统畸形,其余4例为单纯性永存左上腔静脉。结论产前超声诊断胎儿永存左上腔静脉具有很好的可操作性和较高的临床价值。     

胎儿左上腔静脉提示主动脉缩窄及发育不良的比值比分析

目的 探讨永存左上腔静脉与胎儿心脏畸形,尤其是主动脉缩窄及发育不良之间的关系.方法 回顾性分析4 664例胎儿超声心动图结果,计算左上腔静脉和先天性心脏畸形的比值比(OR值)与左上腔静脉和先天性主动脉缩窄及发育不良的比值比等.结果 4 664胎儿超声心动图结果中,无结构性胎儿心脏畸形4 004例,合并左上腔静脉(LSVC) 38例(0.95％);结构性心脏畸形660例,合并LSVC 62例(9.39％)(OR=10.8).660例心脏畸形中,主动脉缩窄和主动脉发育不良47例,同时合并LSVC 17例(36.17％)(OR=7.15).结论 胎儿永存左上腔静脉同样提示胎儿结构性心脏畸形的危险性明显增加.并且胎儿患主动脉缩窄及发育不良的可能性亦增加.提示如果发现LSVC,应追踪观察,除外先天性心脏畸形,尤其是主动脉缩窄及发育不良.     

胎儿永存左上腔静脉的产前超声诊断

目的 探讨胎儿永存左上腔静脉的声像图特征及产前超声诊断胎儿永存左上腔静脉的价值.方法 对6例产前超声诊断为永存左上腔静脉胎儿的超声图像特征进行回顾性分析,并结合文献探讨产前超声诊断胎儿永存左上腔静脉的价值.结果 6例中1例为双胎之一,5例为单胎.1例为单纯性永存左上腔静脉,5例还合并其他心脏异常.1例合并染色体异常.结论 胎儿心脏超声三血管切面可准确诊断胎儿永存左上腔静脉.     

胎儿冠状静脉窦扩张合并心内外畸形的的超声诊断特点

【】 目的：探讨胎儿冠状静脉窦扩张及其合并心内外畸形的产前超声诊断特点。 方法：回顾性分析2011年7月~2015年4月于广州市妇女儿童医疗中心诊断胎儿冠状静脉窦扩张病例,并与出生后随访或引产后病理解剖结果进行对照分析。 结果：共有202例胎儿冠状静脉窦扩张,其中合并永存左上腔静脉194例(194/202, 96%),合并肺静脉异位引流4例,合并无顶冠状静脉窦1例,同时合并永存左上腔静脉及肺静脉异位引流1例,余2例为单纯冠状静脉窦扩张。其中合并其他畸形78例(78/202, 38.6%)。其中永存左上腔静脉合并其他心内畸形52例,合并心外畸形17例,合并心内外畸形7例;肺静脉异位引流合并其他心内畸形1例;无顶冠状静脉窦合并其他心内畸形1例。室间隔缺损、肺动脉狭窄、法洛氏四联症是最常见的并发心内畸形,单脐动脉是最常见的并发心外异常。 结论：永存左上腔静脉是胎儿冠状静脉窦扩张的主要原因,其中室间隔缺损、肺动脉狭窄、法洛氏四联症是最常见的并发心内畸形。产前超声对胎儿冠状静脉窦扩张及合并心内外畸形进行鉴别诊断具有重要诊断意义。     

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。     

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.     

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.     

Ductus venosus blood flow resistance and congenital heart defects in the second trimester

PURPOSE: To study the association between fetal cardiac defects and the presence of abnormal blood flow resistance of the ductus venosus during the second trimester of pregnancy. METHODS: This retrospective case-control study included 72 pregnancies with fetal cardiac anomalies and 267 normal pregnancies. In fetuses with cardiac anomalies, Doppler velocimetry resistances of the ductus venosus were serially determined prior to birth. The Doppler velocimetry resistances obtained from fetuses with cardiac anomalies were in turn compared with median values derived from 267 normal pregnancies to compensate for biometric bias due to gestational age. RESULTS: Among the 72 pregnancies with fetal cardiac anomalies, 26 fetuses showed isolated congenital heart disease (CHD) without nonimmune fetal hydrops (NIFH) (group A), 10 fetuses showed isolated CHD with severe heart defects, including atrioventricular valve insufficiency and consecutive cardiogenic NIHF (group B), and 36 fetuses showed chromosomal abnormalities, nonchromosomal extracardiac malformations, noncardiogenic NIHF, and fetal growth restriction summarized as nonisolated CHD (group C). Based on the pulsatility index for the vein of the ductus venosus, the area under the receiver operating characteristic (ROC) curves was 0.71, 0.73, and 0.86 for groups A, B, and C, respectively. CONCLUSION: In the 36 fetuses from group C, increased pulsatility index for vein of the ductus venosus (DVPIV) yielded a significant area under the ROC curve (0.86) with a sensitivity of 0.78 and a specificity of 0.78. Increased DVPIVs during the second trimester of pregnancy are highly correlated with fetal cardiac anomalies associated with chromosomal and extracardiac anomalies.     

单脐动脉胎儿伴发先天异常的产前超声诊断分析

目的探讨胎儿单脐动脉产前超声表现及伴发先天异常的临床意义。 方法对2014年10月至2015年12月在南京医科大学附属苏州医院产前检查发现单脐动脉的121例胎儿的超声表现、合并其他异常及脐动脉缺失的侧别进行分析。 结果单脐动脉胎儿超声特征：（1）121例单脐动脉胎儿产前二维超声横断面扫查时膀胱周围左侧或右侧仅见1条脐动脉。（2）胎儿单脐动脉检出结果：121例单脐动脉胎儿中左侧脐动脉缺失59例（48.8%,59/121）,右侧脐动脉缺失62例（51.2%,62/121）,左侧与右侧单脐动脉的检出率相近。（3）合并先天异常：121例单脐动脉胎儿中合并其他先天异常16例,以心脏异常占多数（43.8%,7/16）,包括室间隔缺损,法洛四联症,永存左上腔静脉,主动脉弓发育不良,右位主动脉弓,右心房、右心室增大。其中右侧脐动脉缺失胎儿合并其他先天异常的检出率（68.8%,11/16）高于左侧脐动脉缺失的胎儿（31.3%,5/16）。 结论单脐动脉胎儿左侧与右侧脐动脉缺失的检出率相近,但右侧脐动脉缺失合并先天异常的胎儿多于左侧脐动脉缺失的胎儿。     

Extracardiac anomalies, aneuploidy and growth retardation in 100 consecutive fetal congenital heart defects.

The importance of extracardiac anomalies in 100 consecutive fetuses with congenital heart disease was evaluated. The most common cardiac diagnoses were hypoplastic left heart syndrome in 35% and atrioventricular canal defect in 15%. Extracardiac anomalies were present in 42%, abnormal karyotype in 19% and intrauterine growth retardation in 20%. There were 45 survivors (35 required neonatal surgery, ten did not require early surgery) and 80% (36/45) of the survivors had isolated congenital heart disease. Extracardiac anomalies and abnormal karyotype were more frequently present in non-survivors (p < 0.001). Also, intrauterine growth retardation was more frequent in non-survivors (p < 0.05).     

Sonographic screening for renal tract anomalies associated with congenital heart disease

The incidence of structural renal tract abnormalities in 109 children with documented congenital heart disease (CHD) was studied using real-time sonography. In these children, 11.9% had associated urologic anomalies, which included hydronephrosis, duplication, ectopia, agenesis, and dysplasia of kidneys. Children with associated extracardiac anomalies had a significantly higher incidence of renal tract anomalies (39.1%) compared to those with isolated CHD (4.7%). Therefore, sonographic screening of the renal tract should be performed routinely in patients with CHD, especially in those with multiple congenital defects.     

The association between congenital heart disease and Down syndrome in prenatal life.

OBJECTIVE: To assess the relationship between congenital heart disease (CHD) and Down syndrome (DS) in utero. DESIGN: Retrospective case series. SUBJECTS: Fifty-two fetuses with a cytogenetic diagnosis of DS managed at our Fetal Cardiology Unit in the study period. In particular, two populations of fetuses with DS were studied: a group of 41 DS fetuses referred to our unit for fetal echocardiography due to the chromosomal anomaly and a second group of 274 fetuses referred because of suspected CHD, 11 of which were found to have DS. METHODS: All fetuses were submitted to detailed ultrasound evaluation of fetal anatomy. Associated extracardiac anomalies, and presence and type of CHD, were recorded for all fetuses. Karyotyping was obtained by means of cordocentesis or amniocentesis. Necropsy or neonatal echocardiograms were sought for confirmation of the prenatal diagnosis. RESULTS: In the group of 41 fetuses with known DS, the incidence of CHD was 56% ([atrioventricular septal defect (AVSD) 44%, ventricular septal defect (VSD) 48%], the remainder having other heart defects). Conversely, considering the incidence of DS in fetuses with CHD, 43% of all AVSDs (53% of AVSD with normal visceral situs) were associated with DS, whereas none of the 39 cases of VSD was associated with trisomy 21. Ventricular septal defects were diagnosed only in fetuses referred to our center with a known diagnosis of aneuploidy. CONCLUSIONS: We have confined that more than half of the fetuses with DS bear a CHD, which is an AVSD in 44% of cases. Conversely, 43% of fetuses with an AVSD have trisomy 21. For VSDS, the situation is controversial, due to the relatively low detection level of this heart defect at the routine mid-trimester obstetric scan.     

Nuchal translucency and congenital heart defects: heart failure or not?

OBJECTIVE: To determine whether heart failure is the mechanism underlying the association between increased fetal nuchal translucency and congenital heart defects. METHODS: Retrospective analysis of the types of congenital heart defect observed in fetuses with increased nuchal translucency and those with normal nuchal scans. Retrospective quantitative analysis of cardiac size and left ventricular ejection fraction in fetuses with ventricular septal defects or the hypoplastic left heart syndrome. RESULTS: Eighty-three fetuses with congenital heart defects had undergone nuchal screening of which 51 had increased nuchal translucency and 32 had normal nuchal translucency. A wide variety of different congenital cardiac lesions with different hemodynamic effects were observed in fetuses with increased nuchal translucency and those with normal nuchal scans. Defects primarily characterized by left heart obstruction, right heart obstruction and septal defects occurred in both groups. All measurements of cardiothoracic ratio and left ventricular ejection fraction fell within the normal range and there was no significant difference between fetuses with increased nuchal translucency and those with normal nuchal scans. CONCLUSIONS: No specific type of congenital heart lesion is associated with increased nuchal translucency. The contention that heart failure explains the association between congenital heart defects and increased nuchal translucency is not supported by this study.     

Isolated ventricular septal defects detected by color Doppler imaging: evolution during fetal and first year of postnatal life.

OBJECTIVE: To evaluate the development during gestation and up to 1 year postnatally of isolated small ventricular septal defects (VSDs) not visible by gray-scale imaging and detected only on color Doppler fetal echocardiography. METHODS: This was a retrospective analysis of 146 fetuses with isolated VSDs detectable only on color Doppler echocardiography. Complete sequential gray-scale, color Doppler and spectral Doppler examination of the fetal heart were performed. The following variables were documented: site of the VSD, presence of extracardiac or chromosomal anomalies, outcome of the pregnancy and evolution of the defect up to 1 year postnatally. RESULTS: A total of 113 fetuses reached their first year of postnatal life, 23 pregnancies were terminated, there were three stillbirths/neonatal deaths, and seven were lost to follow-up. It was observed that 32.7% (n = 37) of all defects in neonates alive after 1 year closed in utero, 44.3% (n = 50) of defects closed spontaneously within the first postnatal year, and 23.0% (n = 26) of defects did not close. In all, a comparable number of perimembranous and muscular septal defects closed spontaneously in utero and during the first year of postnatal life. Among 35 fetuses with extracardiac anomalies 51.4% (n = 18) were euploid. CONCLUSION: Small VSDs, detectable only by color Doppler echocardiography, show a high spontaneous intrauterine and postnatal closure rate. These findings might be of value for prenatal parental counseling.