产前超声诊断持续性左上腔静脉临床意义的探讨

目的:探讨产前超声诊断一般孕妇人群中胎儿持续性左上腔静脉(PLSVC)的临床意义。方法:回顾性分析2011年1月至2015年7月在上海交通大学附属第六人民医院产前检查并分娩或终止妊娠的连续性孕妇资料15241例。结果:PLSVC胎儿43例,发生率0.28%,均为双上腔静脉。单纯型PLSVC占67.44%(29/43),非单纯型PLSVC(合并除PLSVC外的畸形)占32.56%(14/43)。14例非单纯型PLSVC中,4例仅合并心脏畸形,1例仅合并心外畸形,9例同时合并心脏及心外畸形。单纯型PLSVC 96.55%(28/29)妊娠结局良好,而非单纯型64.29%(9/14)妊娠结局不良(P0.05)。结论:在一般孕妇人群PLSVC有一定的发生率,约1/3合并心内外畸形。非单纯型PLSVC胎儿的预后明显差于单纯型胎儿。产前超声诊断PLSVC是发现更多胎儿畸形和警示胎儿发育不良的重要线索。     

产前超声诊断胎儿持续性右脐静脉及其合并畸形的临床价值

目的 探讨产前超声诊断胎儿持续性右脐静脉及其合并结构畸形的临床价值.方法 回顾性分析2007年4月至2011年8月在复旦大学附属妇产科医院产前检查的38 827例孕妇中,产前超声检出的109例持续性右脐静脉胎儿及其合并结构畸形情况.结果 胎儿持续性右脐静脉的发生率为0.28％(109/38 827),其中单胎100例,双胎9例.109例产前超声诊断的持续性右脐静脉胎儿中,未合并结构畸形者95例(单胎90例,双胎5例),孕妇均正常妊娠分娩,新生儿预后良好,其中有4例行染色体检查,结果均正常.合并结构畸形者14例,发生率为12.8％(14/109),其中合并心血管系统结构畸形者10例,占71.4％(10/14).胎儿合并的结构畸形大多比较严重,例如心内膜垫缺失、右心室双流出道、单心房和单心室等,9例(64.3％,9/14)引产终止妊娠.14例合并结构畸形者仅有1例行胎儿染色体检查,结果正常.结论 产前超声检查发现胎儿持续性右脐静脉后,应仔细检查胎儿各系统结构,了解有无合并结构畸形,尤其是心血管系统.     

胎儿右房增大原因超声诊断研究

目的探讨胎儿右房增大原因的超声诊断思路。方法回顾性分析2005年1月至2014年12月中国医科大学附属盛京医院产前诊断胎儿右房增大的225例病例资料,二维超声结合彩色多普勒扫查胎儿四腔心切面、左右室流出道切面、三血管切面及三血管-气管切面等横断面,扫查腔静脉长轴切面、主动脉弓切面及动脉导管弓切面等矢状面。采用节段分析法除外胎儿先天性心脏病。结果产前超声正确诊断139例(61.8%)胎儿右房生理性增大,11例(4.9%)胎儿三尖瓣下移畸形,21例(9.3%)右房前负荷增大相关畸形,41例(18.2%)右房后负荷增大相关畸形及11例(4.9%)不明原因右房增大。2例(0.9%)产前超声诊断为生理性右房增大的胎儿出生后右房仍大。结论胎儿心脏超声检查发现心脏房室腔改变时应考虑到其他病变所引起的继发改变,分析时应以心脏血流动力学改变为主线,按照节段分析法由内脏-心房水平、心室水平以及大动脉水平逐一分析,以明确真正病因所在。     

胎儿部分型及过渡型房室间隔缺损的产前超声诊断特点

目的 探讨产前超声诊断胎儿部分型及过渡型房室间隔缺损的价值。方法 回顾性分析11例经尸体解剖和产后超声心动图证实的部分型及过渡型房室间隔缺损胎儿的产前超声表现,总结其声像图特征。 结果胎儿超声心动图显示,7例部分型和4例过渡型房室间隔缺损在四腔心切面均显示原发孔型房间隔缺损,二尖瓣和三尖瓣在室间隔的附着点位于同一水平,关闭时与室间隔构成“T”字形图像。7例部分型房室间隔缺损中1例伴有左室-右房通道;4例过渡型房室间隔缺损均伴有室间隔上部缺损。11例胎儿中伴有二尖瓣反流4例,二、三尖瓣反流1例,双侧桡骨缺如1例。 结论产前超声观测心脏“T”字形声像图特征对诊断胎儿部分型和过渡型房室间隔缺损具有重要临床价值,但应注意与冠状静脉窦形成的假象鉴别。     

超声早期诊断胎儿单心室、单心房、永存动脉干一例

胎儿心血管异常以复杂和多种畸形并存,是新生儿死亡的一大关键要素。产前联合应用多种超声成像技术早期准确诊断胎儿先天性心脏畸形,判断其类型和检出合并的畸形,对孕期咨询、评估、抉择及出生后救治具有重要意义,从而达到优生优育的目的。报道1例超声早期诊断胎儿单心室、单心房、永存动脉干的临床资料,观察其在超声下特征性的结构改变,以提高超声医师诊断该疾病的技术水平,使得胎儿复杂畸形在妊娠早中期就能被检查出来。     

超声早期诊断胎儿单心室、单心房、永存动脉干一例

胎儿心血管异常以复杂和多种畸形并存,是新生儿死亡的一大关键要素。产前联合应用多种超声成像技术早期准确诊断胎儿先天性心脏畸形,判断其类型和检出合并的畸形,对孕期咨询、评估、抉择及出生后救治具有重要意义,从而达到优生优育的目的。报道1例超声早期诊断胎儿单心室、单心房、永存动脉干的临床资料,观察其在超声下特征性的结构改变,以提高超声医师诊断该疾病的技术水平,使得胎儿复杂畸形在妊娠早中期就能被检查出来。     

产前超声诊断单脐动脉与胎儿异常的相关性研究

目的探讨超声检测胎儿脐带血管数目预测胎儿先天异常的价值。方法2000年1月至2006年10月在中山大学附属第一医院采用超声检测胎儿脐血管数目，对单脐动脉胎儿行产前超声系统筛查及胎儿染色体检查；分析单脐动脉合并畸形类型，与染色体异常的关系及胎儿结局。结果产前超声诊断胎儿单脐动脉119例，包括左侧72例（60．5％），右侧47例（39．5％）。单纯性单脐动脉59例（49.6％）；合并其他结构畸形60例（50．4％），其中泌尿系统畸形25例（41．7％），心血管系统畸形17例（28.3％），中枢神经系统畸形15例（25.0％），下肢畸形9例（15．0％），前腹壁和消化道畸形各5例（各占8．3％），唇裂或唇腭裂畸形3例（5．0％），其他畸形3例（5．0％）。行胎儿染色体检查41例，发现染色体异常8例，包括18-三体综合征3例、13-三体综合征1例、21．三体综合征1例、染色体片段异常3例，均合并其他畸形。结论胎儿单脐动脉左侧发生率高于右侧，约50％合并其他畸形；合并畸形时，染色体异常发生率较高；大部分单纯性单脐动脉胎儿结局良好。     

产前超声诊断在胎儿左侧异构中的应用

目的:探讨胎儿左侧异构的产前超声征象及其诊断价值。方法:回顾分析21例产前诊断为胎儿左侧异构的病例资料,对比总结其超声征象与随访结果。结果:21例左侧异构胎儿中,内脏心脏异位16例,先天性心脏病19例(伴心脏传导阻滞5例),下腔静脉离断伴奇静脉异常连接19例,其中两种及两种以上异常诊断胎儿左侧异构的敏感性为90.5%。染色体异常3例,18三体2例,13三体1例。18例终止妊娠,1例胎死宫内,2例存活。结论:产前超声诊断胎儿左侧异构是可行的,重要超声征象是内脏心脏异位、先天性心脏病伴或不伴心脏传导阻滞、下腔静脉离断伴奇静脉异常连接,出现上述两种或两种以上异常可提高该病的诊断敏感性。     

产前超声心动图诊断胎儿永存左上腔静脉合并主动脉或圆锥动脉干发育异常的临床分析

目的:探讨应用产前超声心动图诊断永存左上腔静脉(PLSVC)合并主动脉或圆锥动脉干发育异常的相关问题。方法:回顾性分析2016年12月至2017年7月于重庆市妇幼保健院行产前胎儿超声心动图检查诊断PLSVC胎儿78例的临床情况。结果:78例产前超声心动图诊断PLSVC的胎儿均为双上腔静脉,其中33例为单纯型PLSVC,占42%;45例为非单纯型PLSVC(合并除PLSVC外的畸形),占58%。45例非单纯型PLSVC中合并主动脉发育异常10例(13%)、圆锥动脉干发育异常14例(18%),其中包括法洛四联征7例(9%),永存动脉干3例(4%)、大动脉转位2例(3%),主-肺动脉窗1例(1%),永存第5弓1例(1%)。结论:胎儿患PLSVC的同时合并有主动脉或圆锥动脉干发育异常的可能性增加,对诊断PLSVC的胎儿产前超声心动图应重点检查主动脉及圆锥动脉干,以排除相关异常。     

胎儿先天性膈疝及其相关异常的产前诊断

目的 分析胎儿先天性膈疝与合并其他畸形及染色体异常的相关性,探讨可能影响膈疝预后的因素. 方法 总结2002年1月至2008年11月在我院产前超声诊断的胎儿膈疝病例,分析其类型、合并畸形种类、与染色体异常的关系及临床结局.采用Fisher精确概率法进行统计学分析. 结果 产前超声诊断胎儿膈疝71例,左侧62例(87.3%),右侧9例(12.7%).单纯型膈疝38例(53.5%),复合型膈疝33例(46.5%).复合型膈疝中合并的其他异常中常见的有心血管系统畸形18例(54.5%),神经系统畸形10例(30.3%),泌尿系统畸形9例(27.2%).71例膈疝病例中19例行胎儿染色体核型检查,其中12例单纯型膈疝胎儿的染色体均正常,7例复合型膈疝中4例(4/7)染色体核型异常:18-=体综合征2例,13、21-三体综合征各1例.复合型膈疝病例染色体异常的发生率高于单纯型膈疝(P=0.009).复合型膈疝病例均引产;单纯型膈疝病例中32例引产,6例继续妊娠,其中5例足月出生后接受膈疝修补术,均存活;1例出生后死亡. 结论 胎儿先天性膈疝以左侧多见,近半数合并其他异常且以心血管系统畸形为主.单纯型膈疝发生染色体异常的风险低,复合型膈疝发生染色体异常的风险高.膈疝手术对预后的影响尚不能确定.     

Fetale Heterotaxie-Syndrome

The spectrum of conditions associated with heterotaxy syndromes includes a wide variety of cardiovascular and visceral anomalies that are differently distributed amongst the two clinical variants, left and right isomerism, and which determine the intrauterine as well as the postnatal course and outcome. An exact prenatal diagnosis is therefore warranted. Important sonographic markers in heterotaxy syndromes are the associated anomalies of the situs and cardiac defects. Prenatal differentiation of the two clinical variants can be based on the anomalies, the course of the inferior vena cava and the presence of a heart block.In left isomerism, mortality is highest in the prenatal period due to frequent association with a complete heart block and subsequent intrauterine heart failure. In the postnatal period, the outcome depends mainly on the associated cardiac malformations and their ability to be corrected.In contrast, in right isomerism the mortality is highest in the postnatal period. This is mainly due to the more complex type of associated cardiac malformations.     

Situs ambiguo asociado a cardiopatía e interrupción de la vena cava inferior con continuidad de la ácigos

There is a spectrum of heterotaxic syndromes between normal organ distribution (situs solitus) and congenital conditions in which major organs are mirrored from their normal position (situs inversus). The two main modalities are left isomerism (with polysplenia) and right isomerism (with asplenia). Heterotaxic defects involve ciliary dyskinesia, hampering migration of embryonic organs and leading to malposition of thoracic and abdominal organs, complex cardiac defects, and other malformations. We present a case of prenatal echographic diagnosis of levocardia, with left-sided liver, asplenia, congenital heart disease, and interruption of the inferior vena cava with azygos continuation. Because of the malformations frequently associated with heterotaxy, the position of fetal organs should form part of routine ultrasonographic examination.     

Disorders of laterality and heterotaxy in the foetus

Disorders of laterality and heterotaxy syndromes are rare diseases with an incidence of 1-1.5/10,000 live births. They are associated with numerous viscerocardiac anomalies and malformations. In particular, heterotaxy syndromes are associated with complex cardiac and extracardiac malformations that have an important impact on the prenatal and postnatal course. A prenatal differentiation between the 2 main variants of heterotaxy--left and right isomerism--is possible by assessment of cardiac rhythm, anomalies of caval veins and descending aorta and concomittant cardiac and extracardiac anomalies. An exact diagnosis is mandatory for adequate counselling of the parents and planning of postnatal care. Left isomerism has a high intrauterine mortality, caused by early atrioventricular block with subsequent cardiac failure and hydrops. In contrast, right isomerism has a high postnatal mortality due to the more complex type of cardiac defects and splenic disorders. The type of associated cardiac and extracardiac anomalies determines the postnatal morbidity and mortality. Polysplenia and asplenia may be associated with immunological disorders, that cannot be ruled out in the prenatal period, and further complicate the postnatal course.     

子宫外脉管内平滑肌瘤病的诊治

脉管内平滑肌瘤病发生与雌激素密切相关。该类子宫肌瘤可以超出子宫范围,可在静脉内延伸达下腔静脉,甚至心脏。由于临床症状多变且不典型,早期诊断十分困难。病变侵及下腔静脉者及右心或肺动脉者可引起充血性右心衰的表现和猝死。此时需要与静脉血栓栓塞症、心房黏液瘤等鉴别。CT和MRI是重要的影像诊断方法。治疗原则是手术彻底切除所有病变,以降低复发率。如病变局限于盆腔,建议行全子宫切除术+盆腔病灶切除术+双侧附件切除,如果累及大血管甚至心腔,完整手术切除病灶需要多学科合作完成。     

Coupe des trois vaisseaux et de la trachée en période prénatale : aspects anormaux

Good knowledge of normal characteristics of prenatal three vessels and trachea view allows not only to detect, but also to identify abnormalities such as normal variants and malformations of interest to main vessels (trunk of the pulmonary artery and its branches, the aorta and the superior vena cava) and various vascular structures in the anterior-superior mediastinum. These abnormalities may be isolated or associated with other anomalies of cardiac architecture.     

Left atrial isomerism in the adolescence: report of two cases.

Atrial isomerism is very rare in adolescence. Two cases of left atrial isomerism are reported here in 2 females, aged 21 and 19 years. They had presented with cyanosis and dyspnea since childhood. High kilovoltage filter films showed a bilateral morphologically left bronchus. Cardiac catheterization in Case 1 revealed normal pulmonary artery pressure, severe subvalvular pulmonic stenosis, a double outlet right ventricle, a significant oxygen step-up at the atrial level and moderate systemic oxygen desaturation; while Case 2 disclosed pulmonary hypertension and mild systemic oxygen desaturation. Both cases had the following anatomical features: ipsilateral connection of pulmonary veins to the bilateral morphological left atrium; interrupted inferior vena cave with azygos or hemiazygos continuation; total anomalous hepatic venous return to the right-sided atrium; complete atrioventricular canal. The diagnoses were confirmed in both cases at surgical correction.     

3D ultrasound and Doppler angiography for evaluation of fetal cardiovascular anomalies

ObjectiveTo highlight the value of 3D ultrasound in the prenatal assessment of fetal cardiovascular anomalies through offline diagnosis and/or second opinion (e.g. via internet link).MethodsA retrospective offline analysis of volume datasets of fetuses diagnosed with cardiovascular anomalies by 2D ultrasound was conducted.ResultsThirty-three fetuses with 38 cardiac malformations were evaluated. Mean gestational age at diagnosis was 26 weeks (range, 20–34 weeks). Isolated cardiovascular malformations were detected in 23 fetuses. Extracardiac abnormalities were identified in 8 fetuses, of which 2 had trisomy 21 and 1 had trisomy 18. One fetus exhibited 22q11 microdeletion. Ten pregnancy terminations were performed.ConclusionOffline analysis of cardiovascular anomalies conferred significant diagnostic advantages over 2D ultrasound. 3D ultrasound is invaluable for the prenatal diagnosis and management of congenital heart diseases. It may be used to facilitate scientific cooperation between high- and low-income countries.