产前超声诊断胎儿动脉导管提前闭合1例

正孕妇37岁,单胎妊娠,孕37周,常规产检超声发现胎儿右心增大;既往孕1产0,无特殊病史,否认近期用药史,近5天日均进食葡萄约500g。胎儿超声:四腔心切面(图1A)见心胸比0.46,右心增大,右心室壁运动良好,有极少量心包积液;三尖瓣口见瓣叶活动,血流暗淡,流速46cm/s,关闭时大量反流,反流束约占右心房面积50%,反流速度2.9m/s;卵圆孔增大,直径8.4mm,并见右向左分流;三血管切面见动脉导管呈沙漏样,主动脉端纤细,直径仅1.2mm,CDFI未见血流信号通过(图1B),频谱未测及;肺动脉增宽,与主动脉比值约1.5,见窄带样低速高阻型频谱,流速29cm/s;静脉导管a波反向;超声心动图心衰评分8分。     

胎儿Galen动静脉畸形的彩色多普勒超声诊断及分析

目的探讨彩色多普勒超声诊断胎儿颅内Galen动静脉畸形的价值及临床意义。方法对2004年6月至2010年12月经本院行常规及系统产科超声诊断为胎儿颅内Galen动静脉畸形的3例孕妇进行回顾性分析。结果超声检查发现3例胎儿颅内脑中线附近,第三脑室上方单一囊性灶。彩色多普勒见病灶内彩色血流信号,脉冲多普勒为双相湍流静脉频谱。胎儿心脏超声心动图检查发现2例胎儿右心增大,上腔静脉扩张,三尖瓣返流,其中1例还伴有心包积液、胸腹水,皮肤水肿及羊水过多。结论彩色多普勒超声检查是产前诊断胎儿颅内Galen动静脉畸形必不可少的检查手段。     

鲁登巴赫综合征合并多瓣膜联合病变外科治疗一例

<正>临床资料患者,女,50岁,因"活动后气促、乏力20余年,加重伴下肢水肿1年"入院。超声心动图示房间隔缺损(继发孔-中央型),左向右分流为主双向分流,多瓣膜联合病变:二尖瓣重度狭窄,三尖瓣重度反流伴中度狭窄,主动脉瓣中度狭窄伴轻度反流,中-重度肺动脉瓣反流、中-重度肺动脉高压,双心房、右心室显著增大(图1);房颤心律;肺动脉收缩压60 mm Hg,肺血管阻力升高,Wood指数3.1,Qp/Qs=1.7:1。围术期口服贝前列素、地高辛、利尿     

一岁内重度肺动脉瓣狭窄经胸肺动脉瓣球囊扩张成形术疗效分析

目的总结经胸肺动脉瓣球囊扩张成形术对低龄婴儿重度肺动脉瓣狭窄的即刻疗效及近中期随访结果,探讨一站式肺动脉瓣球囊扩张术的有效方法。方法 2006年3月至2010年3月,阜外心血管病医院采用经胸肺动脉瓣球囊扩张成形术治疗32例重度肺动脉瓣狭窄婴儿患者,男23例,女9例;月龄5 d~11个月(4.59±3.21个月);体重2.3~10.5 kg(6.48±2.05 kg)。均在全身麻醉、气管内插管和超声引导下进行肺动脉瓣球囊扩张。术后随访超声心动图,测量肺动脉瓣跨瓣压差,评估肺动脉瓣反流程度。结果手术成功率为100%,均未出现严重并发症,术后血流动力学稳定,跨瓣压差由术前的82±27 mm Hg下降至23±12 mm Hg(t=15.28,P0.05);4例患者出院时复查超声心动图提示:跨瓣压差仍高于40 mm Hg。所有患者三尖瓣反流均明显改善,无反流17例,微小量反流13例,中量反流2例;血氧饱和度均大于95%。随访1个月~4年(16±11个月),疗效满意,所有患者均生长发育良好,肺动脉瓣跨瓣压差17±10 mm Hg,仅1例肺动脉瓣跨瓣压差大于40 mm Hg;发生肺动脉瓣反流24例,主要为少量反流23例,仅1例中量反流。结论经胸肺动脉瓣球囊扩张成形术是治疗新生儿及婴幼儿重度肺动脉瓣狭窄一种安全有效的方法。     

分期全腔静脉-肺动脉连接术治疗复杂先天性心脏病

目的 总结分期全腔静脉.肺动脉连接术(TCPC)治疗复杂先天性心脏病的经验.方法 1998年6月至2008年3月,22例先天性心脏病复杂畸形患者接受分期TCPC.本组中单心室合并肺动脉狄窄9例,合并肺动脉闭锁3例;完全性大动脉转位,十字交叉心,肺动脉瓣狭窄1例;完全性房室通道,左心窒发育不良,肺动脉闭锁,房室瓣少-中量反流1例;完全性房室通道,动脉导管未闭,肺动脉狭窄,双向Glenn术后伴吻合口狭窄房室瓣大量反流1例;镜面右位心,功能性单心室,肺动脉闭锁,动脉导管未闭,体肺侧支,双侧双向Glenn术后2年,右下肺动-静脉瘘1例;三尖瓣闭锁并肺动脉狭窄4例;三尖瓣闭锁伴肺动脉闭锁,动脉导管未闭1例;镜面右位心,右心室双出口,窒间隔缺损,左心室发育小良,功能性单心房,肺动脉瓣狭窄,三尖瓣少量反流,粗大体肺侧支1例.第一期体肺分流术后,二期双向Glenn术5例,第一期单侧或双侧双向Glenn手术17例.行双向Glenn手术的年龄为(5.9±4.4)岁,Glenn术前肺动脉压为17～20 mm Hg(1 mm Hg=0.133 kPa);房室瓣膜关闭不全3例;一期手术前Nakata指数小于200 mm2/m2者4例.接受TCPC术平均年龄为(9.6±4.9)岁,与双向Glenn术间隔时间(3.7 ±1.2)年,术前经上腔静脉置管测得肺动脉压力均小于15 mm Hg.全组22例均采用心外管道TCPC,房窜瓣成形1例.结果 全组住院死亡1例,病死率为4.5%.死亡病例为单心室,肺动脉闭锁,第一期体肺分流术后,二期左肺动脉成形双向Glenn术后5年第三期行TCPC,术后左肺反复多次大出血死亡.余患者均顺利出院,术后中心静脉压12～18 mm Hg.出院前经皮测血氧饱和度为90%～96%,活动量明显增加,发绀及气促症状消失,心功能均为NYHA分级Ⅰ～Ⅱ级.结论 对于高危患者根据病情选择分期TCPC,可以使更多有高危因素不能行Fontan手术的患者得到救治的机会,扩大手术适应证,并取得满意疗效.     

Ebstein畸形的外科治疗

目的总结Ebstein畸形(Ebstein anomaly)的手术治疗经验,以提高临床疗效。方法 2005年5月至2010年9月济宁医学院附属医院手术治疗Ebstein畸形21例,其中男7例,女14例;年龄3～46(17±11)岁。心功能分级(NYHA)Ⅰ级7例,Ⅱ级10例,Ⅲ～Ⅳ级4例;超声心动图提示:三尖瓣重度反流12例,中度反流4例,轻度反流5例;全组患者中1例行三尖瓣置换术,20例行三尖瓣成形术,采用Danielson法2例,Carpentier法18例,其中5例行一个半心室矫治术;同期矫治合并畸形。结果术后无死亡,术后发生低心排血量及室性心律失常各1例,经积极治疗痊愈;复查超声心动图提示:三尖瓣反流减轻。术后随访1个月～5年,7例三尖瓣反流消失;13例存在轻度三尖瓣反流;1例三尖瓣反流加重,心功能不全,于术后3年行三尖瓣置换术,术后心功能恢复至Ⅰ～Ⅱ级。结论 Ebstein畸形是一种少见的先天性心脏病,采用Carpentier法施行三尖瓣成形效果良好;对三尖瓣和右心室发育不良患者施行一个半心室矫治,有利于改善右心功能。     

超声心动图诊断胎儿冠状静脉窦扩张

目的探讨胎儿冠状静脉窦(CS)扩张的超声诊断思路。方法回顾性分析145胎正常冠状静脉窦胎儿(正常组)和72胎CS扩张胎儿(CS组)的资料,于非标准四腔心切面下显示CS长轴,测量CS的矢状切面面积。采用二维超声结合彩色多普勒超声对所有胎儿分别进行胎儿四腔心切面、左右心室流出道切面、三血管切面、大动脉短轴切面、主动脉弓切面、动脉导管弓切面及腔静脉长轴切面扫查,观察CS的特点。结果胎儿CS矢状切面面积与孕周呈正相关(正常组:r=0.954,P0.05;CS组:r=0.904,P0.05),同孕周正常组胎儿CS矢状切面面积小于CS组(P均0.01)。产前超声诊断72胎CS扩张的胎儿中,52胎为永存左上腔静脉,15胎为完全型肺静脉异位引流,5胎右心压力负荷增大。结论胎儿CS矢状切面的面积与孕周呈正相关;胎儿心脏超声检查发现CS扩张时应同时考虑其他心内畸形;临床应通过多切面、多角度扫查,分析和诊断引起CS扩张的病因。     

室间隔完整型肺动脉闭锁手术治疗的个体化方案

目的 总结室间隔完整型肺动脉闭锁(pulmonary atresia with intact ventricular septum,PA/IVS)的个体化手术方案.方法 回顾2004年5月至2012年5月,共收治72例PA/IVS患儿(婴),生后1天～5.2岁.平均(1.3±0.6)岁;三尖瓣(TV)中到重度反流58例;均行急诊或亚急诊手术.首次就诊非体外循环下单纯行BT分流术(A组)16例;体外循环下行肺动脉瓣切开后跨瓣补片术加或不加BT分流术(B组)33例;镶嵌治疗方法,闭式肺动脉瓣口的疏通术加或不加BT分流术(C组)22例.其中有16例已完成二期手术.结果 术后死亡10例(13.9％),其中行体外循环下行肺动脉流出道的疏通再加BT术者6例,镶嵌治疗者2例.术后随访,超声检查显示肺动脉瓣的跨瓣压差15～39 mm Hg(1 mm Hg=0.133 kPa),平均(23±5)mm Hg;三尖瓣反流明显改善,中度反流11例,轻到中度13例,其余均为轻度.术后随访2～5年,8例双心室修补,5例中度的三尖瓣反流加重到重度,右心室严重的发育不良者已行1 1/2心室修补,2例分别完成了单纯BDG和Fontan术.结论 PA/IVS的治疗方法中体肺动脉分流术(MBTS)是常用的姑息手术方法,首次就诊镶嵌治疗方法优于传统的体外循环方法纠治,随访三尖瓣的Z值和右心室发育情况决定双室修补、1 1/2心室或Fontan术.     

产前超声诊断胎儿右位主动脉弓并右位动脉导管

目的探讨产前超声诊断胎儿右位主动脉弓并右位动脉导管的价值。方法回顾分析11胎右位主动脉弓并右位动脉导管的胎儿超声心动图特征,重点观察三血管气管切面主动脉弓与肺动脉动脉导管的连接形态、主动脉弓和动脉导管与气管的位置关系。结果右位主动脉弓并右位动脉导管时,主动脉弓和动脉导管均位于气管的右侧,呈反向的"V"型连接,即主动脉弓和动脉导管仍呈"V"型连接,但位于气管的右侧。所有11胎右位主动脉弓并右位导管胎儿均不伴迷走左锁骨下动脉,未形成血管环。结论胎儿右位主动脉弓并右位导管的诊断切面为三血管气管切面,但不表现为右位主动脉弓并左位导管时特征性的"U"型连接,诊断的关键在于仔细观察主动脉弓和动脉导管与气管的位置关系。     

外科治疗二尖瓣、三尖瓣置换术后三尖瓣血栓形成一例

患者女，57岁。因二尖瓣狭窄直视分离术后21年，心悸1^＋年，加重3^＋月入院。临床诊断：风湿性心脏病，二尖瓣狭窄直视分离术后重度狭窄，三尖瓣重度反流，心房颤动，心功能Ⅲ级。于2006年12月14日在全身麻醉体外循环下，采用St．Jude27号和31号机械瓣膜分别行二尖瓣和三尖瓣置换术。出院前彩色超声心动图提示：二尖瓣位、三尖瓣位机械瓣稳定，瓣叶活动好，     

Anatomy of fetal circulation

This article describes the fetal circulation with its three shunts: the ductus venosus; the foramen ovale between the right and left atria; and the ductus arteriosus, which links the descending aorta with the pulmonary trunk. These shunts form an ingenious mechanism, which ensures that oxygenated blood returning to the fetus in the left umbilical vein (the right having regressed early in fetal life) is shunted preferentially into the aortic root, to supply the heart muscle via the coronary arteries, and the cerebral tissues via the vertebral and carotid arteries. At birth, the foramen ovale closes leaving as its relic the fossa ovalis (though this remains probe patent in 10% of patients), the ductus venosus closes and remains as the ligamentum venosum of the adult, while the ductus arteriosus fibroses into the ligamentum arteriosum.     

超声诊断孕早期胎儿颈部水囊状淋巴管瘤

目的探讨孕早期胎儿颈部水囊状淋巴管瘤（NCH）的超声诊断价值。方法收集2005年1月—2011年8月在我院发现的NCH胎儿36胎,超声观察其囊状扩张的部位、大小、内部情况及胎儿有无水肿,头部、胸腹内脏、四肢、脐带有无异常,并进行随访,记录产后新生儿复查情况或染色体、引产的结果。结果 36胎NCH胎儿中,1胎NCH位于腋下,35胎位于颈部。13胎接受染色体检查,其中6胎染色体核型正常;7胎异常染色体核型中,3胎核型为45,XO;1胎为46,XX/45,XO;1胎Bart＇s水肿;1胎21-三体;1胎合并上肢内翻,46,XY。全部36胎中,1胎出生后证实合并尿道下裂;3胎合并全身水肿,中、晚孕胎死宫内;3胎合并心脏畸形,在中、晚孕期接受引产;2胎单房性淋巴水囊瘤自行消失,出生后观察至产后6个月,未见明显结构异常;其余27胎均引产,并经尸体检查证实。结论孕早期超声可在检测胎儿颈部透明层厚度的同时早期诊断NCH,判断有无分隔、合并畸形,对临床医师评估胎儿预后、指导临床处置、适时终止妊娠、实行优生优育有重要临床意义。     

The effect analysis and comparison between gastroschisis and tracheal ligation on experimental diaphragmatic hernia in fetal rabbits

Purpose

The authors analyzed and compared the effects of experimentally induced gastroschisis and tracheal ligation on pulmonary hypoplasia in fetal rabbits with congenital diaphragmatic hernia.

Methods

Twenty-three pregnant rabbits underwent fetal surgery on gestational day 24 through 27. Left diaphragmatic hernia was created in 1 fetus (DH group) from each rabbit, and a left diaphragmatic hernia with gastroschisis (GS group) or tracheal ligation (TL group) was created in another fetuses. The fetuses were delivered on gestational day 27 through 33. Histologic and morphometric examination of the lungs were performed in each group.

Results

In the DH group, the lungs were hypoplastic with a decrease in lung weight to body weight ratio and an increase in pulmonary arterial wall thickness. The alveolar septae were markedly thickened and diminished alveolar air spaces. In GS and TL groups, the alveolar septae were thickened but narrower than those of the DH group, air spaces were increased, and the pulmonary arterial wall was only slightly thickened.

Conclusions

Pulmonary hypoplasia seen in newborn rabbits after experimentally induced diaphragmatic hernia is less severe in those rabbits with concurrently made gastroschisis or tracheal ligation. From the histologic viewpoint, the effects of gastroschisis and tracheal ligation on pulmonary hypoplasia in diaphragmatic hernia have no differences.     

The effect of gastroschisis on experimental diaphragmatic hernia in fetal rabbits

Purpose

In this study, the authors analyzed the effect of experimentally induced gastroschisis on pulmonary hypoplasia in fetal rabbits with congenital diaphragmatic hernia (CDH).

Methods

Twenty-three pregnant rabbits underwent fetal surgery on gestational day 24 through 27. A left diaphragmatic hernia was created in 1 fetus (DH group) from each rabbit, and a left diaphragmatic hernia with gastroschisis was created in another fetus (GS group). The fetuses were delivered on gestational day 27 through 33. Histologic and morphometric examination of the lungs in each group was done.

Results

In the DH group, the lungs were hypoplastic with a decrease in lung weight to body weight ratio and an increase pulmonary arterial medial wall thickness. The alveolar septae were markedly thickened with increased interstitial tissue and diminished alveolar air spaces. In the GS group, the alveolar septae were thickened but narrower than those of DH group, and air spaces were increased. The pulmonary arterial wall was markedly thickened in the DH group but only slightly thickened in the GS group.

Conclusions

Pulmonary hypoplasia seen in newborn rabbits after experimentally induced diaphragmatic hernia is less severe in those rabbits with both gastroschisis and DH.     

孕早期胎儿心脏结构的超声筛查

目的探讨孕11~13~(+6)周经腹部超声筛查胎儿心脏畸形的可行性及临床价值。方法于孕11~13~(+6)周对3360胎行心脏四切面扫查,包括胎儿四腔心切面、三血管切面、左心室流出道切面及右心室流出道切面。根据孕周分为A(11~11~(+6)周)、B(12~12~(+6)周)、C(13~13~(+6)周)3组,比较3组胎儿心脏切面满意显示率。并于该时期筛查胎儿严重心脏畸形并随访至中孕期。结果 C组心脏各切面显示率明显高于A、B组,差异有统计学意义(P0.05)。3360胎中于早孕期筛查出心脏异常12胎,并于中孕期得以证实。结论孕早期经腹部超声筛查胎儿严重心脏畸形是可行的。     

时间-空间相关成像技术联合反转模式诊断胎儿先天性心脏病

目的评价时间-空间相关成像(STIC)技术联合反转模式在产前诊断胎儿先天性心脏病中的应用价值。方法收集15胎先天性心脏病胎儿和54胎正常胎儿,每胎获取两组容积数据,分别以四腔心胸腔横断切面和主动脉弓长轴切面为初始采集平面,再用反转模式对容积数据进行重建。结果 54胎正常胎儿共获取108组容积数据,对其中103组容积数据成功进行了反转重建;对15胎先天性心脏病胎儿均成功进行反转重建;重建图像均能直观地显示心内结构和心外血管的空间位置关系。结论 STIC技术联合反转模式重建图像可提供更多重要解剖信息,有助于更好地产前可视化诊断胎儿复杂性先心病。     

胎儿主动脉弓离断的产前超声诊断

目的探讨胎儿主动脉弓离断的超声诊断特点。方法回顾性分析产前超声诊断为胎儿主动脉弓离断的35胎胎儿资料,同时纳入180胎正常胎儿。二维超声判断胎儿内脏及心脏位置,获取四腔心切面、左右心室流出道切面、三血管切面及三血管-气管切面等横断面,并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切面。测量主动脉弓离断胎儿左右心室内径比值及主动脉与肺动脉内径比值,并与相应孕周的正常胎儿进行比较。结果产前二维超声诊断A型、B型及C型主动脉弓离断分别为16、18及1胎,均伴室间隔缺损,共27胎经尸体检查或产后检查证实产前诊断,3胎诊断错误,余5例新生儿失访。四腔心切面及三血管切面发现主动脉弓离断胎儿左右心室内径比值和主动脉与肺动脉内径比值显著小于相应孕周正常胎儿,差异有统计学意义(P均0.01)。矢状切面对主动脉追踪连续扫查可确定主动脉的分支及走行,可明确诊断及分型。A型、B型及C型离断升主动脉走行及分支分别呈现典型的"W型"、"Y型"及"I型"。矢状面扫查及横断面扫查对胎儿主动脉弓离断的诊断率分别为90.00%(27/30)及56.67%(17/30),差异有统计学意义(P0.01)。结论产前二维超声可诊断胎儿主动脉弓离断,横断面扫查可发现某些线索,矢状面连续扫查对诊断胎儿主动脉弓离断具有重要意义。     

Severe pulmonary hypoplasia associated with giant cervical teratomas

Background

The use of the ex utero intrapartum treatment (EXIT) procedure has salvaged many fetuses with giant neck masses. Despite an adequate airway, a subset of these patients die from an inability to achieve adequate gas exchange.

Methods

We reviewed our experience with the EXIT procedure from 1996 to 2004. The EXIT was used to deliver 23 fetuses with giant neck masses.

Results

Three fetuses with giant cervical teratomas died of severe pulmonary hypoplasia. On postmortem, these patients had severe airway distortion by the mass. The carina was retracted superiorly to the first or second rib resulting in compression of the lungs in the apices of the chest and pulmonary hypoplasia. Hypoplasia was reflected in the lung weights of 24 vs 38 g and 17 vs 34 g for age-matched normal lung.

Conclusions

Unsuspected obstructive fetal neck masses can be fatal because of an inability to secure an airway. Prenatal ultrasonography can identify fetuses at risk, allowing the fetus to be salvaged using the EXIT procedure. Despite obtaining airway control, a subset of these patients will die because of pulmonary hypoplasia. When counseling patients with large cervical masses it is important to discuss potential pulmonary hypoplasia in these patients.     

The effects of tracheal occlusion on Wnt signaling in a rabbit model of congenital diaphragmatic hernia

PurposeTracheal occlusion (TO) reverses pulmonary hypoplasia (PH) in congenital diaphragmatic hernia (CDH), but its mechanism of action remains poorly understood. Wnt signaling plays a critical role in lung development, but few studies exist. The purpose of our study was to a) confirm that our CDH rabbit model produced PH which was reversed by TO and b) determine the effects of CDH +/− TO on Wnt signaling.MethodsCDH was created in fetal rabbits at 23 days, TO at 28 days, and lung collection at 31 days. Lung body weight ratio (LBWR) and mean terminal bronchiole density (MTBD) were determined. mRNA and miRNA expression was determined in the left lower lobe using RT-qPCR.ResultsFifteen CDH, 15 CDH + TO, 6 sham CDH, and 15 controls survived and were included in the study. LBWR was low in CDH, while CDH + TO was similar to controls (p = 0.003). MTBD was higher in CDH fetuses and restored to control levels in CDH + TO (p < 0.001). Reference genes TOP1, SDHA, and ACTB were consistently expressed within and between treatment groups. miR-33 and MKI67 were increased, and Lgl1 was decreased in CDH + TO.ConclusionTO reversed pulmonary hypoplasia and stimulated early Wnt signaling in CDH fetal rabbits.Type of studyBasic science, prospective.Level of evidenceII.