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。     

胎儿主动脉弓离断的产前超声诊断现状

主动脉弓离断是一种罕见的严重先天性心脏病,胎儿期死亡率极高,其胚胎学起源复杂,表现形式多样,且多合并其他畸形,产前超声诊断困难。超声心动图作为目前临床常用的胎儿心脏检查手段,在先天性心脏病的筛查中发挥重要作用。本文主要从二维超声、三维超声在诊断中的应用及超声鉴别诊断方面对胎儿主动脉弓离断的产前超声诊断现状进行综述。     

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。     

智能三维超声成像在胎儿领域的应用进展

胎儿产前超声检查对胎位和诊断医师的技术水平依赖性较强,尤其当胎儿存在复杂的心脏和颅脑结构异常时,超声检查难度更大。智能三维超声可对容积数据进行"自动"分析和测量,简化操作流程,获得诊断所必须的标准切面,减少对操作医师技术的依赖性,提高对疾病的诊断准确率。本文对智能三维超声成像技术在胎儿心脏切面、颅脑正中矢状面、颈项透明层切面的自动获取及参数自动测量等方面的应用特点和新进展进行综述。     

超声诊断胎儿先天性血管环1例

<正>孕妇26岁,孕1产0,乙肝表面抗原阳性,婚前接触苯、甲苯3年,无先天性心脏病家族史。孕34周产前超声:胎儿双顶径8.74cm,头围30.51cm,股骨长约6.67cm,肝、胃、肾脏、膀胱及四肢正常;胸骨旁四腔心切面示心房正位,心室右襻,大动脉起源正常,左右心室基本对称,降主动脉位于脊柱前方;升主动脉长轴切面示主动脉根部发出左侧颈总动脉,沿气管左侧绕行(图1A);主动脉弓切面示主动脉弓部仅见右侧颈总动脉、右侧锁骨下动脉(图1B);三血管-气管切面示肺动脉和动脉导管位于气管左侧,主动脉弓位于气管右侧,左锁骨下动脉发自降     

中晚孕期超声显示鼻骨异常伴舌外伸检出唐氏综合征

目的探讨中晚孕期超声显示胎儿颜面部鼻骨异常伴伸舌辅助诊断唐氏综合征(DS)的价值。方法纳入资料完整的经胎儿系统超声筛查及羊水或脐带血染色体检查的中晚孕期孕妇5657名。观察并分析正中矢状切面胎儿鼻骨发育情况及舌尖位置,比较鼻骨异常、伸舌及鼻骨异常伴伸舌检出DS的效能。结果经染色体检查确诊49胎DS,其中鼻骨异常伴伸舌33胎、单纯鼻骨异常10胎、单纯伸舌2胎,无阳性表现4胎。根据鼻骨异常伴伸舌诊断DS的敏感度、特异度、阳性预测值、阴性预测值分别为67.35%(33/49)、99.91%(5603/5608)、86.84%(33/38)和99.72%(5603/5619),其中阳性预测值较鼻骨异常、伸舌及鼻骨异常或伸舌明显增高(P0.0001)。结论中晚孕期颜面部正中矢状切面观察胎儿鼻骨异常和伸舌的表现,可提高DS的产前检出率。     

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。     

产前超声诊断肺动脉瓣闭锁伴室间隔缺损1例

正孕妇29岁,孕2产1,体健,平素月经规律。孕24周~(+2)超声检查示胎儿心脏位于左侧胸腔内,心轴左移,四腔心对称,十字交叉结构存在,心房正位,心室右襻,房室连接一致;室间隔膜周部中断约3.5mm,主动脉内径明显增宽约7.7 mm,前移骑跨于室间隔之上(图1A);降主动脉短轴切面示降主动脉两侧探及体肺动脉侧支(图1B)。超声诊断:胎儿肺动脉瓣闭锁伴     

超声图像定量分析在胎儿肺发育评估中的应用

目的探讨DFY-Ⅱ型超声图像定量分析在评估不同孕周胎儿肺/肝回声比值中的价值。方法收集孕24~41周健康孕妇315名,获取胎儿右侧旁矢状切面图像,采用DFY-Ⅱ型超声图像定量分析仪分析胎儿肺、肝回声强度并计算两者的比值,分析肺/肝回声比值与孕周的相关性。结果胎儿肺/肝回声比值与孕周呈正相关性(r=0.94,P0.05),直线回归方程为Y=0.60+0.07 X(r~2=0.883)。结论采用DFY-Ⅱ型超声图像定量分析仪分析胎儿肺/肝回声比值可作为评估胎儿肺发育的有效方法。     

产前超声发现胎儿透明隔腔异常的临床意义

目的分析产前超声发现胎儿透明隔腔(CSP)异常的临床意义。方法回顾性对比分析53胎产前超声发现CSP异常胎儿的超声及头部MRI。结果产前超声检出53胎CSP异常,包括26胎CSP未显示、18胎CSP狭小、6胎CSP增宽及3胎CSP形态异常,其中12胎产前超声及头部MRI均提示神经系统发育异常。产前超声未能显示CSP的26胎中,8胎存在神经系统异常,包括单纯性完全型胼胝体缺如3胎,完全型胼胝体缺如合并脑膨出和四肢长骨短小1胎,完全型胼胝体缺如伴脑裂畸形或叶状前脑无裂畸形各1胎,脑积水或额叶多小脑回致CSP受压未显示各1胎。产前超声显示CSP狭小的18胎中,1胎为单纯性部分型胼胝体缺如并经MRI证实。产前超声发现CSP增宽的6胎中,MRI均未检出其他神经系统异常。3胎超声显示CSP形态异常胎儿中,MRI示2胎孤立性透明隔部分发育不良及1胎部分型胼胝体缺如。结论产前超声发现胎儿CSP异常是诊断神经系统发育异常的重要线索。     

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。     

Repair of Truncus Arteriosus and Interrupted Aortic Arch

A total of seven patients with truncus arteriosus and interrupted aortic arch (IAA) comprises our surgical experience in this condition. All underwent primary complete repair via median sternotomy between June 1985 and December 1989. Median age at repair was 8 days and median weight, 3.2 kg. Anatomy of these seven patients was truncus arteriosus type "1 1/2" in five patients and type II in two patients, IAA type B in six patients and type A in one patient. Aortic arch was reconstructed by direct anastomosis of ascending aorta and descending aorta. Right ventricle to pulmonary artery continuity was established with a porcine valved conduit in four patients, aortic homograft in two, and aortic homograft monocusp patch in one. Three patients have required five reoperations (three in one patient). One reoperation was due to compression of the left main bronchus from the reconstructed aorta, one was due to obstruction of the aorta at the site of IAA repair, and one was due to compression of the left main bronchus, right pulmonary artery, and residual stenosis across the hypoplastic ascending aorta. There were no early or late deaths and all seven survivors are currently well with a mean follow-up of 29 months from initial repair.     

产前超声心动图诊断胎儿心肌致密化不全

目的探讨产前超声心动图诊断胎儿心肌致密化不全的价值。方法收集12胎胎儿心肌致密化不全(FNVM)的超声心动图资料,并与病理结果进行对照分析。结果 12胎FNVM,5胎累及双心室,5胎累及左心室,2胎累及右心室;累及节段以左心室心尖段为主(n=10);7胎合并心脏结构异常,5胎合并浆膜腔积液,3胎合并心律失常。对8胎进行单基因及拷贝数变异检测,其中6胎检出致病性单基因变异,1胎疑似致病单基因变异KCNH2,1胎染色体微缺失。结论 FNVM可同时累及左右心室,并易合并右心系统结构异常、心律不齐及浆膜腔积液。产前超声心动图在FNVM的诊断、预后咨询中有重要作用。     

早孕期颅脑超声诊断胎儿颅内畸形

目的探讨早孕期颅脑超声诊断胎儿颅内畸形的价值。方法回顾性分析3800胎早孕期胎儿的常规颅脑超声资料,与随访结果相对照。结果产前胎儿颅脑超声共发现颅脑畸形51胎。其中无脑儿13胎,未见圆形颅骨环、无大脑组织结构;露脑畸形6胎,见大部分颅盖骨缺失,脑组织外露;脊柱裂2胎,见"柠檬头"征;全前脑5胎,见单个扩张的脑室,无大脑镰;脑膜脑膨出13胎,见颅骨高回声环延续性中断,缺损处见局部向外突出囊性物;颈部水囊瘤12胎,见头颅颈后多房性囊肿。漏诊露脑畸形2胎、全前脑6胎、脊柱裂2胎,均于中孕期检出。所有畸形胎儿均经引产获证实,其中28胎合并染色体异常。结论早孕期胎儿颅脑超声在检出胎儿颅脑畸形中具有重要临床价值。     

Off-pump aortic arch repair through a median sternotomy for type B interrupted aortic arch with single ventricle physiology

BACKGROUND: The aortic arch repair for interrupted aortic arch (IAA) with the hypoplastic ascending aorta through a median sternotomy requires cardiopulmonary bypass (CPB), which is very invasive in neonates and complicates pulmonary artery banding (PAB) is staged repair. METHODS: A 22-day-old neonate with a type B IAA having a functional single ventricle underwent arch repair and PAB through a median sternotomy without CPB. A partial occlusion clamp could be placed on the ascending aorta without cerebral malperfusion and the descending aorta could be directly anastomosed to the ascending aorta in an end-to-side fashion under stable circulatory condition. Thereafter, the tight PAB was performed with a circumference of 23mm without any difficulty. RESULTS: The postoperative echocardiogram revealed no stenosis on the anastomotic site and the patient was discharged uneventfully. CONCLUSION: This approach is effective in neonates with IAA who require staged repair, and least invasive for them.     

Reverse subclavian flap and aorto-pulmonary window technique for repair of interrupted aortic arch and truncus arteriosus

We report a surgical strategy for repairing an interrupted aortic arch (IAA) with truncus arteriosus (TA) by using a reverse subclavian flap and an aorto-pulmonary (A-P) window technique for preserving the pulmonary artery architecture. A 10-day-old neonate with type B IAA and type I TA with echocardiographic evidence of a significant distance between the ascending and descending aorta underwent surgical repair at the Bristol Royal Hospital for Children. The superior part of the arch was reconstructed using a reverse subclavian flap and the undersurface with a pulmonary homograft patch. The ascending aorta was separated from the pulmonary arteries using a Gore-Tex patch (A-P window type of repair) without disconnecting the branch pulmonary arteries, in order to preserve their architecture. The continuity between the right ventricle and the pulmonary artery bifurcation was established using a 12 mm Contegra conduit. The postoperative course was uneventful, and the neonate was discharged after 12 days. At follow-up, the patient remains well, gaining weight, with no echocardiographic evidences of obstruction. Reverse subclavian flap with homograft patch combined with and 'A-P window' technique for preservation of the pulmonary artery architecture is a useful and effective surgical strategy for neonates presenting with IAA associated with TA.     

Prenatal Sonography in Suspected Proximal Gastrointestinal Obstructions: Diagnostic Accuracy and Neonatal Outcomes

BackgroundThe purpose of this study was to assess diagnostic accuracy and neonatal outcomes in fetuses with a suspected proximal gastrointestinal obstruction (GIO).MethodsAfter IRB approval, a retrospective chart review was conducted on prenatally suspected and/or postnatally confirmed cases of proximal GIO at a tertiary care facility (2012–2022). Maternal-fetal records were queried for presence of a double bubble ± polyhydramnios, and neonatal outcomes were assessed to calculate the diagnostic accuracy of fetal sonography.ResultsAmong 56 confirmed cases, the median birthweight and gestational age at birth were 2550 g [interquartile range (IQR) 2028–3012] and 37 weeks (IQR 34–38), respectively. There was one (2%) false-positive and three (6%) false-negatives by ultrasound. Double bubble had a sensitivity, specificity, positive predictive value, and negative predictive value for proximal GIO of 85%, 98%, 98%, and 83%, respectively. Pathologies included 49 (88%) with duodenal obstruction/annular pancreas, three (5%) with malrotation, and three (5%) with jejunal atresia. The median postoperative length of stay was 27 days (IQR 19–42). Cardiac anomalies were associated with significantly higher complications (45% vs 17%, p = 0.030).ConclusionsIn this contemporary series, fetal sonography has high diagnostic accuracy for detecting proximal gastrointestinal obstruction. These data are informative for pediatric surgeons in prenatal counseling and preoperative discussions with families.Level of EvidenceDiagnostic Study, Level III.