产前超声诊断双胎反向动脉灌注综合征

目的 探讨多普勒超声在产前诊断双胎反向动脉灌注综合征(TRAP)中的临床价值,提高对此类罕见畸形的认识. 方法 我院1995年1月至2008年9月产前超声诊断及疑似诊断19例TRAP,比较超声与生后随访或引产后病理结果. 结果 19例TRAP中,无心畸胎(受血儿)均有严重结构畸形,产前超声对胎儿皮肤水肿、脊柱、腹腔结构及下肢显示准确性高;但对头颅发育不良及特别短小的上肢显示困难,颜面部畸形基本上显示不清.泵血儿(供血胎)中死胎及死产16例,存活3例;心功能不全9例.胎儿附属结构特征:(1)存活的无心畸胎均为脐动脉供血,血流方向朝向胎儿体内,与正常胎儿脐动脉血流方向相反;(2)两胎儿脐带胎盘附着处可见大的血管相交通;(3)无心畸胎多为单脐动脉;(4)羊水过多为预后不良征象;(5)单羊膜囊双胎常发生脐带缠绕及打结. 结论 多普勒超声可为评价TRAP血流动力学特征、评估泵血儿心功能、附属物异常及预后提供重要信息,为产前诊断TRAP的首选和重要的诊断方法.     

产前超声诊断双胎反向动脉灌注综合征

目的 探讨多普勒超声在产前诊断双胎反向动脉灌注综合征(TRAP)中的临床价值,提高对此类罕见畸形的认识. 方法 我院1995年1月至2008年9月产前超声诊断及疑似诊断19例TRAP,比较超声与生后随访或引产后病理结果. 结果 19例TRAP中,无心畸胎(受血儿)均有严重结构畸形,产前超声对胎儿皮肤水肿、脊柱、腹腔结构及下肢显示准确性高;但对头颅发育不良及特别短小的上肢显示困难,颜面部畸形基本上显示不清.泵血儿(供血胎)中死胎及死产16例,存活3例;心功能不全9例.胎儿附属结构特征:(1)存活的无心畸胎均为脐动脉供血,血流方向朝向胎儿体内,与正常胎儿脐动脉血流方向相反;(2)两胎儿脐带胎盘附着处可见大的血管相交通;(3)无心畸胎多为单脐动脉;(4)羊水过多为预后不良征象;(5)单羊膜囊双胎常发生脐带缠绕及打结. 结论 多普勒超声可为评价TRAP血流动力学特征、评估泵血儿心功能、附属物异常及预后提供重要信息,为产前诊断TRAP的首选和重要的诊断方法.     

彩色多普勒超声诊断血管前置7例临床分析

目的探讨彩色多普勒超声（CDFI）检查对胎儿血管前置的诊断价值。方法回顾性分析7例血管前置的彩色多普勒超声声像图特点。结果7例血管前置患者中,帆状脐带附着胎盘4例（其中双胎1例）,副胎盘2例,分叶状胎盘1例。产前CDFI正确诊断脐带血管前置5例,漏诊2例。7例患者均行剖宫产,正常新生儿8例（1例双胎）,无并发症发生。结论彩色多普勒超声是胎儿血管前置诊断的重要诊断方法。     

双胎反向动脉灌流综合征的诊治进展

双胎反向动脉灌流(TRAP)又称双胎之一无心畸形,是一种特发单绒毛膜多胎妊娠的畸形。单绒毛膜双胎妊娠中,TRAP的发生率为1/100,是双胎输血综合征(TTTS)的一种特殊类型。TRAP主要表现为双胎中一胎发育相对正常,成为供血儿(或泵血儿);另一胎为受血儿(或无心畸形),是无心脏且伴发其他严重畸形的胎儿。随着双胎TRAP诊断技术的不断提高、双胎发生率的升高以及胎儿镜技术和超声的发展,其治疗也随着发展和提高。本文就现有知识,对TRAP治疗方法的选择和结局做一个综述。     

超声造影在双胎反向动脉灌注序列征伴心脏遗迹的诊断探讨

目的探讨双胎反向动脉灌注序列征伴心脏遗迹的超声造影诊断价值。方法对2例无心畸胎行常规超声检查后,采用造影剂SonoVue行超声造影,造影剂注射方法为静脉缓慢推注,选用微血管成像技术进行实时超声造影,观察造影剂在双胎及脐血管、胎盘中的灌注情况。结果2例无心畸胎胸腔内均可见心脏遗迹搏动,越近遗迹心脏心率越缓慢,越近泵血儿心率越快。超声造影灌注顺序为:泵血儿脐静脉→泵血儿体内→泵血儿脐动脉→胎盘(动脉与动脉吻合)→无心畸胎单一脐动脉→无心畸胎体内→无心畸胎脐静脉→胎盘(静脉与静脉吻合)→泵血儿脐静脉。病理解剖结果:遗迹心脏结构原始,类似原始心管结构。结论遗迹心脏无正常功能,循环仍依赖于泵血儿心脏。超声造影使我们实时观察到无心畸胎、脐血管及胎盘内的血液灌注顺序,为进一步了解无心畸胎循环特点提供重要信息。     

双胎反向动脉灌注综合征二例临床分析

双胎反向动脉灌注综合征(twin reversed arterial perfusion sequence，TRAP)是单绒毛膜多胎妊娠的严重并发症，表现为双胎中一胎为发育相对正常，并成为供血胎儿，另一胎为无心脏且严重畸形的受血胎儿，因此，又称为双胎妊娠一胎无心畸形(acardiac twin)，临床上罕见。我院近1年内收治了2例TRAP，现将临床情况报道分析如下。     

彩色多普勒超声在非高危胎儿产前心脏筛查中的临床意义

目的：探讨彩色多普勒超声在非高危胎儿产前心脏筛查中的临床意义。方法：选用我院1125例产前孕检的孕妇，通过多普勒超声的多切面检查，获得较为清晰的二维和彩色多普勒声像图，检查心脏畸形。结果：检查出9例先天性心脏病患者，其中通过产前超声筛查出7例，漏诊2例（室间隔缺损、法洛四联症），经伦理委员会和家属的同意，对引产后患儿进行尸解证实主要原因是由于母体的羊水少、胎儿体位异常、母体腹壁厚。多普勒超声对非高危胎儿的检出率0．6％，对心脏畸形的检查率是78％，诊断准确率是100％。结论：多普勒超声对非高危胎儿进行产前心脏超声筛查，检查率比较高，能够及时发现心脏畸形和做出相应的干预处理。     

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.     

胎儿心脏锥干畸形产前诊断评价

目的评价胎儿心脏锥干畸形产前超声心动图诊断准确性。方法运用胎儿超声心动图进行先天性心脏病产前诊断，并与心脏病理诊断或新生儿心脏超声诊断进行比较分析。结果2063例胎儿进行心脏超声检查，产前发现胎儿心脏锥干畸形28例，诊断时平均孕周26．6周（16～40周），产前诊断包括大血管转位5例，法洛四联症5例，右室双出口6例，永存动脉干9例，室间隔缺损合并主动脉骑跨可能1例，2例检查发现大血管位置异常但胎儿超声心动图未予明确诊断。24例经胎儿心脏病理检查或新生儿心脏超声检查确诊，4例失访（包括1例TOF／TA？，2例“大血管发育异常”，1例TGA）。比较产前诊断与产后诊断，分析大血管位置关系诊断准确性为75．0％（18／24）。结论胎儿超声心动图可正确诊断胎儿心脏锥干畸形，需运用多种超声切面及彩色脉冲多普勒确定大血管位置关系。     

双胎反向动脉灌流综合征4例分析

<正>双胎反向动脉灌流(twin reversed arterial perfusion sequence,TRAP)又称双胎之一无心畸形,是一种特发单绒毛膜多胎妊娠的畸形,发生率在所有妊娠中为1/35000,在单绒毛膜双胎妊娠中为1/100,是双胎输血综合征(twin-to-twin transfusion syndrome,TTTS)的一种特殊类型[1]。TRAP主要表现为双胎中一胎发育相对正常,成为供血儿(或泵血儿),另一胎为受血儿(或无心畸形),为无心脏且伴其他严重畸形的胎儿。本文对我院2010~2013年诊断的4例病例进行回顾性分析,现报道如下。     

Sonographic findings of acardiac twin in the first trimester of pregnancy

Twin Reversed Arterial Perfusion (TRAP) sequence, or the acardiac anomaly, is a rare complication of monozygotic twins, that occurs in approximately 1 in 35,000 births. In the first trimester, once the diagnosis of monochorionic twins is made, particularly if one embryo shows abnormal development or generalized edema, the TRAP sequence must be considered. Moreover, malformations reported in the perfused twin are often severe. Diagnosis is confirmed by using Doppler ultrasound which demonstrated the presence of retrograde perfusion in the umbilical cord of the abnormal twin. The more complete the body form is, the more similarity the vascular relationships have to the pattern found in a full-term fetus. Final outcome and treatment decisions can be determined based on hemodynamic criteria. We report a case of a misdiagnosed TRAP sequence with no structural malformations detected by ultrasonography at 12 weeks of gestation.     

Percutaneous ultrasonographically guided ablation of an acardiac twin

Acardiac twinning is caused by twin reversed arterial perfusion (TRAP). Normal "pump" twins may face early delivery and cardiac decompensation and have a high perinatal mortality. A primagravid patient had serial evaluation of a TRAP pregnancy beginning early in the second trimester. Rapid growth of the acardiac sibling, high diastolic velocity Doppler waveforms in the perfusing vessel, and early hydramnios prompted ablation of blood flow by a percutaneous ultrasonographically guided infusion of absolute alcohol. A term birth of a normal pump twin was the outcome. Ablation of blood flow into an acardiac sibling of a TRAP pregnancy may be indicated in cases with a poor prognosis by use of an effective percutaneous ultrasonographically guided technique.     

胎儿心脏超声Yagel法扫描在产前诊断双胎妊娠胎儿先天性心脏病中的价值

目的探讨胎儿心脏超声Yagel法扫描对双胎妊娠胎儿先天性心脏病的产前诊断价值。方法1103例双胎妊娠孕妇中,有胎儿先天性心脏病的高危病史孕妇127例（高危双胎组）,正常双胎妊娠孕妇976例（低危双胎组）。应用超声Yagel法对两组双胎妊娠胎儿进行心脏5个心脏横切面（胎儿腹部胃泡平面、四腔心平面、五腔心平面、三血管气管平面和动脉弓平面）快速扫描,并对诊断为先天性心脏病而引产的胎儿进行尸体解剖,以核对产前超声Yagel法诊断的正确性,同时进行胎儿染色体分析;对未引产或产前诊断未发现明显异常的胎儿进行临床随访,胎儿出生后进行新生儿或婴儿的心脏超声检查,判定产前超声Yagel法诊断的正确性。结果（1）1103例双胎妊娠中,有12例产前发现有先天性心脏病,检出率为1．09％,其中4例（33．3％,4／12）来自高危双胎组,8例（66．7％,8／12）来自低危双胎组。（2）2例双胎为两个胎儿同时患同一种先天性心脏病,其中1例双胎胎儿均为法洛四联症（TOF）,另1例双胎两个胎儿均为心脏横纹肌瘤;1例双胎为两个胎儿患不同类型异常,一胎儿为TOF,另一胎儿心脏正常,但十二指肠闭锁。上述3例患者均选择终止妊娠放弃胎儿,尸体解剖结果与产前超声诊断相同。9例双胎仅一胎儿受累为先天性心脏病,另一胎儿正常,均足月分娩或自然早产,其中7例在行新生儿心脏检查时得出的诊断与产前相同。（3）12例中,2例有先天性心脏病的双胎被发现染色体异常。（4）1091例双胎妊娠孕妇产前超声检查未发现胎儿心脏异常,但其中1例双胎中的1个胎儿出生后被诊断为室间隔缺损（VSD）,染色体异常（为21三体）。另1个胎儿正常。另1例双胎中的1个胎儿出生后被诊断为动脉导管未闭,另1个胎儿正常。（5）应用超声Yagel法产前诊断双胎先天性心脏病的敏感度为82．4％,特异度为100％。结论心脏超声Yagel法是筛查和诊断双胎妊娠中胎儿先天性心脏病的简单、有效和可靠方法,值得在临床产前诊断中推广应用。     

Twin-Reversed Arterial Perfusion (TRAP) Sequence: Case Reports and Review of Literature

Summary: The twin reversed-arterial-perfusion (TRAP) sequence found in monozygotic twins is a consequence of primary or secondary cardiac development disruption and direct arterioarterial and venovenous placental anastomoses. Associated findings include the presence of a single umbilical artery (66%) and chromosomal abnormalities in the acardiac twin (33%). Morphological abnormalities in the acardiac twin are consistent with perfusion of tissues supplied by the common iliac and lower branches of the aorta with deoxygenated blood. The pump or donor twin may develop cardiac failure because of the anomalous perfusion circuit. Polyhydramnios is significantly associated with the presence of renal tissue in the acardiac twin. An acardiac pump twin weight ratio (>50%) is associated with the development of polyhydramnios and preterm labour. Identified high-risk factors for poor obstetrical outcome include: acardiac anceps, polyhydramnios, acardiac twin with ears, and pump twin cardiac failure. Management options include elective termination, observation (serial cardiotocography (CTG), ultrasonography and echocardiography) and selective nonsurgical interventions (indomethacin, digitalis, tocolysis). Additionally, surgical interventions (hysterotomy with selective delivery of the acardiac twin or ligation of the acardiac twin's umbilical cord), and ultrasound-guided embolization of the acardiac twin's umbilical artery with absolute alcohol, platinum coils, or thrombogenic coils have been reported. The most appropriate interventions for the varous clinical presentations of this disorder are as yet undetermined, and conservative nonintervention is often appropriate. Long-term follow-up data on surviving pump twins are lacking. It is anticipated that centres with active study protocols for these conditions will best serve patient care and clinical research needs.     

Feto acardio en gestación gemelar a término

Twin reversed arterial perfusion sequence (TRAP) is a serious complication of monochorionic twin pregnancies, in which one twin perfuses the other twin (an acardiac fetus) via large arterio-arterial and veno-venous anastomoses.We report a case of monochorionic twin pregnancy at term, in which the second twin was an acardiac fetus with only an undeveloped lower limb and rudimentary colonic tissue. The first twin was born healthy, a very rare outcome in this entity as the pump twin usually shows distinct degrees of cardiac overload, leading to intrauterine death from heart failure in more than 50% of cases.     

Prenatal diagnosis of fetal heart failure in twin reversed arterial perfusion syndrome

Diagnosis of twin reversed arterial perfusion (TRAP) syndrome is a rare fetal anomaly that can be misdiagnosed on prenatal ultrasound. We confirmed the use of colour-flow Doppler for prenatal diagnosis of TRAP syndrome and used serial fetal echocardiography for non-invasive evaluation of the fetus. A patient with twin intrauterine pregnancy was referred to our centre with suspected intrauterine fetal demise following a 16 week ultrasound. Serial colour-flow Doppler ultrasonography demonstrated retrograde arterial flow in an acardiac twin. Following diagnosis of TRAP syndrome, serial fetal echocardiography was employed to follow the normal twin for signs of heart failure, including right atrial dilation, tricuspid regurgitation and pericardial effusion. When early signs of fetal heart failure were suspected a viable female infant was delivered at 32 weeks' gestation. We suggest that serial fetal echocardiography represents a non-invasive approach that can be used to follow fetal cardiac function of the normal twin in TRAP syndrome.     

Twin reversed arterial perfusion sequence: two different heartbeats in one umbilical cord - rare finding in monochorionic twin pregnancy

The development of twin-twin transfusion syndrome complicates 5-35% of twin pregnancies with monochorionic placentation. Acardiac twinning is the most extreme manifestation of pathological vascular anastomoses between twins. An acardiac twin is a rare complication of multifetal pregnancy, reported in the literature with an incidence of 1% in monochorionic twin pregnancies, i.e. 1/35 000 pregnancies, and more than 400 cases have been described. We review the literature on this subject and report a special case of twin reversed arterial perfusion (TRAP) sequence in a twin pregnancy with the rare finding of a functional univentricular circulation pump in the acranius, its antenatal ultrasound diagnosis and postnatal findings. Remarkably, prenatally we could demonstrate two different arterial pulsations in the umbilical cord of the acranius. Etiological hypotheses of the TRAP sequence and new implications for risk-adapted therapeutic options are discussed.     

15例双胎反向动脉灌注序列症的自然病程及围生儿预后探讨

目的:了解双胎反向动脉灌注序列症(TRAP序列症)的自然病程,分析其围生儿预后。方法:回顾性分析我院2008年7月至2011年12月收治的TRAP序列症15例患者的临床资料,对足月产、早产、流产及平均分娩孕周等资料进行统计,分析无心畸胎/供血儿的腹围比(AC比值)、脐动脉搏动指数(UA-PI)比、供血儿心脏情况等,并追踪供血儿出生后1年的健康状况。结果:15例TRAP序列症患者无心无头型11例,无心无脑型4例。10例孕早期超声检查误诊为双胎之一死亡(3例)、胎儿水肿(2例)和颈部淋巴水囊瘤(5例)。供血儿情况:羊水过多2例(1例引产,1例自然流产);心功能不全2例(1例因合并先天性心脏病引产,1例早产);羊水过多合并心功能不全1例(自然流产)。无心畸胎情况:无心畸胎血流自然阻断2例(13.3%),其中1例孕37周突发宫内死胎,1例继续妊娠(情况稳定);无心畸胎血流微弱(UA-PI比值<0.7)8例(53.3%),其中自然流产2例,引产1例,分娩活胎3例(追踪1年,3例新生儿均健康),继续妊娠2例(情况稳定);无心畸胎血流丰富5例(33.3%)(均引产)。另外无心畸胎出现迅速增长(AC比值>50%)共10例(66.7%),其中引产7例,自然流产1例,仅2例剖宫产分娩存活。结论:无心畸胎较小、血流不丰富者,自然病程良好。出现无心畸胎迅速生长、供血儿心力衰竭或水肿,提示胎儿死亡风险高或预后不良。