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First trimester diagnosis of Absent Pulmonary Valve (APV): a case report of prenatal treatment with a review of literature and data from The Polish National Registry of Fetal Cardiac Pathology 妊娠早期诊断肺动脉瓣缺失(APV): 1例产前治疗报告,并回顾波兰国家胎儿心脏病理登记处的文献和数据
Pub Date : 2014-09-01 DOI: 10.12847/09143
A. Nawara, Beata Radzymińska-Chruściel, Maria Respondek Liberska, K. Januszewska, E. Malec
Abstract Absent of pulmonary valve syndrome is a rare congenital heart defect, which is diagnosed prenataly in 0,8% of fetuses with congenital heart defect based on the data from National Polish Registry Of Fetal Cardiac Anomalies. We present a case of pregnat woman and fetus with that heart defect, which was detected in the 1st trimester and treated prenatally with digoxin, amnioreduction, tocolysis and steroids following by the cardiac sugery in the neonatal period. Despite an intensive therapy, the infant died on the 3rd month of age. We belive that the main reason of poor outcome was premature delivery at the 35th week of gestation. We present unique cardiac images proving the changing characterists of this type anomay since 1 st trimester
摘要:肺动脉瓣缺失综合征是一种罕见的先天性心脏缺陷,根据波兰国家胎儿心脏异常登记处的数据,先天性心脏缺陷胎儿的产前诊断率为0.8%。我们提出一个病例的孕妇和胎儿与心脏缺陷,这是在妊娠早期发现和治疗地高辛,羊膜还原,胎解和类固醇后,在新生儿期心脏手术。尽管进行了强化治疗,婴儿还是在3个月大时死亡。我们认为结局不佳的主要原因是妊娠第35周早产。我们提出了独特的心脏图像证明这种类型的异常变化的特点,自1孕早期
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引用次数: 2
An Unusual Complication of an Invasive Intrauterine Therapy: 6 year follow-up 侵入性宫内治疗的一种罕见并发症:6年随访
Pub Date : 2014-09-01 DOI: 10.12847/09144
A. Świercz, S. Lewandowska, K. Szaflik, A. Oleś, A. Piaseczna-Piotrowska, Maria Respondek ‐ Liberska
Abstract Shunt implantation in utero being a life-saving procedure, may be connected with a wide range of complications. One of them is catheter dislocation, a side effect which may impair the state of the fetus and child. We present a case of a boy with Congenital Adenomatoid Lung Malformation (CALM) type II. Invasive prenatal therapy, complicated by shunt migration did not influence our patient’s health. Moreover, the abnormally located shunt stayed within the child’s chest up to the age of 6 years. No symptoms linked to foreign body presence were observed. Although similar cases were published, we report a situation, in which the catheter was left in the patient’s body for 6 years, for the first time.
子宫内分流着床是一种挽救生命的手术,可能与广泛的并发症有关。其中之一是导管脱位,这是一种可能损害胎儿和儿童状态的副作用。我们报告一个男孩先天性腺瘤样肺畸形(CALM) II型。有创产前治疗,合并分流迁移没有影响患者的健康。此外,异常位置的分流管在孩子的胸腔内一直存在到6岁。没有观察到与异物存在有关的症状。虽然类似的病例也有报道,但我们首次报道了导管在患者体内放置6年的情况。
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引用次数: 1
DORV in fetuses: how to consult future parents at a prenatal cardiology center? Retrospective analysis of 39 cases 胎儿DORV:如何在产前心脏病中心咨询未来的父母?回顾性分析39例病例
Pub Date : 2014-09-01 DOI: 10.12847/09142
M. Soroka, M. Słodki, H. Moczulska, M. Respondek-Liberska
Abstract DORV [double outlet right ventricle] is defined as a defect in which the great vessels leave entirely or mostly from above the morphologically right ventricle. The proposed by us new prenatal classification of heart defects for the DORV defect including the division into isolated and coexisting with extracardiac defect, facilitates consultation and predicting prognosis for the fetus and newborn. Isolated DORV in fetuses is classified as a severe elective defect (expected cardiac intervention or surgery at 1 month of age) with a relatively good prognosis for newborns, regardless of the type of intracardiac anomalies (in our series of cases 100% survival). DORV in the fetus with coexisting extracardiac defects (ECM) regardless of type of anomaly had poor prognosis (in our study group 100% demise rate).
摘要:双出口右心室是指大血管全部或大部分从形态上的右心室上方离开的一种缺损。我们对DORV缺陷提出了新的产前心脏缺陷分类,将DORV缺陷分为孤立的心外缺陷和共存的心外缺陷,便于对胎儿和新生儿进行会诊和预测预后。孤立的胎儿DORV被归类为严重的选择性缺陷(1月龄时预期的心脏干预或手术),无论心内异常类型如何,新生儿预后相对较好(在我们的一系列病例中,存活率为100%)。同时存在心外缺陷(ECM)的胎儿DORV,无论异常类型如何,预后都很差(在我们的研究组中死亡率为100%)。
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引用次数: 0
Duodenal obstruction in prenatal ultrasound examinations - duodenal atresia versus annular pancreas - reports on two cases and literature review 产前超声检查中的十二指肠梗阻——十二指肠闭锁与环状胰腺——附2例报告并文献复习
Pub Date : 2014-06-01 DOI: 10.12847/06145
Klaudia Korecka, M. Respondek-Liberska
Abstract Obstruction of the duodenum is the most common intestinal obstruction of the fetus and newborn. A typical symptom of duodenal atresia is the double bubble sign. However, in order to diagnose annular pancreas, it is also required to locate a hyperechogenic band around the double bubble. We analysed the possibility of prenatal differential diagnosis of intestinal atresia, such as duodenal atresia and annular pancreas based on the analysis of two cases examined in the 26th week of pregnancy. This material was analysed by two ultrasonographers (one pediatric surgeon). Conclusions: "Double bubble" symptom in prenatal sonography is typical of high level intestinal obstruction, but it can occur both in classical duodenal atresia, and in the cases of annular pancreas, and in other rare anomalies. Classic prenatal ultrasound examination using 2D option seems to be insufficient for accurate differential diagnosis.
摘要十二指肠梗阻是胎儿和新生儿最常见的肠梗阻。十二指肠闭锁的典型症状是双泡征。然而,为了诊断环状胰腺,还需要在双泡周围定位高回声带。通过对2例妊娠26周检查的十二指肠闭锁、环状胰腺等肠道闭锁的产前鉴别诊断的可能性进行分析。该材料由两名超声医师(一名儿科外科医生)进行分析。结论:产前超声“双泡”症状是高水平肠梗阻的典型表现,但在经典十二指肠闭锁、环状胰腺及其他罕见异常中均可出现。传统的产前超声检查使用二维选项似乎不足以准确鉴别诊断。
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引用次数: 0
Comparison of the clinical status and the effects of treatment of newborns with interrupted aortic arch diagnosed prenatally and postnatally in the "Polish Mother's Memorial" Institute in Lodz in the years 2003-2012 罗兹“波兰母亲纪念”研究所2003-2012年产前和产后诊断的新生儿主动脉弓中断的临床状况及治疗效果比较
Pub Date : 2014-06-01 DOI: 10.12847/06142
Anna Augustyniak, M. Słodki, W. Krajewski, J. Moll, M. Respondek-Liberska
Abstract Material and Methods: A retrospective analysis of 41 patients with congenital heart defect IAA from ICZMP in Lodz in the years 2003-2012, including 15 (36.6%) after prenatal diagnosis. Results: An average of 4 newborns with IAA were operated annually, in the years 2003 -2009 postnatal diagnosis dominated, from 2010 prenatal diagnosis. In the group, n = 15 fetuses with IAA, heart size was normal: Avg 0.33 + / -0.05 HA / CA. Cardiovascular endurance in the CVPS : 8 - 10 points Avg. 9.56 + / -0.73. Infants in the "prenatal" group were often delivered by CS (64.3%), and infants diagnosed postnatally, often by spontaneous delivery (76.9%). In the prenatal group 100% of newborns received prostaglandin E1 from day one, and the "postnatal" group at an average of 6th day. In the "prenatal" group the dose of PGE: 0.02 ug / kg / min, in the "postnatal" group the average dose was> 2 x higher 0,042 mg / kg / min. A statistically significant relationship was shown: between the day of administering prostaglandin E1 and the number of postoperative recovery hospitalization days of the newborn with IAA. Di George Syndrome occurred in 40% in the "prenatal" and in 15.4% in the "postnatal" group. Cardiac operations in the "prenatal" group were performed at average 19th day, and at average 21st day in the "postnatal" group. Infants in the "prenatal" group remained in the postoperative recovery room an average of 7.85 + / -3.98 days, and those from the “postnatal” group an average of 8.38 + / -3.94 days (p = 0.6212). There were 3 deaths (7.3%): 1 patient diagnosed prenatally and 2 postnatally. Neonates with IAA in the "prenatal" group remained in the hospital on average: 43.69 + / - 4.82 days and 39.54 + / -3.75 days in the "postnatal" group. After cardiac surgery, hospitalization in the "prenatal" group amounted to an average of 25.2 days in the "postnatal" 26.7 days. Conclusions: 1. The prenatal diagnosis of IAA was grounds to start the administration of prostaglandin E1 on the first day of life, and in the "postnatal" group the administration of prostaglandin followed on average 6 days after birth, the "postnatal" dose was 2 x higher. 2. A statistically significant correlation was demonstrated between the day of prostaglandin E1 commencement and the number of hospitalization days of the newborn with IAA in the postoperative recovery room, the length of hospital stay in the ICU and sometimes full hospitalization.
材料与方法:回顾性分析2003-2012年罗兹ICZMP先天性心脏缺陷IAA患者41例,其中产前诊断15例(36.6%)。结果:平均每年4例IAA新生儿手术,2003 -2009年产后诊断为主,2010年产前诊断为主。在IAA组n = 15例胎儿,心脏大小正常:平均0.33 + / -0.05 HA / CA,心血管耐力:8 - 10点,平均9.56 + / -0.73。“产前”组的婴儿通常通过CS分娩(64.3%),而产后诊断的婴儿通常通过自然分娩(76.9%)。产前组100%新生儿从第一天开始接受前列腺素E1治疗,产后组平均第6天接受前列腺素E1治疗。“产前”组PGE剂量为0.02 ug / kg / min,“产后”组平均剂量为0.042 mg / kg / min,高出2倍以上。给予前列腺素E1的天数与IAA新生儿术后恢复住院天数有统计学意义。产前组和产后组的Di George综合征发生率分别为40%和15.4%。“产前”组平均第19天进行心脏手术,“产后”组平均第21天进行心脏手术。“产前”组患儿在术后恢复室的平均停留时间为7.85 + / -3.98天,“产后”组患儿在术后恢复室的平均停留时间为8.38 + / -3.94天(p = 0.6212)。死亡3例(7.3%):1例产前诊断,2例产后诊断。“产前”组IAA新生儿平均住院时间为43.69 + / - 4.82天,“产后”组为39.54 + / -3.75天。心脏手术后,“产前”组平均住院25.2天,“产后”组平均住院26.7天。结论:1。产前诊断为IAA者在出生第一天开始给予前列腺素E1,“产后”组平均在出生后6天开始给予前列腺素E1,“产后”剂量高2倍。2. 前列腺素E1起始日与IAA新生儿术后恢复室住院天数、ICU住院天数、有时完全住院天数有统计学意义。
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引用次数: 1
The 1st and 2nd trimester scan of the fetal heart in the case of interrupted aortic arch - case report 主动脉弓中断的妊娠1、2月胎儿心脏扫描1例报告
Pub Date : 2014-06-01 DOI: 10.12847/06143
A. Wójtowicz, Hubert Huras
Abstract Interrupted aortic arch is a rare and very difficult to diagnose congenital heart defect, which is divided into three types according to the place of interruption. The most common is type B, where the interruption is between the left carotid artery and left subclavian artery. This case report shows the description of the anomaly during the first and second trimester scan and review of the literature regarding interrupted aortic arch.
主动脉弓中断是一种罕见的、非常难诊断的先天性心脏缺陷,根据中断的部位分为三种类型。最常见的是B型,中断在左颈动脉和左锁骨下动脉之间。本病例报告显示了在孕早期和孕中期扫描异常的描述,并回顾了有关主动脉弓中断的文献。
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引用次数: 0
Trisomy 9 In Prenatal Diagnosis - Case Report 产前诊断中的9三体病例报告
Pub Date : 2014-03-01 DOI: 10.12847/03145
M. Soroka, M. Słodki
Abstract Trisomy 9 is a rare chromosomal disorder that often results in significant mortality. We present a case report in a low-risk pregnancy. The prenatal ultrasonography at 12 weeks of gestation showed normal nuchal translucency and the presence of the nasal bone. The anatomy scan performed by an experienced doctor revealed an abnormal four chamber view and abnormal posterior cranial fossa. First trimester biochemical analysis ( free βhCG and PAPP-A) showed high risk for trisomy 18. By amniocenthesis ( at 16 weeks of gestation ) and karyotype evaluation trisomy 9 was diagnosed and at 20 weekstermination was conducted on maternal request.
摘要9三体是一种罕见的染色体疾病,通常会导致严重的死亡率。我们报告一例低风险妊娠。妊娠12周的产前超声检查显示颈部正常半透明,鼻骨存在。由一位经验丰富的医生进行的解剖扫描显示异常的四腔视图和异常的后颅窝。妊娠早期生化分析(游离β - hcg和ppap - a)显示18三体的高危。经羊膜穿刺(孕16周)和核型评估诊断为9型三体,孕20周应产妇要求进行流产。
{"title":"Trisomy 9 In Prenatal Diagnosis - Case Report","authors":"M. Soroka, M. Słodki","doi":"10.12847/03145","DOIUrl":"https://doi.org/10.12847/03145","url":null,"abstract":"Abstract Trisomy 9 is a rare chromosomal disorder that often results in significant mortality. We present a case report in a low-risk pregnancy. The prenatal ultrasonography at 12 weeks of gestation showed normal nuchal translucency and the presence of the nasal bone. The anatomy scan performed by an experienced doctor revealed an abnormal four chamber view and abnormal posterior cranial fossa. First trimester biochemical analysis ( free βhCG and PAPP-A) showed high risk for trisomy 18. By amniocenthesis ( at 16 weeks of gestation ) and karyotype evaluation trisomy 9 was diagnosed and at 20 weekstermination was conducted on maternal request.","PeriodicalId":415760,"journal":{"name":"Prenatal Cardiology","volume":"6 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2014-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121231947","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Fetal Thymus - Review 胎儿胸腺-回顾
Pub Date : 2014-03-01 DOI: 10.12847/03142
M. Respondek-Liberska
Abstract This is review of the literature regarding fetal thymus development, its role in immune system, research regarding prenatal thymus evaluation in fetal congenital heart defects, abnormal karyotypes, intrauterine growth restriction. The methods of fetal type measurements both in singelton and multiple pregnancies are discussed and presented.
本文综述了有关胎儿胸腺发育、胸腺在免疫系统中的作用、胎儿先天性心脏缺陷、异常核型、宫内生长受限的产前胸腺评价研究等方面的文献。在单胎和多胎妊娠胎儿类型测量的方法进行了讨论和介绍。
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引用次数: 4
Pulmonary Valvar Stenosis in Polish National Registry for Fetal Cardiac Malformations 波兰国家胎儿心脏畸形登记中心的肺动脉瓣狭窄
Pub Date : 2014-03-01 DOI: 10.12847/03143
P. Kordjalik, M. Respondek-Liberska, M. Słodki, Z. Tobota, Beata Radzymińska-Chruściel, A. Siwińska, A. Włoch, J. Szymkiewicz-Dangel
Abstract Prenatal cardiologists for councelling, most often are using both terminology and data from pediatric cardiology, which is a population of different age. As there is lack in polish literature data about pulmonary stenosis in prenatal population, we retrospectively evaluated our data from National Registry of Cardiac Problems in Fetuses.
产前心脏病专家咨询,最经常使用的术语和数据从儿科心脏病学,这是一个不同年龄的人口。由于波兰缺乏关于产前人群肺狭窄的文献资料,我们回顾性地评估了来自国家胎儿心脏问题登记处的数据。
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引用次数: 1
Persistent Cloaca - A Challenge for an Experienced Sonographer 持续泄殖腔-对经验丰富的超声医师的挑战
Pub Date : 2014-03-01 DOI: 10.12847/03144
Marek Blitek, M. Słodki, H. Moczulska, A. Piaseczna-Piotrowska, M. Respondek-Liberska
Abstract This was a retrospective analysis of ultrasonographic and echocardiographic examinations in 12 fetuses with postnatally confirmed Cloacal Malformation. All examinations were conducted at the tertiary fetal diagnostic center (none of the preliminary screening diagnoses were correct). The main manifestations of cloacal malformation were: signs of urinary tract malformations in 10 fetuses (83,33%), pelvic cyst, diagnosed or suspected as hydrocolpos in 9 fetuses (75%), signs of lower part of digestive tract obstruction in 6 fetuses (50%). In addition there were abnormalities in echocardiographic examinations in 6 cases, such as symptoms of cardiac failure (cardiomegaly, tricuspid regurgitation, monophasic inflow, pericardial effusion, ascites) and one case with AS ( aortal stenosis). Functional abnormalities in fetal echocardiography suggest increased preload or afterload and early circulation failure in this severe prenatal malformation. This malformation have never been published before. The data suggest, that fetal functional echocardiography abnormalities in cases of abnormal pelvic structures should alert the clican to possibility of the presence of cloaca.
摘要回顾性分析了12例产后确诊的胎儿的超声和超声心动图检查结果。所有检查均在第三胎诊断中心进行(初筛诊断均不正确)。肛管畸形的主要表现为:有尿路畸形征象的胎儿10例(83,33%),有盆腔囊肿,诊断或怀疑为阴积水的胎儿9例(75%),有消化道下段梗阻征象的胎儿6例(50%)。6例超声心动图检查出现心衰症状(心脏肥大、三尖瓣反流、单相流、心包积液、腹水),1例出现as(主动脉狭窄)。胎儿超声心动图的功能异常表明,在这种严重的产前畸形中,负荷前或负荷后和早期循环衰竭增加。这种畸形以前从未发表过。数据提示,胎儿功能超声心动图异常的情况下,盆腔结构异常应提醒临床可能存在泄殖腔。
{"title":"Persistent Cloaca - A Challenge for an Experienced Sonographer","authors":"Marek Blitek, M. Słodki, H. Moczulska, A. Piaseczna-Piotrowska, M. Respondek-Liberska","doi":"10.12847/03144","DOIUrl":"https://doi.org/10.12847/03144","url":null,"abstract":"Abstract This was a retrospective analysis of ultrasonographic and echocardiographic examinations in 12 fetuses with postnatally confirmed Cloacal Malformation. All examinations were conducted at the tertiary fetal diagnostic center (none of the preliminary screening diagnoses were correct). The main manifestations of cloacal malformation were: signs of urinary tract malformations in 10 fetuses (83,33%), pelvic cyst, diagnosed or suspected as hydrocolpos in 9 fetuses (75%), signs of lower part of digestive tract obstruction in 6 fetuses (50%). In addition there were abnormalities in echocardiographic examinations in 6 cases, such as symptoms of cardiac failure (cardiomegaly, tricuspid regurgitation, monophasic inflow, pericardial effusion, ascites) and one case with AS ( aortal stenosis). Functional abnormalities in fetal echocardiography suggest increased preload or afterload and early circulation failure in this severe prenatal malformation. This malformation have never been published before. The data suggest, that fetal functional echocardiography abnormalities in cases of abnormal pelvic structures should alert the clican to possibility of the presence of cloaca.","PeriodicalId":415760,"journal":{"name":"Prenatal Cardiology","volume":"4 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2014-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"130269588","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
期刊
Prenatal Cardiology
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