vascular ring

血管环
  • 文章类型: Case Reports
    我们使用高清(HD)血流渲染模式和时空图像相关性(STIC)描述了一例具有主动脉下左头臂静脉(LBCV)和右侧动脉导管的双主动脉弓(DAA)病例。尽管诊断为右侧动脉导管,但我们对这个有趣的病例感到不确定。动脉导管起源于右肺动脉(PA)并汇入降主动脉(DAO),而血管起源于PA并汇入升主动脉(AAO)。因此,我们假设连接PA和AAO的血管可能是C型持续性第五主动脉弓(PFAA).
    We described a case of a double aortic arch (DAA) with a subaortic left brachiocephalic vein (LBCV) and right-side ductus arteriosus using high-definition (HD) flow render mode and spatiotemporal image correlation (STIC). We experienced uncertainty regarding this interesting case despite the diagnosis of right-sided ductus arteriosus. The ductus arteriosus originates from the right pulmonary artery (PA) and converges into the descending aorta (DAO), whereas the vessel originated from the PA and converged into the ascending aorta (AAO). Therefore, we assumed that the vessel connecting the PA to AAO may be a type-C persistent fifth aortic arch (PFAA).
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  • 文章类型: Journal Article
    背景:很少有描述右主动脉弓(RAA)或双主动脉弓(DAA)和食管癌(EC)患者的病例报道。
    方法:我们分析了我们中心数据库中接受食管切除术治疗的RAA和DAA病例,并以英语研究报告,直至2023年4月1日。我们的研究评估了患有RAA和DAA的EC患者的畸形特征和手术细节。
    结果:我们提取了24例患有RAAs的EC患者和10例患有DAAs的EC患者的数据。在这两组中,患者更可能是日本人和男性,有鳞状细胞癌,肿瘤位于胸上食管或胸中食管。左开胸手术常用于RAA患者。对于DAA患者,左侧和右侧方法的比例相似。近年来,在RAA或DAA患者中常规进行了电视胸腔镜手术(VATS)下的食管切除术。每组有两个吻合口漏。具体来说,1例RAA患者发生Kommerel憩室破裂;1例DAA患者发生胃管扩张;2例RAA患者发生喉返神经(RLN)损伤。对于两个队列中的大多数患者来说,术后过程都是顺利的。
    结论:食管癌在RAA或DAA患者中很少见。为了充分解剖上纵隔LN,辅助切口(如胸骨切开术),应适当使用左开门方法或之前的宫颈手术。食管切除术,无论是通过开胸手术还是胸腔镜手术,可以安全地进行RAA和DAA。
    Few cases describing patients with a right aortic arch (RAA) or double aortic arch (DAA) and esophageal cancer (EC) have been reported.
    We analyzed RAA and DAA cases treated with esophagectomy in our center\'s database and reported in English-language studies until April 1, 2023. Our study assessed the malformation characteristics and surgical details of EC patients with RAA and DAA.
    We extracted data of 24 EC patients with RAAs and 10 EC patients with DAAs. In both groups, the patients were more likely to be Japanese and male, to have squamous cell carcinoma and to have tumors located in the upper thoracic esophagus or middle thoracic esophagus. Left thoracotomy was commonly applied for RAA patients. For DAA patients, the proportions of left-sided and right-sided approaches were similar. Esophagectomy under video-assisted thoracoscopic surgery (VATS) in RAA or DAA patients had been performed on a routine basis in recent years. There were two anastomotic leakages in each group. Specifically, Kommerell diverticulum rupture occurred in 1 RAA patient; gastric tube dilation occurred in 1 DAA patient; and recurrent laryngeal nerve (RLN) injury occurred in 2 RAA patients. The postoperative course was uneventful for most patients in both cohorts.
    Esophageal carcinoma is rarely seen in patients with an RAA or DAA. To adequately dissect superior mediastinal LNs, an auxiliary incision (such as sternotomy), the left door open method or a preceding cervical procedure should be used appropriately. Esophagectomy, whether via thoracotomy or thoracoscopic surgery, can be performed safely for both RAA and DAA.
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    目的:本研究旨在表征病理类型,诊断,染色体异常,胎儿右主动脉弓和双主动脉弓畸形的产后临床表现。
    方法:在这项回顾性研究中,纳入2012年12月至2021年12月在我们的三级转诊中心进行常规二维超声心动图结合时空图像相关性的所有诊断为右主动脉弓或双主动脉弓异常的胎儿.
    结果:总计,确定了234例主动脉弓异常的胎儿。41例失去了后续行动。本研究纳入了93例病例。88例右主动脉弓。双主动脉弓6例。右主动脉弓伴异常左锁骨下动脉的大多数病例(77/101,76.2%)为孤立性病变。而大多数具有镜像分支的患者(45/75,60%)与心内或心外异常相关。113例右主动脉弓胎儿产前筛查染色体异常,其中3例异常左锁骨下动脉(3/63,4.8%)和8例镜像分支(8/50,16%)染色体异常(p<0.05)。此外,3例有微缺失22q11.2,这些与心内畸形显著相关。
    结论:除了孤立的左锁骨下动脉和孤立的左头臂干,大多数孤立的右主动脉弓病例没有临床症状。此外,孤立的右主动脉弓患者发生染色体异常的风险非常低.我们建议应告知孕妇进行侵入性产前染色体检测的风险和益处。
    OBJECTIVE: This study aimed to characterize the pathological types, diagnosis, chromosomal abnormalities, and postnatal clinical manifestations of right and double aortic arch malformations in fetuses.
    METHODS: In this retrospective study, all fetuses diagnosed with right or double aortic arch anomalies for whom conventional two-dimensional echocardiography combined with spatio-temporal image correlation was performed at our tertiary referral center between December 2012 and December 2021 were included.
    RESULTS: In total, 234 fetuses with aortic arch abnormalities were identified. Forty-one cases lost to follow-up. One hundred ninety-three cases were included in this study. One hundred eighty-seven cases with right aortic arch. Six cases with double aortic arch. Most cases of right aortic arch with aberrant left subclavian artery (77/101, 76.2%) were isolated lesions, whereas most of those with mirror-image branching (45/75, 60%) were associated with intracardiac or extracardiac anomalies. Chromosomal abnormalities were screened prenatally in 113 fetuses with right aortic arch, among whom three with aberrant left subclavian artery (3/63, 4.8%) and eight with mirror-image branching (8/50, 16%) had chromosome anomalies (p < 0.05). Furthermore, three cases had microdeletion 22q11.2 and these were significantly associated with intracardiac malformations.
    CONCLUSIONS: Most cases of isolated right aortic arch do not present with clinical symptoms except isolated left subclavian artery and isolated left brachiocephalic trunk. In addition, the risk of chromosomal abnormalities in patients with isolated right aortic arch is very low. We recommend that pregnant women should be informed of the risks and benefits of undergoing invasive prenatal chromosomal detection.
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    背景:血管环(VR)表现出复杂而多样的形式,使用传统的二维(2D)示意图很难概念化。缺乏经验的医学生和缺乏医疗技术背景的父母在理解VR方面面临着重大挑战。这项研究的目的是开发VR的三维(3D)打印模型,为医学教育和父母咨询提供新的技术成像支持。
    方法:本研究包括42例诊断为VRs的胎儿。胎儿超声心动图,进行了建模和3D打印,并对模型的尺寸精度进行了分析。通过对48名医学生进行教学干预前后的测试结果比较及满意度调查,分析3D打印在VR教学中的应用价值。对40位父母进行了简短的调查,以评估3D打印模型在产前咨询中的价值。
    结果:成功获得了40种VR模型,它以很高的尺寸精度再现了VR空间的解剖形状。在3D打印组和2D图像组之间没有注意到讲座前测试结果的差异。讲座结束后,两组的知识都有所提高,但是在3D打印组中,课后得分以及讲座前得分与课后得分的变化更大,3D打印组主观满意度调查反馈也较好(P<0.05)。从父母问卷中观察到类似的结果,绝大多数家长对使用3D打印模型持热情和积极的态度,并建议在未来的产前咨询中使用它们。
    结论:三维打印技术为有效显示不同类型的胎儿VR提供了一种新工具。这个工具帮助医生和家庭了解胎儿大血管的复杂结构,积极影响医疗指导和产前咨询。
    BACKGROUND: Vascular rings (VRs) exhibit complex and diverse forms that are difficult to conceptualize using traditional two-dimensional (2D) schematic. Inexperienced medical students and parents who lack a medical technology background face significant challenges in understanding VRs. The purpose of this research is to develop three-dimensional (3D) printing models of VRs to provide new technical imaging support for medical education and parental consultation.
    METHODS: This study included 42 fetuses diagnosed as VRs. Foetal echocardiography, modeling and 3D printing were performed, and the dimensional accuracy of models was analyzed. The value of 3D printing in the teaching of VRs was analyzed based on comparing the test results before and after the teaching intervention of 48 medical students and the satisfaction survey. A brief survey was conducted to 40 parents to assess the value of the 3D printed model in prenatal consultations.
    RESULTS: Forty models of VRs were successfully obtained, which reproduced the anatomical shape of the VRs space with high dimensional accuracy. No differences in the prelecture test results were noted between the 3D printing group and the 2D image group. After the lecture, the knowledge of both groups improved, but the postlecture score and the change in the prelecture versus postlecture score were greater in the 3D printing group, and the subjective satisfaction survey feedback in the 3D printing group was also better (P < 0.05). Similar results were observed from the parental questionnaire, the vast majority of parents have an enthusiastic and positive attitude towards the use of 3D printed models and suggest using them in future prenatal consultations.
    CONCLUSIONS: Three-dimensional printing technology providing a new tool for effectively displaying different types of foetal VRs. This tool helps physicians and families understand the complex structure of foetal great vessels, positively impacting medical instruction and prenatal counselling.
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  • 文章类型: Journal Article
    背景:成人双主动脉弓(DAA)合并降主动脉弓夹层(DAAD)是一种罕见的主动脉血管疾病。由于双弓和弓血管的解剖结构异常,临床症状和手术方法与典型主动脉夹层不同。
    方法:本研究是对涉及三名患者(平均年龄,47.3年),从2010年9月至2019年6月,DAA联合DAAD进行了全弓置换或混合主动脉修复。病人的人口统计学,最初的症状,合并症,外科手术,并对结果进行了总结。
    结果:对2例患者进行深低温停循环下的全足弓置换加冷冻支架植入术,其中1例术后出现弥散性血管内凝血和多器官功能衰竭。病例3接受了左锁骨下动脉血运重建和胸主动脉腔内修复的混合手术。随访期间声音嘶哑、吞咽困难症状明显改善。
    结论:除了典型的突发胸痛和背痛,DAA和DAAD患者可出现声音嘶哑和吞咽困难.基于DAA的发展,全弓置换或杂交手术可能是一种可选的治疗方法。
    BACKGROUND: Double aortic arch (DAA) combined with descending aortic arch dissection (DAAD) in adults is a rare aorta vascular disease. Due to the abnormal anatomy of the double arch and arch vessels, the clinical symptoms and surgical methods differ from those of typical aortic dissection.
    METHODS: This study was retrospective analysis of a case series involving three patients (mean age, 47.3 years) with DAA combined DAAD underwent total arch replacement or hybrid aortic repair from September 2010 to June 2019. The patients\' demographics, initial symptoms, comorbidities, surgical procedures, and outcomes are summarized.
    RESULTS: Total arch replacement plus frozen stent implantation under deep hypothermic circulatory arrest was performed for 2 patients, one of them developed disseminated intravascular coagulation and multiple organ failure postoperatively. Case 3 underwent a hybrid procedure with left subclavian artery revascularization and thoracic endovascular aortic repair. The symptoms of hoarseness and dysphagia were obviously improved during the follow up.
    CONCLUSIONS: In addition to typical sudden chest and back pain, patients with DAA and DAAD may have hoarseness and dysphagia. Based on the development of DAA, total arch replacement or hybrid surgery may be is an optional treatment.
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  • 文章类型: Journal Article
    背景:就胚胎起源而言,血管环是一种先天性异常,其中主动脉弓及其分支完全或不完全环绕并压迫气管或食道。早期准确诊断血管环是治疗的关键。产前诊断主要依靠胎儿超声心动图,但是漏诊和误诊率仍然很高,预后尚未评估。这项研究的目的是研究产前诊断的准确性,并根据环的形状和血管与气管之间的距离半定量地评估预后。
    方法:从2019年到2021年,我们中心对37,875例胎儿进行了产前超声检查。所有胎儿心脏检查均使用美国超声医学研究所(AIUM)提出的胎儿超声心动图方法结合动态序贯横断面观察(SCS)进行。对于SCS,标准腹部切片作为初始切片,探头沿着身体的长轴向头部移动,直到上纵隔消失。如果发现了血管环,观察环的形状和分支到气道的距离。与气道的距离关系分为三个等级:I-III;距离越近,等级越低。出生前每4周监测血管环。所有患者均在手术前或出生后1年进行监测。
    结果:共检出血管环418例。SCS没有漏诊或误诊。容器根据其起源和路线形成不同形状的环。一级,\"\"和\"O\"环预后不良,并且与呼吸道症状的最高风险相关。
    结论:SCS可以在分娩前准确诊断血管环,评估戒指的形状和大小,以对儿童进行产前监测,直到出生,对出生后气道压缩有指导作用。
    BACKGROUND: In terms of embryonic origin, vascular ring is a congenital anomaly in which the aortic arch and its branches completely or incompletely encircle and compress the trachea or esophagus. Early and accurate diagnosis of a vascular ring is the key to treatment. Prenatal diagnosis mainly relies on fetal echocardiography, but the rate of missed diagnosis and misdiagnosis is still very high, and the prognosis has not been evaluated. The aim of this study was to investigate the accuracy of prenatal diagnosis and to evaluate the prognosis semi-quantitatively according to the shape of the ring and the distance between the vessel and the trachea.
    METHODS: From 2019 to 2021, 37,875 fetuses underwent prenatal ultrasound examination in our center. All fetal cardiac examinations were performed using the fetal echocardiography method proposed by the American Institute of Ultrasound in Medicine (AIUM) combined with dynamic sequential cross-sectional observation (SCS). For SCS, the standard abdominal section was taken as the initial section, and the probe was moved cephalically along the long axis of the body until the superior mediastinum had disappeared. If a vascular ring was found, the shape of the ring and the distance of the branch to the airway were observed. The distance relationship with the airway was divided into three grades: I-III; the closer the distance, the lower the grade. The vascular rings were monitored every 4 weeks before birth. All were monitored before surgery or 1 year after birth.
    RESULTS: A total of 418 cases of vascular rings were detected. There was no missed diagnoses or misdiagnoses by SCS. The vessels formed different shaped rings according to their origin and route. Grade I, \"\" and \"O\" rings have a poor prognosis and are associated with the highest risk of respiratory symptoms.
    CONCLUSIONS: SCS can accurately diagnose vascular rings before delivery, evaluate the shape and size of the rings to conduct prenatal monitoring of children until birth, which plays a guiding role in airway compression after birth.
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  • 文章类型: Journal Article
    背景:继发性甲状旁腺功能亢进(SHPT)是终末期肾病的常见并发症。甲状旁腺切除术(PTx)通常用于治疗严重的SHPT。然而,PTx可能通过未知的机制引起低血压。甲状旁腺中含有COMM结构域的蛋白质5(COMMD5)与自发性高血压大鼠的血压调节有关。
    目的:探讨COMMD5水平与血液透析(HD)患者PTx后血压降低的关系。
    结果:(1)研究队列包括31例接受PTx的HD患者。血清COMMD5水平在PTx后较高。pre-PTx.(2)SD大鼠(n=22)分为5/6肾切除组和假手术组,CKD大鼠胸主动脉血管环与COMMD5孵育,比较血管张力的变化.COMMD5抑制完整内皮的血管环的血管收缩,但对没有内皮的血管环没有影响。(3)用COMMD5或小干扰RNA(siRNA)刺激人脐静脉内皮细胞。心钠素(ANP)和内皮型一氧化氮合酶(eNOS)表达上调,分别。
    结论:SHPT患者PTx后血清COMMD5水平升高。COMMD5促进血管内皮细胞ANP和eNOS的高表达,导致血管舒张并导致低血压。
    BACKGROUND: Secondary hyperparathyroidism (SHPT) is a common complication of end-stage renal disease. Parathyroidectomy (PTx) is often employed for treatment of severe SHPT. However, PTx may cause hypotension via unknown mechanisms. COMM domain-containing protein 5 (COMMD5) in the parathyroid glands has been linked to blood pressure regulation of spontaneously hypertensive rats.
    OBJECTIVE: To explore the relationship between COMMD5 levels and reduced BP after PTx in patients receiving hemodialysis (HD).
    RESULTS: (1) The study cohort included 31 patients receiving HD who underwent PTx. Serum COMMD5 levels were higher post-PTx vs. pre-PTx. (2) Sprague-Dawley rats (n = 22) were assigned to a 5/6 nephrectomy group or sham surgery group, vascular rings of the thoracic aorta from rats with CKD were incubated with COMMD5, and changes in vascular tension were compared. COMMD5 inhibited vasoconstriction of vascular rings with intact endothelium, but had no effect on vascular rings without the endothelium. (3) Human umbilical vein endothelial cells were stimulated with COMMD5 or small interfering RNA (siRNA). The expression levels of atrial natriuretic peptide (ANP) and endothelial nitric oxide synthase (eNOS) were up-regulated and down-regulated, respectively.
    CONCLUSIONS: Serum COMMD5 levels were increased after PTx in SHPT patients. COMMD5 promoted high expression of ANP and eNOS in endothelial cells, leading to vasodilation and resulting in hypotension.
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  • 文章类型: Case Reports
    分娩前未报告双主动脉弓(DAA)伴主动脉左头臂静脉(LBCV)和右侧动脉导管(RDA)。仅在具有解剖学课程发现的成年人中。我们使用高清(HD)血流渲染模式和时空图像相关性(STIC)介绍了一例具有主动脉下LBCV和RDA的胎儿DAA病例。
    Double aortic arch (DAA) with subaortic left brachiocephalic vein (LBCV) and right-side ductus arteriosus (RDA) was not reported before delivery, only in adults with anatomy course findings. We present a case of fetal DAA with subaortic LBCV and RDA using high-definition (HD) flow render mode and spatiotemporal image correlation (STIC).
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