vascular ring

血管环
  • 文章类型: Case Reports
    已发布的数据估计血管环的患病率约为每10,000例活产7。文献中很少描述双主动脉弓与大动脉D转位的关联。在这项研究中,我们报告了一名28岁女性的产前诊断.胎龄为24周6天的胎儿超声心动图检查显示大动脉D移位和双主动脉弓伴室间隔缺损和肺动脉狭窄。出生后的第一个晚上,婴儿经历了乳酸水平的增加,氧饱和度始终低于80%。出生后几个小时,患者接受了Rashkind手术.超声心动图,胸部X线CT,CT血管造影证实诊断为气管腔严重缩小(>85%)和支气管软化。然后,患者接受了后气管固定术和主动脉固定术,随后进行了动脉转换手术,室间隔缺损闭合术,切除漏斗状隔膜的一部分,接受潜在的新主动脉阻塞的风险。文献仅报道了2例胎儿超声心动图诊断的患者。因此,我们的病人只有第三个有胎儿诊断,第二个有复杂的心内解剖,不仅表现为室间隔缺损,而且表现为阻塞的两个独立部分(二尖瓣和发育不良瓣膜,漏斗状隔膜后偏)。总之,大动脉的D转位与双主动脉弓仍然是一个非常不寻常的关联。这些患者的临床结果表现出高度的变异性,并且在产前生活中是完全不可预测的。作为胎儿和围产期心脏病专家,我们的最大目标是通过胎儿诊断来改善这些患者的管理和预后。识别需要早期新生儿侵入性手术的新生儿先天性心脏病的类型。
    Published data estimate the prevalence of the vascular ring at approximately 7 per 10,000 live births. The association of a double aortic arch with a D-transposition of the great arteries has been rarely described in the literature. In this study, we report the prenatal diagnosis of a 28-year-old woman. A fetal echocardiography at a gestational age of 24 weeks + 6 days showed a D-transposition of the great arteries and a double aortic arch with a ventricular septal defect and pulmonary stenosis. On the first night after birth, the baby experienced an increase in lactate levels, with the rate of oxygen saturation consistently below 80%. A few hours after birth, the patient underwent a Rashkind procedure. An echocardiography, CT chest x-ray, and CT angiogram confirmed a diagnosis with a severe reduction of the tracheal lumen (>85%) and bronchomalacia. Then, the patient underwent posterior tracheopexy and aortopexy and later an arterial switch operation, ventricular septal defect closure, and resection of a part of the infundibular septum, accepting the risk of potential neoaortic obstruction. The literature has reported only two cases of patients with a fetal echocardiogram diagnosis. Therefore, our patient is only the third one with a fetal diagnosis and the second one with a complex intracardiac anatomy, characterized not only by a ventricular septal defect but also by two separate components of the obstruction (a bicuspid valve and a dysplastic valve with a posterior deviation of the infundibular septum). In conclusion, a D-transposition of the great arteries with a double aortic arch remains an extremely unusual association. The clinical outcome of these patients presents a high degree of variability and is entirely unpredictable in prenatal life. Our greatest aim as fetal and perinatal cardiologists is to improve the management and outcome of these patients through a fetal diagnosis, recognizing types of congenital heart disease in newborns who require early neonatal invasive procedures.
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    文章类型: Case Reports
    双主动脉弓是主动脉弓形成血管环的胚胎异常。已经注意到,右回神经在主动脉弓重复的患者中的行进方式不同,并且可能与肺癌的上纵隔淋巴结清扫区域非常接近。我们报告了一例与主动脉弓重复相关的右中肺癌患者的手术病例。一名64岁的男子被转诊到我们医院,原因是在体检期间在胸部X光片上发现右肺区域有结节状阴影。经支气管针吸活检显示诊断为腺癌,行右侧中叶切除及淋巴结清扫术。当解剖主动脉弓重叠的患者的上纵隔淋巴结时,有必要仔细地进行手术,注意右回神经的运行。
    Double aortic arch is an embryological abnormality of the aortic arch forming a vascular ring. It has been noted that the right recurrent nerve travels differently in patients with a duplicated aortic arch and may be in close proximity to the area of superior mediastinal lymph node dissection in lung cancer. We report a surgical case of a patient with right middle lung cancer associated with a duplicated aortic arch. A 64-year-old man was referred to our hospital because of a nodular shadow in the right lung field noted on chest X-ray during a medical checkup. A transbronchial needle biopsy revealed a diagnosis of adenocarcinoma, and right middle lobe resection and lymph node dissection were performed. When dissecting the superior mediastinal lymph nodes in a patient with an overlapping aortic arch, it was necessary to carefully perform the operation, paying attention to the running of the right recurrent nerve.
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  • 文章类型: Case Reports
    带有血管环的双主动脉弓是一种罕见但有记载的主动脉弓变体,传统上由于外在压迫而出现吞咽或呼吸困难。从非冠状窦到左心房的管道非常罕见,与有限的病例报告进行比较。我们报告了一位因右侧中风症状而接受脑血管造影的老年妇女的双主动脉弓偶然发现,在随后的非冠状窦和左心房之间的可能管道的CT门控主动脉造影上发现了进一步的异常。值得注意的是,主动脉弓异常在以前的X线平片上漏诊,这甚至在经验丰富的放射科医生中也可能发生。这个案例说明了一个彻底的,系统的方法来解释胸片,以避免错过纵隔病变,如主动脉异常。
    The double aortic arch with vascular ring is a rare but documented aortic arch variant, traditionally presenting with difficulty swallowing or breathing due to extrinsic compression. Tracts from the noncoronary sinus to left atrium are very rare, with limited case reports to compare against. We report an incidental finding of double aortic arch in an elderly woman who underwent a cerebral angiogram for symptoms of a right-sided stroke, with a further anomaly identified on subsequent CT gated aortogram of a possible tract between the non-coronary sinus and left atrium. It is worth noting that the aortic arch abnormality was missed on previous plain radiographs, which can happen even among experienced radiologists. This case illustrates the need for a thorough, systematic approach to interpreting chest radiographs to avoid missing mediastinal lesions, such as aortic abnormalities.
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  • 文章类型: Case Reports
    主动脉弓的动脉分支的异常很少见,右锁骨下动脉异常是这种异常中最常见的。大多数异常是无症状的,通常是偶然发现的。在绝大多数有症状的病例中,表现可能是呼吸困难或吞咽困难或两者兼有。这是除了异常血管的固有动脉疾病的性质之外,尤其是在成年患者中;除非考虑到,诊断经常被遗漏,导致延误和错误的治疗。在本报告中,我们介绍了一名尼日利亚成年男性吞咽困难的病例,该病例最初被诊断为因摄入草药药水而导致的食管狭窄,但对他的影像学检查进行了最终诊断,即从异常的右锁骨下动脉吞咽困难。强调了诊断的困难以及对影像学检查进行多学科审查的必要性。患者通过经胸和颈部联合入路成功治疗,将异常血管分开并重新植入右颈总动脉。他在手术后2年内无症状。尽管这些异常中的大多数通常是无症状的,重要的是,在影像学检查以及涉及上内脏纵隔结构的手术中,都应牢记它们。在有症状的治疗中已经记录了各种手术方法;但是,建议选择确保患肢血运重建的方案。
    Anomalies of the arterial branches of the arch of the aorta are rare, with the aberrant right subclavian artery being the most common of this anomaly. Majority of the anomalies are asymptomatic and often discovered as incidental findings. In the great majority of the symptomatic cases, the presentation may be either with breathlessness or dysphagia or both. This is in addition to the nature of the intrinsic arterial disease of the aberrant vessel, especially in adult patients; and unless borne in mind, the diagnosis is often missed leading to delays and wrong treatment. In this report we present a case of dysphagia in an adult male Nigerian initially diagnosed as œsophageal stricture from herbal potion ingestion but review of his imaging investigations gave a final diagnosis of dysphagia lusoria from an aberrant right subclavian artery. The difficulty in making a diagnosis and the need for a multidisciplinary review of the imaging investigations are highlighted. The patient was successfully treated by a combined trans-thoracic and cervical approach with division and re-implantation of the aberrant vessel unto the right common carotid artery. He has remained symptom-free for 2 years after surgery. Although the great majority of these anomalies are often asymptomatic, it is important they are borne in mind both in imaging investigations as well as in procedures involving structures in the upper visceral mediastinum. Various surgical approaches have been documented in the management of symptomatic ones; it is however recommended that options that ensure revascularization of the affected limb be selected.
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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: Case Reports
    一个女婴,出生在37周5天的母亲通过诱导阴道分娩先兆子痫,产前诊断为右主动脉弓有血管环。生命的第三天,婴儿表现出青铜色的灰色,检测到直接胆红素为1.7mg/dL。腹部超声无法显示胆囊。临床上,婴儿表现出与Alagille综合征一致的特征,包括不寻常的面部外观,蝴蝶椎骨,心血管缺陷,和胆汁淤积。遗传学家指出,患者的母亲也表现出类似的特征。婴儿和母亲都被诊断出患有Alagille综合征,两者都具有相同的杂合JAG1基因(NM_000214.2)变体(c.1890_1893del,p.Ile630Metfs*112)。我们认为,在我们的患者中观察到的血管环是与Alagille综合征相关的血管环的首次报道实例。
    A female infant, born at 37 week 5 days to a mother via induced vaginal delivery for preeclampsia, was prenatally diagnosed with a right aortic arch with vascular ring. On the third day of life, the infant exhibited a bronze-gray coloration, and a direct bilirubin of 1.7 mg/dL was detected. The abdominal ultrasound did not visualize the gallbladder. Clinically, the infant displayed features consistent with Alagille syndrome, including unusual facial appearance, butterfly vertebrae, cardiovascular defects, and cholestasis. The geneticist noted that the mother of the patient also exhibited similar features. Both the infant and the mother were diagnosed with Alagille syndrome, both having the same heterozygous JAG1 gene (NM_000214.2) variant (c.1890_1893del, p.Ile630Metfs*112). We believe that the vascular ring observed in our patient is the first reported instance of a vascular ring associated with Alagille syndrome.
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  • 文章类型: Case Reports
    本文描述了一名5个月大的女性的成功临床结果,该女性诊断为异常右锁骨下动脉和继发孔房间隔缺损的不完整血管环,并伴有弯刀综合征类型的部分肺静脉异常回流,与右肺隔离症共存。住院期间,进行心脏缺损的手术矫正和肺隔离症的切除。据我们所知,描述的星座缺陷是一种独特的现象,对复杂的治疗和疾病管理构成挑战。
    The article describes a successful clinical outcome in the case of a 5-month old female with a diagnosis of incomplete vascular ring of aberrant right subclavian artery and ostium secundum atrial septal defect associated with partial anomalous pulmonary venous return of scimitar syndrome type, coexisting with right pulmonary sequestration. During hospitalization, surgical correction of the heart defect and resection of the lung sequestration were performed. To the best of our knowledge, described constellation of defects is a unique phenomenon, posing a challenge for complex treatment and disease management.
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  • 文章类型: Case Reports
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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: Case Reports
    先天性主动脉疾病(CAoD)包括从无症状的发现到危及生命的疾病的各种疾病。多种成像技术可用于CAoD的评估。
    我们提供了7例先天性主动脉疾病的病例报告,包括主动脉弓阻塞(缩窄,发育不全,和中断)和血管环,其中讨论了整个病例的临床表现,突出症状的异质性。
    多重成像技术对于CAoD的评估是必不可少的,其中,心脏计算机断层扫描血管造影是快速采集三维体积渲染图像以实现最佳手术计划的主要方式。
    UNASSIGNED: Congenital aortic diseases (CAoD) encompass a wide variety of disorders that range from asymptomatic findings to life-threatening conditions. Multiple imaging techniques are available for the assessment of CAoD.
    UNASSIGNED: We present seven case reports of congenital aortic diseases, including obstructions in the aortic arch (coarctation, hypoplasia, and interruption) and vascular rings, in which the clinical manifestations throughout the cases are discussed, highlighting the heterogeneity of the symptoms.
    UNASSIGNED: Multi-imaging techniques are indispensable for the assessment of CAoD, where cardiac computed tomography angiography is the main modality for rapid acquisition of three-dimensional volume-rendered images for optimal surgical planning.
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