儿童先天性主动脉弓畸形的MSCT诊断

目的：分析儿童先天性主动脉弓畸形的 MSCT 表现,提高对该病的认识及诊断水平。方法：回顾性分析经MSCT诊断的83例先天性主动脉弓畸形患儿的CT横轴面图像,并进行多平面重组（MPR）、最大密度投影（MIP）、最小密度投影（MinIP）、容积再现（VR）后处理。结果：83例中梗阻性先天性主动脉弓畸形22例,包括主动脉缩窄17例（合并迷走右锁骨下动脉4例）,主动脉弓离断5例;非梗阻性先天性主动脉弓畸形61例,包括双主动脉弓8例,右位主动脉弓、右位降主动脉44例,右位主动脉弓伴迷走左锁骨下动脉7例,左位主动脉弓、右位降主动脉2例（合并迷走右锁骨下动脉1例）。结论：MSCT可清晰显示主动脉弓畸形及合并畸形,对诊断先天性主动脉弓畸形具有重要价值。     

永存第5对主动脉弓的心血管造影及磁共振血管造影诊断

目的 分析并比较永存第5对主动脉弓的心血管造影（ACG）及对比增强磁共振血管造影（CE－MRA）的表现。方法 对2例永存第5对主动脉弓患者行选择性升主动脉及肺动脉总干正侧位造影；2例均作了MRI检查。结果 CE－MRA可清晰显示永存第5对主动脉弓，2例诊断明确并经手术证实。结论 永存第5对主动脉弓是主动脉弓畸形的1种罕见类型，ACG仍是诊断金标准，但MRI中CE－MRA是目前最好的非创伤性诊断方法。     

婴幼儿先天性血管环的MSCT诊断及分析

目的:探讨MSCT对婴幼儿先天性血管环的诊断及分析。方法:回顾性分析本院2012-2018年间60例先天性血管环患儿的MSCT的表现,记录其组成、形态及病变与周围结构的关系。结果:9例双主动脉弓;1例右弓合并左动脉导管未闭或动脉韧带;2例左弓合并右位、中位降主动脉;18例左位主动脉弓伴迷走的右锁骨下动脉;1例无名动脉后位;21例右弓伴迷走左锁骨下动脉;7例肺动脉吊带;1例无名静脉形成静脉环。结论:MSCT能明确血管环的组成及与周围结构的关系,对外科手术有重要意义。     

MSCT对Kommerell憩室的诊断和临床应用价值

Kommerell憩室是先天性主动脉弓畸形的一种,指降主动脉起始部与迷走左或右锁骨下动脉间的瘤样膨凸,也称为主动脉憩室[1].自1936年德国放射学家Kommerell报道1例主动脉左弓左降,迷走右锁骨下动脉起始于主动脉弓处异常膨大后,此后类似病例均被命名为“Kommerell憩室”[2].此类畸形,可引起一些较严重的临床并发症,但因症状隐匿,国内外报道均较少.笔者分析近年来经本院MSCT检查证实的10例患者,发现其影像学有着较为特异的征象,现报道如下.     

先天性主动脉弓畸形的MRI诊断

目的：评价MRI在主动脉弓畸形诊断中的作用。资料与方法：回顾分析228例心脏MR检查中61例先天性主动脉弓畸形的MRI表现，37例尚有心血管造影（CAG），44例经手术证实，结果：61例先天性主动脉畸形中，主动脉缩窄（COA）25例，右位主动脉弓24例，主动脉弓离断（IAA）5例，单纯动脉导管未闭（PDA）4例，迷走右锁骨下动脉（伴COA）2例，孤立性左有下动脉（伴右位主动脉弓）1例，永存第5对主动脉弓（伴IAA）1例，主动脉扭曲畸形（Kink A)2例，升主动脉扭曲1例，结论：比较MRI与CAG，MRI在对先天性主动脉畸形的诊断方面已达到CAG水平。     

右位主动脉弓伴起源于Kommerell憩室的迷走左锁骨下动脉的影像表现

目的探讨右位主动脉弓伴起源于Kommerell憩室的迷走左锁骨下动脉的影像学表现及其临床意义。方法回顾性分析25例右位主动脉弓伴起源于Kommerell憩室的迷走左锁骨下动脉患者的影像学资料,全部患者行胸片和胸部CT检查,其中4例患者行胸主动脉CTA检查,1例行胸腹主动脉CTA和MRA检查。结果 25例患者胸部平片见右位主动脉弓,12例见"双主动脉弓",9例误诊为纵隔病变。CT和MR能显示左锁骨下动脉起源于主动脉弓降段左侧壁,起始段膨大形成Kommerell憩室,迷走左锁骨下动脉横跨食管后方,"双主动脉弓"的左弓为膨大的Kommerell憩室及其远侧的迷走左锁骨下动脉。2例左锁骨下动脉近段动脉硬化并形成动脉瘤。1例患者偶觉吞咽不适。结论右位主动脉弓伴起源于Kommerell憩室的迷走左锁骨下动脉是一种罕见的先天变异,胸片容易误诊为纵隔病变,CT和MR能明确诊断并显示变异血管继发病变。正确认识此变异,有助于避免误诊。     

罕见类型的主动脉弓畸形

主动脉弓畸形的罕见类型包括右或左骨下动与左或右主动脉弓彼此不连接（分离）。无名或颈动脉分离和左无名动脉异常走行（迷走）伴随右位主动脉弓，其它畸形还有永存第五动脉弓，作为主动脉弓第五分支的锁骨下动脉，肺动脉和动脉导管悬带，中讨论了这些畸形的心血管造影特点和胚胎学改变。     

主动脉弓离断的心血管造影影像分析

目的分析主动脉弓离断(interruption of aortic arch,IAA)患者的心血管造影影像,提高对本病的认识和诊断准确性。资料与方法 8例患者均经股动脉、股静脉穿刺插管,行右心室、左心室、降主动脉造影。其中3例经右颈总动脉穿刺插管行升主动脉造影;3例从右心室经室间隔缺损至升主动脉造影。结果 8例升主动脉与降主动脉之间均无血流连续。升主动脉细小7例,升主动脉扩张1例,降主动脉顶端与未闭的动脉导管连接7例,降主动脉与右锁骨下动脉连接1例。8例均合并心内畸形,其中室间隔缺损+动脉导管未闭并肺动脉高压5例,右室双出口+动脉导管未闭并肺动脉高压2例,法乐四联症1例。结论心血管造影是IAA确诊的重要方法,能够为IAA的早期有效诊断和外科手术适应证的选择提供重要的临床依据。     

64排螺旋CT血管造影对迷走右锁骨下动脉的临床应用

迷走右锁骨下动脉又称异位右锁骨下动脉,是较常见的主动脉弓变异,一般无临床症状,但出现症状后易与食管癌混淆,影像学上常误诊为主动脉弓部的夹层动脉瘤等.但迷走右锁骨下动脉的影像学研究对相关临床疾病的诊断、胸部外科手术、头颈部血管的介入治疗或动脉插管路径的选择等方面具有重要意义.本研究旨在分析迷走右锁骨下动脉的CT血管造影的征象和特点,探讨CT血管造影对迷走右锁骨下动脉的诊断价值.     

MR快速多时相造影增强与时间飞跃血管成像在颈部血管疾病中诊断价值的对比研究

目的　对比研究磁共振时间飞跃血管成像 (timeofflightmagneticresonanceangiograhy,TOF MRA)与快速多时相造影增强成像 (contrastenhancemagneticresonanceangiography ,CE MRA)在颈部血管疾病中的诊断价值。方法　临床怀疑颈部血管疾病患者 12 6例 ,同时进行颈部TOF MRA和CE MRA检查 ,所有病例均行DSA或者手术 ,并以此为金标准对TOF MRA和CE MRA的图像质量 [信噪比(SNR)、对比噪声比 (CNR)、伪影 ]、颈总动脉分叉区狭窄诊断 (准确性、特异性、敏感性 )及其在血管畸形中的诊断价值进行对比研究。结果　经DSA或手术证实阳性病例共 89例 (动脉瘤 3例、动静脉畸形 2例、颈总动脉分叉区动脉硬化狭窄 84例 ,共 12 2处狭窄 ) ,CE MRA在颈总动脉分叉区的SNR、CNR分别为 2 0 94± 2 5 7、11 10± 3 84 ,TOF MRA为 12 5 5± 1 71、2 70± 0 74 ,二者差异具有显著性意义 (t值分别为 13 36和 11 5 0 ,P值均 <0 0 0 1)。 12 6例的TOF MRA均出现伪影 ,而CE MRA未发现伪影。CE MRA对颈总动脉分叉区狭窄诊断特异性为 99 2 3% ,TOF MRA为 2 1 5 4 % ,二者差异具有显著性意义 (χ2 =12 0 85 ,P <0 0 0 1) ;CE MRA对颈总动脉分叉区狭窄诊断的敏感度为 90 88% ,TOF MRA为93 4 4 % ,二者差异无显     

Aberrant subclavian arteries: cross-sectional imaging findings in infants and children referred for evaluation of extrinsic airway compression

OBJECTIVE: The purpose of our study was to describe patterns of airway compression identified on cross-sectional imaging in infants and children with either right aortic arch and aberrant left subclavian artery or left aortic arch with aberrant right subclavian artery. MATERIALS AND METHODS: Data from MR imaging and CT performed to evaluate pediatric patients for extrinsic airway compression were reviewed for cases that revealed an aberrant right or left subclavian artery. Clinical, endoscopic, and imaging findings in identified cases were reviewed. Recurrent patterns of extrinsic compression were reviewed among cases. RESULTS: Twelve patients with right aortic arch with aberrant left subclavian artery and nine patients with left aortic arch and aberrant right subclavian artery were identified. All 12 with right aortic arch with aberrant left subclavian artery had airway compression shown, with multiple sites or diffuse compression in six. Of these 12 patients, nine had compression at the level of the arch and aberrant subclavian artery (10 had Kommerell's diverticulum), and nine had compression of the distal airway in association with a midline descending aorta. Five of the nine patients with left aortic arch and aberrant right subclavian artery had airway compression shown, all at the level of the arch and aberrant subclavian artery. None of these compressions was associated with either Kommerell's diverticulum or midline descending aorta. CONCLUSION: Both right and left aberrant subclavian arteries can be associated with symptomatic airway compression, but the patterns of compression are different. The airway compression in right aortic arch with aberrant left subclavian artery is often associated with either Kommerell's diverticulum or midline descending aorta, whereas compression associated with left aortic arch and aberrant right subclavian artery is not.     

MR imaging of congenital anomalies of the aortic arch

Eighteen patients with congenital aortic arch anomalies were evaluated by ECG-gated MR imaging. Transverse images encompassing the heart and thoracic aorta were available in all patients; sagittal or coronal studies were available in 12 patients. Visualization of the aortic arch, its orientation, and the origin and course of the arch vessels was assessed. Associated intracardiac abnormalities were noted, and the effect of aberrant vessels on the trachea or esophagus was determined. Thirteen patients had a right aortic arch. Mirror-image branching was found in 10 cases, and an aberrant left subclavian artery was found in three of these. Three patients had a left aortic arch with aberrant right subclavian artery, and two patients had a double arch. Tracheal compression caused by vascular rings was found in two patients with respiratory symptoms. Corroborating studies (angiography, surgery, CT, and autopsy) in 16 patients confirmed the MR diagnoses in all but one. We conclude that MR could substitute for other techniques as an effective, noninvasive method for the evaluation of congenital aortic arch anomalies.     

Congenital subclavian steal associated with an unusual type of right aortic arch

A case of congenital subclavian steal caused by a previously unreported aortic arch anomaly was demonstrated by angiography. The patient was shown to have a right aortic arch with a retroesophageal segment, a stenotic proximal portion of the left subclavian artery, and a left descending aorta.     

Follow-up of patients with previous treatment for coarctation of the thoracic aorta: comparison between contrast-enhanced MR angiography and fast spin-echo MR imaging

Regular follow-up is required in patients with previous intervention for coarctation of the aorta to detect recoarctation or aneurysm formation. In this study we describe the findings encountered on routine follow-up exams and we compare the use of contrast-enhanced 3D MR angiography (CE MRA) with fast spin-echo MRI (FSE) to study the thoracic aorta after previous intervention. In 51 consecutive patients previously treated for aortic coarctation, 74 MR studies of the thoracic aorta were performed during a 2-year period using CE MRA and FSE MRI. The thoracic aorta was evaluated for abnormalities of course, caliber, shape, and pathology of side branches. The CE MRA and FSE MRI studies were evaluated side by side by consensus of two reviewers evaluating which MR technique depicted the abnormalities of the thoracic aorta the best. Of 74 exams, six clinically important abnormalities were found: four aneurysms and two restenoses. Two small pseudoaneurysms were missed on the FSE studies. Contrast-enhanced MRA was judged to visualize aortic abnormalities better than FSE (47 of 74 MR studies) especially for the transverse aortic arch, coarctation site, left subclavian artery, and aortic arch configuration. For the ascending aorta and distal descending aorta, CE MRA and FSE performed equally well. Aortic diameters measured at four levels in the first 18 MRI studies showed no significant differences in diameter when measured by FSE or CE MRA (p = not significant). Clinically important abnormalities, such as aneurysm formation and restenosis, can be present years after treatment for aortic coarctation. In the regular follow-up of these patients, CE MRA may provide additional diagnostic information compared with FSE and should be included as part of the routine exam. Received: 3 April 2000; Revised: 5 July 2000; Accepted: 7 July 2000     

Pitfalls of digital angiography in the diagnosis of right aortic arch

The authors describe the difficulties using intravenous digital subtraction angiography, in diagnosing on a left anterior oblique view a right aortic arch with aberrant left subclavian artery and left descending aorta. The proximal topography of the left common carotid artery ostium is a useful sign in the diagnosis of this kind of abnormality.     

Circumflex retroesophageal right aortic arch simulating mediastinal tumor or dissecting aneurysm

The authors present three cases of circumflex right aortic arch, a retroesophageal arch in which the aorta crosses from right to left behind the esophagus to descend on the left side. The findings on the frontal chest films resembled those of a left arch with mediastinal widening, simulating a mass. Aortography revealed four-vessel branching of the arch vessels, typical of right aortic arch with aberrant left subclavian artery. Computed tomography and barium studies of the esophagus demonstrated the retroesophageal course of the aorta. In two patients, obstruction of the thoracic portion of the left subclavian artery resulted in differences in blood pressure and pulse between the arms, supporting the clinical impression of dissecting aortic aneurysm.     

MSCTA对先天性血管环的诊断价值

目的：探讨儿童先天性血管环的MSCTA表现,提高临床对本病的认识水平。方法：搜集2011年1月-2012年2月经手术确诊30例先天性血管环患者的病例资料,年龄1个月～9岁。主要临床表现为反复呼吸道感染者19例,反复呕吐、进食困难等消化道症状4例,6例合并心内畸形,1例为偶然发现。MSCTA原始图像以0．6mm层厚重建,使用多平面重组（MPR）、最大密度投影（MIP）、容积再现（VR）等技术对图像进行后处理。结果：30例中双主动脉弓4例,右位主动脉弓伴迷走左锁骨下动脉9例,左位主动脉弓伴迷走右锁骨下动脉7例,肺动脉吊带9例,右位主动脉弓伴迷走左头臂动脉1例。合并气管病变26例,主要为先天性气道发育畸形（4例）和气管狭窄（22例）。结论：MSCTA能清楚显示心外大血管情况,对先天性血管环明确诊断的同时,可了解气管受压程度及狭窄范围,有助于临床明确诊断及选择治疗方法。     

MR angiography of left-sided cervical aortic arch with aberrant right subclavian artery

Cervical aortic arch is a rare anomaly. We report the contrast-enhanced MR angiography features of left-sided cervical aortic arch with contralateral descending aorta and aberrant right subclavian artery in a 16-year-old girl.     

Endovascular Repair of a Right-Sided Descending Thoracic Aortic Aneurysm Associated with a Right Aortic Arch and a Left Subclavian Artery Arising from a Kommerell’s Diverticulum

This case report describes the endovascular repair of a right-sided descending thoracic aortic aneurysm associated with a right aortic arch and an aberrant left subclavian artery. A 76-year-old male with multiple comorbidities was incidentally found to have a right-sided descending thoracic aortic aneurysm with a maximum diameter of 6.2 cm. Additionally, there was a right aortic arch with a retroesophageal segment and separate arch branches arising in the following order: left common carotid artery, right common carotid artery, right subclavian artery, and left subclavian artery that was aberrant, arising from a Kommerrell’s diverticulum. The aneurysm was successfully excluded by deployment of a Zenith TX1 36 × 32 × 20-mm stent-graft using wire traction technique via the left femoral and right brachial arteries in order to deal with two severe aortic angulations. At 18-month follow-up the patient was doing well, with aneurysm sac shrinkage to 5.9 cm and no signs of endoleak or migration. Endovascular repair of right-sided descending thoracic aortic aneurysms with a right arch and aberrant left subclavian artery is feasible, safe, and effective. In such rare configurations, which demand considerably increased technical dexterity and center experience, endovascular repair emerges as an attractive therapeutic option.