Syncope as Initial Presentation of Kommerell Diverticulum

Kommerell diverticulum (KD) is an aortic arch diverticulum at the origin of an aberrant subclavian artery. It is a rare anatomical anomaly that can be associated with a double aortic arc, a left aortic arch, and anomalous origin of the right subclavian artery or a right aortic arch with anomalous left subclavian artery. We present a case of KD presenting initially as syncope, as well as a review of the literature of this rare syndrome, including diagnosis, imaging techniques, and current surgical treatments.     

Subclavian Steal Phenomenon Associated With Vascular Ring in an Infant Who Had a Prenatally Diagnosed Right Aortic Arch With an Atretic Aberrant Left Subclavian Artery

Vascular ring is a rare congenital anomaly in which the abnormal origin of the aorta or its branches and pulmonary arteries leads to encircling and compression of the trachea and esophagus. A right aortic arch (RAA) with an aberrant left subclavian artery is one of the most common forms of vascular ring. Here, we report a case of a prenatally diagnosed vascular ring resulting from an RAA with an aberrant left subclavian artery. When the infant was 7 months of age, the development of noisy breathing prompted further evaluation with cardiac magnetic resonance imaging that showed an atretic left subclavian artery associated with collateral retrograde flow from the left vertebral artery to the distal portion of the subclavian artery. Our findings indicate that an untreated RAA with an aberrant left subclavian artery may be associated with an increased risk of developing subclavian artery steal syndrome.     

迷走右锁骨下动脉合并主动脉夹层1例

迷走右锁骨下动脉（ARSA）是发自降主动脉近端较常见的主动脉弓大血管异常。ARSA在起始部位可出现内膜撕裂而导致主动脉夹层的发生,从而引起血流受限和后期的动脉瘤样变性。笔者报道了1例起源于ARSA的急性A型主动脉夹层。ARSA合并A型主动脉夹层实属罕见,术前经CT血管造影（CTA）得以确诊,CTA对本病进行外科治疗决策非常有价值。临床医师应对迷走右锁骨下动脉合并主动脉夹层引起注意,以做到早期诊断,早期处理。     

Arteria lusoria: Developmental anatomy, clinical, radiological and surgical aspects

The left aortic arch with an aberrant right subclavian artery, or arteria lusoria, is the most common aortic arch anomaly, occuring in 0.5-2.5% of individuals. Four vessels arise sequentially from the aortic arch: the right common carotid artery, the left common carotid artery, the left subclavian artery and the aberrant right subclavian artery, which crosses upwards and to the right in the posterior mediastinum. It results from a disruption in the complex remodelling of the paired branchial arches, typically of the right dorsal aorta distal to the sixth cervical intersegmental artery. The diagnosis and differentiation of arch anomalies is based on findings at chest radiography in association with those at esophagography. It is usually asymptomatic. When symptomatic, it produces dysphagia lusoria or dyspnea and chronic coughing. Treatment is indicated for symptomatic relief of dysphagia lusoria and for prevention of complications due to aneurysmal dilatation.     

Thoracic Endovascular Aortic Repair With Subclavian Revascularization for Symptomatic Nonaneurysmal Aberrant Right Subclavian Artery

Aberrant right subclavian artery is a common aortic arch anomaly that can cause dysphagia as a result of compression by the aberrant artery. For patients with an aneurysm associated with an aberrant right subclavian artery, surgical or endovascular intervention is a well-described treatment. However, for patients with a nonaneurysmal aberrant right subclavian artery, treatment with thoracic endovascular aortic repair has been limited. We describe the use of thoracic endovascular aortic repair and subclavian revascularization to treat esophageal stricture in a patient with a symptomatic nonaneurysmal aberrant right subclavian artery. The patient''s dysphagia was successfully relieved after the operation.     

Utility of endoscopic ultrasound in the diagnosis of aberrant right subclavian artery

BACKGROUND AND AIMS: Aberrant right subclavian artery (ARSA) is the most common congenital anomaly of the aortic arch occurring in 0.4-2.0% of the general population. Instead of arising from the brachiocephalic artery, the ARSA arises as the last branch from the aortic arch. The aim of this study was to determine the prevalence of ARSA in patients undergoing upper endoscopic ultrasound (EUS) and to describe the EUS characteristics of ARSA. METHODS: A retrospective review was conducted of 7513 upper EUS exams performed from 1 July 2000 to 1 February 2005. RESULTS: In total, 27 (0.36%, 95% CI 0.22-0.50%) of 7513 patients undergoing upper EUS were found to have an ARSA (10 male, 17 female; mean age 58 years, range 23-81 years). Of the 27 patients, 16 had only radial EUS, 10 had radial and linear EUS, and one had only linear EUS. In all 26 patients who underwent radial imaging, a well-defined, anechoic tubular structure was seen originating from the aortic arch and passing between the esophagus and spine. In all 11 patients undergoing linear imaging, the abnormal anatomy was detected and the vascular nature confirmed by Doppler. In one patient, both radial and linear imaging identified unsuspected aneurismal dilatation of the ARSA; a rare condition referred to as Kommerell's diverticulum (KD). None of the other 26 patients had symptoms to suggest an ARSA. Of the 14 patients who had computed tomography (CT) prior to EUS, only four were initially reported to have ARSA. However, later review of the CT scans verified an ARSA in all 14 patients. Of the two patients who underwent a barium swallow, only one had findings suggestive of ARSA. CONCLUSIONS: This report highlights the utility of both radial and linear EUS imaging in identifying previously unsuspected ARSA and associated anomalies. This report also suggests the need for careful review of the CT in patients suspected of having ARSA due to the frequent failure of radiologists to identify, or report, this anomaly when particular focus is not given.     

Double aortic arch with atretic left arch distal to the origin of left subclavian artery accompanied by dyspnea: A case report and literature review

Double aortic arch with atretic left arch distal to the origin of left subclavian artery is a rare type of vascular ring, and it can be easily confused with the right aortic arch with mirror branching. We provided a rare case of a 10‐month‐old infant with dyspnea. Echocardiography showed a suspicious double aortic arch with atretic left arch distal to the origin of left subclavian artery, which was confirmed intra‐operatively. We summarize ultrasonic image characteristics of the disease and combine it with computed tomography angiography, bronchoscopy, and clinical symptoms in order to improve the detection rate and treatment strategy.     

Isolation of the Left Subclavian Artery in D-Transposition of the Great Arteries with Right Aortic Arch

Abnormal branching of the aorta associated with the right aortic arch (RAA) has been reported as isolation of left subclavian artery (ILSA), isolation of left common carotid artery, isolation of brachiocephalic artery. ILSA is a rare aortic branch anomaly that originates in the left subclavian artery from the pulmonary artery via ductus arteriosus. Several reports have described ILSA associated with 22q11.2 deletion syndrome and tetralogy of Fallot. Here, we present a very unusual case of RAA with ILSA associated with D-transposition of the great arteries and inferior vena cava interrupted with azygos continuation.     

主动脉右弓右降合并Stanford B型主动脉夹层的外科治疗

目的:总结主动脉右弓右降合并Stanford B型主动脉夹层的外科治疗经验。方法:3例右位主动脉弓、右位降主动脉、迷走左锁骨下动脉(迷走左锁骨下动脉型)合并Stanford B型主动脉夹层的患者经胸部右后外切口行胸降主动脉置换术、迷走左锁骨下动脉缝扎术。结果:3例患者均痊愈出院,住院天数7～10 d,无左上肢缺血症状及神经系统并发症。结论:主动脉右弓右降合并Stanford B型主动脉夹层患者行胸降主动脉置换术方法可行,临床疗效满意,术中判断后行迷走左锁骨下动脉缝扎术,可简化手术方式,但应避免术后左上肢缺血坏死。     

Aberrant left subclavian artery associated with Kommerell's diverticulum: chance finding in a 75-year-old patient

The most common anomaly of the aortic arch is the occurrence of a left aortic arch with an aberrant right subclavian artery. Other, less common anomalies have also been described. These include the occurrence of a right aortic arch with an aberrant left subclavian artery that, in addition, has a diverticulum at its site of origin known as Kommerell's diverticulum. All cases described in the literature have occurred in individuals younger than 35 years of age, generally with symptoms related to tracheal or esophageal compression. We present the case of a patient diagnosed by chance with this rare anomaly at 75 years of age.     

Aneurysms of an aberrant right subclavian artery: report of two cases

Aneurysm of the aberrant right subclavian artery is rare. We report two patients who underwent successful repair using hypothermic circulatory arrest and retrograde cerebral perfusion. One patient presented with progressive dysphagia to solids and hoarseness of voice due to pressure exerted by the expanding aneurysm, and the second patient, with a dissected subclavian aneurysm, presented with transient ischaemic attacks and a previous cerebral infarct secondary to embolism. Repair in both patients was done through a median sternotomy with femoral arterial and right atrial venous cannulation. Under hypothermic circulatory arrest, the origin of the subclavian aneurysm was divided off the aorta and closed with a Dacron patch from outside the aorta in one patient, and from within the aorta in the other patient. Concomitant revascularisation of the right upper limb was achieved using a 12-mm Hemishield Dacron graft from the ascending aorta to the distal right subclavian artery. The graft to subclavian anastomosis was done under hypothermic circulatory arrest as distal control was not possible. The graft to the aortic anastomosis was done over a side-biting clamp during rewarming. Both patients were successfully treated without major morbidity.     

The Survival of a Newborn with an Interrupted Aortic Arch and a Closed Ductus Arteriosus

Interrupted aortic arch is a rare congenital cardiovascular anomaly with a wide spectrum of anatomical and clinical presentation. We report the case of a newborn presenting at 11 days of age with a type B‐2 aortic arch interruption, a closed ductus arteriosus, and an unusual large collateral vessel arising from the ascending aorta proximal to the right carotid artery and joining an aberrant right subclavian arising from the descending aorta.     

Importance of Absent Ductus Arteriosus in Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

Tetralogy of Fallot without pulmonary valve syndrome is almost always associated with an absent ductus arteriosus. Patients with right aortic arch and retroesophageal left subclavian artery have a vascular ring if the left ductus arteriosus or its remnant and the Kommerell diverticulum are present. We report the cases of 2 infants in whom the role of an absent ductus arteriosus or its remnant is noteworthy. Both patients had a combination of tetralogy of Fallot with absent pulmonary valve syndrome and right aortic arch with retroesophageal left subclavian artery without a vascular ring. The absence of the ductus arteriosus has a role in the pathogenesis of tetralogy of Fallot with absent pulmonary valve syndrome. The absence of a ductus arteriosus in the right aortic arch with retroesophageal left subclavian artery precludes a vascular ring.     

Aberrant subclavian artery causing difficulty in transhiatal esophageal dissection

The right subclavian artery normally arises from the brachiocephalic artery. Anomalies in development may lead to peculiar problems during surgery. We report a patient with esophageal carcinoma who had an aberrant right subclavian artery, posing specific difficulties during a transhiatal esophagectomy, requiring conversion of the procedure into a transthoracic approach. The embryologic basis of this anomaly and the clinical significance are discussed.     

Double Aortic Arch with Dominant Left Arch and Right Ligamentum Arteriosum

This case report describes a rare example of double aortic arch with a dominant left aortic arch, patent minor right aortic arch, left descending aorta, and right ligamentum arteriosum causing tracheobronchial compression in a twin baby girl with DiGeorge syndrome. She also had large right subclavian artery arising from right‐sided diverticulum of Kommerell, aplastic thymus, T cell lymphopenia with normal immunoglobulin, hypocalcemia, and hypomagnesemia. The diverticulum of Kommerell was resected and minor right aortic arch, right ligamentum arteriosum, and right subclavian artery were divided through right posterolateral thoracotomy. Aortopexy was performed under bronchoscopic guidance to relieve the airway compression. We strongly suggest a right‐sided approach in this type of vascular arrangement for easy access and better outcome.     

Cervical origin of the right subclavian artery in aortic arch interruption: pathogenesis and significance

In 14 of 21 infant hearts (66%) with aortic arch interruption between the left common carotid and left subclavian arteries (type B of Celoria and Patton), the right subclavian artery (SA) arose anomalously. In 9 (43%) it arose from the descending aorta and in 1 heart from the right pulmonary artery (5%). In 4 hearts (19%), it arose high in the neck from the right common carotid artery as a trifurcation with the internal and external carotid arteries, an anomaly not previously reported. In these latter cases, the SA descended down the neck to enter the right arm, with the right recurrent laryngeal nerve coursing around its take-off. Anomalous origin of the right SA is common in type B aortic arch interruption, and we believe this to be the result of a strong tendency for both fourth aortic arches to disappear early in development. If involution occurs in embryos of less than 14 mm crown-rump length, before the right ductus caroticus and dorsal segment of the right sixth arch have become attenuated, 3 alternate routes are available to the embryo to form a right SA.     

Solitary interruption of the aortic arch with stenotic origin of the left subclavian artery in 45, XO/46, XY/47, XYY mosaicism

Summary A four-year-old boy whose karyotype was 45, XO/46, XY/47, XYY mosaicism was diagnosed as having interruption of the aortic arch without ventricular septal defect or patent ductus arteriosus, complicated by stenotic origin of the left subclavian artery, which resembled coarctation of the aorta hemodynamically. Solitary interruption of the aortic arch is a very rare anomaly.     

Type B Interrupted Left Aortic Arch with Isolated Right Subclavian Artery

Interrupted aortic arch is a rare congenital heart malformation occurring in approximately three per 1 million births. Type B interrupted aortic arch (interruption between the second carotid artery and the ipsilateral subclavian artery) is the most common of three major types (A, B, and C). We report an extremely rare finding: a case of left‐sided type B interrupted aortic arch with isolation of the right subclavian artery (origin from the right pulmonary artery).     

Dissected aberrant right subclavian artery with Kommerell diverticulum

Aberrant right subclavian artery is the most frequent anomaly of the aortic arch, and in 60% of cases is associated with Kommerell diverticulum. It is usually asymptomatic but may present with dysphagia or asthma due to esophageal and tracheal compression. Indications for surgical repair have not been established; however, when Kommerell diverticulum is complicated by aortic dissection the treatment is surgery. We present the case of a 54-year-old man with thoracic pain due to dissection of an aberrant right subclavian artery associated with Kommerell diverticulum. Elective surgical treatment was performed.     

Congenital subclavian steal syndrome. Apropos of 14 cases. Review of the literature

Fourteen cases of the congenital subclavian steal syndrome are presented with a review of 99 cases in the literature. This abnormality is usually asymptomatic and a fortuitous finding (clinical: asymmetric blood pressure; radiological: investigation of an associated cardiovascular malformation). The possible malformations are numerous and may be diagnosed by angiography: however, they can be suspected from three simple investigations, clinical examination indicating the side of lower blood pressure, chest X-ray and barium swallow. Seventy one per cent congenital subclavian steal syndromes are associated with a right-sided aortic arch, the steal being then almost always left-sided via a left subclavian artery anomaly. In this group, a left subclavian artery isolated from the aorta represents about a half of the cases. A cardiac malformation is present in one out of two cases and a patent ductus arteriosus uniting the left subclavian artery and the left pulmonary artery is observed in one out of four cases. Twenty nine per cent of congenital subclavian steal syndromes are associated with a left-sided aortic arch. In these cases the abnormalities usually involve the aortic arch (57% of cases) or a right or left subclavian artery. Therefore, in this group, there is no preferential side for the subclavian steal which can be right, left or bilateral. These abnormalities usually only require medical surveillance. When they become symptomatic, vertebro-subclavian revascularisation is justified. The presence of a subclavian steal may have surgical implications: it is important to exclude a subclavian steal before performing a Blalock anastomosis.(ABSTRACT TRUNCATED AT 250 WORDS)