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Comprehensive three-dimensional analysis of right-sided aortic arch with multiple vascular anomalies

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      Right-sided aortic arch is a rare congenital defect usually diagnosed incidentally in adults; it is often asymptomatic unless aneurismal disease develops. In half the cases, an aberrant left subclavian artery arises from a Kommerell’s diverticulum; in these cases, congenital heart anomaly is very rarely present.

      Case presentation

      We report a case of incidentally-detected right-sided aortic arch with multiple vascular anomalies including left subclavian artery originating from a Kommerell’s diverticulum, supra-sinus origin of coronary arteries and coronary arteriovenous fistula.


      Through comprehensive 3-dimensional reconstruction of the aortic arch and surrounding structures we defined anatomical relationships, which is useful for follow-up and treatment.

      Related collections

      Most cited references 8

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      Anomalies of the derivatives of the aortic arch system.

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        Kommerell's diverticulum and right-sided aortic arch: a cohort study and review of the literature.

        We report four consecutive cases of Kommerell's aneurysm of an aberrant left subclavian artery in patients with a right-sided aortic arch and the results of a systematic review of the literature. In our cohort of patients, three had an aneurysm limited to the origin of the aberrant subclavian artery, causing dysphagia and cough, and one had an aneurysm involving also the distal arch and the entire descending thoracic aorta, causing compression of the right main-stem bronchus. A left subclavian-to-carotid transposition was performed in association with the intrathoracic procedure, and a right thoracotomy was used in all patients. One of the patients underwent surgery with deep hypothermia and circulatory arrest, and the others with the adjunct of a left-heart bypass. The repair was accomplished with an interposition graft in two patients and with endoaneurysmorrhaphy in the others. The postoperative course was complicated by respiratory failure and prolonged ventilation in one patient, and one patient died because of severe pulmonary emboli. The survivors are alive and well at a follow-up of 1 to 3 years. Only 32 cases of right-sided aortic arch with an aneurysm of the aberrant subclavian artery have been reported: 12 were associated with aortic dissection, and 2 presented with rupture. Surgical repair was accomplished in 29 patients. A number of operative strategies were described: right thoracotomy, bilateral thoracotomy, left thoracotomy with sternotomy, sternotomy with right thoracotomy, and left thoracotomy. In only 12 cases was the subclavian artery reconstructed. We believe that a right thoracotomy provides good exposure and avoids the morbidity associated with bilateral thoracotomy or sternotomy and thoracotomy. We feel that a left subclavian-to-carotid transposition completed before the thoracic approach revascularizes the subclavian distribution without increasing the complexity of the intrathoracic procedure.
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          Burckhard F. Kommerell and Kommerell's diverticulum.

          Burckhard Friedrich Kommerell's scholarly description of the aortic diverticulum that bears his name was published in 1936. In the international literature, however the name of Kommerell survives only as an eponym. We present biographical information about Kommerell, as supplied by family members, and comment on the surgical relevance of his 1936 report.

            Author and article information

            [1 ]Department of Cardiology, Yeungnam University Hospital, 170, Hyeonchungro, Nam-gu, Daegu 705-717, Korea
            BMC Cardiovasc Disord
            BMC Cardiovasc Disord
            BMC Cardiovascular Disorders
            BioMed Central
            20 August 2014
            : 14
            : 104
            25138741 4236589 1471-2261-14-104 10.1186/1471-2261-14-104
            Copyright © 2014 Lee et al.; licensee BioMed Central Ltd.

            This is an Open Access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver ( applies to the data made available in this article, unless otherwise stated.

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