Ann Thorac Surg 2002;73:1640-1642
© 2002 The Society of Thoracic Surgeons
Case report
Aortic coarctation, vascular ring, and right aortic arch with aberrant subclavian artery
Juan-Miguel Gil-Jaurena, MD*a,
Marcos Murtra, MD, PhDa,
Arturo Gonçalves, MDa,
Luis Miró, MDa
a Department of Cardiac Surgery, Hospital Valle de Hebrón, Barcelona, Spain
Accepted for publication September 25, 2001.
* Address reprint requests to Dr Gil-Jaurena, Cirugía Cardiaca, Hospital Valle de Hebrón, Barcelona 08035, Spain
e-mail: giljaurena{at}yahoo.com
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Abstract
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Right aortic arch, in all situations, is relatively rare. In association with coarctation and vascular compression, it is extremely rare. We present a patient with a right aortic arch and an aberrant left subclavian artery, in addition to coarctation. This was dealt with through a left thoracotomy by dividing the ligamentum arteriosum and placing a Dacron graft from the ascending aorta to the descending aorta.
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Introduction
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Congenital obstructive lesions of the right aortic arch are very rare. Few cases have been reported. The arrangement of the brachiocephalic vessels and the descending part of the aorta in such a setting can lead to compression of the tracheoesophageal pedicle because of the vascular ring syndrome. In this article, we have collected data from previous reports, discussing anatomical and pathogenic considerations, in relation to the surgical issues.
A 9-year-old girl weighing 29 kg presented with recurrent bronchitis as the only outstanding symptom. In December 1998, it became evident that she was pulseless in all four limbs, while carotid pulses were present. Aortic coarctation was suspected and, on echocardiography, a right aortic arch was found, with four branches arising in the following order: left carotid, right carotid, right subclavian, and left subclavian (so-called aberrant in a mirror-image arrangement). The right arch was extended retroesophageally to a left-sided descending aorta, and coarctation was noted between the right carotid and right subclavian arteries, with a gradient of 35 mm Hg across the site of narrowing. Deletion of chromosome 22q11 was demonstrated by karyotyping. Magnetic resonance imaging and angiography (Fig 1)
confirmed these findings, showing an aneurysmal right subclavian artery, with its blood derived from the vertebral artery. Kommerell’s diverticulum was seen at the origin of the hypoplastic left subclavian artery, and compression of the trachea and esophagus was produced by the vascular ring. Bronchoscopy revealed dynamic narrowing 2 cm above the carina, and a posterior indentation was seen on an esophagogram.
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Fig 1. Angiography: precoarctation (A) and postcoarctation (B). (AA = ascending aorta; CoD = coarctation site, distal end; CoP = coarctation site, proximal end; DA = descending aorta; LC = left carotid artery; LS = left subclavian artery; RC = right carotid artery; RS = right subclavian artery.)
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Surgical relief was undertaken on April 27, 1999, approaching through a left thoracotomy. The arterial ligament between the left subclavian and the left pulmonary artery was divided, with immediate relief of the compression produced by the vascular ring. Relief of the tracheal narrowing was assessed by intraoperative bronchoscopy. A 14-mm Dacron graft (Hemashield; Meadox Medical Inc, Oakland, NJ) was placed between the ascending and descending parts of the aorta, using a side-biting clamp, without extracorporeal circulation (Fig 2).
The patient was extubated 72 hours later, the postoperative course being complicated by tracheomalacia. The subsequent postoperative course was uneventful, and she was discharged on the 14th day.
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Fig 2. Scheme (A) pre-repair and (B) post-repair. Coarctation site and arterial ligament are circled. (AA = ascending aorta; DA = descending aorta; K = Kommerell’s diverticulum; LC = left carotid artery; LS = left subclavian artery; PA = pulmonary artery; RC = right carotid artery; RS = right subclavian artery; VA = vertebral artery.)
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Comment
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A right aortic arch is said to exist in about 0.1% of the population [1]. It is seen more frequently as a mirror-image pattern of the usual arrangement, or in association with aberrant retroesophageal origin of the left subclavian artery. Associated intracardiac anomalies, such as tetralogy of Fallot or common arterial trunk, are more likely in the setting of mirror-image arrangement. Congenital obstruction of a right aortic arch is extremely rare, can be at different sites, and not be necessarily juxtaductal. The association is explained in two ways. On the one hand, it is known that chromosome 22q11 deletion [2], such as is present in interrupted aortic arch type B with right aortic arch, or as part of CATCH 22 syndrome, can result in coarctation. On the other hand, flow-related factors during the intrauterine life may play a role in the predominance of the right aortic arch [3].
In a recent review, McElhinney and associates collected 42 cases of right aortic arch associated with either coarctation or interruption of the aortic arch [4]. Of these, relatively few cases [4–8] specifically involve an interruption between the right common carotid and subclavian arteries, or aortic coarctation existing proximal to both subclavian arteries. Of those described, the descending aorta was right-sided in three, and this pattern was free of vascular compression. As stated by McElhinney and colleagues [4], bronchial compression is more likely to happen when the right arch leads to a left-sided descending aorta, as in our patient. The link to deletion of chromosome 22q11 has been mentioned by Momma [6], but the association was not sought in the other reports [4, 5, 7, 8]. Regarding treatment, most authors have approached by right thoracotomy [4, 7, 8], excepting one by midline sternotomy and laparotomy [5]. Our patient was approached by left thoracotomy; placement of a graft between ascending and descending (or abdominal) aorta has been reported elsewhere [5].
Irrespective of the surgical approach, division of the arterial ligament would relieve the compression produced by the vascular ring. The aneurysmal consistency of the tissues surrounding the coarctation dissuaded us from attempting to perform an end-to-end repair. Through a left thoracotomy, however, we were able successfully to divide the vascular ring, and to place a graft between the ascending and descending parts of the aorta, leaving the aneurysmal segment in place along with the retroesophageal arch. After repair, flow to the right carotid and both subclavian arteries was provided by the graft rather than by the vertebral artery, as it was preoperatively. In this fashion, in one stage, and by a single approach, we corrected both associated anomalies. In a different setting, two thoracotomies or a thorough dissection of distant and aneurysmal structures via a right thoracotomy or sternotomy might have been required. Concerns may arise about potential complications of this inadvertedly created new vascular ring. Should this happen in the future, division of the coarctation site through a right thoracotomy could be considered.
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Acknowledgments
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The authors acknowledge Professors Robert H. Anderson and Marc de Leval (GOSH, London, UK) for the final review of the manuscript.
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References
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McElhinney D.B., Tworetzky W., Hanley F.L., Rudolph A.M. Congenital obstructive lesions of the right aortic arch. Ann Thorac Surg 1999;67:1194-1202.[Abstract/Free Full Text]
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Skutnik M., Religa Z., Bialkowski J., Wojtalik M. Coarctation of the aorta with right aortic arch. Tex Heart Inst J 1998;25:212-215.[Medline]
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Momma K. Right aortic arch with coarctation proximal to the right subclavian artery and Kommerell’s diverticulum. Cardiol Young 1998;8:413-414.[Medline]
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Vaillant L., Lorette G., Chantepie A., et al. Multiple cutaneous hemangiomas and coarctation of the aorta with right aortic arch. Pediatrics 1988;81:707-710.[Abstract/Free Full Text]
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Abstract
Introduction
