Ann Thorac Surg 2003;76:927-929
© 2003 The Society of Thoracic Surgeons
Case report
Aberrant right subclavian artery with preductal coarctation of the aorta
Yoshihiro Ko, MDa*,
Yuzuru Nakamura, MDa,
Michio Yoshitake, MDa,
Takahiro Inoue, MDa
a Department of Cardiovascular Surgery, Saitama Childrens Medical Center, Saitama, Japan
Accepted for publication February 14, 2003.
* Address reprint requests to Dr Ko, Department of Cardiovascular Surgery, Saitama Childrens Medical Center, 2100 Magome, Iwatsuki-city, Saitama 339-0077, Japan
e-mail: 109434{at}pref.saitama.jp
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Abstract
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A 9-day-old boy diagnosed with an aberrant right subclavian artery, a preductal coarctation, and a ventricular septal defect underwent a subclavian flap coarctectomy. Subsequently, he underwent surgical closure of the ventricular septal defect at the age of 11 months. This case is extremely rare with regard to having an aberrant right subclavian artery originating from the proximal site of a preductal coarctation of the aorta.
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Introduction
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Numerous accounts in the literature have described the association of an aberrant right subclavian artery (ARSA) and a coarctation of the aorta (CoA). However, in all previous reports, the ARSA originated from the descending aorta distal to the CoA, or combined with the postductal CoA. Here we report a surgical case in which the ARSA originated from the proximal site of a preductal CoA.
A 9-day-old boy with Down syndrome was diagnosed with multiple anomalies including umbilical hernia, CoA, ventricular septal defect (VSD), and patent ductus arteriosus. The umbilical hernia required urgent repair because of a hernia sac rupture at birth. An echocardiogram revealed a preductal CoA (1.67 mm) and a perimembranous VSD (4 mm). In addition, a left-to-right shunt was restricted by the tricuspid valve. A left radial angiogram revealed an aberrant right subclavian artery proximal to a preductal coarctation (Fig 1).
There was no pressure gradient between the upper and lower extremities. No medication was required to maintain a patent ductus arteriosus blood flow.

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Fig 1. Left radial angiogram showing the aberrant right subclavian artery located on the most distal side of the aortic arch vessels. The preductal coarctation is located on the distal site of the aberrant right subclavian artery. Asterisk = aberrant right subclavian artery; diamond = patent ductus arteriosus.
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The patient weighed 3.0 kg at the time of surgery. A left thoracotomy through the third intercostal space was made. After ligation of the ductus arteriosus, the aorta was cross-clamped and the right subclavian artery was divided behind the esophagus. The subclavian artery was opened along its length and the incision was extended across the coarctation, and the right subclavian flap coarctectomy was then carried out (Fig 2).
The pressure gradient through the coarctectomy site was only 5 mm Hg.

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Fig 2. The aberrant right subclavian artery was used as a flap and was sutured in place. Asterisk = aberrant right subclavian artery; diamond = patent ductus arteriosus.
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A postoperative angiogram taken at 7 months demonstrated that blood flow was not restricted at the descending aorta, and collateral flow to the right arm had developed (Fig 3).
Because of a progression of pulmonary hypertension, the VSD was closed when the patient reached the age of 11 months.

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Fig 3. Postoperative aortogram showing the smooth reconstruction of the aorta (large arrow) and the collateral flow to the right subclavian artery (small arrows).
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Comment
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An aberrant right subclavian artery can be recognized comparatively easily in the so-called vascular ring, and it has been found in 0.4% to 2.0% [1] of autopsy samples. Most cases are asymptomatic, but some may present with dysphagia [2], and these patients rarely suffer from an aneurysm in the future [3]. In a previous report [4], 1% of the individuals with an aberrant right subclavian artery were shown to also have a postductal CoA. However, no reports have described patients having an aberrant right subclavian artery as well as a preductal CoA. In addition, according to a hypothetical model of the primitive aortic arch system [5], this disorder is produced by interrup-tions of the right fourth aortic arch and the right ductus arteriosus in the fetus. According to this model, the aberrant right subclavian artery should be located on the distal side rather than at the ductus arteriosus or ligament. All previous reports [2, 6] have supported this model, and thus the present case is extremely rare.
Our operative procedure was almost identical to the usual subclavian flap method, and the course of this case progressed favorably without postoperative complications. However, particular care should be taken in surgical cases of CoA with ARSA because collateral vessels from the right subclavian artery or the right subclavian artery in itself can sometimes function as the main collateral channel to the descending aorta [7].
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References
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- Felson B., Cohen S., Courter S.R., McGuire J. Anomalous right subclavian artery. Radiology 1950;54:340-348.
- Stone W.M., Brewster D.C., Moncure A.C., et al. Aberrant right subclavian artery: varied presentations and management options. J Vasc Surg 1990;11:812-817.[Medline]
- Verkroost M.W., Hamerlijnck R.P., Vermeulen F.E. Surgical management of aneurysms at the origin of an aberrant right subclavian artery. J Thorac Cardiovasc Surg 1994;107:1469-1471.[Abstract/Free Full Text]
- Reid D.A., Foster E.D., Stubberfield J., Alley R.D. Anomalous right subclavian artery arising proximal to a postductal thoracic aortic coarctation. Ann Thorac Surg 1981;32:85-87.[Abstract/Free Full Text]
- Stewart J.R., Kinkaid O.W., Edwards J.E. An atlas of vascular rings and related malformations of the aortic arch system. Springfield, IL: Charles C Thomas, 1964.
- Odell J.A., Spilkin S. Anomalous right subclavian artery and coarctation of the aorta. Br Heart J 1984;51:666-669.[Abstract/Free Full Text]
- Lerberg D.B., Hardesty R.L., Siewers R.D., Zuberbuhler J.R., Bahnson H.T. Coarctation of the aorta in infants and children. 25 years of experience. Ann Thorac Surg 1982;33:159-170.[Abstract/Free Full Text]
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