Asian Cardiovasc Thorac Ann 2008;16:e37-e39
© 2008 Asia Publishing EXchange Ltd
Right Cervical Aortic Arch with Aberrant Left Subclavian Artery
Yanto S Tjang, MD,
José I Aramendi, MD,
Alejandro Crespo, MD,
Gadah Hamzeh, MD,
Roberto Voces, MD,
Miguel A Rodríguez, MD
Division of Cardiac Surgery Hospital de Cruces Barakaldo Bilbao, Spain
For reprint information contact: José I Aramendi, MD, Tel: 34 94 600 6339, Fax: 34 94 600 6076, Email: jiaramendi{at}hcru.osakidetza.net, Division of Cardiac Surgery, Hospital de Cruces, Plaza de Cruces, Barakaldo, Spain 48903.
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ABSTRACT
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The combination of right cervical aortic arch, aberrant retroesophageal left subclavian artery originating from a Kommerell’s diverticulum, and a ligamentum arteriosum, constitutes a rare form of vascular ring. Two patients aged 21 days and 54 years, who were diagnosed by multislice 3-dimensional computed tomography and magnetic resonance imaging, underwent surgical division of a vascular ring. The adult required resection of a Kommerell’s aneurysm and subclavian artery reimplantation.
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INTRODUCTION
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Right cervical aortic arch is a rare malformation in which a right-sided aortic arch is unusually long, forms a loop and ascends into the right supraclavicular region, then descends and crosses behind the esophagus to continue as a left descending aorta.1 With an aberrant subclavian artery contralateral to the aortic arch, and a persistent ligamentum arteriosum, a vascular ring can occur around the trachea and esophagus.2 We describe 2 patients who underwent surgery with a diagnosis of right cervical aortic arch associated with an aberrant left subclavian artery arising from a Kommerell’s diverticulum.
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CASE 1
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A 54-year-old woman was referred because of dysphagia and dyspnea on exertion, which had progressed slightly in recent years. Chest radiography showed widening on the right side of the superior mediastinum and an aortic knob located above the tracheal bifurcation (Figure 1A
). A barium esophagogram revealed a posterolateral indentation of the esophagus. Given the high suspicion of a vascular ring, multislice 3-dimensional computed tomography was performed (Figure 2A). A right cervical aortic arch with a retroesophageal aberrant left subclavian artery arising from an aneurysmal Kommerell’s diverticulum was identified (Figure 2B). The patient underwent a limited left posterolateral thoracotomy at the 3rd intercostal space. A left ligamentum arteriosum was confirmed intraoperatively, which originated from the proximal left subclavian artery arising from a diverticulum with 25-mm aneurysmal dilatation. The ligamentum ended in the left pulmonary artery and completed a vascular ring encircling the esophagus and trachea. The ligamentum was first divided, resulting in complete decompression of the esophagus. The aberrant left subclavian artery was ligated and divided, followed by resection of the diverticulum and aneurysmorrhaphy. Extraanatomic bypass of the left common carotid-to-subclavian artery was carried out through a separate left supraclavicular incision, using a 6-mm Gore-Tex vascular graft. The patient made an uneventful recovery. She experienced immediate resolution of dysphagia, and was discharged on the 6th postoperative day, on antiplatelet medication. Postoperative magnetic resonance imaging showed complete resolution of the compression, with a normal aortic diameter (Figure 2C).
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Figure 1. (A) Chest radiograph of patient no. 1. Note the high right aortic arch reaching the supraclavicular region. (B) Esophagogram of patient no. 2, showing right lateral and posterior indentation.
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Figure 2. (A) Multislice computed tomography in patient no. 1 in lateral view, showing the gothic shape of the aortic arch and the Kommerell’s aneurysm, and (B) in posterior view showing the aberrant left subclavian artery arising from the Kommerell’s aneurysm. (C) Postoperative magnetic resonance imaging in patient no. 1. In the lateral view, the aortic arch is wider than preoperatively and there is no Kommerell’s aneurysm. In frontal view, note the extraanatomic Gore-Tex graft between the left carotid and subclavian arteries. (D) Magnetic resonance imaging in patient no. 2, showing the right cervical arch with Kommerell’s diverticulum.
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CASE 2
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A neonate was referred at 2 days old because of inspiratory stridor. Her weight was 2,850 g, and apart from stridor, her physical examination was normal. An esophagogram showed posterior compression of the esophagus (Figure 1B
). Magnetic resonance imaging revealed a right cervical aortic arch with a left descending thoracic aorta (Figure 2D). The aortic arch branches were: left common carotid artery, right common carotid artery, right subclavian artery, and an aberrant left subclavian artery originating from a Kommerell’s diverticulum, with a retroesophageal course. The baby was operated on at the age of 21 days through a limited left lateral minithoracotomy. A ligamentum arteriosum originating from the left subclavian artery closed a vascular ring that compressed the trachea and esophagus. The ligamentum was divided, and this resolved the compression. She was extubated 24 hours postoperatively and discharged on the 8th postoperative day, with no medication.
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DISCUSSION
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The prevalence of cervical aortic arch is less than 1 in 10,000 live births.3 Since its first description by Reid4 in 1914, less than 50 cases have been reported.5 The clinical presentation of cervical aortic arch varies from asymptomatic to a pulsatile cervical mass in the lower neck, dysphagia, dyspnea, recurrent infection, discrepancy of arterial blood pressure between upper and lower extremities, symptoms associated with congenital heart disease, or rupture.6 Its development can be explained by persistence of the 2nd or 3rd branchial arch, combined with resorption of the 4th branchial arch. The aortic arch is narrow and elongated, producing a gothic-shaped arch. The area under the arch is further reduced by a ligamentum arteriosum, resulting in a vascular ring. In the neonate, the main symptom is dysphagia related to the vascular ring.
Routine chest radiography is diagnostic and allows recognition of the supraclavicular location of the cervical aortic arch, and associated aneurysms as mediastinal masses. Computed tomography and magnetic resonance imaging studies are complementary. We believe that computed tomography offers the best spatial and contrast resolution, providing excellent depiction of the arch and venous anomaly with respect to the surrounding soft tissues. Like magnetic resonance imaging, computed tomography allows imaging and data analysis in different planes through 3-dimensional reconstruction.5 The ligamentum arteriosum is not identified by these imaging techniques, but must be suspected.
Surgical repair is simple ligation and section of the ligamentum. The repair can be performed easily through a minimally invasive approach, without the need for more complex procedures, such as resection of the diverticulum and reimplantation of the left subclavian artery into the left carotid artery, as suggested by Backer and colleagues.7 This latter repair may be the procedure of choice in infancy to prevent recurrence of symptoms, but it may not be necessary if the defect is repaired in the neonatal period because there is a very short exposure of the trachea and esophagus to the vascular ring, and complete expansion is easily achieved. At a later stage, aneurysmal dilatation of the Kommerell’s diverticulum requires aortic arch repair. We were successful in repairing the arch by simple aneurysmorrhaphy without the need for hypothermic circulatory arrest. Kouchoukos and Masetti8 reported 2 cases of right aortic arch and Kommerell’s diverticulum repaired by tube graft interposition under hypothermic circulatory arrest. These patients were much older than our adult patient, and they had developed aneurysms of the adjacent descending thoracic aorta. Unlike our cases, a ligamentum arteriosum was not present, and the aneurysms mandated surgery. We chose an extraanatomic bypass graft to restore blood flow to the left subclavian artery in the adult. This type of repair is easily accomplished in cases of subclavian steal syndrome. In infants, the subclavian artery is healthy and flexible, permitting reimplantation to the left carotid artery.7 In adults, this type of repair requires tube graft interposition.
Reports of surgical management of the vascular ring formed by the right cervical aortic arch with an aberrant retroesophageal left subclavian artery arising from a Kommerell’s diverticulum and ligamentum arteriosum are relatively few. If the descending thoracic aorta is not dilated, it can be easily managed without cardiopulmonary bypass and circulatory arrest.
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REFERENCES
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- Reid DG. Three examples of a right aortic arch. J Anat Physiol 1914;48:174–81.[Medline]
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- Backer CL, Hillman N, Mavroudis C, Holinger LD. Resection of Kommerell’s diverticulum and left subclavian artery transfer for recurrent symptoms after vascular ring division. Eur J Cardiothorac Surg 2002;22:64–9.[Abstract/Free Full Text]
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ABSTRACT
INTRODUCTION
