Ann Thorac Surg 1999;67:248-250
© 1999 The Society of Thoracic Surgeons
Case Reports
Cervical aortic arch with pseudocoarctation: presentation with spontaneous rupture
John. S. Ikonomidis, MD, PhDa,
Robert C. Robbins, MDa
a Department of Cardiothoracic Surgery, Stanford Health Services, Stanford, California, USA
Accepted for publication June 23, 1998.
Address reprint requests to Dr Robbins, Department of Cardiothoracic Surgery, Falk Cardiovascular Research Center, Stanford Health Services, Stanford, CA 94305-5247
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Abstract
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A 23-year-old man presented with hypotension secondary to a left hemothorax. Diagnostic studies revealed a left cervical arch associated with a redundant tortuous pseudocoarctation in the proximal descending thoracic aorta. Operative exploration revealed an aortic rupture just proximal to the pseudocoarctation. Resection of the diseased aorta and tube graft replacement was performed under circulatory arrest. The patient was discharged home on the 12th postoperative day.
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Introduction
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The cervical aortic arch is a rare entity, with fewer than 50 patients collected in the literature since its first description by Reid in 1914 [1]. These cases may be further complicated by distal aneurysm formation or the various cardiovascular and other anomalies comprising the catch 22 syndrome. We describe a unique case of a left cervical arch with rupture proximal to an associated tortuous pseudocoarctation of the descending thoracic aorta.
A 23-year-old male ex-marine was transferred to the Stanford Health Center emergency department from an outside hospital where he had presented with a sudden onset of crushing retrosternal chest pain, abdominal pain, diaphoresis, and labile blood pressure. There was no antecedent history of thoracic trauma. A chest roentgenogram revealed a left pleural effusion suggestive of a left hemothorax. An electrocardiogram showed a sinus tachycardia with nonspecific S-T segment and T-wave changes in the leads I, II, and V4–6. After resuscitation, a thoracic aortogram was performed, which showed a tortuous distal aortic arch and proximal descending thoracic aorta with extravasation of contrast. A spiral computed tomogram was obtained (Fig 1) that revealed evidence of a left cervical aortic arch with a "corkscrew" appearance from which the left subclavian artery originated. In addition, the proximal descending thoracic aorta was configured in a tortuous mass. There was no poststenotic dilatation of the remainder of the descending thoracic aorta.
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Fig 1. Three-dimensional spiral computed tomographic reconstruction of thoracic aorta. (A) Arrow points to Medusa-like ball of descending thoracic aorta with pseudocoarctation. (B) Arrow points to "cork-screw" cervical aortic arch. (I = innominate artery; LCC = left common carotid artery; LS = left subclavian artery.)
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The patient was brought to the operating room and a left posterolateral thoracotomy was performed based on the fourth intercostal space. After suctioning of a copious amount of clotted and nonclotted blood from the pleural cavity, a large mediastinal hematoma was noted. Proximal control of the aorta was obtained at the origin of the cervical arch and distal control was obtained at the left subclavian artery and the mid descending thoracic aorta. Following this, cardiopulmonary bypass was instituted through the left common femoral artery and vein. The patient was cooled to a bladder temperature of 22°C and cross-clamps were placed proximal and distal to the Medusa-like ball of descending thoracic aorta. The site of rupture was identified on the lesser curvature of the aorta between the cervical arch and the pseudocoarctation. The clamped portion of aorta was then resected and a 16-mm double velour-woven Dacron graft was used to fashion a distal end-to-end anastomosis with a running 4-0 polypropylene suture. The patient was then placed on circulatory arrest and the cervical arch was resected. The proximal aortic arch was clamped distal to the left common carotid artery and cardiopulmonary bypass was resumed. The proximal end-to-end anastomosis was then performed with a running 4-0 polypropylene suture. The left subclavian artery was then revascularized with a 7-mm limb of a double velour-woven Dacron aorto-bifemoral graft. After rewarming, the patient was weaned from cardiopulmonary bypass. Hemostasis was obtained, and the wound was closed in layers. The patient was discharged home on postoperative day 12 with his thoracotomy incision healing well. Pathologic examination of the resected aorta showed evidence of medial degeneration and atheromatous changes.
Although the normal left-sided aortic arch is derived from persistence of the fourth branchial arch, a cervical aortic arch develops most likely from persistence of the third branchial arch [2]. Cervical aortic arches are classified into two main types: one with the descending thoracic aorta and an aberrant subclavian artery contralateral to the side of the aortic arch, and the other with ipsilateral descending thoracic aorta and a virtually normal great vessel branching pattern [3]. These rare entities usually present between the ages of 20 and 40 and have an equal sex distribution except when associated with aneurysm formation, which occurs in approximately 20% of patients, predominantly women [2]. Available pathological studies suggest an association with cystic medial degeneration of the aortic wall [2]. Recent work suggests that the cervical aortic arch is associated with deletions in chromosome 22q11 [3, 4], and hence these lesions could be included in the spectrum of defects known as catch 22. This syndrome, described by Wilson and colleagues in 1993 [5], is characterized by cardiac defects, abnormal facies, thymic hypoplasia, cleft palate, hypocalcemia, and a deletion in chromosome 22. Although a variety of concomitant lesions have been documented with cervical aortic arches, we believe that the present case describing an associated pseudocoarcation is unique in the modern literature. In 1972, Smyth and Edwards [6] suggested that pseudocoarcation, or kinking, of the aorta represented a variant of true coarctation. Pseudocoarctation has been described as being a benign entity requiring no specific therapy. In the more recent literature, however, pseudocoarctation has been associated with aneurysm formation [7] and, in the present case, aortic rupture, perhaps meriting annual surveillance of these patients.
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References
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Reid D.G. Three examples of a right aortic arch. J Anat Physiol 1914;48:174-181.[Medline]
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Pearson G.D., Kan J.S., Neill C.A., Midgley F.M., Gardner T.J., Hougen T.J. Cervical aortic arch with aneurysm formation. Am J Cardiol 1997;79:112-114.[Medline]
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Kumar A., McCombs J.L., Sapire D.W. Deletions in chromosome 22q11 region in cervical aortic arch. Am J Cardiol 1997;79:388-390.[Medline]
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Kazuma N., Murakami M., Suzuki Y., Umezu R., Murata M. Cervical aortic arch associated with 22q11.2 deletion. Pediatr Cardiol 1997;18:149-151.[Medline]
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Wilson D.I., Burn J., Scambler P., Goodship J. DiGeorge syndrome: part of CATCH 22. J Med Genet 1993;30:852-856.[Abstract/Free Full Text]
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Smyth P.J., Edwards J.E. Pseudocoarctation: kinking or buckling of the aorta. Circulation 1972;46:1027-1032.[Abstract/Free Full Text]
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Grigsby J.L., Galbraith T., Shurmur S., Deligonul U. Pseudocoarctation of the aorta complicated by saccular aneurysm: treatment by aortic arch replacement. Am Heart J 1996;131:200-202.[Medline]
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Abstract
Introduction
