Ann Thorac Surg 2001;71:2030-2032
© 2001 The Society of Thoracic Surgeons
Case report
Atherosclerotic aneurysm in the circumflex retroesophageal right aortic arch
Ryuji Tominaga, MDa,
Takahiro Nishida, MDa,
Shigeki Morita, MDa,
Munetaka Masuda, MDa,
Hisataka Yasui, MDa
a Department of Cardiovascular Surgery, Kyushu University Faculty of Medicine, Fukuoka, Japan
Accepted for publication April 21, 2000.
Address reprint requests to Dr Tominaga, Department of Cardiovascular Surgery, Kyushu University Faculty of Medicine, 3-1-1, Maidashi, Higashi-ku, Fukuoka 8128582, Japan
e-mail: tomina{at}heart.med.kyushu_u.ac.jp
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Abstract
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A 63-year-old man had a distal arch aneurysm combined with a circumflex retroesophageal right aortic arch, which is a rare form of vascular ring. By using combined median sternotomy and left thoracotomy under deep hypothermia and retrograde cerebral perfusion, total arch replacement with a vascular graft was successful. We describe our surgical strategy for arch aneurysm associated with an uncommon congenital vascular malformation.
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Introduction
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A right aortic arch with left descending aorta and retroesophageal aortic segment (circumflex aorta) is an uncommon congenital vascular malformation that can cause symptoms of compression from a vascular ring [1–3]. We report a case of distal arch aneurysm in this rare vascular malformation.
A 63-year-old man with a long-standing history of hypertension was suspected to have a mediastinal tumor or thoracic aortic aneurysm because of a wide mediastinum in his chest x-ray. A lateral view of it with barium swallow showed compression of the esophagus from the posterior wall, although he had no history of airway obstruction or dysphagia. A computed tomographic (CT) scan (Fig 1) showed a distal arch aneurysm measuring 7 cm in maximal diameter with right aortic arch and left descending aorta with retroesophageal segment. Three-dimensional computed tomographic reconstruction of the thoracic aorta (Fig 2A) and aortography showed the left common carotid artery to be the first brachiocephalic vessel arising from the aorta. In addition the right common carotid artery, right subclavian artery, and the aberrant left subclavian artery, the origin of which showed aneurysmal dilatation, also branched one by one (Fig 2B). A persistent left superior vena cava also was diagnosed. No significant coronary stenosis was found by coronary angiography. Two-dimensional echocardiography also showed no cardiac anomalies.
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Fig 1. Preoperative computed tomographic scan showing a circumflex retroesophageal aortic arch, which displaces both the trachea and esophagus anteriorly from the vertebral body. Aneurysmal dilatation of the distal portion of aortic arch is also demonstrated. A small arrow indicates the left common carotid artery arising from the ascending aorta. (T = trachea; E = esophagus; A = aneurysm.)
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Fig 2. (A) Three-dimensional computed tomographic reconstruction of thoracic aorta. The left common carotid artery (LCA) is shown to be the first brachiocephalic vessel arising from the aorta (arrow). (B) Schematic drawing of operative findings. The origin of the aberrant subclavian artery (aLSA) shows mild aneurysmal dilatation. (A = aneurysm; Ao = aorta; E = esophagus; PA = pulmonary artery; RCA = right common carotid artery; RSA = right subclavian artery; T = trachea.)
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The operation was done through median sternotomy and fourth intercostal left thoracotomy with the patient in the left anterolateral position. A 10-mm sealed graft (Gelseal, Vascutek, Inchinnian, Renfrewshire, Scotland) was anastomosed end to side to the left common femoral artery for arterial perfusion. Venous cannulas were inserted into the superior and inferior vena cavas. A left ventricular venting tube was inserted from the right upper pulmonary vein. Because intraoperative two-dimensional echography showed no significant atheromatous plaque in the ascending aorta, aortic cross-clamping and administration of antegrade cardioplegia were done. A 24-mm knitted Dacron arterial prosthesis sealed with gelatin with four branches (Branched Gelseal, Vascutek Ltd, Inchinnian, Renfrewshire, Scotland) was anstomosed end to end to the ascending aorta, and subsequently the heart was reperfused from the first branch of the graft. After the rectal temperature decreased to below 18°C, circulatory arrest and retrograde cerebral perfusion with 300 to 400 mL/minute of pump flow rate were instituted from both the right and left superior vena cavas. The right jugular venous pressure was maintained between 15 and 20 mm Hg. The descending aorta was clamped, and lower body perfusion was restored. The arch vessels except the aberrant subclavian artery were anastomosed with branches of the graft one by one and subsequently reperfused. After terminating the lower perfusion from the femoral artery, the aneurysm was opened, and distal anastomosis was done at the fifth thoracic vertebra level using the open distal method with a 20-mm Gelseal graft. Severe atheromatous changes were seen in the wall of the aneurysm and descending aorta; therefore, a modified elephant trunk technique was used to ensure hemostasis at the anastomosis. The graft that was anastomosed proximally was passed through the native circumflex aorta, which measured 30 mm in inner diameter. There appeared to be no space to pass the graft in an anatomically normal place. The left subclavian artery was reconstructed using an 8-mm sealed graft. Graft-to-graft anastomosis was done after flushing debris from distal graft. Cardiac arrest time, retrograde cerebral perfusion time, and lower body ischemic time was 28, 43, and 31 minutes, respectively. The heartbeat recovered spontaneously and hemostasis was accomplished easily. The patient recovered uneventfully and was discharged 4 weeks after the operation. Two years after the operation the patient is doing well without any symptoms of esophageal or airway compression.
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Comment
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The circumflex retroesophageal aortic arch was first described by D’Cruz and associates [1], who treated patients with a left aortic arch that turned medially, crossed the midline behind the esophagus, and descended to the right of the thoracic spine. The vascular anomaly in the present patient was a mirror image of that rare malformation [4, 5].
There were two major problems in treating such a rare anomaly. One was the extent of the diseased aorta to be resected. Aneurysmal dilatation was localized at the distal arch where the orifice of the aberrant left subclavian artery arose. It was presumably possible to resect only the distal arch (hemiarch replacement) with or without a reconstruction of the left subclavian artery through the left thoracotomy with deep hypothermia and circulatory arrest. However, the predicted proximal anastomosis site of the aorta was considered to be behind the esophagus and trachea, and thus might adhere with aneurysmal wall. We thus chose a total arch replacement through a combined median sternotomy and left thoracotomy.
Another problem was choice of the route for the graft. It seemed reasonable to pass through the anatomically correct route, which runs anterior to the trachea and esophagus, and thus would never induce any undue pressure on both organs. However, there was no space between the chest wall in order to put the graft anterior of the trachea, because the trachea had remarkably shifted anteriorly. We therefore chose the posterior route. Care should be taken not to use a larger size sealed graft when it was passed posteriorly through in the native aorta to prevent tracheal obstruction and esophageal stenosis, because sealed grafts, especially knitted grafts, expand gradually late after the operation.
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References
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D’Cruz I.A., Cantez T., Namin E.P., Licata R., Hastreiter A.R. Right-sided aorta. : Part II: Right aortic arch, right descending aorta, and associated anomalies. Br Heart J 1966;28:725-739.
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Shuford W.H., Sybers R.G., Gordon I.J., Baron M.G., Carson G.C. Circumflex retroesophageal right aortic arch simulating mediastinal tumor or dissecting aneurysm. Am J Roentgenol 1986;146:491-496.[Abstract/Free Full Text]
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Blieden L.C., Schneeweiss A., Deutsch V., Neufeld H.N. Right aortic arch with left descending aorta (circumflex aorta). Roentgenographic diagnosis. Pediatr Radiol 1978;6:208-210.[Medline]
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Sissman N.J. Anomalies of the aortic arch complex. In: Adams F.H., Emmanouilides G.C., eds. Moss’ heart disease in infants, children, and adolescents, 3rd ed. Baltimore: Williams & Wilkins, 1983:199-215.
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Park S.C., Siewers R.D., Neches W.H., Lenox C.C., Zuberbuhler J.R. Left aortic arch with right descending aorta and right ligamentum arteriosum. A rare form of vascular ring. J Thorac Cardiovasc Surg 1976;71:779-784.[Abstract]
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Abstract
Introduction
