Ann Thorac Surg 2001;72:2121-2123
© 2001 The Society of Thoracic Surgeons
Case report
Surgical treatment of persistent esophageal compression by an unusual form of right aortic arch
Igor E. Konstantinov, MDa,
Francisco J. Puga, MD*a
a Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota, USA
Accepted for publication February 14, 2001.
* Address reprint requests to Dr Puga, Division of Cardiovascular Surgery, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA
e-mail: puga.francisco{at}mayo.edu
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Abstract
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A 32-year-old woman with dysphagia due to an unusual form of right aortic arch and anomalous left subclavian artery had successful repair after two previous failures. The definitive repair was accomplished by resection of the retroesophageal portion of the right aortic arch. The continuity of the aorta was established with a prosthetic graft. The operation was performed through a median sternotomy with cardiopulmonary bypass and circulatory arrest.
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Introduction
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Esophageal compression by a vascular structure is a rare cause for dysphagia. Symptomatology associated with vascular rings is more commonly respiratory in nature and is seen in infants and younger children. Anomalous left subclavian artery in combination with right aortic arch may cause esophageal compression, but it rarely causes swallowing difficulties. We encountered a patient with an unusual form of a right aortic arch accompanied by severe swallowing difficulties that did not improve after the standard surgical maneuvers.
A 32-year-old woman with dysphagia was referred to our institution because of persistent dysphagia that had progressed to the point that she was unable to swallow solid food. Two years before her referral she underwent an extensive investigation in another institution. Esophageal manometry performed on September 22, 1998, was normal. A right aortic arch with an anomalous left subclavian artery arising from the diverticulum of Kommerell was found and was thought to cause esophageal compression by an expected vascular ring (Fig 1). On April 4, 1998, the patient underwent left thoracotomy with division of a left-sided ligamentum arteriosum at her local hospital. The distal aortic arch and left pulmonary artery were mobilized so as to free the esophagus from any possible compression. Unfortunately, her symptoms did not improve. She continued to have dysphagia and a pressure sensation at the base of the neck. She underwent reevaluation by another surgical team. It was concluded that her persistent esophageal compression could be caused by the diverticulum of Kommerell. On March 19, 1999, she underwent repeat left thoracotomy at another institution. The periesophageal tissues were divided. The diverticulum of Kommerell was resected. The left subclavian artery was mobilized and anastomosed to descending aorta using an interposition graft (10-mm Hemashield; Meadox Medicals, Inc, Oakland, NJ). The surgical note indicated that the esophagus appeared to be free of any significant compression at the end of the procedure. This procedure was also ineffective in relieving her symptoms and she continued to experience severe dysphagia. The patient was referred to Mayo Clinic for further evaluation and treatment. She underwent aortography and contrast esophageal studies (Fig 2). These studies confirmed the presence of persistent esophageal compression by the right aortic arch. The course of the right aortic arch was unusual in that is crossed the midline immediately after the take-off of the right subclavian artery; therefore, the entire descending aorta was to the left of the spine (Fig 2A). The lateral projection demonstrated posterior esophageal indentation (Fig 2B). The esophageal compression appeared to be caused by the abnormally positioned right aortic arch, as it coursed to the right and behind the esophagus. The left subclavian artery originated from the previously placed Hemoshield graft and was far from the area of compression (Fig 2C).
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Fig 1. Initial anatomy of the anomaly. (LCA = left common carotid artery; LSA = left subclavian artery; RCA = right common carotid artery; RSA = right subclavian artery.) (By permission of Mayo Foundation.)
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Fig 2. Contrast swallow study demonstrating severe esophageal compression in posteroanterior (A) and lateral projections (B). (A) Aortic arch had an unusual course immediately crossing the spine to the left and descending on the left side. (B) Posterior indentation of the esophagus. (C) Angiographic study demonstrating the arch vessels branching pattern. Left subclavian artery anastomosed to descending aorta by mean of a 10-mm Hemashield graft.
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On March 24, 2000, the patient underwent a third operation. A median sternotomy approach was used. Cardiopulmonary bypass was instituted by cannulating the distal ascending aorta and right atrium. A 22-mm Hemashield graft was anastomosed to the right aspect of the ascending aorta. The heart was elevated and the descending aorta was exposed through the incision in the posterior pericardium. The Hemashield graft was brought around the right atrium, in front of the inferior vena cava and distal esophagus. It was anastomosed to the descending aorta over a side-biting clamp (Fig 3). The patient’s temperature was lowered to 18°C, the aorta was cross-clamped and cardioplegic arrest of the heart was instituted. A short period of circulatory arrest was then established (25 minutes). The aortic arch was transected just beyond the origin of the right subclavian artery. The proximal end was oversewn in two layers over Teflon felt strip (Impra, Inc, Tempe, AZ). The distal aortic arch was then dissected behind the esophagus to a point just proximal to the origin of the left subclavian artery graft and excised. The distal end was oversewn in the same fashion as previously. After rewarming, extracorporeal circulation was discontinued without difficulty. The postoperative recovery was uncomplicated. She underwent contrast study of the esophagus before hospital discharge, which showed no residual obstruction. She was discharged home on April 1, 2000. Her symptoms were markedly improved. The choking and pressure sensation during eating disappeared completely. She still had mild residual dysphagia at 1 month after operation, which, we believe, is secondary to intrinsic esophageal abnormalities due to long-standing esophageal compression.
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Fig 3. Final repair. Right aortic arch division immediately below the origin of the right subclavian artery (RSA). A 22-mm Hemashield graft was positioned from the ascending to the descending aorta. (LCA = left common carotid artery; LSA = left subclavian artery; RCA = right common carotid artery.) (By permission of Mayo Foundation).
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Comment
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Surgical management of the right aortic arch with aberrant retroesophageal left subclavian artery has been reported before [1–3]. The clinical presentation and degree of compression is dependent on the nature of the anomaly [4]. Significant esophageal compression caused by the right aortic arch with an aberrant left subclavian artery and left ligamentum arteriosum has been described by Jung and colleagues [3]. Although there was no vascular ring in their patient, the esophagus appears to be compressed due to the tension in the left subclavian artery, which was pulling the aortic arch toward the left. The division of the left subclavian artery has resulted in complete relief of symptoms. In the case described, the division of the left ligamentum arteriosum did not relieve the compression. Often patients with the right aortic arch and left ligamentum arteriosum have the diverticulum of Kommerell at the origin of the left subclavian artery [5]. The diverticulum can enlarge and form an aneurysm compressing surrounding structures. In our patient, the standard surgical maneuvers were used in an effort to relieve the compression. It was only after these procedures failed that the unusual features of this right aortic arch was appreciated. The arch crossed the midline after giving rise to the right subclavian artery, coursed behind the esophagus causing severe compression and descended entirely to the left of the spine. This unusual anatomy was in contrast to most cases of right aortic arch, where the aorta descends for some distance in the right chest before crossing the midline above the diaphragm and competing its inferior course to the left of the spine.
Excision and reposition of the aortic arch, as it was done in our patient, seemed to be an extensive procedure as it committed the distal aortic circulation to an extra-anatomically placed tubular prosthesis. Yet, given the severity of this patient’s symptomatology, it appeared justified. Exposure of the descending thoracic aorta through the posterior pericardium in performing ascending aorta-to-descending aorta bypass grafting was described by Vijayanagar and associates [6] in 1980 in an adult patient with coarctation of the aorta associated with severe aortic valve insufficiency. In this patient, the graft was placed around the left margin of the heart, traversing the pericardium and the left pleural cavity, anterior to the left pulmonary hilum, and was anastomosed proximally to the median aspect of the ascending aorta. Powell and colleagues [7] modified this technique so that the graft was routed around the right margin of the heart and anastomosed proximally to the right lateral aspect of the ascending aorta. The latter technique appeared eminently suitable for our patient, who had undergone two previous left thoracotomies and also needed the actual excision of the right aortic arch. Circulatory arrest allowed precise closure of the transected aortic ends, not easily achieved with on-going perfusion.
In summary, this case represents an unusual persistent form of esophageal compression in a patient with right aortic arch and aberrant left subclavian artery. Conventional surgical approaches failed to relieve the symptoms. Excision of the distal aortic arch and extra-anatomical aortic reconstruction through the median sternotomy was used to correct the problem.
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References
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Yap J., Hayward P.A., Lincoln C. Right aortic arch with aberrant subclavian arteries: a cause of esophageal compression. Ann Thorac Surg 1999;68:2331-2332.[Abstract/Free Full Text]
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Luetmer P.H., Miller G.M. Right aortic arch with isolation of the left subclavian artery: case report and review of the literature. Mayo Clin Proc 1990;65:407-413.[Medline]
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Jung J.Y., Almond C.H., Saab S.B., Lababidi L. Surgical repair of the right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum. J Thorac Cardiovasc Surg 1978;75:237-243.[Abstract]
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Van Son J.A., Julsrud P.R., Hagler D.J., et al. Surgical treatment of vascular rings: the Mayo Clinic experience. Mayo Clinic Proc 1993;86:1056-1063.
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Kommerell B. Verlagerung des osophagus durch eine abnorm verlaufende arteria subclavia dextra (arteria lusoria). Fortschr Geb Rontgenstr 1936;54:590-597.
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Vijayanagar R., Natarajan P., Eckstein P.F., Bognolo D.A., Toole J.C. Aortic valvular insufficiency and postductal aortic coarctation in the adult. Combined surgical management through median sternotomy: a new surgical approach. J Thorac Cardiovasc Surg 1980;79:266-268.[Abstract]
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Powell W.R., Adams P.R., Cooley D.A. Repair of coarctation of the aorta with intracardiac repair. Tex Heart Inst J 1983;10:409-413.[Medline]
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Abstract
Introduction
