Interact CardioVasc Thorac Surg 2006;5:18-19. doi:10.1510/icvts.2005.113894
© 2006 European Association of Cardio-Thoracic Surgery
Case report - Vascular thoracic |
Surgical treatment of aneurysm of right aortic arch with cervical aorta
Marina Jueza,
Juan Martínez Leóna,
Javier Habab and
Eduardo Otero Cotoa,*
a Hospital Clínico Universitario, Avda. Blasco Ibañez 17–19, 46010 Valencia, Spain
b Cardiovascular Surgery and Cardiology, Clínica Quirón, Avda. Blasco Ibañez 14, 46010 Valencia, Spain
Received 31 May 2005;
received in revised form 12 October 2005;
accepted 17 October 2005
*Corresponding author. Tel./fax: +34-963987848.
E-mail address: otero_edu{at}gva.es (E. Otero Coto).
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Abstract
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A patient with a cervical aorta and an aneurysm of a right sided aortic arch was treated without cardiopulmonary bypass by ascending to distal descending aorta bypass grafting, excision of the aneurysm and its transverse connection to the descending aorta and of the cervical segment of the aorta, and connection of the right subclavian artery to the ascending aorta. The clinical result is excellent after 9 years of follow-up.
Key Words: Aneurysm; Aorta; Aortic arch
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1. Introduction
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Cervical aorta [1] is a rare congenital anomaly due to persistence of the third branchial arch with regression of the fourth branchial arch. We report a different approach to surgical treatment of this anomaly, with long-term follow-up.
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2. Case report
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A 39-year-old woman with a pulsating mass on the neck and a history of dysphagia came to our clinic in 1996 for evaluation of a possible carotid artery aneurysm. Physical examination showed a vigorous pulsation in the right supra-clavicular space and a II/VI systolic murmur in the upper sternal area. Other physical findings were normal and no anomalies of the CATCH 22 group of defects were found. Chest X-ray showed a large mass in the right para-tracheal region and absence of the usual left aortic arch shadow. Magnetic resonance angiography showed the ascending aorta giving rise to both carotid arteries before passing into the right side of the neck, where the right subclavian artery originated; after entering again the thorax the aorta formed a right aortic arch with an aneurysm of 6.7 cm of diameter. The aorta then crossed transversally the medial line behind the esophagus to descend to the left of the spine (Fig. 1). The left subclavian artery arose from the upper part of the descending aorta.
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Fig. 1. A. The aneurysm of the right aortic arch (ANEURYSM) extended up to the thoracic outlet, and connected with the descending aorta (DESC.AO) through a retro-esophageal transverse connection (TRASV.AO). B and C. Both carotid arteries (CAROTID ART) originated from a common trunk arising on the left side of the ascending aorta (ASC.AO). The cervical segment (CERV.AO) is placed clearly above the upper end of the sternum (STERN), and its relationship to the ascending aorta and the aneurysm of the right aortic arch (ANEURYSM) is shown.
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The operation was performed on June 14, 1996, through a median sternotomy extended into the lower right neck. Anatomical examination confirmed the findings of angiography. Attempted dissection and control of the proximal descending aorta was unsuccessful, but incision of the diaphragm allowed easy access to the distal descending aorta, where an end-to-lateral anastomosis of a 26-mm wowen-dacron graft was performed under lateral clamping. The graft was then passed beneath and to the right of the heart and anastomosed end-to-side to the right side of the ascending aorta. The diaphragm was closed, the right subclavian artery was connected to the ascending aorta with an 8-mm knitted-dacron graft and the ascending aorta distal to the origin of the carotid arteries was sectioned and suture-closed. The aneurysm was still under tension and attempts to dissect and ligate the transverse connection with the descending aorta were unsuccessful; a lateral 5th space limited left thoracotomy, allowed easy dissection of the descending aorta and control of the transverse connection with the arch, that was suture-closed and sectioned. Finally, the aneurysm was opened and its wall excised with the remnants of the transverse connection. No cardiopulmonary bypass was used; platelet-rich plasma was obtained after anesthesia and used to cover all the anastomoses; all recovered blood was washed and re-infused.
The patient followed an uncomplicated course and was discharged 6 days after operation. Nine years after the operation she is asymptomatic, without evidences of repair dysfunction (Fig. 2).
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Fig. 2. A. Postoperative angio-CAT scan. A frontal projection shows the vertical segment of the graft (GRAFT) interposed between ascending aorta (AO) and distal descending aorta. B. A transverse cut shows the transverse, diaphragmatic, segment of the graft (GRAFT) going from behind the sternum to the descending aorta (AO). C. A sagital projection shows the anastomosis of the graft (GRAFT) to the distal descending aorta (AO). D. 3-D reconstruction of the angio-CAT scan shows the course of the graft in relation to the heart and the ascending and descending aorta.
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3. Discussion
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Cervical aorta is a rare congenital anomaly, possibly part of the CATCH 22 group of defects, characterized by a deletion in chromosome 22q11 region [2], that can be confused with an aneurysm of the supra-aortic vessels and has received very little surgical attention. A right aortic arch is frequently associated and aneurysms of the arch are frequent with both a right or left aortic arch.
Successful surgical treatment of this condition with a right aortic arch was reported by Cooley et al. [3]. Other authors have reported surgical [4,5] and endovascular [6] correction of aneurysms of a right aortic arch without cervical aorta, and of vascular rings including a right cervical aortic arch without aneurysm [2,7]. Reports of misdiagnoses that prompted inadequate therapeutic attempts resulting in death of the patient have also been published. There are also reports of repair in cases with a left aortic arch.
Cooley et al. [3] performed a right-sided aortic arch graft replacement and a bifurcated graft to the right carotid and subclavian arteries through a median sternotomy. This technique leaves in place the transverse segment, following a retro-esophageal course, a relationship that was the probable cause of dysphagia in our patient. For this reason, and to avoid possible new vascular complications at this level, we believe that patients with this anomaly are better served by resection of the transverse segment in addition to resection of the aneurysm and aortic reconstruction.
Anatomical repair (with a graft in the right arch configuration) may produce esophageal compression and direct damage to the esophagus. Conventional repair with a left sided prosthetic arch graft may be inadequate in cases like ours because of the origin of the carotid arteries on the left-side of the aorta and the rightwards position of the distal ascending aorta, which could have produced compression of a graft placed in the left arch location. Ascending aorta to distal descending aorta bypass grafting [8] has been used for treatment of different conditions with good results, and was easily applied in our patient; the possible risk of an aorto-esophageal fistula with this approach has been mentioned in the literature [9], but direct contact between both structures is avoided by making the anastomosis above the diaphragmatic crura, and covering it with pericardium.
Disconnection of the descending aorta from the transverse segment required an associated limited lateral thoracotomy in our patient, which seems a significant drawback (although we preferred this option to going on bypass after extensive dissection). Cardiopulmonary bypass and hypothermic arrest could allow resection of the aneurysm and direct aorto-aortic grafting, but leaving in place the transversal retro-esophageal aortic segment may predispose to other complications, as pointed out above. Direct excision of the transverse segment through the sternotomy may be possible in smaller aneurysms or with a different anatomy.
Full early recovery and an excellent result after 9 years of follow-up may justify consideration of ascending aorta to distal descending aorta bypass grafting for similar cases. Other technical aspects should probably be individualized for each patient.
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References
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Abstract
1. Introduction