Asian Cardiovasc Thorac Ann 1998;6:135-137
© 1998 Asia Publishing EXchange Pte Ltd
Aneurysm of Right Cervical Aortic Arch with Abnormal Branching Pattern
Sandeep Shrivastava, MCh,
Shipra Shrivastava, MCh,
Kurur Sankaran Neelakandhan, MCh
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Department of Cardiovascular & Thoracic Surgery Sree Chitra Tirunal Institute for Medical Sciences & Technology Thiruvananthpuram, India
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For reprint information contact: Kurur Sankaran Neelakandhan, MCh Dept. of Cardiovascular & Thoracic Surgery Sree Chitra Tirunal Institute for Medical Sciences & Technology Thiruvananthpuram 695011, India Tel: 91 471 44 3152 Fax: 91 471 44 6433 or 55 0728 Email: sctimst{at}ren.nic.in
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ABSTRACT
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We report a rare case of congenital saccular aneurysm of the right cervical aortic arch in a 16-year-old girl. There were no branches arising from the aortic arch but 3 branches arose from the ascending aorta: the left innominate artery, the right common carotid artery, and the right subclavian artery. The aneurysm was successfully repaired with a plasma-preclotted woven Dacron interposition graft during profound hypothermic cardiopulmonary bypass without total circulatory arrest.
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INTRODUCTION
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Cervical aortic arch is a rare congenital anomaly that may be associated with cardiac or other aortic anomalies including an abnormal branching pattern.1 We present an extremely rare case of aneurysm of the right cervical aortic arch where all the arch branches arose from the ascending aorta. Repair of an aortic arch aneurysm is usually performed under cardiopulmonary bypass with total circulatory arrest. In this case, it was possible to repair the aortic arch aneurysm on cardiopulmonary bypass without total circulatory arrest because there were no branches from the aortic arch.
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CASE REPORT
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A 16-year-old female presented with a pulsatile swelling at the root of the neck, which had gradually increased in size over the previous 7 years. She had a hemangioma on the left side of the face, scalp, and back, which had incompletely regressed with steroids. Examination revealed an expansile swelling of 3.5 x 3.5 cm in size. Carotid pulsations were normal. A bruit was present over the swelling in the right supraclavicular region. Hematological and biochemical investigations were within normal limits. Chest radiograph revealed widening of the superior mediastinum. Echocardiography showed a large aneurysm of the aortic arch. A digital subtraction aortogram confirmed the diagnosis of a large aneurysm of the aortic arch and revealed a right cervical aortic arch with an abnormal branching pattern, a left descending thoracic aorta, and a saccular aneurysm of the cervical arch. There were no vessels arising from the arch. The following vessels arose from the long ascending aorta: the left innominate artery; the right common carotid artery; and the right subclavian artery (Figure 1
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Figure 1. Preoperative digital subtraction angiogram showing the right cervical aortic arch aneurysm. LIA = left innominate artery, RCCA = right common carotid artery, RSCA = right subclavian artery.
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Femorofemoral bypass was established and the patient was cooled to 18°C nasal temperature. A sternotomy was performed under low flows. The anatomy was confirmed visually; there were no branches from the arch but the ascending aorta was long and all the arch branches arose from it. The aneurysm was located just distal to the origin of the right subclavian artery and extended to the beginning of the descending thoracic aorta. The pericardium was marsupialized. An additional cannula was introduced into the ascending aorta. Proximal control was obtained between the right common carotid artery and the right subclavian artery. The right subclavian artery was occluded separately. The femoral arterial cannula was clamped to prevent flooding because distal control could not be achieved. Thus, during the period of aortic clamping, circulation was maintained to the left upper extremity via the subclavian branch of the left innominate artery and to the brain via the left common carotid artery, a branch of the left innominate artery and via the right common carotid artery, a direct branch of the ascending aorta (Figure 2). The aneurysm was opened and found to be free of clots. Repair was undertaken with a no. 22 plasma-preclotted woven Dacron interposition graft. The patient's postoperative course was uneventful and a digital subtraction aortogram showed a successful repair (Figure 3). After 2 years of follow-up, the patient was asymptomatic and pursuing her studies.
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Figure 3. Postoperative digital subtraction angiogram showing the interposition graft repair. LIA = left innominate artery, RCCA = right common carotid artery, RSCA = right subclavian artery.
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DISCUSSION
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Rare types of aortic arch malformation include isolation of the right or left subclavian arteries in conjunction with a left or right aortic arch, respectively. Isolation of the innominate or carotid arteries and an aberrant left innominate artery may exist in association with a right aortic arch. Other anomalies are persistent fifth aortic arch, subclavian artery as the first branch of the aortic arch, ductus arteriosus sling, and pulmonary artery sling.2 A right aortic arch is not uncommon. However, a right cervical aortic arch is rare. It is usually associated with a right descending thoracic aorta. Aortic arch anomalies arise from failure of the normal development of the left fourth dorsal arch. A cervical aortic arch results from persistence of the third dorsal arch that reroutes the aortic arch in the cervical region.3 A right cervical aortic arch results from persistence of the ventral horn of the aortic sac, its right horn, right third dorsal arch, and the ductus caroticus.
Pulsatile neck swellings are usually related to carotid vessels and include aneurysms, pseudoaneurysms, and tortuous atherosclerotic vessels.4 Congenital extension of the aortic arch into the neck causing a pulsatile neck swelling is very rare. A cervical aortic arch can be associated with other aortic anomalies and cardiopathies.5 In this patient, the right cervical aortic arch was associated with a left-sided descending aorta. The patient had an embryological right aortic arch because of its location on the right side and a mirror-image branching pattern. However, the mirror-image branches arose from the long ascending aorta. The patient had an aneurysm of the aortic arch with an anomalous aortic arch branching pattern as an associated anomaly. There was no associated cardiac anomaly but the patient had another congenital anomaly in the form of a hemangioma of the face, scalp, and back.
Femorofemoral bypass was established to cool the patient and allow the rapid establishment of total circulatory arrest in the event of accidental dissection of the aneurysm during sternotomy. We decided not to manage this case simply by cross-clamping the aorta without cardio-pulmonary bypass because of the risk of catastrophic bleeding in the event of accidentally opening the aneurysm. The anatomy enabled us to achieve proximal control distal to the left innominate artery and the right common carotid artery. Thus, blood supply to the brain was maintained during the clamping period and we were able to avoid total circulatory arrest. In the postoperative aortogram (Figure 3
), the aorta distal to the distal anastomosis appears mildly dilated. At surgery, this section of the aorta appeared to be relatively large but healthy. Therefore, we did not consider it necessary to replace this portion.
This case is reported because of the extremely rare congenital anomaly of the aortic arch, its branching pattern, the associated aneurysm of the aortic arch, and the good surgical outcome. We are not aware of any similar case reported previously.
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REFERENCES
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Kumar S, Mandalam KR, Unni M, et al. Left cervical arch and associated abnormalities. Cardiovasc Intervent Radiol
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Moes CA, Freedom RM. Rare types of aortic arch anomalies. Pediatr Cardiol
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Moller-Hartman W, Pereira P, Salbeck R, Reinbold WD. Diagnosis of a left-sided cervical aortic arch with nuclear magnetic resonance tomography. Radiology
1994;34:331–9.
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Murray J, Negrette J. An unusual cause of a pulsatile neck swelling: report of a case of cervical aortic arch. Scott Med J
1989;34:568–9.[Medline]
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Sobrino Marquez JM, Gozalves I, Gomez A, et al. Cervical aortic arch associated with cardiopathy. Rev Esp Cardiol
1992;45:537–40.[Medline]
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ABSTRACT
INTRODUCTION
