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Isolated interrupted aortic arch in an adult male
Isolated interrupted aortic arch in an adult male Francisco Javier Garcipérez de Vargas, MD, Gonzalo Marcos, MD, María Victoria Mogollón, MD, and Jose Javier Gomez-Barrado, MD, Cáceres, Spain
A 38-year-old male was referred to our hospital for evaluation of hypertension that was diagnosed 1 year ago. The patient did not have any symptoms. On physical examination, bilateral femoral and popliteal pulses were weak, and there was an ejection systolic murmur on the left second intercostal area. Chest radiograph showed rib notching with cardiomegaly (A). Transthoracic echocardiography showed concentric left ventricular hypertrophy, and the ascending aorta was dilated. The descending aorta appeared to terminate 2 to 3 cm after the left subclavian artery in suprasternal echocardiographic view. Aortography showed a complete interruption of the aortic arch just distal to the origin of the left subclavian artery and the absence of patent ductus arteriosus. Magnetic resonance angiography confirmed the diagnosis of isolated interrupted aortic arch (IAA) with extensive collaterals (B and C). An extra-anatomic bypass procedure in a single-stage repair through a median sternotomy extended into an upper midline laparotomy was performed by placing a graft-assisted anastomosis between the ascending aorta and the supraceliac abdominal aorta. The postoperative course was uneventful. IAA is a rare congenital anomaly with an incidence of three per million live births. In most cases, IAA is associated with other congenital cardiac anomalies. The classification made by Celoria and Patton in 1959 is the most widely used,1 based on the position of interruption that may be distal to the left subclavian artery (type A), between the left common carotid and left subclavian arteries (type B), and between the innominate and left common carotid arteries (type C). IAA is associated with a mortality rate of more than 90% at 1 year of age if untreated.2 In adults, a substantial collateral circulation must be present to maintain flow and enable survival. It is also thought that, in some cases, the initial affection would be a coarctation of the aorta, which evolves to the progressive closing of the lumen. Surgery is the definitive treatment of choice. Extra-anatomic bypass procedures are the most frequently preferred method of reconstruction in adult patients with IAAs. Most reported cases in adults have been reported in a single-stage repair.3 REFERENCES 1. Celoria GC, Patton RB. Congenital absence of the aortic arch. Am Heart J 1959;58:407-13. 2. Collins-Nakai RL, Dick M, Parisi-Buckley L, Fyler DC, Castaneda AR. Interrupted aortic arch in infancy. J Pediatr 1976;88:959-62. 3. Sai Krishna C, Bhan A, Sharma S, Kiran U, Venugopal P. Interruption of aortic arch in adults: surgical experience with extra-anatomic bypass. Tex Heart Inst J 2005;32:147-50. Submitted Aug 18, 2011; accepted Apr 10, 2012. From the Department of Cardiology, Hospital San Pedro de Alcántara. Author conflict of interest: none. E-mail: [email protected]. The editors and reviewers of this article have no relevant financial relationships to disclose per the JVS policy that requires reviewers to decline review of any manuscript for which they may have a conflict of interest. J Vasc Surg 2013;58:1399 0741-5214/$36.00 Copyright © 2013 by the Society for Vascular Surgery. http://dx.doi.org/10.1016/j.jvs.2012.04.047
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A 38-year-old male was referred to our hospital for evaluation of hypertension that was diagnosed 1 year ago. The patient did not have any symptoms. On physical examination, bilateral femoral and popliteal pulses were weak, and there was an ejection systolic murmur on the left second intercostal area. Chest radiograph showed rib notching with cardiomegaly (A). Transthoracic echocardiography showed concentric left ventricular hypertrophy, and the ascending aorta was dilated. The descending aorta appeared to terminate 2 to 3 cm after the left subclavian artery in suprasternal echocardiographic view. Aortography showed a complete interruption of the aortic arch just distal to the origin of the left subclavian artery and the absence of patent ductus arteriosus. Magnetic resonance angiography confirmed the diagnosis of isolated interrupted aortic arch (IAA) with extensive collaterals (B and C). An extra-anatomic bypass procedure in a single-stage repair through a median sternotomy extended into an upper midline laparotomy was performed by placing a graft-assisted anastomosis between the ascending aorta and the supraceliac abdominal aorta. The postoperative course was uneventful. IAA is a rare congenital anomaly with an incidence of three per million live births. In most cases, IAA is associated with other congenital cardiac anomalies. The classification made by Celoria and Patton in 1959 is the most widely used,1 based on the position of interruption that may be distal to the left subclavian artery (type A), between the left common carotid and left subclavian arteries (type B), and between the innominate and left common carotid arteries (type C). IAA is associated with a mortality rate of more than 90% at 1 year of age if untreated.2 In adults, a substantial collateral circulation must be present to maintain flow and enable survival. It is also thought that, in some cases, the initial affection would be a coarctation of the aorta, which evolves to the progressive closing of the lumen. Surgery is the definitive treatment of choice. Extra-anatomic bypass procedures are the most frequently preferred method of reconstruction in adult patients with IAAs. Most reported cases in adults have been reported in a single-stage repair.3 REFERENCES 1. Celoria GC, Patton RB. Congenital absence of the aortic arch. Am Heart J 1959;58:407-13. 2. Collins-Nakai RL, Dick M, Parisi-Buckley L, Fyler DC, Castaneda AR. Interrupted aortic arch in infancy. J Pediatr 1976;88:959-62. 3. Sai Krishna C, Bhan A, Sharma S, Kiran U, Venugopal P. Interruption of aortic arch in adults: surgical experience with extra-anatomic bypass. Tex Heart Inst J 2005;32:147-50. Submitted Aug 18, 2011; accepted Apr 10, 2012. From the Department of Cardiology, Hospital San Pedro de Alcántara. Author conflict of interest: none. E-mail: [email protected]. The editors and reviewers of this article have no relevant financial relationships to disclose per the JVS policy that requires reviewers to decline review of any manuscript for which they may have a conflict of interest. J Vasc Surg 2013;58:1399 0741-5214/$36.00 Copyright © 2013 by the Society for Vascular Surgery. http://dx.doi.org/10.1016/j.jvs.2012.04.047
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