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A Rare Congenital Anomaly: Interrupted Aortic Arch and Giant Ascending Aortic Aneurysm in a Young Male

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(1)Images in Cardiovascular Medicine http://dx.doi.org/10.4070/kcj.2013.43.8.578 Print ISSN 1738-5520 • On-line ISSN 1738-5555. Korean Circulation Journal. A Rare Congenital Anomaly: Interrupted Aortic Arch and Giant Ascending Aortic Aneurysm in a Young Male Muhammed Hakan Tas, MD, Ziya Simsek, MD, Husnu Degirmenci, MD, Hayri Ogul, MD, Ugur Aksu, MD, and Abdulmecit Kantarci, MD Department of Cardiology, Ataturk University, Turkey. Interrupted aortic arch (IAA) is a rare congenital cardiac defect defined as complete loss of luminal and anatomical continuity between the ascending and descending segments of the aorta. Very few patients can reach adult age without surgical correction. These cases showed diagnosis of the nearly asymptomatic Type A IAA with aortography and multislice computed tomography (MSCT). A 27- year-old male patient was applied to the internal medicine department with complaint of fatigue. They heard a diastolic murmur on the aortic focus and referred the patient to our cardiology polyclinic. Physical examination showed reguler heart beat with blood pressures of 150/90 mm Hg in the right arm, 140/90 mm Hg in the left arm, 100/60 mm Hg in the right limb and 100/60 mm Hg in the left limb, and there was a diastolic murmur prominently on the aortic fo-cus. Electrocardiogram was in sinus rhytm and normal. His chest ro-entgenogram (anteroposterior) showed a dilated aortic root. When we made echocardiography, we saw bicuspit aortic valve with. moderate aortic regurgitation and dilatation of the ascending aorta (70 mm). Then an attemp to perform aortography via femoral artery fa-iled because the aortic arch was not able to go through. When we gave contrast, we saw that the aortic arch was interrupted. Aortography via the right brachial artery showed a giant ascending aortic aneurysm (70 mm), severe aortic regurgitation, interruption of the aortic arch distal to the left subclavian artery (Fig. 1, arrow) and prominent collateral circulation (Fig. 2, arrows). After aortography, the patient underwent a MSCT. MSCT confirmed the diagnosis of the IAA distal to the orgin of the left subclavian artery and measured the ascending aorta as 75 mm (Fig. 3, arrow). The patient was referred to an experienced surgeon on adult congenital heart diseases, but was denied surgery due to lack of symptoms. In this case, a rare congenital anomaly was detected with transcatheter aortography and MSCT diagnostic techniques, which complement each other.. Received: January 7, 2013 / Revision Received: February 26, 2013 / Accepted: April 1, 2013 Correspondence: Muhammed Hakan Tas, MD, Department of Cardiology, Ataturk University, Turkey Tel: 904422318481, Fax: 904422318481, E-mail: [email protected] • The authors have no financial conflicts of interest. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/ by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.. 578. Copyright © 2013 The Korean Society of Cardiology.

(2) Muhammed Hakan Tas, et al. 579. Fig. 1.. A  . B  . Fig. 2.. A  . B  . C  . Fig. 3. www.e-kcj.org. http://dx.doi.org/10.4070/kcj.2013.43.8.578.

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