Interrupted aortic arch with isolated persistent left superior vena cava in patient with Turners syndrome

M Obadah Kattea; Osama A Smettei; Abdulrahman Kattea; Rami M Abazid

doi:10.4103/2231-0770.191449

Avicenna Journal of Medicine, Inhaltsverzeichnis

CC BY-NC-ND 4.0 · Avicenna J Med 2016; 06(04): 117-119
DOI: 10.4103/2231-0770.191449

CASE REPORT

Interrupted aortic arch with isolated persistent left superior vena cava in patient with Turners syndrome

M Obadah Kattea

Department of Cardiology, Prince Sultan Cardiac Centre, Al-Qassim, Buraydah, Saudi Arabia

,

Osama A Smettei

Department of Cardiology; Department of Cardiac Imaging, Prince Sultan Cardiac Centre, Al-Qassim, Buraydah, Saudi Arabia

,

Abdulrahman Kattea

Department of Internal Medicine, Northwestern Air Force Hospital, Tabuk, Saudi Arabia

,

Rami M Abazid

Department of Cardiology; Department of Cardiac Imaging, Prince Sultan Cardiac Centre, Al-Qassim, Buraydah, Saudi Arabia

› Institutsangaben

Abstract

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Abstract

We present a case of 13-year-old female with Turner syndrome (TS), who presented with unexplained lower limbs swelling and ejection systolic murmur at the left second intercostal space. Suspicion of mild aortic coarctation was made by echocardiography. Computed tomography angiography (CTA) showed a complete interruption of the aortic arch (IAA) below the left subclavian artery with persistent left superior vena cava (PLSVC) and absent right SVC, defined as an isolated PLSVC. The patient underwent successful surgical correction after unsuccessful trial of transcatheter stent placement. We present this case of asymptomatic IAA to draw attention to the importance of CTA in diagnosing such rare anomalies and ruling out asymptomatic major cardiovascular abnormalities in patient with TS.

Keywords

Coarctation of aorta - interrupted aortic arch - left-sided superior vena cava - Turner syndrome

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Referenzen

References
1 Stochholm K, Juul S, Juel K, Naeraa RW, Gravholt CH. Prevalence, incidence, diagnostic delay, and mortality in Turner syndrome. J Clin Endocrinol Metab 2006;91:3897-902.
2 Messner G, Reul GJ, Flamm SD, Gregoric ID, Opfermann UT. Interrupted aortic arch in an adult single-stage extra-anatomic repair. Tex Heart Inst J 2002;29:118-21.
3 Marino B, Digilio MC, Persiani M, Di Donato R, Toscano A, Giannotti A, et al. Deletion 22q11 in patients with interrupted aortic arch. Am J Cardiol 1999;84:360-1, A9.
4 Mutlu M, Dilber E, Aslan Y, Okten A, Oztürk O. A Turner syndrome case associated with anal atresia, interrupted aortic arch and multicystic dysplastic kidney. Turk J Pediatr 2010;52:215-7.
5 Celoria GC, Patton RB. Congenital absence of the aortic arch. Am Heart J 1959;58:407-13.
6 Mishra PK. Management strategies for interrupted aortic arch with associated anomalies. Eur J Cardiothorac Surg 2009;35:569-76.
7 Brown JW, Ruzmetov M, Okada Y, Vijay P, Rodefeld MD, Turrentine MW. Outcomes in patients with interrupted aortic arch and associated anomalies: A 20-year experience. Eur J Cardiothorac Surg 2006;29:666-73.
8 Gordon EA, Person T, Kavarana M, Ikonomidis JS. Interrupted aortic arch in the adult. J Card Surg 2011;26:405-9.
9 Varghese A, Gatzoulis M, Mohiaddin RH. Images in cardiovascular medicine: Magnetic resonance angiography of a congenitally interrupted aortic arch. Circulation 2002;106:E9-10.
10 Sheikh AS, Mazhar S. Persistent left superior vena cava with absent right superior vena cava: Review of the literature and clinical implications. Echocardiography 2014;31:674-9.