Telescopic aortic arch a new entity in marfan syndrome

Teleskopik arkus aorta, Marfan sendromunda aortik diseksiyon sonu- cu oluşabilen ve daha önceden rapor edilmemiş bir antitedir. Burada bir adli otopside tespit edilen ve kronik aort diseksiyonuna ikincil ilk teleskopik arkus aorta olgusu bildirilmiştir. 20 yaşında bir erkekte as- sendan aorta rüptüründen kaynaklanan kalp tamponadı sonucu ani ölüm olgusu sunulmuştur. Otopside, serbest ve pıhtılı kandan oluşan 800 cclik bir hemoperikardium olduğu dikkati çekmiştir. Assendan torasik aortanın dilate olduğu ve ön duvar mediailinde 1,2 cmlik tam kat duvar rüptürü olduğu tespit edilmiştir. Aort muayenesinde, arkus aorta içerisinde bir tüp gibi yerleşmiş ikinci bir arkus aorta olduğu dikkati çekmiştir. İçteki bu ikinci arkın aort kapaklarının 2,5 cm üzeri- nden başladığı ve desseden aorta başlangıcında sonlandığı; ayrıca 10 cm uzunluğunda ve 2 cm çapında olup trunkus brakiosefalikus, sol karotis kommonis ve sol subklavian arterle ilişkili olduğu saptanmıştır.

Teleskopik arkus aorta: Marfan sendromunda yeni bir antite

Telescopic aortic arch is a consequence of aortic dissection in Marfan s syndrome, which has not been reported previously. This paper pres- ents the first case of telescopic aortic arch secondary to chronic aortic dissection, as an incidental finding of a forensic autopsy. Here we present a sudden death of a 20 years old male due to cardiac tampon- ade sourcing from the rupture of ascending aorta. At autopsy, there was a haemopericardium of 800 cc, which comprised blood and clot. The ascending thoracic aorta was dilated and a 1.2 cm ragged linear complete rupture was noticed on the medial side of the front wall of ascending aorta. Examination of aorta showed a second/inner aortic arch just as a tube lying inside the aortic arch. The inner aortic arch was arrised 2.5 cm above aortic valves and lasted at the beginning of the descending aorta. It was 10 cm in length with a lumen 2 cm in diameter and was associated with truncus brachiosephalicus, left common carotid, and left subclavian arteries.

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  • 1. Aoyagi S, Akashi H, Fujino T, et al. Spontaneous rup- ture of the ascending aorta. Eur J Cardiothorac Surg 1991;5:660-2.
  • 2. Columbia University Collage of Physicians and Surgeons. Thoracic aortic aneurysms. Available at URL: http:// www.slrctsurgery.com/Thoracic%20aortic%20 aneurysms. htm Accessed on: 17.07.2009
  • 3. Klintschar M, Bilkenroth U, Arslan-Kirchner M, Schmidtke J, Stiller D. Marfan syndrome: clinical consequences re- sulting from a medicolegal autopsy of a case of sudden death due to aortic rupture. Int J Legal Med 2009;123:55–8.
  • 4.Hudge DP, Dietz HC. Marfan’s syndrome. Lancet 2005:3; 366: 1965–76.
  • 5. Elefteriades JA. Thoracic Aortic Aneurysm: Reading the Enemy’s Playbook. Yale J Biol Med 2008;81:175-86.
  • 6.Frydman M. The Marfan Syndrome. IMAJ 2008;10:175–8.
  • 7.Gott VL, Cameron DE, Alejo DE, et al. Aortic root replace- ment in 271 Marfan patients: a 24-year experience. Ann Thorac Surg 2002;73:438–43.
European Journal of General Medicine-Cover
  • Başlangıç: 2015
  • Yayıncı: Sağlık Bilimleri Araştırmaları Derneği
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