Marfan syndrome with ascending aortic aneurysm and Stanford A, DeBakey I aortic dissection

Case contributed by Dr Mohammad A. ElBeialy

Presentation

25 years old tall man with arachnodactyly and marfanoid features is presenting with severe acute chest pain.

Patient Data

Age: 25 years
Gender: Male
  • Aneurysmal dilatation of the aortic annulus and ascending aorta is seen measuring upto 6.5 X 6.2 cm.  with an intimal dissection flap is seen involving the ascending aorta just above the aortic root and the LVOT  and spiraling across the entire ascending aorta, aortic arch and the descending aorta down to the right and left common iliac and common femporal  arteiries. No gross abnormality related to the coronary arteies.
  • No evidence of periaortic leakage or collections.
  • Normal celiac, mesenteric and renal arteries which arise from the true lumen.
  • Mild cardiomegaly is noted with mild dilatation of the left atrium as well mild dilatation and concenteric hypertrophy of the left ventricle. Evidence of prostheric mitral valve.
  • No pericardial effusion.
  • No mediastinal masses.
  • Mild left dorsolumbar scolitoic list. 
  • Posterior vertebral scalloping of the lumbo-sacral spine (indicative of dural ectasia).

Case Discussion

A case of Marfan's syndrome with aneurysmal diltation of  aortic annulus and ascending aorta as well as Stanford A, DeBakey I aortic dissection.

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Case information

rID: 34752
Case created: 7th Mar 2015
Last edited: 11th Jul 2016
System: Vascular
Inclusion in quiz mode: Included

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