Marfan syndrome with ascending aortic aneurysm and Stanford A, DeBakey I aortic dissection
25 years old tall man with arachnodactyly and marfanoid features is presenting with severe acute chest pain.
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- 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).
- 1. Budde RP, Kluin J, van Herwerden LA et-al. Comprehensive dynamic aortic and cardiac analysis by 256-slice computed tomography in type A dissection. Ann. Thorac. Surg. 2011;92 (3): e47-8. doi:10.1016/j.athoracsur.2011.03.126 - Pubmed citation
- 2. Scholte AJ, Hilhorst-Hofstee Y, Versteegh MI et-al. Rapid aortic aneurysm formation in Marfan patient with dissection of the entire aorta. Eur Heart J Cardiovasc Imaging. 2013;14 (5): 507. doi:10.1093/ehjci/jes289 - Pubmed citation