Name : Hamid Reza
Family :Pour Aliakbar
Affiliation : Radiology Department,ShahidRajaeeHospital,TUMS
Academic Degree: Assistant Professor of Radiology
Resident : Seyed Kamaleddin Hadei
Gender : Male
Age : 34
A 34 year- old man with acute chest pain
CT angiography of the aorta: Multiple axial,coronal and sagittal images with MIP and MPR reconstruction show intimal flap from aortic root up to aortic arch infavor of dissection type A and aneurysmal dilatation of the aortic root and the ascending aorta . Dilatation of the aortic annulus with 33mm in size (normal range 23-27mm) is noted. The ascending aorta measured 67mm maximally. The aortic arch and descending aorta appear normal. Pectus excavatum with depression and angulation of sternum, kyphoscoliosis and dural ectasia due to Marfan syndrome are seen.
Aneurysme and dissection of the ascending aorta in Marfan syndrome
Discussion (Related Text)
Thoracic aortic aneurysms are best classified by the portion of the aorta involved-that is, the ascending, arch, or descending thoracic aorta. This anatomic distinction is important because it allows an etiologic classification scheme. those involving the ascending aorta include cystic medial necrosis, Marfan syndrome, Ehlers-Danlos syndrome and syphilis . Aneurysms of the arch itself are more often atherosclerotic, as are descending thoracic aortic aneurysms. In general, the ascending and transverse aorta are considered aneurysmal when their diameter exceeds 4 cm, and the descending aorta is considered aneurysmal when its diameter exceeds 3.5 cm. Marfan syndrome is an autosomal dominant disorder; recent studies in molecular genetics have identified the fibrillin gene product as the responsible defective connective tissue protein. Marfan syndrome affects approximately 1 per 10,000 individuals throughout the world, including both genders and all races and ethnic groups. The effects are widespread, including the eyes, skeleton, heart, and aorta, where >50% have cardiovascular complications. There is weakening of the aortic root, producing aortic ectasia and aortic insufficiency, making the patient prone to aortic dissection. Dissection or LVfailure causes death in one third of patients by 32 years of age and in two thirds by age 50 if untreated. The classic aortogram appearance is that of a very large aneurysmal aortic root with sinotubularectasia (the "tulip bulb" appearance).Whenpresent, aortic dissection involves the ascending aorta with or without extension into the descending. Annuloaorticectasia may be seen in Marfan syndrome ,forme-frusts Marfan ,Ehlers-Danlos syndrome and osteogenesis imperfecta.
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