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- W2053253014 abstract "B ecause bicuspid aortic valve (BAV) and coarctation of the aorta (COA) frequently coexist,1-3 it is reasonable to believe that these malformations result from a single developmental diathesis. In 1972 McKusick4 added a third component. He suggested that manifestations of an abnormal aortic media are so frequently encountered in patients with COA and with BAV as to allow postulation of a common underlying defect. In his clinical note he said ‘I... BAV may be an expression of a developmental defect of the arterial tree which is also expressed in COA in some, in cystic medial necrosis in others, or in various combinations of these three.‘14 If McKusick’s suggestion is true, aortic aneurysm, rupture and dissection join valvular aortic stenosis and infective endocarditis as potential hazards for patients with BAV and continue to threaten patients with COA even after successful surgical repair. Numerous investigators have described one or another of these combinations, yet their clinical significance does not seem to be widely appreciated. This essay will examine the information that supports McKusick’s concept. “Cystic medial necrosis,” McKusick’s marker of an intrinsically abnormal aortic media, is commonly identified in patients with COAn5 This finding may not, however, be a definite marker of intrinsic weakness, for this histologic pattern is seen in older patients with no gross abnormality of the aortas.7 and may be absent in other patients with clear evidence of intrinsic fragility of the aortic wal1.8vg Schlatmann and Becker778 suggested that it is a nonspecific consequence of injury to the aortic wall resulting from hydraulic stress and subsequent repair. The wall stress may, in this construct, result either from externally generated forces (e.g., hypertension] or from dilatation attendant on intrinsic" @default.
- W2053253014 created "2016-06-24" @default.
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- W2053253014 date "1988-01-01" @default.
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- W2053253014 title "Coarctation of the aorta, biscuspid aortic valve and abnormal ascending aortic wall" @default.
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- W2053253014 doi "https://doi.org/10.1016/0002-9149(88)91327-6" @default.
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