Key result
Congenital complex of great vessel and ostial stenosis shows variable expression and hemodynamic impact.
Case Report (n=4)
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A developmental complex is described characterized by (1) supravalvular stenosis of the aorta and pulmonary trunk, (2) dysplasia of valves and (3) stenosis of ostia of coronary arteries and branches of the aortic arch. From the four cases described, it is evident that not all of the features of the complex need be present in a given case and, when present, some lesions are not of functional significance. The variations underly the potential for differences in the hemodynamic states of affected subjects.
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Blieden et al. (1974) conducted a case report in Developmental complex including supravalvular stenosis of the aorta and pulmonary trunk (n=4). Developmental complex of supravalvular stenosis, valvular dysplasia, and ostial stenosis was evaluated. A developmental complex of supravalvular aortic and pulmonary stenosis, valvular dysplasia, and ostial stenosis in 4 cases showed variable feature expression and hemodynamic impact.
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