Supravalvar aortic stenosis syndrome with stenosis of all aortic arch branches.
نویسندگان
چکیده
A 9-year-old girl with the supravalvar aortic stenosis syndrome is presented. In addition to the classical features of the syndrome, namely supravalvar aortic stenosis, peripheral pulmonary stenosis, typical facies, and mental retardation, she was also found to have stenosis at the origin of all aortic arch main branches, a hitherto undescribed feature.
منابع مشابه
SUPRAVALVAR AORTIC STENOSIS IN CHILDREN REPORT OF THIRTEEN CASES
During 15 years from 1975 to 1990, thirteen cases of supravalvar aortic stenosis were admitted at the pediatrics department of Shahid Rajai Heart Hospital, Tehran. All patients were subjected to cardiac catheterization and a angiocardiography. Patients ranged in age from 3.5 years to 14 years with a mean of 8.7 years. Seventy seven percent of childen were male. Eight cases (61.5%) had Wil...
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AIMS Supravalvar aortic stenosis is a rare form of left ventricular outflow tract obstruction that is often progressive in childhood. Little data are available on outcomes in the adult population. Our aim was to define cardiac outcomes in adults with supravalvar aortic stenosis. METHODS AND RESULTS This is a multicentre retrospective study of cardiac outcomes in adults (≥18 years) with suprav...
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one manifestation of the idiopathic hypercalcaemia of infancy syndrome, and the possible aetiologies are discussed. Another presentation of supravalvar aortic stenosis is in the so-called familialform, but there may exist considerable confusion between these two forms, as less than 50 per cent of those without physical stigmata have a positive family history, and the severeform of the idiopathi...
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RECENT REPORTS have indicated that idiopathic infantile hypercalcemia, a disease related to deranged vitamin D metabolism, -4 may be a feature of the nonfamilial, congenital supravalvar aortic stenosis syndrome.5 8 Other manifestations of this syndrome include mental retardation, a peculiar "elfin" facies, narrowing of peripheral pulmonary and systemic arteries, strabismus, inguinal herniae, an...
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ورودعنوان ژورنال:
- Thorax
دوره 30 4 شماره
صفحات -
تاریخ انتشار 1975