Supravalvar aortic stenosis syndrome with stenosis of all aortic arch branches.
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چکیده
منابع مشابه
Supravalvar aortic stenosis syndrome with stenosis of all aortic arch branches.
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.
متن کاملSUPRAVALVAR 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...
متن کاملSupravalvar aortic stenosis.
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...
متن کاملCardiac outcomes in adults with supravalvar aortic stenosis.
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...
متن کاملThe natural course of supravalvar aortic stenosis and peripheral pulmonary artery stenosis in Williams's syndrome.
The haemodynamic findings from two serial cardiac catheterisations in ten patients with Williams's syndrome were reviewed. The median ages at study were one and nine years. Raised mean (SD) left ventricular peak systolic pressures associated with supravalvar aortic stenosis increased significantly with time from 121 (25) mm Hg to 153 (29) mm Hg. The peak systolic pressure drop between the left ...
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ژورنال
عنوان ژورنال: Thorax
سال: 1975
ISSN: 0040-6376
DOI: 10.1136/thx.30.4.481