background:  pericardial effusion (pe) in down syndrome (ds) patients  usually occur s   secondary to hypothyroidism,  but  we have   no  report of massive pericardial effusion in   euthyroid   down syndrome patient with  supravalvar  pulmonary stenosis . case presentation:  we  report ed   an   11-month-old  male with down syndrome   with  massive  pericardial  effusion that  had conservative ...

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.

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...

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 williams&...

Eight balloon dilatations were performed in five patients (aged from 10 to 37 months) in whom supravalvar pulmonary stenosis developed after anatomical correction for complete transposition. The ratio of the maximum diameter of the inflated balloon to the narrowest pulmonary arterial diameter varied from 1.6 to 3.3. In three patients with an initial ratio of less than or equal to 2 dilatation w...

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 ...

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...

OBJECTIVE To assess the medium term outcome in infants and children after surgical resection of supravalvar mitral stenosis with special reference to risk factors for mortality or recurrence of supravalvar mitral stenosis. No detailed follow up has been previously reported in this uncommon condition. DESIGN Prospective cross sectional clinical and echocardiographic follow up. SETTING Paedia...

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...