Key result
Williams syndrome features progressive supravalvar aortic stenosis alongside improving peripheral pulmonary stenosis over time.
Observational (n=10)
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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 ventricle and aorta (measured in seven patients) increased significantly from 25 (25) mm Hg to 50 (35) mm Hg. Raised right ventricular peak systolic pressure associated with peripheral pulmonary artery stenosis (in eight patients) decreased significantly with time from 52 (26) mm Hg to 28 (6) mm Hg. Supravalvar aortic stenosis in Williams's syndrome seems to progress rapidly, and careful monitoring is indicated. There is a strong likelihood that peripheral pulmonary artery stenosis in Williams's syndrome will improve spontaneously with time.
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Giddins et al. (1989) conducted an observational in Williams's syndrome with supravalvar aortic stenosis and peripheral pulmonary artery stenosis (n=10). Natural course of supravalvar aortic stenosis and peripheral pulmonary artery stenosis vs. Baseline cardiac catheterisation at median age 1 year was evaluated on Left ventricular and right ventricular peak systolic pressures and left ventricle-to-aorta peak systolic pressure drop. Williams's syndrome showed worsening supravalvar aortic stenosis (LV pressure 121 to 153 mm Hg) and improving peripheral pulmonary stenosis (RV pressure 52 to 28 mm Hg) over time.
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