Natural course of supravalvar aortic stenosis and peripheral pulmonary arterial stenosis in Williams' syndrome.

Full Abstract

We investigated the catheterization and angiographic findings of 26 patients with Williams' syndrome to evaluate the natural course of supravalvar aortic stenosis and peripheral pulmonary arterial stenosis. The severity of the stenosis was correlated with age and body surface area in terms of the pulmonary arterial index, right ventricular systolic pressure, sinutubular ratio (ratio of measured to mean normal diameter of sinutubular junction), and systolic pressure gradient across the sinutubular junction. In patients with pulmonary arterial stenosis (n=20), right ventricular systolic pressure tended to decrease, and pulmonary arterial index increased, with increase in age and body surface area. Between the groups with and without pulmonary arterial stenosis, there was significant difference in age (mean 4.70 vs. 9.87, p=0.019), body surface area (0.62 vs. 1.16, p=0.002), pulmonary arterial index (152 vs. 317, p=0.002) and right ventricular systolic pressure (73.9 vs. 33.0, p=0.006). As all patients showed similar diameters at the sinutubular junction regardless of age and body size, sinutubular ratio decreased with increases in age and body surface area. The group with abnormal coronary arteries (n=7) had smaller sinutubular ratio (0.46 vs. 0.61, p=0.021) and higher pressure gradients between the left ventricle and the aorta (67.6 vs. 42.2, p=0.023) than did the group with normal coronary arteries. Stenosis of a coronary artery, or a branch of the aortic arch, was observed only in three patients with diffuse aortic stenosis. Our results suggest that, with time, peripheral pulmonary arterial stenosis tends to improve, and supravalvar aortic stenosis to progress. Failure of growth of the sinutubular junction might be responsible for the progression of the aortic lesion. Progression of the aortic lesion may be associated with involvement of the coronary arteries.

Author information

Author/s: Kim, Y M (YM); Yoo, S J (SJ); Choi, J Y (JY); Kim, S H (SH); Bae, E J (EJ); Lee, Y T (YT);

Affiliation: Department of Radiology, Sejong Heart Institute, Korea.

Journal and publication information

Publication Type: Comparative Study; Journal Article

Journal: Cardiology in the young (Cardiol Young), published in ENGLAND. (Language: eng)

Reference: 1999-Jan; vol 9 (issue 1) : pp 37-41

Dates: Created 1999/07/01; Completed 1999/07/01; Revised 2006/11/15;

PMID: 10323536, status: MEDLINE (last retrieval date: 2/18/2009, IMS Date: )

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