caa a22 4500 388115874 CHVBK 20180307125406.0 cr unu---uuuuu 161130e199901 xx s 000 0 eng 10.1017/S1047951100007356 doi S1047951100007356 pii (NATIONALLICENCE)cambridge-10.1017/S1047951100007356 Natural course of supravalvar aortic stenosis and peripheral pulmonary arterial stenosis in Williams‘ syndrome [Elektronische Daten] 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. Copyright © Cambridge University Press 1999 Williams‘ syndrome nationallicence supravalvar aortic stenosis nationallicence peripheral pulmonary arterial stenosis nationallicence angiography nationallicence Kim Yang Min Departments of Radiology Sejong Heart Institute, Kyunggi-do Yoo Shi-Joon Departments of Radiology Sejong Heart Institute, Kyunggi-do Choi Jung Yun Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea Kim Seong Ho Departments of Pediatrics Sejong Heart Institute, Kyunggi-do Bae Eun Jung Departments of Pediatrics Sejong Heart Institute, Kyunggi-do Lee Young Tak Departments of Thoracic and Cardiovascular Sugery Sejong Heart Institute, Kyunggi-do Cardiology in the Young Cambridge University Press 9/1(1999-01), 37-41 1047-9511 9:1<37 1999 9 CTY https://doi.org/10.1017/S1047951100007356 text/html Onlinezugriff via DOI 1 other jats NATIONALLICENCE 856 40 https://doi.org/10.1017/S1047951100007356 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Kim Yang Min Departments of Radiology Sejong Heart Institute, Kyunggi-do NATIONALLICENCE 700 1- Yoo Shi-Joon Departments of Radiology Sejong Heart Institute, Kyunggi-do NATIONALLICENCE 700 1- Choi Jung Yun Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea NATIONALLICENCE 700 1- Kim Seong Ho Departments of Pediatrics Sejong Heart Institute, Kyunggi-do NATIONALLICENCE 700 1- Bae Eun Jung Departments of Pediatrics Sejong Heart Institute, Kyunggi-do NATIONALLICENCE 700 1- Lee Young Tak Departments of Thoracic and Cardiovascular Sugery Sejong Heart Institute, Kyunggi-do NATIONALLICENCE 773 0- Cardiology in the Young Cambridge University Press 9/1(1999-01), 37-41 1047-9511 9:1<37 1999 9 CTY CC0 http://creativecommons.org/publicdomain/zero/1.0 nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-cambridge