GP37 Echocardiographic follow-up of children with supravalvular aortic stenosis

Abstract

Introduction Supravalvular aortic stenosis (SVAS) is the congenital stenosis of the ascending aorta, which may be discrete or diffuse. It can be seen in children with no other underlying disease, especially in syndromic cases such as Williams syndrome. Supravalvular aortic stenosis is also seen as an autosomal dominant type of familial and sporadic cases. In this study, long-term and postoperative follow-up results of patients who were followed up between 1990 and 2017 were presented. Materials and methods The study included 31 patients who were evaluated and followed with echocardiography at our institution between 1990 and 2017 with SVAS. Results Twelve of the cases were female (39%). Twenty patients were diagnosed with Williams syndrome. Thirty patients remained after exclusion of one case who was lost during cardiac catheterization, were followed for an average of 6.7 ± 5.8 years. The severity of SVAS detected in the first echocardiographic examination was very mild in 6 cases (19%), mild in 8 cases (26%), moderate in 5 cases (16%) and severe in 12 cases (39%). Six patients with severe SVAS were operated after the first echocardiographic examination. Right ventricular outflow tract stenosis was detected in the first examination in 6 cases and appeared in the third year of follow-up in one case. The mean right ventricular outflow tract obstruction gradient was 46.4 ± 18.5 mmHg on Doppler examination. According to the recent echocardiographic examination, the distribution of the degree of SVAS; Six patients (19.3%) were very mild, 5 (16%) were mild, 6 (19%) were moderate, and 12 (38.7%) were severe. SVAS progressed from very mild to mild in one patient, mild to moderate in 3 patients and moderate to severe in 3 patients. On the other hand, in 2 cases with severe SVAS were decreased during follow-up. Right ventricular outflow tract stenosis was lost in four cases and there was no change in three cases. Peripheral pulmonary stenosis (PPS) was present in 14 patients and PPS progressed in 2 patients, remained same in 7 cases and regressed in 5 cases. In the first examination, 21 of the cases had no aortic regurgitation; one patient had very mild, 5 had mild, and 5 had moderate aortic regurgitation gradually. Fourteen patients did not develop aortic regurgitation up to last examination. Conclusion SVAS is a rare cause of left ventricular outflow tract obstruction requiring echocardiographic close follow-up