Pediatric cardiology
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Pediatric cardiology · Nov 2006
Echocardiographic follow-up of children with supravalvular aortic stenosis.
This study evaluates the course of supravalvular aortic stenosis (SVAS)-associated right ventricular outflow tract (RVOT) obstruction and the results of surgery in children. We reviewed the medical records of 24 patients diagnosed with SVAS at initial echocardiographic examination or during the following period of RVOT obstruction. Very mild SVAS was defined as a transvalvular Doppler peak systolic instantanous gradient (PSIG) less than 25 mmHg, mild stenosis as 25-49 mmHg, moderate stenosis as 50-75 mmHg, and severe stenosis as more than 75 mmHg. ⋯ Newborns with SVAS should be followed for rapid progression of SVAS. In some patients, RVOT obstruction may disappear, and SVAS may develop in others with RVOT obstruction. Patients with RVOT obstruction (at the valvular, supravalvular, or peripheral pulmonary arterial level) should be evaluated carefully for development of SVAS at follow-up.