NATURAL COURSE OF ISOLATED PULMONARY VALVE STENOSIS IN INFANTS AND CHILDREN UTILIZING DOPPLER-ECHOCARDIOGRAPHY

Most natural history data regarding pulmonary stenosis (PS) were obtai ned from cardiac catheterization studies over 15 to 20 years ago. Sele ction bias in these studies often excluded patients with mild disease and infants. Today, Doppler echocardiography allows accurate serial as sessments of stenotic lesions in patients of all ages. This study eval uates the natural history of PS utilizing serial Doppler examinations in the pediatric population. A total of 147 patients with PS and seria l echocardiographic data were identified. Age at initial echocardiogra m ranged from 2 days to 15 years, with a mean follow-up of 2.4 years. Sixteen of 56 patients (29%) initially evaluated within 1 month had a greater than or equal to 20 mm Hg increase in their peak systolic pres sure gradient. Only 7 of 89 patients (8%) initially evaluated over 1 m onth had a greater than or equal to 20 mm Hg increase. Eleven of 40 ne wborn infants (28%) with mild obstruction had progression to moderate or severe PS compared with 10 of 68 patients (15%) initially evaluated over 1 month. Moderate PS in the newborn was also more likely to prog ress compared with older children, Of the 16 newborns with greater tha n or equal to 20 mm Hg increases, 8 developed the increase in less tha n or equal to 6 months. In contrast, no patient aged >2 years whose in itial gradient was <50 mm Hg developed severe PS. Mild PS may not be s tatic, particularly in young infants. Progression in this age group oc curs more often and more rapidly than in older infants and children. ( C) 1997 by Excerpta Media, Inc.