Abstract

Pulmonary valvular motion has recently been characterized for adults with pulmonary vascular obstructive disease (PVOD). Echocardiographic features of PVOD include loss of the presystolic posterior motion (the pulmonary valve “a wave”) flattening of the diastolic segment and premature systolic anterior motion (PSAM) of the pulmonary valve, which is almost always followed by a secondary systolic posterior motion. The systolic pulmonary valve motion thus inscribed resembles the letter “w”. We postulated that PSAM would occur earlier in children with a site for right to left shunting. Our study population consisted of 8 children with ventricular septal defect plus PVOD of severe degree, 18 patients with cor pulmonale and pulmonary hypertension due to cystic fibrosis and 500 children with various other forms of congenital cardiac disease studied by consecutive examination, approximately 20% of whom had pulmonary vascular obstruction of some degree. All children with ventricular septal defect and severe PVOD had PSAM of the pulmonary valve. Those with combined cor pulmonale and PVOD had variable pulmonary valve motion, but most were not separable from normals. Two of those children had PSAM. Of the 500 consecutive examinations of children, we found only 8 with PSAM of the pulmonary valve. With respect to PSAM, two groups of children emerged with differences, those with severe PVOD plus VSD (group 1) and those with PVOD without VSD (group 2).

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