Severe pulmonary stenosis or atresia with intact ventricular septum falls within the group of cardiac malformations which are characterized functionally by obstruction to pulmonary flow at the right side of the heart. This malformation is commonly manifested clinically by intense cyanosis, cardiac failure, and death in the neonatal period. Whereas the diagnosis and management of pulmonary stenosis in older children and adults is well established, there are relatively few published data relating to atresia or severe stenosis in infants having neonatal difficulties. In 1951, Johnson and Johnson1 reported 5 patients with pulmonary stenosis who died between 3 and 19 months of age. Subsequently, others2-4 having similar experiences have stressed the importance of early diagnosis and surgical correction.
Pulmonary atresia (complete fusion of valve cusps) and severe pulmonary stenosis (extremely tiny or pinpoint orifice) may present similar or identical pictures. Cases with pulmonary atresia or severe stenosis may be
BENTON JW, ELLIOTT LP, ADAMS P, ANDERSON RC, HONG CY, LESTER RG. Pulmonary Atresia and Stenosis with Intact Ventricular Septum. Am J Dis Child. 1962;104(2):161–168. doi:10.1001/archpedi.1962.02080030163009
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