Pulmonary Venous Stenosis in a Premature Infant with Bronchopulmonary Dysplasia: Clinical and Autopsy Findings of these Newly Associated Entities

Abstract

Pulmonary venous stenosis is rare and is most commonly found in association with cardiac malformations. Recent studies have associated pulmonary venous stenosis with prematurity, especially with bronchopulmonary dysplasia, although no such case has been documented at autopsy. We report the case of a 26-week-gestation infant who required ventilation at birth and who, among other complications, developed chronic lung disease of prematurity by the age of 3 months. Imaging showed suprasystemic right-sided pressures and pulmonary venous stenosis. Despite aggressive management of respiratory status and surgical marsupialization of stenoses, the infant expired after 3 weeks. At autopsy, 3 of 4 pulmonary veins showed a fibrous ridge obstructing atrial ostia with otherwise normal anatomy. The lungs showed bronchopulmonary dysplasia, pulmonary hypertensive vascular changes, and features of venous obstruction. Pulmonary venous stenosis and bronchopulmonary dysplasia in premature infants may be pathogenetically related. Coincidence of these diseases is likely underrecognized, and careful cardiac examination in these patients is warranted.