Steven Christopher Smith, Raja Rabah
University of Michigan Health System.
United States
Pediatric and Developmental Pathology
Pediatr Dev Pathol 2012; 15: 160-164
DOI: 10.2350/11-09-1099-CR.1
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.
Category
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Pulmonary Hypertension Associated with Stenosis or Atresia of Pulmonary Veins
Right Heart Failure Associated with Stenosis or Atresia of Pulmonary Veins
Surgical Interventions for Pulmonary Venous Obstruction After the Onset of Disease
Length of Life Associated with Pulmonary Venous Obstruction
Year of Publication: 2012
Age Focus: Pediatric
Article Type: Case Reports or Retrospective Observations in Small Groups of Patients (≤10 patients)
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: No