Chen-Chih J. Sun, Thomas Doyle, Richard E. Ringel
University of Maryland.
United States
Human Pathology
Human Pathol 1995; 26: 880-886
DOI: 10.1016/0046-8177(95)90011-x
Abstract
Pulmonary vein stenosis (PVS) is a rare disorder. Accurate diagnosis often requires anatomical examination. We report four children with pulmonary vein stenosis. Autopsy showed bilateral lesions in two patients who were thought clinically to have unilateral disease. A diagnosis of PVS was made at autopsy in the third case. Intimal and medial fibromuscular proliferation was noted in extrapulmonary and intrapulmonary veins. Some of the fibromuscular proliferation were eccentric, resembling organized thrombi. In one case a focal organizing thrombus was found in a clinically unobstructed but anatomically narrowed veno-atrial junction. In another case injection of contrast medium into the stenotic pulmonary vein (PV) showed anastomosis between PV and bronchial vessels as well as small pulmonary arteries. Bilateral hypertensive arteriopathy was observed in unilateral and bilateral PVS. Our histological finding of intrapulmonary venous lesions in the lobes in which PVS was not detected clinically suggests that during surgical correction of unilateral PVS multiple biopsies of the opposite lung may help to evaluate possible bilateral disease. Our study also suggests that thrombosis in a stenotic pulmonary vein may further compromise the lumen and contribute to the progression of pulmonary vein obstruction. The possible pathogenesis of bilateral pulmonary hypertensive arteriopathy in unilateral PVS also is discussed.
Category
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Pulmonary Hypertension Associated with Stenosis or Atresia of Pulmonary Veins
Pulmonary Venous Pathology
Year of Publication: 1995
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