Iran M. Sadr, Puay E. Tan, Mark W. Kieran, Kathy J. Jenkins
Children’s Hospital, Boston.
United States
American Journal of Cardiology
Am J Cardiol 2000; 86: 577-579
DOI: 10.1016/s0002-9149(00)01022-5
Abstract
Stenosis of individual pulmonary veins in infants has the most aggressive pattern of reobstruction of any of the congenital obstructive defects, making it an ideal model to study the neoproliferative mechanism leading to restenosis. Importantly, pulmonary vein stenosis can be found in normally connected veins in otherwise normal hearts, suggesting that the neoproliferative mechanism may be a “primary” pathologic process, rather than embryologic anatomic derangement or a response to hemodynamic or postsurgical stress. When the disease presents in infancy, restenosis seems to be universal, usually within weeks to months, despite all forms of treatment attempted thus far. We studied the infantile version of isolated pulmonary vein stenosis to explore the histopathologic process leading to reobstruction. We examined all pathologic material available at Children’s Hospital over a 12-year period.
We used microscopy, immunohistochemistry, and cell culture to identify the mechanism of restenosis in 4 infants with isolated pulmonary vein stenosis. Recurrent obstruction appears to be due to myofibroblastic proliferation in this fatal disease.
Category
Segmental Pulmonary Venous Disease. Without a Focus on Pulmonary Hypertension
Pulmonary Vascular Pathology
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No