M. E. McConnell, A. D. Pacifico, W. H. Johnson Jr, E. Mroczek
University of Alabama in Birmingham.
United States
Pediatric Cardiology
Pediatr Cardiol 1994; 15: 95-99
DOI: 10.1007/BF00817617
Abstract
Stenosis of individual pulmonary veins is a rare condition associated with a very high mortality. The present report is the first known case of progressive pulmonary venous obstruction associated with the tetralogy of Fallot. This clinical and anatomical study suggests that initial dynamic and possibly reversible occlusion occurs at the junction of the pulmonary vein and left atrium. Anatomical lesions in pulmonary veins then develop, possibly secondary to functional stenosis. The usually fatal outcome of these cases is due to the development of fixed pulmonary venous occlusion and possibly pulmonary interstitial fibrosis. The rapid progression of pulmonary venous stenosis strongly suggests that discrete veno-atrial stenosis should be surgically corrected as early as possible to prevent the irreversible diffuse pulmonary venous obstruction associated with an invariably fatal prognosis.
Category
Segmental Pulmonary Venous Disease. Without a Focus on Pulmonary Hypertension
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
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