Thomas L. Spray, Nancy D. Bridges
The Children’s Hospital of Philadelphia.
United States
Seminars in Thoracic and Cardiovascular Surgery Pediatric Cardiac Surgery Annual
Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 1999; 2: 177-188
DOI: 10.1016/s1092-9126(99)70015-3
Abstract
Pulmonary vein stenosis describes a variety of entities with a variable clinical course. The basic pathologic process appears to be fibrous intimal thickening, which can gradually cause obliteration of the lumen of the pulmonary veins at the atrial junction. In its most severe form, congenital pulmonary vein stenosis is a progressive disease with rapid pulmonary hypertension and rare survival beyond the first year of life. Surgical intervention has not been successful in this group. Other forms of pulmonary vein stenosis, including unilateral types associated with congenital heart disease, may be managed by various surgical treatments or pneumonectomy in some cases. Although multiple surgical techniques have been described for repair of congenital vein stenosis, the “sutureless” techniques recently described may have a place in the surgical treatment of this condition. In the most severe forms, lung transplantation appears to be an important option that should be considered early in the course of the disease.
Category
Segmental Pulmonary Venous Disease. Without a Focus on Pulmonary Hypertension
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
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