Pulmonary Vein Stenosis Medical Therapy: Current and on the Horizon

Mudit Gupta, David B. Frank
Perelman School of Medicine at the University of Pennsylvania and 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 2026;
DOI: 10.1053/j.pcsu.2026.02.006

Abstract
Pulmonary vein stenosis (PVS) is a severe vascular obstructive lesion historically associated with poor survival. As outcomes have improved in the current era, PVS has become a chronic disease requiring frequent surgeries, catheter-based interventions, and experimental therapies to prevent progression and recurrence. Despite this, PVS survival remains low and has pushed experimental research into new therapies into the forefront. In this paper, we briefly examine the pathophysiology and etiology of PVS and provide a more comprehensive review of computational and animal modeling of PVS and therapies derived from these studies, both current and on the horizon.

Category
Segmental Pulmonary Venous Disease. Without a Focus on Pulmonary Hypertension
Review Articles Concerning Pulmonary Vascular Disease

Age Focus: Pediatric Pulmonary Vascular Disease

Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication

Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes

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