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
Surgical Interventions for Pulmonary Venous Obstruction After the Onset of Disease
Catheter-mediated Interventions: Efficacy or Lack of Efficacy
Medical Therapy to Prevent or Reverse the Onset of Disease. Efficacy or Lack of Efficacy
Medical Therapy to Prevent Progression of Disease. Efficacy or Lack of Efficacy
Medical Therapy to Prevent Recurrence of Disease after an Intervention. Efficacy or Lack of Efficacy
Year of Publication: 2026
Age Focus: Pediatric
Article Type: Review
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: Yes