Joice Prodigios, Stephanie El Omeiri, Marcus Meneses, Bethelhem Belachew, Hansel J. Otero, David M. Biko, Jordan B. Rapp
Children’s Hospital of Philadelphia and Perelman School of Medicine at The University of Pennsylvania.
United States
Seminars in Roentenology
Semin Roentenol 2025;
DOI: 10.1053/j.ro.2025.09.006
Abstract
Pulmonary vein stenosis (PVS) is an uncommon but often progressive condition in children, associated with high morbidity and mortality despite advances in diagnosis and treatment. Etiologies include primary congenital disease and secondary causes such as postoperative total or partial anomalous pulmonary venous return repair, bronchopulmonary dysplasia, and anatomic compression. PVS can occur in isolation or in association with complex congenital heart disease and is frequently characterized by restenosis after intervention. Multimodality imaging is essential for diagnosis, treatment planning, and follow-up in pediatric PVS. Transthoracic echocardiography remains the first-line screening tool, although limited acoustic windows and small vessel caliber may reduce accuracy. Cross-sectional imaging with cardiac magnetic resonance and computed tomography angiography provides high-resolution anatomical assessment and is increasingly complemented by dual-energy computed tomography with iodine perfusion mapping to evaluate the functional impact of stenosis. Cardiac catheterization remains the reference standard for hemodynamic assessment and offers therapeutic capabilities. This review summarizes the epidemiology, anatomy, pathophysiology, and imaging features of PVS in children, with emphasis on the role of multimodality imaging in both congenital and acquired forms. We discuss current interventional, surgical, and medical treatment strategies, highlight challenges in pediatric imaging, and outline recommendations for long-term surveillance to detect restenosis and guide timely reintervention.
Category
Segmental Pulmonary Venous Disease. Without a Focus on Pulmonary Hypertension
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing
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: No