Work

A translational framework for patient-specific modeling of pulmonary vein stenosis

Year: 2026

Type: article

Abstract: Pulmonary vein stenosis (PVS) is a rare but aggressive pediatric vascular disorder characterized by progressive intimal thickening, myofibroblast proliferation, and venous obstruction. 1 Infants typically present within the first year of life, and despite advances in imaging and interventional techniques, PVS continues to be associated a poor prognosis.Mortality can approach 50% within 2 to 3 years of life, reflecting both the rapid disease progression and the high rate of restenosis after interventions. 1 Reintervention is common, and many patients require multiple catheterizations or operations during early childhood, underscoring the chronic and recurrent nature of the disease.Management remains challenging.Current treatment strategies-including balloon angioplasty, stent placement, and surgical repair-often provide only temporary relief, because fibroproliferative occurrence and recurrent narrowing are frequent occurences. 1 Disease biology varies across phenotypes such as primary PVS and postrepair total anomalous pulmonary venous connection-associated PVS, adding further heterogeneity to clinical outcomes.1 Although recent reports have improved understanding of clinical course and treatment strategies, 2 the field still lacks reliable predictors of recurrence or progression.These challenges underscore the need to couple current clinical knowledge with emerging translational frameworks capable of defining mechanisms, predicting recurrence risk, and guiding more individualized management.The underlying molecular mechanism of PVS remains unknown.Emerging evidence suggests that endothelial injury, smooth muscle proliferation, and extracellular matrix remodeling are drivers of recurrent phenotype.3 Hemodynamic stressors potentially have a role in mediating phenotype, 4 although the exact nature of this stimulus remains unknown.

Source: JTCVS Open

DOI: 10.1016/j.xjon.2026.101858

Authors: Manish Bansal, Jeffrey S. Heinle, Athar M. Qureshi, Ravi K. Birla

Institutions: Baylor College of Medicine, Texas Children's Hospital

Language: English

Topic: Congenital Heart Disease Studies

Subfield: Cardiology and Cardiovascular Medicine

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $2,431