DOI: 10.3390/children13111511 ISSN: 2227-9067
The Role of Mechanotransduction in Pediatric Pulmonary Vein Stenosis
Alessia Di Nardo, Marta Mhanna, Rachel D. VanderlaanPediatric pulmonary vein stenosis (PVS) is a progressive vascular disease that is challenging to treat. Multiple factors are believed to contribute to pathogenic vascular remodeling in PVS, and a growing body of evidence implicates the contribution of mechanotransduction. The pulmonary veins possess a unique mechanical environment and the response of pulmonary venous cells to atypical mechanical stimuli is currently being investigated. This review aims to summarize the current evidence supporting the existence of an atypical mechanical environment in PVS, emerging methods that can be used to quantify mechanical indices within the pulmonary veins, and potential cellular mechanisms contributing to adverse vascular remodeling.