The Role of Elevated Wall Shear Stress in Progression of Pulmonary Vein Stenosis: Evidence from Two Case Studies

Pulmonary vein stenosis is a serious condition characterized by restriction or blockage due to fibrotic tissue ingrowth that develops in the pulmonary veins of infants or children. It is often progressive and can lead to severe pulmonary hypertension and death. Efforts to halt or reverse disease pro...

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Main Authors: Peter E. Hammer, Kerry McEnaney, Ryan Callahan, Christopher W. Baird, David M. Hoganson, Kathy J. Jenkins
Format: Article
Language:English
Published: MDPI AG 2021-08-01
Series:Children
Subjects:
Online Access:https://www.mdpi.com/2227-9067/8/9/729
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author Peter E. Hammer
Kerry McEnaney
Ryan Callahan
Christopher W. Baird
David M. Hoganson
Kathy J. Jenkins
author_facet Peter E. Hammer
Kerry McEnaney
Ryan Callahan
Christopher W. Baird
David M. Hoganson
Kathy J. Jenkins
author_sort Peter E. Hammer
collection DOAJ
description Pulmonary vein stenosis is a serious condition characterized by restriction or blockage due to fibrotic tissue ingrowth that develops in the pulmonary veins of infants or children. It is often progressive and can lead to severe pulmonary hypertension and death. Efforts to halt or reverse disease progression include surgery and catheter-based balloon dilation and stent implantation. Its cause and mechanism of progression are unknown. In this pilot study, we propose and explore the hypothesis that elevated wall shear stress at discrete pulmonary venous sites triggers stenosis. To assess this theory, we retrospectively analyzed cardiac catheterization, lung scan, and X-ray computed tomography data to estimate wall shear stress in the pulmonary veins at multiple time points during disease progression in two patients. Results are consistent with the existence of a level of elevated wall shear stress above which the disease is progressive and below which progression is halted. The analysis also suggests the possibility of predicting the target lumen size necessary in a given vein to reduce wall shear stress to normal levels and remove the trigger for stenosis progression.
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spelling doaj.art-91c51f7c432f42259f7f250c9a1d02ae2023-11-22T12:29:08ZengMDPI AGChildren2227-90672021-08-018972910.3390/children8090729The Role of Elevated Wall Shear Stress in Progression of Pulmonary Vein Stenosis: Evidence from Two Case StudiesPeter E. Hammer0Kerry McEnaney1Ryan Callahan2Christopher W. Baird3David M. Hoganson4Kathy J. Jenkins5Department of Cardiac Surgery, Boston Children’s Hospital, Boston, MA 02115, USADepartment of Cardiology, Boston Children’s Hospital, Boston, MA 02115, USADepartment of Cardiology, Boston Children’s Hospital, Boston, MA 02115, USADepartment of Cardiac Surgery, Boston Children’s Hospital, Boston, MA 02115, USADepartment of Cardiac Surgery, Boston Children’s Hospital, Boston, MA 02115, USADepartment of Cardiology, Boston Children’s Hospital, Boston, MA 02115, USAPulmonary vein stenosis is a serious condition characterized by restriction or blockage due to fibrotic tissue ingrowth that develops in the pulmonary veins of infants or children. It is often progressive and can lead to severe pulmonary hypertension and death. Efforts to halt or reverse disease progression include surgery and catheter-based balloon dilation and stent implantation. Its cause and mechanism of progression are unknown. In this pilot study, we propose and explore the hypothesis that elevated wall shear stress at discrete pulmonary venous sites triggers stenosis. To assess this theory, we retrospectively analyzed cardiac catheterization, lung scan, and X-ray computed tomography data to estimate wall shear stress in the pulmonary veins at multiple time points during disease progression in two patients. Results are consistent with the existence of a level of elevated wall shear stress above which the disease is progressive and below which progression is halted. The analysis also suggests the possibility of predicting the target lumen size necessary in a given vein to reduce wall shear stress to normal levels and remove the trigger for stenosis progression.https://www.mdpi.com/2227-9067/8/9/729pulmonary vein stenosiswall shear stressneointimal hyperplasia
spellingShingle Peter E. Hammer
Kerry McEnaney
Ryan Callahan
Christopher W. Baird
David M. Hoganson
Kathy J. Jenkins
The Role of Elevated Wall Shear Stress in Progression of Pulmonary Vein Stenosis: Evidence from Two Case Studies
Children
pulmonary vein stenosis
wall shear stress
neointimal hyperplasia
title The Role of Elevated Wall Shear Stress in Progression of Pulmonary Vein Stenosis: Evidence from Two Case Studies
title_full The Role of Elevated Wall Shear Stress in Progression of Pulmonary Vein Stenosis: Evidence from Two Case Studies
title_fullStr The Role of Elevated Wall Shear Stress in Progression of Pulmonary Vein Stenosis: Evidence from Two Case Studies
title_full_unstemmed The Role of Elevated Wall Shear Stress in Progression of Pulmonary Vein Stenosis: Evidence from Two Case Studies
title_short The Role of Elevated Wall Shear Stress in Progression of Pulmonary Vein Stenosis: Evidence from Two Case Studies
title_sort role of elevated wall shear stress in progression of pulmonary vein stenosis evidence from two case studies
topic pulmonary vein stenosis
wall shear stress
neointimal hyperplasia
url https://www.mdpi.com/2227-9067/8/9/729
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