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Distinct types of plexiform lesions identified by synchrotron-based phase-contrast micro-CT

Westoo, Christian LU ; Norvik, Christian LU ; Peruzzi, Niccolo LU ; van der Have, Oscar LU orcid ; Lovric, Goran ; Jeremiasen, Ida LU ; Tran, Phan Kiet LU ; Mokso, Rajmund LU ; de Jesus Perez, Vinicio and Brunnstrom, Hans LU orcid , et al. (2021) In American Journal of Physiology - Lung Cellular and Molecular Physiology 321(1). p.17-28
Abstract

In pulmonary arterial hypertension, plexiform lesions are associated with severe arterial obstruction and right ventricular failure. Exploring their structure and position is crucial for understanding the interplay between hemodynamics and vascular remodeling. The aim of this research was to use synchrotron-based phase-contrast micro-CT to study the three-dimensional structure of plexiform lesions. Archived paraffin-embedded tissue samples from 14 patients with pulmonary arterial hypertension (13 idiopathic, 1 with known BMPR2-mutation) were imaged. Clinical data showed high-median PVR (12.5 WU) and mPAP (68 mmHg). Vascular lesions with more than 1 lumen were defined as plexiform. Prior radiopaque dye injection in some samples... (More)

In pulmonary arterial hypertension, plexiform lesions are associated with severe arterial obstruction and right ventricular failure. Exploring their structure and position is crucial for understanding the interplay between hemodynamics and vascular remodeling. The aim of this research was to use synchrotron-based phase-contrast micro-CT to study the three-dimensional structure of plexiform lesions. Archived paraffin-embedded tissue samples from 14 patients with pulmonary arterial hypertension (13 idiopathic, 1 with known BMPR2-mutation) were imaged. Clinical data showed high-median PVR (12.5 WU) and mPAP (68 mmHg). Vascular lesions with more than 1 lumen were defined as plexiform. Prior radiopaque dye injection in some samples facilitated 3-D rendering. Four distinct types of plexiform lesions were identified: 1) localized within or derived from monopodial branches (supernumerary arteries), often with a connection to the vasa vasorum; 2) localized between pulmonary arteries and larger airways as a tortuous transformation of intrapulmonary bronchopulmonary anastomoses; 3) as spherical structures at unexpected abrupt ends of distal pulmonary arteries; and 4) as occluded pulmonary arteries with recanalization. By appearance and localization, types 1–2 potentially relieve pressure via the bronchial circulation, as pulmonary arteries in these patients were almost invariably occluded distally. In addition, types 1–3 were often surrounded by dilated thin-walled vessels, often connected to pulmonary veins, peribronchial vessels, or the vasa vasorum. Collaterals, bypassing completely occluded pulmonary arteries, were also observed to originate within plexiform lesions. In conclusion, synchrotron-based imaging revealed significant plexiform lesion heterogeneity, resulting in a novel classification. The four types likely have different effects on hemodynamics and disease progression.

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organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Imaging, Lung, Plexiform lesion, Pulmonary arterial hypertension, Synchrotron
in
American Journal of Physiology - Lung Cellular and Molecular Physiology
volume
321
issue
1
pages
17 - 28
publisher
American Physiological Society
external identifiers
  • pmid:33881927
  • scopus:85109525088
ISSN
1040-0605
DOI
10.1152/AJPLUNG.00432.2020
language
English
LU publication?
yes
id
03aa0dd8-345b-4e11-b736-65e3c17517f9
date added to LUP
2021-08-23 13:04:58
date last changed
2024-05-04 11:04:04
@article{03aa0dd8-345b-4e11-b736-65e3c17517f9,
  abstract     = {{<p>In pulmonary arterial hypertension, plexiform lesions are associated with severe arterial obstruction and right ventricular failure. Exploring their structure and position is crucial for understanding the interplay between hemodynamics and vascular remodeling. The aim of this research was to use synchrotron-based phase-contrast micro-CT to study the three-dimensional structure of plexiform lesions. Archived paraffin-embedded tissue samples from 14 patients with pulmonary arterial hypertension (13 idiopathic, 1 with known BMPR2-mutation) were imaged. Clinical data showed high-median PVR (12.5 WU) and mPAP (68 mmHg). Vascular lesions with more than 1 lumen were defined as plexiform. Prior radiopaque dye injection in some samples facilitated 3-D rendering. Four distinct types of plexiform lesions were identified: 1) localized within or derived from monopodial branches (supernumerary arteries), often with a connection to the vasa vasorum; 2) localized between pulmonary arteries and larger airways as a tortuous transformation of intrapulmonary bronchopulmonary anastomoses; 3) as spherical structures at unexpected abrupt ends of distal pulmonary arteries; and 4) as occluded pulmonary arteries with recanalization. By appearance and localization, types 1–2 potentially relieve pressure via the bronchial circulation, as pulmonary arteries in these patients were almost invariably occluded distally. In addition, types 1–3 were often surrounded by dilated thin-walled vessels, often connected to pulmonary veins, peribronchial vessels, or the vasa vasorum. Collaterals, bypassing completely occluded pulmonary arteries, were also observed to originate within plexiform lesions. In conclusion, synchrotron-based imaging revealed significant plexiform lesion heterogeneity, resulting in a novel classification. The four types likely have different effects on hemodynamics and disease progression.</p>}},
  author       = {{Westoo, Christian and Norvik, Christian and Peruzzi, Niccolo and van der Have, Oscar and Lovric, Goran and Jeremiasen, Ida and Tran, Phan Kiet and Mokso, Rajmund and de Jesus Perez, Vinicio and Brunnstrom, Hans and Bech, Martin and Galambos, Csaba and Tran-Lundmark, Karin}},
  issn         = {{1040-0605}},
  keywords     = {{Imaging; Lung; Plexiform lesion; Pulmonary arterial hypertension; Synchrotron}},
  language     = {{eng}},
  month        = {{07}},
  number       = {{1}},
  pages        = {{17--28}},
  publisher    = {{American Physiological Society}},
  series       = {{American Journal of Physiology - Lung Cellular and Molecular Physiology}},
  title        = {{Distinct types of plexiform lesions identified by synchrotron-based phase-contrast micro-CT}},
  url          = {{http://dx.doi.org/10.1152/AJPLUNG.00432.2020}},
  doi          = {{10.1152/AJPLUNG.00432.2020}},
  volume       = {{321}},
  year         = {{2021}},
}