4.5 Article

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

Publisher

AMER PHYSIOLOGICAL SOC
DOI: 10.1152/ajplung.00432.2020

Keywords

imaging; lung; plexiform lesion; pulmonary arterial hypertension; synchrotron

Funding

  1. Swedish Heart-Lung Foundation
  2. Swedish Society of Medicine
  3. Fanny Ekdahl's Foundation for Pediatric Research
  4. Crafoord Foundation
  5. Knut and Alice Wallenberg Foundation
  6. Skane County Council
  7. National Institutes of Health [R01HL06702]
  8. Jayden DeLucia Foundation for Pulmonary Hypertension
  9. Sten K. Johnson Foundation

Ask authors/readers for more resources

This study used synchrotron-based imaging to study the three-dimensional structure of plexiform lesions in patients with pulmonary arterial hypertension, identifying four distinct types of plexiform lesions which likely have different effects on hemodynamics and disease progression. Types 1-3 may potentially relieve pressure via the bronchial circulation in these patients, as their pulmonary arteries were almost always occluded distally.
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 3D 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.

Authors

I am an author on this paper
Click your name to claim this paper and add it to your profile.

Reviews

Primary Rating

4.5
Not enough ratings

Secondary Ratings

Novelty
-
Significance
-
Scientific rigor
-
Rate this paper

Recommended

No Data Available
No Data Available