Lung - nontumor
Other nonneoplastic disease
Plexiform arteriopathy


Topic Completed: 1 December 2011

Minor changes: 15 December 2019

Copyright: 2003-2019, PathologyOutlines.com, Inc.

PubMed search: plexiform arteriopathy

Elliot Weisenberg, M.D.
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Cite this page: Weisenberg, E. Plexiform arteriopathy. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lungnontumorpulmonaryhyperplexart.html. Accessed May 26th, 2020.
Definition / general
  • Historical term based on lesions induced by congenital heart disease
  • Heath and Edwards Classification from 1958 (modified below) still in widespread use
  • Open lung biopsy in patients with congenital heart disease may be performed to determine if corrective vascular surgery will be beneficial
Microscopic (histologic) description
Grade I (early):
  • Muscularization and media hypertrophy (> 7% of lumen) of pulmonary arteries

Grade II:
  • Intimal hyperplasia causing attenuation of vascular lumen

Grade III:
  • Subintimal fibrosis with onion ring appearance
  • Marked reduplication of internal elastic membrane
  • Arteries and arterioles resemble pipes

Grade IV to V:
  • Dilation and plexiform lesions, aneurysmal dilation of small pulmonary arteries, plexiform and glomeruloid nodules
  • Fibrin thrombi within plexiform lesion, old / new hemorrhage present

Grade VI:
  • Uncommon, acute necrotizing arteritis with fibrinoid necrosis and acute inflammation of vessel wall, similar to polyarteritis nodosa
  • Associated with extreme pulmonary hypertension
Microscopic (histologic) images

Images hosted on other servers:

Plexiform lesion

Differential diagnosis
  • Secondary pulmonary hypertension: similar histology, different history
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