Other nonneoplastic disease

Plexiform arteriopathy

Last author update: 1 December 2011
Last staff update: 30 September 2020

Copyright: 2003-2023,, Inc.

PubMed search: plexiform arteriopathy

Elliot Weisenberg, M.D.
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Cite this page: Weisenberg E. Plexiform arteriopathy. website. Accessed April 2nd, 2023.
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

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Plexiform lesion

Plexiform lesion

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