Early aberrant angiogenesis due to elastic fiber fragmentation in aortic valve disease

Robert B. Hinton, Amy L. Juraszek, Amy M. Opoka, Benjamin J. Landis, J. Michael Smith, Robert P. Mecham, Kevin E. Bove

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

Elastic fiber fragmentation (EFF) is a hallmark of aortic valve disease (AVD), and neovascularization has been identified as a late finding related to inflammation. We sought to characterize the relationship between early EFF and aberrant angiogenesis. To examine disease progression, regional anatomy and pathology of aortic valve tissue were assessed using histochemistry, immunohistochemistry, and electron microscopy from early-onset (<40 yo) and late-onset (≥40 yo) non-syndromic AVD specimens. To assess the effects of EFF on early AVD processes, valve tissue from Williams and Marfan syndrome patients was also analyzed. Bicuspid aortic valve was more common in early-onset AVD, and cardiovascular comorbidities were more common in late-onset AVD. Early-onset AVD specimens demonstrated angiogenesis without inflammation or atherosclerosis. A distinct pattern of elastic fiber components surrounded early-onset AVD neovessels, including increased emilin-1 and decreased fibulin-5. Different types of EFF were present in Williams syndrome (WS) and Marfan syndrome (MFS) aortic valves; WS but not MFS aortic valves demonstrated angiogenesis. Aberrant angiogenesis occurs in early-onset AVD in the absence of inflammation, implicating EFF. Elucidation of underlying mechanisms may inform the development of new pharmacologic treatments.

Original languageEnglish (US)
Article number75
JournalJournal of Cardiovascular Development and Disease
Volume8
Issue number7
DOIs
StatePublished - Jul 2021
Externally publishedYes

Keywords

  • Angiogenesis
  • Aortic root
  • Elastic fiber
  • Heart valves
  • Pediatrics

ASJC Scopus subject areas

  • Pharmacology, Toxicology and Pharmaceutics(all)
  • Pharmacology (medical)

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