Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: II. Noninflammatory degenerative diseases - Nomenclature and diagnostic criteria

Marc K. Halushka, Annalisa Angelini, Giovanni Bartoloni, Cristina Basso, Lubov Batoroeva, Patrick Bruneval, L. Maximilian Buja, Jagdish Butany, Giulia D'Amati, John T. Fallon, Patrick J. Gallagher, Adriana C. Gittenberger-De Groot, Rosa H. Gouveia, Ivana Kholova, Karen L. Kelly, Ornella Leone, Silvio H. Litovsky, Joseph J. Maleszewski, Dylan V. Miller, Richard N. MitchellStephen D. Preston, Angela Pucci, Stanley J. Radio, E. Rene Rodriguez, Mary N. Sheppard, James R. Stone, S. Kim Suvarna, Carmela D. Tan, Gaetano Thiene, John P. Veinot, Allard C. Van Der Wal

Research output: Contribution to journalReview articlepeer-review

91 Scopus citations

Abstract

Surgical aortic specimens are usually examined in Pathology Departments as a result of treatment of aneurysms or dissections. A number of diseases, genetic syndromes (Marfan syndrome, Loeys-Dietz syndrome, etc.), and vasculopathic aging processes involved in vascular injury can cause both distinct and nonspecific histopathologic changes with degeneration of the media as a common denominator. Terminology for these changes has varied over time leading to confusion and inconsistencies. This consensus document has established a revised, unified nomenclature for the variety of noninflammatory degenerative aortic histopathologies seen in such specimens. Older terms such as cystic medial necrosis and medionecrosis are replaced by more technically accurate terms such as mucoid extracellular matrix accumulation (MEMA), elastic fiber fragmentation and/or loss, and smooth muscle cell nuclei loss. A straightforward system of grading is presented to gauge the extent of medial degeneration and synoptic reporting tables are provided. Herein we present a standardized nomenclature that is accessible to general pathologists and useful for future publications describing these entities.

Original languageEnglish (US)
Pages (from-to)247-257
Number of pages11
JournalCardiovascular Pathology
Volume25
Issue number3
DOIs
StatePublished - May 2016

Keywords

  • Aneurysm
  • Aorta
  • Bicuspid aortic valve
  • Consensus document
  • Cystic medial degeneration
  • Degenerative
  • Dissection
  • Histopathology
  • Lamellar unit
  • Loeys-Dietz syndrome
  • Marfan syndrome
  • Medial degeneration
  • Noninflammatory

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Cardiology and Cardiovascular Medicine

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