title: 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.
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.
authors.affiliation: Johns Hopkins University, Baltimore, MD, USA.
authors.forename: Marc K
authors.initials: MK
authors.lastname: Halushka
authors.affiliation: University of Padua, Padua, Italy.
authors.forename: Annalisa
authors.initials: A
authors.lastname: Angelini
authors.affiliation: University of Catania, Catania, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Bartoloni
authors.affiliation: University of Padua, Padua, Italy.
authors.forename: Cristina
authors.initials: C
authors.lastname: Basso
authors.affiliation: Russian Academy of Medical Sciences, Irkutsk, Russia.
authors.forename: Lubov
authors.initials: L
authors.lastname: Batoroeva
authors.affiliation: Paris Descartes University, Paris, France.
authors.forename: Patrick
authors.initials: P
authors.lastname: Bruneval
authors.affiliation: University of Texas Health Science Center at Houston, Houston, TX, USA.
authors.forename: L Maximilian
authors.initials: LM
authors.lastname: Buja
authors.affiliation: Toronto General Hospital, Toronto, Ontario, Canada.
authors.forename: Jagdish
authors.initials: J
authors.lastname: Butany
authors.affiliation: Sapienza University of Rome, Rome, Italy.
authors.forename: Giulia
authors.initials: G
authors.lastname: d'Amati
authors.affiliation: New York Medical College, Valhalla, NY, USA.
authors.forename: John T
authors.initials: JT
authors.lastname: Fallon
authors.affiliation: Southampton University Hospital, Southampton, United Kingdom.
authors.forename: Patrick J
authors.initials: PJ
authors.lastname: Gallagher
authors.affiliation: Leiden University, Leiden, the Netherlands.
authors.forename: Adriana C
authors.initials: AC
authors.lastname: Gittenberger-de Groot
authors.affiliation: Hospital Santa Cruz, Carnaxide, Portugal.
authors.forename: Rosa H
authors.initials: RH
authors.lastname: Gouveia
authors.affiliation: Department of Pathology, Fimlab Laboratories, Tampere, Finland.
authors.forename: Ivana
authors.initials: I
authors.lastname: Kholova
authors.affiliation: East Carolina University, Greenville, NC, USA.
authors.forename: Karen L
authors.initials: KL
authors.lastname: Kelly
authors.affiliation: Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy.
authors.forename: Ornella
authors.initials: O
authors.lastname: Leone
authors.affiliation: University of Alabama at Birmingham, Birmingham, AL, USA.
authors.forename: Silvio H
authors.initials: SH
authors.lastname: Litovsky
authors.affiliation: Mayo Clinic, Rochester, MN, USA.
authors.forename: Joseph J
authors.initials: JJ
authors.lastname: Maleszewski
authors.affiliation: University of Utah, Salt Lake City, UT, USA.
authors.forename: Dylan V
authors.initials: DV
authors.lastname: Miller
authors.affiliation: Brigham and Women's Hospital, Boston, MA, USA.
authors.forename: Richard N
authors.initials: RN
authors.lastname: Mitchell
authors.affiliation: Papworth Hospital, Cambridgeshire, United Kingdom.
authors.forename: Stephen D
authors.initials: SD
authors.lastname: Preston
authors.affiliation: Pisa University Hospital, Pisa, Italy.
authors.forename: Angela
authors.initials: A
authors.lastname: Pucci
authors.affiliation: University of Nebraska, Lincoln, NE, USA.
authors.forename: Stanley J
authors.initials: SJ
authors.lastname: Radio
authors.affiliation: Cleveland Clinic, Cleveland, OH, USA.
authors.forename: E Rene
authors.initials: ER
authors.lastname: Rodriguez
authors.affiliation: St. George's Medical School, University of London, London, United Kingdom.
authors.forename: Mary N
authors.initials: MN
authors.lastname: Sheppard
authors.affiliation: Massachusetts General Hospital, Boston, MA, USA.
authors.forename: James R
authors.initials: JR
authors.lastname: Stone
authors.affiliation: Sheffield Teaching Hospitals, Sheffield, United Kingdom.
authors.forename: S Kim
authors.initials: SK
authors.lastname: Suvarna
authors.affiliation: Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Carmela D
authors.initials: CD
authors.lastname: Tan
authors.affiliation: University of Padua, Padua, Italy.
authors.forename: Gaetano
authors.initials: G
authors.lastname: Thiene
authors.affiliation: University of Ottawa, Ottawa, Ontario, Canada.
authors.forename: John P
authors.initials: JP
authors.lastname: Veinot
authors.affiliation: University of Amsterdam, Amsterdam, the Netherlands.
authors.forename: Allard C
authors.initials: AC
authors.lastname: van der Wal
mesh_terms: D001018:Aortic Diseases / Q000175:diagnosis*; D002309:Cardiology / Q000592:standards*; D006801:Humans; D010340:Pathology, Surgical / Q000592:standards*; D009626:Terminology as Topic
keywords: Aneurysm; Aorta; Bicuspid aortic valve; Consensus document; Cystic medial degeneration; Degenerative; Dissection; Histopathology; Lamellar unit; Loeys–Dietz syndrome; Marfan syndrome; Medial degeneration; Noninflammatory