Severe elastic fiber fragmentation/loss is correlated with Marfan syndrome in patients with an ascending aortic aneurysm

CONTEXT AND OBJECTIVES: Aortic wall degeneration was described by a consensus statement published in 2016 by the Society for Cardiovascular Pathology (SCP) and Association for European Cardiovascular Pathology (AECP). The aim of this study was to determine if histopathologic features could predict the presence of genetic syndrome, to assess the prognostic value of histological findings, and to correlate it with clinical data. METHODS: This study included 85 patients operated in the CHRU of Nancy. When available (n=80), all histological sections were reviewed and scored following the SCP/AECP recommendations. RESULTS: Translamellar mucoid extracellular matrix accumulation (MEMA-T) was significantly associated with MFS, only in patients