AORTIC PERIVASCULAR ATTENUATION ON CHEST COMPUTED TOMOGRAPHY ANGIOGRAPHY AND MARFAN SYNDROME

Abstract Introduction Marfan syndrome (MFS, OMIM #154700) is an autosomal dominant inherited disorder with a prevalence in the general population of about 3/10,000. It mainly affects connective tissue due to mutations in the FBN1 gene (15q21.1) that codes for fibrillin–1. The prognosis of patients w...

Full description

Saved in:
Bibliographic Details
Published in:European heart journal supplements 2024-05, Vol.26 (Supplement_2), p.ii19-ii20
Main Authors: Tuttolomondo, D, Gaibazzi, N, Pini, A, Secchi, F
Format: Article
Language:English
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Abstract Introduction Marfan syndrome (MFS, OMIM #154700) is an autosomal dominant inherited disorder with a prevalence in the general population of about 3/10,000. It mainly affects connective tissue due to mutations in the FBN1 gene (15q21.1) that codes for fibrillin–1. The prognosis of patients with MFS is dominated by the high risk of aortic dissection or rupture. Peri–vascular adipose tissue attenuation (PVAT) applied to thoracic angioTC (CTA) is a marker of localised vascular inflammation that is greater in ascending aortic aneurysm than in healthy controls and is associated with in–situ increase of fibrotic tissue. In patients with MFS there is excessive deposition of collagen and proteoglycans with increased wall stiffness that could be identified by PVAT analysis prior to aneurysm development. Material We enrolled 54 consecutive patients with a genetic diagnosis of MFS undergoing control CTA and 43 subjects at low cardiovascular risk undergoing CTA for chest pain. Exclusion Criteria Presence of aortic pathology; presence of at least one cardiovascular risk factor; previous vascular surgery/cardiac surgery; ischaemic cardiovascular pathology; presence of significant coronary atherosclerotic pathology; current or previous neoplasm; chronic inflammatory/infectious diseases. Results There were no statistically significant differences in age, gender and diameter of the ascending aorta between patients in the MFS group and controls. In contrast, PVAT in the ascending aorta appears greater in patients diagnosed with MFS (p
ISSN:1520-765X
1554-2815
DOI:10.1093/eurheartjsupp/suae036.040