Assessment of Myocardial Fibrosis in Marfan Syndrome Using Cardiac Magnetic Resonance Imaging.

ABSTRACT:

Background

Impaired myocardial function and arrhythmia are important manifestations of Marfan syndrome (MFS). Studies assessing myocardial fibrosis in relation to these manifestations are scarce.

Methods

This cross-sectional, single-center study assessed ventricular volumes, ventricular function, and myocardial fibrosis by cardiac magnetic resonance imaging (CMR) in patients with MFS harboring a (likely) pathogenic FBN1 variant. The presence and extent of fibrosis were assessed by late gadolinium enhancement (LGE) and extracellular volume measurement (ECV). Data on 24-h Holter monitoring and clinical data were extracted from electronic patient records.

Results

The study included 32 unselected patients with MFS (median age 38 years [range 10-69], 41% women). No focal m

SUBMITTER: Demolder A 

PROVIDER: S-EPMC11568239 | biostudies-literature | 2024 Nov

REPOSITORIES: biostudies-literature