Imaging features of desmoplakin arrhythmogenic cardiomyopathy: A comparative cardiovascular magnetic resonance study.

ABSTRACT:

Background

Arrhythmogenic cardiomyopathy (ACM) related to Desmoplakin (DSP) mutations is a distinct condition associated with particularly severe outcomes, more frequent left ventricular (LV) involvement, including fibrosis, dysfunction, and inflammatory episodes. Whether DSP-ACM is associated with specific imaging features remains elusive. This study aims to provide a comprehensive description of cardiovascular magnetic resonance (CMR) findings in patients with DSP-ACM and to compare them to RV-dominant ACM with LV involvement (LV+ right-dominant-ACM).

Methods

Patients with DSP-ACM matched with patients with ACM related to a non-DSP desmosomal mutation and ≥1 feature of LV involvement underwent CMR in two institutions. Biventricular metrics and segmental wall motion abnorma

SUBMITTER: Laredo M 

PROVIDER: S-EPMC12138554 | biostudies-literature | 2025 Summer

REPOSITORIES: biostudies-literature