Three-dimensional Mapping of Fibrosis Distribution in Desmoplakin Cardiomyopathy from Cardiac MRI.

Abstract

Purpose To generate high-resolution fibrosis maps using universal ventricular coordinates for spatial characterization of fibrotic patterns across disease severity in desmoplakin cardiomyopathy. Materials and Methods This retrospective study included patients with desmoplakin cardiomyopathy who underwent cardiac MRI between March 2012 and October 2024. Three-dimensional ventricular models were reconstructed from cine MRI acquisitions. Fibrosis identified at late gadolinium enhancement MRI was mapped to ventricular geometry using universal ventricular coordinates, enabling analysis within a common reference framework. Patients were classified as having mild, moderate, or severe disease based on fibrosis extent. Associations between fibrosis burden and left ventricular structural and functional metrics were evaluated using Tukey and Wald tests. Results Twenty-nine patients (mean age, 37.39 years [range, 10-77 years]; 20 female patients) were included. In mild disease, fibrosis was primarily located in the subepicardial midinferior region. With increasing fibrosis burden, moderate and severe disease demonstrated subepicardial circumferential involvement with a ringlike pattern in severe cases. Fibrosis burden correlated negatively with left ventricular ejection fraction (r = -0.80, P < .001) and positively with left ventricular end-diastolic volume index (r = 0.54, P = .002). Group comparisons showed significant differences between moderate and severe disease groups across all metrics (P < .05) but not between mild and moderate groups. Conclusion Use of universal ventricular coordinates enabled high-resolution mapping of fibrosis and demonstrated characteristic spatial patterns across disease severity in desmoplakin cardiomyopathy. Fibrosis was observed in nondilated ventricles, whereas higher fibrosis burden was associated with left ventricular dilatation and impaired systolic function. Keywords: Cardiomyopathies, Left Ventricle, Computer Applications-3D, MR Imaging Supplemental material is available for this article. © RSNA, 2026.