Trabecular complexity as an early marker of cardiac involvement in Fabry disease
European Heart Journal – Cardiovascular Imaging Jan 28, 2021
Camporeale A, Moroni F, Lazzeroni D, et al. - Researchers used cardiac magnetic resonance to determine if phenotype evolution in Fabry cardiomyopathy can be tracked by myocardial trabecular complexity which is quantified by endocardial border fractal analysis. The study sample comprised 20 healthy controls (12 men, age 32 ± 9) and 45 Fabry patients grouped into three categories: 15 left ventricular hypertrophy (LVH)-negative patients with normal T1 (Group 1); 15 LVH-negative patients with low T1 (Group 2); 15 LVH-positive patients (Group 3). Experts assessed trabecular fractal dimensions (Dfs) (total, basal, mid-ventricular, and apical) on cine images. According to findings, a progressive increase in Df of endocardial trabeculae together with shortening of T1 values were identified as the characteristics of Fabry cardiomyopathy. An increase in myocardial trabeculation was evident prior to the presence of detectable sphingolipid storage, therefore representing an early sign of cardiac involvement.
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