Speckle Tracking Echocardiography- derived parameters as new prognostic markers in Hypertrophic Cardiomyopathies

Hypertrophic Cardiomyopathies (HCM) are caused in 30- 60% by mutations in cardiac sarcomere genes, but can also be an expression of cardiac involvement in multisystemic metabolic diseases, such as Anderson-Fabry disease (AFD). HCM entails a risk of sudden cardiac death (SCD) of 0.9%/year and is the most common cause of SCD in young adults. Recent studies suggested Mechanical Dispersion (MD) by Speckle-Tracking Echocardiography (STE) as an additional arrhythmic risk marker. Aim of the study was to evaluate left ventricle global longitudinal strain (LV-GLS) and MD, in patients with HCM or AFD-Cardiomyopathy and the association with ventricular arrhythmias. Methods and results We evaluated 40 patients with HCM, 57 with AFD (12 with LV-hypertrophy and 45 without) and 40 healthy subjects, between January 2014 and June 2022. We performed a comprehensive echocardiographic study, analyzed systolic and diastolic function, LV-GLS and MD. We also analyzed ventricular arrhythmias (V-AR), including ventricular fibrillation and sustained/non-sustained ventricular tachycardia, by Holter-EKG, in a subset of hypertrophic patients. Data were analyzed by unpaired Student t-test or chi-square/Fisher’s exact test as appropriate, and binary logistic regression (SPSS Statistics ver.26). LV-GLS was signicantly lower in the V-AR group compared to patients without V-AR (median - 10.2% versus -14%, P = 0.038), MD was signicantly higher in the V-AR group (85.5 ms versus 61.1 ms, P = 0.004). V-AR were found signicantly associated with MD (OR, 1.030, 95% CI, 1.003-1.058, P = 0.03) Conclusions MD is a useful additional index in the evaluation of patients with Hypertrophic Cardiomyopathies, and may be a promising prognostic predictor of increased arrhythmic risk.