Speckle tracking echocardiography-derived parameters as new prognostic markers in hypertrophic cardiomyopathies

Abstract

Aims 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 significantly lower in the V-AR group compared to patients without V-AR (median - 10.2% versus -14%, P = 0.038), MD was significantly higher in the V-AR group (85.5 ms versus 61.1 ms, P = 0.004). V-AR were found significantly 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.

Exaggerated myocardial torsion may contribute to dynamic left ventricular outflow tract obstruction in hypertrophic cardiomyopathy

“…While the relationship between peak twist and other speckle tracking measures was not explored in detail in this study, we note that other studies have suggested that mechanical dispersion may be a useful prognostic marker for arrhythmia risk in HCM. 40 …”

Myocardial strain of the left ventricle by speckle tracking echocardiography: From physics to clinical practice

“…Among these parameters, mechanical dispersion (MD) was associated with an increased risk of ventricular arrhythmia, providing a promising tool for risk stratification. 133 The assessment of regional strain seems to be useful for the detection of some HCM phenocopies, in particular Fabry disease and cardiac amyloidosis, as specific strain patterns are suggestive of distinct diseases. 65 In Fabry disease, for example, regional longitudinal strain is typically decreased at the basal posterior wall (where replacement fibrosis is typically located).…”

“…The use of STE‐derived parameters has been suggested for the evaluation of patients with LV hypertrophy where sarcomeric or storage diseases are suspected. Among these parameters, mechanical dispersion (MD) was associated with an increased risk of ventricular arrhythmia, providing a promising tool for risk stratification 133 …”

Sex Differences in Anderson–Fabry Cardiomyopathy: Clinical, Genetic, and Imaging Analysis in Women

“…Recent literature shows advanced echocardiographic techniques, like left-ventricular global longitudinal strain (LV-GLS) and mechanical dispersion (MD) by speckle tracking echocardiography (STE), provide early diagnostic and prognostic markers [ 28 , 29 ], correlating with fibrosis, ventricular arrhythmias, and SCD [ 30 ]. A study recently published by our group demonstrated MD as a reliable prognostic predictor due to its association with increased ventricular arrhythmias [ 31 ].…”