Myocardial strain measured via two-dimensional speckle-tracking echocardiography in a family diagnosed with arrhythmogenic left ventricular cardiomyopathy

Abstract

Background Arrhythmogenic cardiomyopathy is a myocardial disorder characterized by ventricular arrhythmias, right and/or left ventricular involvement, and fibrofatty infiltrations in the myocardium. We report a family diagnosed with arrhythmogenic left ventricular cardiomyopathy (ALVC) and depict their echocardiographic characteristics. Methods and results Fifteen family members were divided into three groups based on whether they carried the TMEM43 mutation and had been diagnosed with ALVC. Eight of them had TMEM43 mutations, and four were diagnosed with ALVC according to the Padua criteria. Only the proband experienced sudden cardiac death and had a dilated left ventricle. Left ventricular ejection fraction was reduced in two patients; however, left ventricular global longitudinal strain was depressed in three patients. Low QRS voltages in limb leads were evident in three patients, and five patients had frequent ventricular premature contractions. Late gadolinium enhancement was evident in three patients. Left ventricular layer-specific strain showed that the transmural strain gradient ratio was increased in patients diagnosed with ALVC, and it was elevated in the genotype-positive and phenotype-negative groups compared with healthy individuals. Conclusion Global left ventricular longitudinal strain better evaluated left ventricular function than left ventricular ejection fraction. The transmural strain gradient ratio was elevated in patients diagnosed with ALVC, suggesting that it was useful for the evaluation of ALVC.

Undiagnosed cardiomyopathy: An incidental finding in a recreational soccer player—A case report and literature review

“…Combining both deformation patterns and mechanical dispersion has been shown to improve the sensitivity for identifying subclinical disease and the predictive value for risk stratification. [34][35][36][37][38][39][40][41] Mast et al and Kirkels et al showed that pattern recognition adds prognostic value and helps to predict disease progression. 32,34 Regional patterns such as delayed onset of shortening, reduced peak systolic strain, and mild post-systolic shortening may be observed in early ACM phenotypes, which often present before fulfilment of electrical or structural 2010 TFC.…”

Effect of hemodialysis on left atrial function in patients with end‐stage renal failure evaluated by two‐dimensional speckle tracking imaging

“…Previous studies have confirmed 1,14 that left ventricular global longitudinal strain (LVGLS) can accurately evaluate left ventricular systolic function, while GLS is affected by volume load 15 . Recently, experts recommended 16–18 to add left atrial strain to the evaluation of left ventricular diastolic function.…”

“…Conduit function is the difference between reservoir function and contractile function. In the early stage of ventricular diastole, blood flows directly into left ventricle through left atrium, which is mainly determined by left atrial compliance, and is also closely related to left ventricular diastolic tension and stiffness 14 . After hemodialysis, the strain value of LAScd also decreased, indicating that the blood flowing through left atrium and entering left ventricle decreased in early diastole.…”

The Challenges of Diagnosis and Treatment of Arrhythmogenic Cardiomyopathy: Are We there yet?

“…Moreover, Mast et al [61] observed that in relatives of ARVC patients normal sub-tricuspid regional strain is associated with no disease progression in the next 4 years follow-up, strain abnormalities possibly anticipating the overt signs of the disease. To date there are only a few reports describing LV strain abnormalities in the setting of ACM [62]. 3D echocardiography enables a more accurate measurement of RV volumes and ejection fraction, thereby overcoming the geometrical limits of 2D RV imaging, that underestimates RV volumes.…”