Hypertrophic Cardiomyopathy (HCM) is burdened by sudden cardiac death (SCD) risk of 0.9%/year, and is the most common cause of SCD in young adults. It is an autosomal dominant inherited disease caused by mutations in cardiac sarcomere genes, but the hypertrophic phenotype can also be an expression of cardiac involvement in multiorgan metabolic storage diseases, such as Anderson-Fabry disease (AFD). Mechanical Dispersion (MD) by Speckle-Tracking Echocardiography (STE) has recently emerged as an additional arrhythmic risk marker.

Speckle-tracking echocardiography for arrhythmic risk. Assessment in hypertrophic and Fabry cardiomyopathy

Margherita Stefania Rodolico;
2022

Abstract

Hypertrophic Cardiomyopathy (HCM) is burdened by sudden cardiac death (SCD) risk of 0.9%/year, and is the most common cause of SCD in young adults. It is an autosomal dominant inherited disease caused by mutations in cardiac sarcomere genes, but the hypertrophic phenotype can also be an expression of cardiac involvement in multiorgan metabolic storage diseases, such as Anderson-Fabry disease (AFD). Mechanical Dispersion (MD) by Speckle-Tracking Echocardiography (STE) has recently emerged as an additional arrhythmic risk marker.
2022
Istituto per la Ricerca e l'Innovazione Biomedica -IRIB
fabry disease
holter electrocardiography
hypertrophic cardiomyopathy
phenotype
ventricular fibrillation
echocardiography
sudden cardiac death
cardiomyopathy
mutation
left ventricular hypertrophy
genetic disorder
diastole
gadolinium
genes
risk assessment
sarcomeres
systole
heart
patient prognosis
non-sustained ventricular tachycardia
ventricular arrhythmia
storage disease
ejection fraction
cardiac mri
young adult
cardiovascular findings
two-dimensional speckle tracking
longitudinal strain
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/20.500.14243/450468
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