Ventricular arrhythmias and primary prevention of sudden cardiac death in Anderson-Fabry disease.

The Anderson-Fabry disease (AFD) is a X-linked lysosomal storage disorder due to the deficiency in the α-galactosidase A enzyme. Cardiovascular mortality is a major cause of death in patients with AFD and sudden cardiac death (SCD) is one of the main causes of death. The storage of glycosphingolipid along with ionic channel impairment, inflammation and fibrosis are involved in the arrhythmogenesis.

Hepatic T1-time, cardiac structure and function and cardiovascular outcomes in patients with dilated cardiomyopathy.

Aim: Liver damage frequently occurs in patients with cardiovascular (CV) disease and is associated with adverse clinical outcomes. The associations of liver damage with cardiac structure/function measures and the risk of adverse CV events in patients with dilated cardiomyopathy (DCM) are poorly known.

Methods: We retrospectively enrolled consecutive patients with DCM undergoing cardiac magnetic resonance imaging (MRI).

Role of arrhythmic phenotype in prognostic stratification and management of dilated cardiomyopathy.

Aims: Dilated cardiomyopathy (DCM) with arrhythmic phenotype combines phenotypical aspects of DCM and predisposition to ventricular arrhythmias, typical of arrhythmogenic cardiomyopathy. The definition of DCM with arrhythmic phenotype is not universally accepted, leading to uncertainty in the identification of high-risk patients. This study aimed to assess the prognostic impact of arrhythmic phenotype in risk stratification and the correlation of arrhythmic markers with high-risk arrhythmogenic gene variants in DCM patients.