Comprehensive Cardiovascular Management of Myotonic Dystrophy Type 1 Patients: A Report from the Italian Neuro-Cardiology Network

Russo, Vincenzo; Antonini, Giovanni; Massa, Roberto; Casali, Carlo; Mauriello, Alfredo; Martino, Anna; Marconi, Roberto; Garibaldi, Matteo; Franciosa, Pasquale; Zecchin, Massimo; Gaudio, Carlo; D’Andrea, Antonello; Strano, Stefano

doi:10.3390/jcdd11020063

Cited by 1 publication

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References 86 publications

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“…The DM1 cardiac phenotype is broad and includes conduction disturbances, arrhythmias, and subclinical diastolic and systolic dysfunction in the early stage of disease; in contrast, severe ventricular systolic dysfunction occurs in the late stage of disease. In fact, dilated cardiomyopathy and end-stage cardiomyopathy are uncommon [95][96][97][98]. SCD occurs in 30% of DM1 patients [99][100][101][102][103][104].…”

Section: Myotonic Dystrophy Type 1 (Dm1)mentioning

confidence: 99%

Arrhythmogenic Left Ventricular Cardiomyopathy: From Diagnosis to Risk Management

Mauriello,

Roma,

Ascrizzi

et al. 2024

JCM

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Purpose of Review: Left ventricular arrhythmogenic cardiomyopathy (ALVC) is a rare and poorly characterized cardiomyopathy that has recently been reclassified in the group of non-dilated left ventricular cardiomyopathies. This review aims to summarize the background, diagnosis, and sudden cardiac death risk in patients presenting this cardiomyopathy. Recent Findings: Although there is currently a lack of data on this condition, arrhythmogenic left ventricular dysplasia can be considered a specific disease of the left ventricle (LV). We have collected the latest evidence about the management and the risks associated with this cardiomyopathy. Summary: Left ventricular arrhythmogenic cardiomyopathy is still poorly characterized. ALVC is characterized by fibrofatty replacement in the left ventricular myocardium, with variable phenotypic expression. Diagnosis is based on a multiparametric approach, including cardiac magnetic resonance (CMR) and genetic testing, and is important for sudden cardiac death (SCD) risk stratification and management. Recent guidelines have improved the management of left ventricular arrhythmogenic cardiomyopathy. Further studies are necessary to improve knowledge of this cardiomyopathy.

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