Arrhythmogenic Left Ventricular Cardiomyopathy: From Diagnosis to Risk Management
Alfredo Mauriello1,2, Anna Selvaggia Roma1, Antonia Ascrizzi1
1Cardiology Unit, Department of Medical Translational Science, University of Campania "Luigi Vanvitelli"-"V. Monaldi" Hospital, 80126 Naples, Italy.
Insights
Left ventricular arrhythmogenic cardiomyopathy (ALVC) is a rare condition characterized by fibrofatty myocardial replacement. Diagnosis and risk stratification are crucial for managing sudden cardiac death risks in ALVC patients.
Area of Science:
- Cardiology
- Genetics
- Pathology
Background:
- Left ventricular arrhythmogenic cardiomyopathy (ALVC) is a rare, poorly understood condition.
- Recently reclassified within non-dilated left ventricular cardiomyopathies.
- Characterized by fibrofatty replacement in the left ventricular myocardium.
Purpose of the Study:
- To review the background of ALVC.
- To outline diagnostic approaches for ALVC.
- To summarize sudden cardiac death (SCD) risk in ALVC patients.
Main Methods:
- Literature review of current evidence.
- Analysis of diagnostic criteria.
- Evaluation of risk stratification strategies.
Main Results:
- ALVC is a distinct left ventricular disease with variable presentation.
- Cardiac magnetic resonance (CMR) and genetic testing are key diagnostic tools.
- Recent guidelines enhance ALVC management and SCD risk stratification.
Conclusions:
- ALVC remains poorly characterized despite recent advances.
- Multiparametric diagnosis is essential for patient management.
- Further research is needed to fully understand ALVC.
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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