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Arrhythmogenic left ventricular cardiomyopathy
Seyedeh Mojdeh Mirmomen1, Andrew Jay Bradley1, Andrew Ernest Arai1
1National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD, USA.
Insights
Arrhythmogenic ventricular cardiomyopathy (AVC) is a genetic heart condition causing fibrofatty tissue in the heart muscle. Primarily affecting the right ventricle, AVC can rarely involve the left ventricle, posing diagnostic challenges.
Area of Science:
- Cardiology
- Genetics
- Pathology
Background:
- Arrhythmogenic ventricular cardiomyopathy (AVC) is an inherited myocardial disorder.
- It involves fibrofatty infiltration of the heart muscle, leading to arrhythmias and sudden cardiac death.
Observation:
- The classic form, arrhythmogenic right ventricular cardiomyopathy (ARVC), primarily affects the right ventricle.
- Left ventricular involvement can occur in advanced ARVC cases.
Findings:
- Predominantly left ventricular AVC is rare.
- Diagnosis is challenging due to lack of specific criteria and potential cardiotoxicity.
Implications:
- Understanding predominantly left ventricular AVC is crucial for accurate diagnosis and management.
- Further research is needed to develop diagnostic criteria for this rare subtype.
Abstract:
Arrhythmogenic ventricular cardiomyopathy (AVC) is a heritable heart muscle disorder characterized by fibrofatty infiltration of the myocardium. Intramyocardial fat deposition is considered arrhythmogenic and predisposes patients to life-threatening arrhythmias and sudden cardiac death. The classic subtype of AVC is characterized by fibrofatty replacement of the right ventricular myocardium (i.e. arrhythmogenic right ventricular cardiomyopathy). In advanced cases of arrhythmogenic right ventricular cardiomyopathy, the left ventricle may be involved as well. Predominantly left ventricular involvement by AVC is exceedingly rare and lack of specific diagnostic criteria as well as its potential cardiotoxic effect make its diagnosis challenging and of high importance.
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