Key result
Cardiac MRI revealed intramyocardial fat and fibrosis in the left ventricle, establishing the rare diagnosis of arrhythmogenic left ventricular cardiomyopathy in a 59-year-old female.
Why the study?
Predominantly left ventricular involvement in arrhythmogenic ventricular cardiomyopathy is exceedingly rare, and the lack of specific diagnostic criteria makes diagnosis challenging.
Case Report (n=1)
This case highlights the utility of cardiac MRI with tissue characterization in diagnosing the exceedingly rare condition of predominantly left ventricular arrhythmogenic cardiomyopathy.
Cardiac MRI aids rare left ventricular cardiomyopathy diagnosis; leaves open larger studies on prevalence and criteria.
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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Mirmomen et al. (2019) conducted a case report in Arrhythmogenic left ventricular cardiomyopathy (n=1). Cardiac MRI was evaluated. Cardiac MRI revealed intramyocardial fat and fibrosis in the left ventricle, establishing the rare diagnosis of arrhythmogenic left ventricular cardiomyopathy in a 59-year-old female.
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