Key result
Cardiac MRI identifies extensive circumferential late gadolinium enhancement, confirming arrhythmogenic left ventricular cardiomyopathy.
Why the study?
Revised task force criteria for arrhythmogenic cardiomyopathy include only right ventricular morphological criteria without left ventricular criteria, despite accumulating evidence of left ventricular involvement such as in Carvajal syndrome.
Case Report (n=1)
No
Highlights the utility of cardiac MRI in identifying structural and fibrotic manifestations of left-dominant arrhythmogenic cardiomyopathy (Carvajal syndrome).
CMR may aid diagnosis of left-dominant arrhythmogenic cardiomyopathy; extends sparse case evidence but leaves broader utility open.
The current revised task force criteria for diagnosis of arrhythmogenic cardiomyopathy (ACM) includes only morphological criteria for right ventricle with no consideration for left ventricle criteria.1 However, accumulating evidence suggests increasing left ventricle involvement in ACM. One such phenotype is Carvajal syndrome, which is a primary left ventricle ACM that is inherited as a familial cardiocutaneous syndrome consisting of woolly hair, palmoplantar keratoderma, and cardiac involvement.
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Akintoye et al. (2021) conducted a case report in Arrhythmogenic left ventricular cardiomyopathy (Carvajal syndrome) (n=1). Cardiac magnetic resonance imaging was evaluated on Cardiac morphological and tissue characteristics on MRI. Cardiac magnetic resonance imaging demonstrated extensive, circumferential epicardial to midmyocardial enhancement of the left ventricle, confirming arrhythmogenic left ventricular cardiomyopathy.
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