A Rare Case of Coincidental LVNC and ALVC According to CMR

Arrhythmogenic left ventricular cardiomyopathy (ALVC) is a rare inherited cardiomyopathy characterized by the progressive fibrofatty replacement of the left ventricular (LV) myocardium in combination with arrhythmias of LV origin. Herein, we present a case of the coincidence of ALVC and left ventricular non-compaction (LVNC) in a 34-year-old woman presenting with the chief complaint of palpitation. The patient had a history of an episode of severe common cold the previous year. Her mother was diagnosed with typical hypertrophic cardiomyopathy. The patient’s preliminary echocardiography demonstrated evidence of LVNC. Subsequent evaluation using cardiac magnetic resonance (CMR) imaging showed a mildly enlarged LV without hypertrophy, along with noticeable myocardial trabeculation (Fig. 1) and a mildly decreased systolic function. Late gadolinium enhancement illustrated a fibrotic layer in the mid-anterior LV segment (Fig. 2). There was an elongated, thick stripe of subepicardial fat deposition at the base-to-mid–anteroseptal, anterior, and anterolateral LV segments (Fig. 3 & Fig. 4). Right ventricular apical trabeculation was prominent, with a regular ventricular function. The CMR tissue characterization criteria indicated the coincidence of ALVC and LVNC. Advances in noninvasive imaging modalities have significantly improved the delineation of the morphologic appearance of ventricles in different cardiomyopathies, facilitating identification and diagnosis. (Iranian Heart Journal 2024; 25(2): 92-95)