Non-compaction cardiomyopathy (NCM) is rare congenital cardiomyopathy characterized on cardiac imaging by a two-layered ventricular wall with prominent trabeculations and intertrabecular recesses. This case highlights a patient in his fifth decade who presented from an outpatient setting for abnormal findings found on a transthoracic echocardiogram for syncopal workup. Cardiac MRI was consistent with non-compaction cardiomyopathy. A loop recorder then inserted, and he was placed on guideline-directed therapy for heart failure with reduced ejection fraction (HFrEF) and discharged with life vest since left ventricular ejection fraction (LVEF) > 35%. There are many areas of controversies in NCM, such as prevalence, diagnostic criteria, clinical features, prognosis, and management strategy. We will discuss the etiology, diagnostic criteria, and management. Physicians should be aware of NCM diagnosis when a patient presents with heart failure and structural heart changes on imaging despite the age. Cardiac magnetic resonance imaging (CMRI) is the best diagnostic modality. Patients should be recognized and started on proper management to prevent complications.

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Copyright © 2019, Musmar et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

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