Perry Wengrofsky, Christopher Armenia, Filip Oleszak, Eric Kupferstein, Chandra Rednam, Cristina A Mitre, Samy I McFarlane
Hypertrabeculation and noncompaction are congenital or acquired abnormalities of myocardial anatomy characterized by prominent trabeculations, intertrabecular recesses, and a thin outer epicardial compacted myocardial layer that are most clinically relevant when presenting as left ventricular noncompaction (LVNC) cardiomyopathy. Manifesting in isolation or in association with development or acquired cardiomyopathies, and primarily extracardiac genetic syndromes, LVNC predisposes patients to major cardiac and systemic complications, including cardioembolic disease, ventricular tachyarrhythmia, and sudden cardiac death. Improvements in cardiac imaging modalities such as echocardiography and magnetic resonance imaging have increased the identification of hypertrabeculation and LVNC, but overall rates of LVNC cardiomyopathy remain very low. We present a review on the embryonic pathogenesis of trabeculations and noncompaction, genetic and epidemiologic profiles of LVNC, clinical manifestations, diagnostic imaging strategies and criteria, and the approach to family medical genetic screening and management of the major complications of hypertrabeculation and LVNC cardiomyopathy.
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