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