Highlights the potential association between Ebstein's anomaly and LVNC, and the risk of sudden cardiac death, emphasizing the importance of familial screening.
May support LVNC screening in Ebstein's anomaly families after SCD; leaves open the association and screening benefit.
Ebstein's anomaly is a congenital disorder characterized by apical displacement of the septal leaflet of the tricuspid valve. Ebstein's anomaly may be seen in association with other cardiac conditions, including patent foramen ovale, atrial septal defect, and left ventricular noncompaction (LVNC). LVNC is characterized by increased trabeculation within the left ventricular apex. Echocardiography is often used to diagnose LVNC; however, magnetic resonance (MR) imaging offers superior characterization of the myocardium. We report a case of sudden cardiac death in a patient with Ebstein's anomaly with unrecognized LVNC noted on post mortem examination with screening documenting the presence of LVNC in one of the patient's twin sons.
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McGee et al. (2015) studied this question.
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