Highlights the possibility of long-term survival in patients with concurrent Ebstein's anomaly and left ventricular noncompaction.
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Benign Ebstein's with LV noncompaction may permit survival to advanced age; leaves open natural history and management questions in this rare overlap.
Ebstein's anomaly is a rare congenital cardiac disease characterized by apical displacement of the tricuspid valve with decreased right ventricular function. Left ventricular noncompaction is a cardiomyopathy characterized by the presence of numerous and prominent trabeculations together with deep intertrabecular recesses in a portion of the ventricular wall, principally at the lateral and apical level. We describe the very rare case of a patient living for 78 years with a benign form of Ebstein's anomaly and left ventricular noncompaction cardiomyopathy with slightly reduced systolic function.
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Tourmousoglou et al. (2018) studied this question.
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