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However, persistent neurohormonal signaling and increased wall stress leads to hypertrophy of surviving cardiomyocytes, dilation of the left ventricle, and deterioration of myocardial contractility and eventually these changes can progress to heart failure (HF) or death [ 5, 6 ].

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This review will focus on clinical manifestations and diagnosis of LVNC as an isolated disorder distinct from other clinical settings in which non-compacted myocardium may be seen in association with other cardiac and noncardiac abnormalities. Management and prognosis of isolated LVNC is discussed separately.

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