Left ventricular noncompaction is a rare structural cardiomyopathy that can occur isolated or in relationship with other conditions, mainly with musculoskeletal diseases or congenital heart defects. The association of left ventricular noncompaction and connective tissue disorders, including systemic lupus erythematosus, was scarcely described in the literature. Reported cases are, more likely, cardiomyopathies mimicking left ventricular noncompaction or transient left ventricular noncompaction with ventricular function improving after appropriate treatment.
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