ABSTRACT
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. We present the case of a 23-year-old woman admitted for cardiac evaluation because of ECG abnormalities observed during a routine check-up. Echocardiography showed severe systolic and diastolic dysfunction, diffuse hypokinesis and hypertrabeculation, suggestive of left ventricular non-compaction. Cardiac magnetic resonance imaging confirmed the diagnosis. She later presented with specific clinical and biological parameters and was diagnosed with systemic lupus erythematosus. Corticosteroid and hydroxychloroquine treatment induced general improvement of signs and symptoms, but no recovery of cardiac function.
