Background
Left ventricular noncompaction (LVNC) is a rare genetic cardiomyopathy with a structural abnormality of the left ventricular (LV) myocardium of unknown cause (1), characterized by excessively prominent trabeculations and associated deep recesses that communicate with the ventricular cavity (2). Heart failure, systemic embolism and arrhythmias are cardinal findings (3). LVNC occurs isolated or in association with neuromuscular disorders (NMDs) (4).LVNC can rarely be asymptomatic and manifest later in life following other unrelated conditions that lead to exacerbation in contractile dysfunction (5). However, its association with jSLE was rarely described (6,7). jSLE is known to be associated with a higher incidence of arthritis, nephritis, haematologic and neurologic manifestations than that seen in adult-onset disease (8). In particular, adolescent-onset systemic lupus erythematosus is associated with more aggressive disease (9). Overall, less than 10% of jSLE patients have severe cardiovascular system involvement at presentation (10). It is an important manifestation as it is now considered a leading cause of morbidity and mortality in jSLE (11). Pericardium, myocardium, endocardium, heart valves, coronary vessels, and cardiac autonomic functions may be affected in jSLE patients (12).