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).