DOI: 10.36347/sjams.2018.v06i11.018

Pages: 4257-4259

Left ventricular noncompaction (LVNC) is a rare genetic cariomyopathy. It is thought to be due to the failure of condensation of the myocardial meshwork of fibers during intrauterine life, leading to persistence of ventricular trabeculations. The major clinical manifestations of LVNC are heart failure, systemic thromboembolism, ventricular arrhythmias, conduction disorders, and neurologic abnormalities. LVNC may be associated with several disorders, especially with neuromuscular disorders. Hypertrabeculation, a cardinal echocardiographic feature of LVNC, might represent a morphological expression of a number of morbidities, nevertheless. The relationship of LVNC with connective tissue disorders such as Systemic lupus erythematosus (SLE) is unknown. We aim to present a case of a patient with SLE who recently showed features compatible with an atypical LVNC. We report a case of a young female with a 5-year history of SLE who developed haematological disease activity and cardiac failure. Echocardiography showed left ventricle dilation, hypertrabeculation/noncompaction, a very low ejection fraction at 25% and pulmonary hypertension. After treatment, all signs of LVNC disappeared in echocardiography.The transitory aspect of the cardiomyopathy made unlikely a “true” LVNC for this patient, but she might have presented a lupus myocarditis with “LVNC-like” features. The occurrence of hypertrabeculated myocardium in patients with SLE warrants further studies.