Cardiomyopathy mimicking left ventricular noncompaction in a patient with lupus nephritis

Abstract

A 37-year-old female patient was admitted with exertional dyspnea. Her serum creatinine was 2.4 mg/dL and anti-nuclear antibody was positive in a titer of 1/320. Renal biopsy revealed diffuse proliferative lupus nephritis. Echocardiography and cardiac magnetic resonance (MR) imaging showed increased apical trabeculations compatible with left ventricular noncompaction (LVNC), which is a rare genetic cardiomyopathy. The patient expressed a marked improvement in exertional dyspnea after the immune-suppressive treatment for systemic lupus erythematosus (SLE). Control echocardiography revealed a significant increase of ejection fraction. SLE may cause a kind of cardiomyopathy with high resemblance to LVNC.Discrimination of these two similar clinical entities is important because SLE-induced cardiomyopathy is potentially reversible after the immune-suppressive treatment for SLE.

Left Ventricular noncompaction in a patient with systemic lupus erythematosus

“…In previously reported cases the proposed imaging criteria for LVNC were present, however the final diagnosis was cardiomyopathy resembling noncompaction [6,7] or myocarditis with noncompaction features [10]. These reports also share a pattern of reversibility, with cardiac function improving after aggressive SLE treatment [6,7,10]. In the case we presented, all the Stöllberger and Finesterer [5] echocardiographic criteria were met, and LVNC diagnosis was confirmed by cardiac MRi.…”

“…Furthermore, the initial diagnosis of LVNC was established before the patient presented the first SLE flair. In other cases, the aspect of LVNC was described as part of SLE activity or after initial SLE diagnosis [6,7]. Considering all the available data, we consider LVNC as the most accurate diagnosis in our case, independent of SLE manifestations.…”

“…Pericarditis, myocarditis, nonbacterial endocarditis, coronary artery disease, premature coronary atherosclerosis or congestive heart failure are known to occur in SLE [9]. However, a cardiac manifestation in the form of a cardiomyopathy mimicking LVNC was rarely described in these patients [6,7,10]. In previously reported cases the proposed imaging criteria for LVNC were present, however the final diagnosis was cardiomyopathy resembling noncompaction [6,7] or myocarditis with noncompaction features [10].…”

“…However, a cardiac manifestation in the form of a cardiomyopathy mimicking LVNC was rarely described in these patients [6,7,10]. In previously reported cases the proposed imaging criteria for LVNC were present, however the final diagnosis was cardiomyopathy resembling noncompaction [6,7] or myocarditis with noncompaction features [10]. These reports also share a pattern of reversibility, with cardiac function improving after aggressive SLE treatment [6,7,10].…”

“…LVNC can occur either isolated, or associated with neuromuscular disorders or congenital heart defects [1]. Case reports describing the link between left ventricular noncompaction and systemic lupus erythematosus (SLE) conclude with the diagnosis of transient LVNC [6] or myocarditis [7].…”