An unusual pattern of late gadolinium enhancement (LGE) in a
bi-ventricular non-compaction patient
Abstract
Myocardial non-compaction (NC) is a rare genetically heterogeneous
cardiomyopathy which is characterized by excessively prominent
ventricular trabeculations with deep intertrabecular recesses. It is
commonly believed to be due to an intrauterine arrest of the
endomyocardial morphogenesis. Left ventricle (LV) is the usual site of
affection, but very rarely right ventricular (RV) or both ventricles may
be involved. It can be isolated or associated with other cardiac
diseases or neuromuscular disorders. It has a wide spectrum of clinical
manifestations that range from asymptomatic status, progressive
ventricular dysfunction, arrhythmias and congestive heart failure that
have been found to be associated with myocardial fibrosis. Cardiac
Magnetic resonance (CMR) is the gold standard non-invasive modality for
the diagnosis of cardiomyopathies. In NC cases, late gadolinium
enhancement (LGE) of the trabeculated layer or mild fibrosis of the
compacted layer was previously described in the literature, however
extensive fibrosis in the compacted layer only with no fibrosis at the
trabeculated layer was not described before. We report a male patient
presented with heart failure symptoms, echocardiogram showed severe LV
and RV systolic dysfunction with prominent bi-ventricular
trabeculations. CMR confirmed the diagnosis of bi-ventricular
non-compaction with peculiar pattern extensive fibrosis of the compacted
layer of LV in LGE. To the best of our knowledge, our case is the first
case to report extensive fibrosis in the compacted layer only with no
fibrosis at the trabeculated layer.