Lack of MR late-enhancement in left ventricular non-compaction in infants and young children

Abstract Objective Noncompaction cardiomyopathy or left ventricular noncompaction is a rare disease that is probably underdiagnosed. The diagnosis is reached by echocardiography, although MRI provides additional morphological and functional information. Late MRI enhancement is a hallmark of the disease that reflects fibrosis or infarction of affected areas in adults and older children. We aimed to review the presence of late enhancement in left ventricular noncompaction in infants and young children. Material and methods We found five very young patients (mean age, 29.4 months; range 1 month to 5 years) with left ventricular noncompaction in our cardiac MRI database. We reviewed the morphological and functional findings, including late enhancement after the administration of contrast material. Results All patients had been previously diagnosed by echocardiography. At MRI, the morphological findings and the ratio of noncompacted myocardium to compacted myocardium were compatible with left ventricular noncompaction. None of the cases showed late enhancement after the administration of contrast material. Conclusions Unlike in adults and older children, none of the infants and young children we studied had late enhancement. This finding might reflect the natural history of the disease, with subendocardial fibrosis developing over time.