Cardiovascular magnetic resonance findings in a pediatric population with isolated left ventricular non-compaction

Abstract

Background: Isolated Left Ventricular Non-compaction (LVNC) is an uncommon disorder characterized by the presence of increased trabeculations and deep intertrabecular recesses. In adults, it has been found that Ejection Fraction (EF) decreases significantly as non-compaction severity increases. In children however, there are a few data describing the relation between anatomical characteristics of LVNC and ventricular function. We aimed to find correlations between morphological features and ventricular performance in children and young adolescents with LVNC using Cardiovascular Magnetic Resonance (CMR). Methods: 15 children with LVNC (10 males, mean age 9.7 y.o., range 0.6 -17 y.o.), underwent a CMR scan. Different morphological measures such as the Compacted Myocardial Mass (CMM), Non-Compaction (NC) to the Compaction (C) distance ratio, Compacted Myocardial Area (CMA) and Non-Compacted Myocardial Area (NCMA), distribution of NC, and the assessment of ventricular wall motion abnormalities were performed to investigate correlations with ventricular performance. EF was considered normal over 53%. Results: The distribution of non-compaction in children was similar to published adult data with a predilection for apical, mid-inferior and mid-lateral segments. Five patients had systolic dysfunction with decreased EF. The number of affected segments was the strongest predictor of systolic dysfunction, all five patients had greater than 9 affected segments. Basal segments were less commonly affected but they were affected only in these five severe cases. Conclusion: The segmental pattern of involvement of non-compaction in children is similar to that seen in adults. Systolic dysfunction in children is closely related to the number of affected segments.

Combination of Left Ventricular Noncompaction and Partial Atrioventricular Canal Defect in a 21-Year-Old Male: A Case Report

“…Poor acoustic windows could mislead even experienced echocardiographers the diagnosis of this disease, resulting in an erroneous label such as dilated or hypertrophic cardiomyopathy 9. Cardiovascular magnetic resonance (CMR) has become the method of choice to confirm or rule out LVNC and may outperform echocardiography in defining the morphology and extension of myocardial noncompaction 7,10. This was demonstrated in a study by Yousef et al11 CMR can also give valuable diagnostic and prognostic information about the disease by depicting fibrosis on delayed contrast-enhanced images 8…”

Differentiation of patients with two-dimensional echocardiography false positive non-compaction of ventricular myocardium by contrast echocardiography

“…The results of this study confirm that LVO enhanced imaging of intracardiac structure is helpful to differentiate and diagnose suspected patients with NVM. Normal left ventricular apex may also have noncompact structure (Uribe et al, 2012). Depending on the ratio of noncompact myocardial thickness to subepicardial compact myocardial thickness, abnormal myocardial trabeculae may also occur, but the shape of normal myocardial trabeculae is different from that of NVM patients.…”

Isolated Noncompaction of the Left Ventricle in Adults

“…It is also generally recommended that asymptomatic relatives of affected individuals be screened using echocardiography (22). (23)(24)(25)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35).…”