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

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%.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.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.Left Ventricular Non-Compaction (LVNC) is morphologically characterized by numerous prominent trabeculations and deep intertrabecular recesses [1]. It is also known as "spongy myocardium" or "persistent embryonic myocardium", and it is probably secondary to an arrest in the normal process of myocardial compaction during fetal life.LVNC may be present as an isolated finding (isolated LVNC) or in association with other congenital heart or neuromuscular disorders [2,3]. Distribution of left ventricular segments with non-compaction (NC) can vary from one patient to another, but it often involves the apical, mid-inferior, and mid-lateral myocardial segments [4]. The clinical manifestations, including age of onset, are variable. Functional cardiac status may range from absence of symptoms to severe cardiac disability, sometimes leading to heart transplantation