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