Cardiovascular magnetic resonance findings in a pediatric population with isolated left ventricular non-compaction
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RESEARCH
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Cardiovascular magnetic resonance findings in a pediatric population with isolated left ventricular non-compaction Sergio Uribe1,3*, Lina Cadavid1, Tarique Hussain5, Rodrigo Parra1, Gonzalo Urcelay4, Felipe Heusser4, Marcelo Andía5, Cristian Tejos2,3 and Pablo Irarrazaval2,3
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. Keywords: isolated left ventricular non-compaction, cardiomyopathy, spongy myocardium, ventricular performance, prominent trabeculations
Background 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. * Correspondence: [email protected] 1 Radiology Department, Pontificia Universidad Católica de Chile. Marcoleta 367, Hospital Clinico Universidad Catolica, Santiago 8330024, Santiago Chile Full list of author information is available at the end of the article
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 anoth
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