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

Sergio Uribe, Lina Cadavid, Tarique Hussain, Rodrigo Parra, Gonzalo Urcelay, Felipe Heusser, Marcelo Andía, Cristian Tejos, Pablo Irarrazaval

Research output: Contribution to journalArticle

16 Scopus citations

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.

Original languageEnglish (US)
Article number9
JournalJournal of Cardiovascular Magnetic Resonance
Volume14
Issue number1
DOIs
StatePublished - 2012

Keywords

  • cardiomyopathy
  • isolated left ventricular non-compaction
  • prominent trabeculations
  • spongy myocardium
  • ventricular performance

ASJC Scopus subject areas

  • Radiological and Ultrasound Technology
  • Radiology Nuclear Medicine and imaging
  • Cardiology and Cardiovascular Medicine

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