Comparison of Echocardiographic Diagnostic Criteria of Left Ventricular Noncompaction in a Pediatric Population

Pediatr Cardiol. 2017 Oct;38(7):1493-1504. doi: 10.1007/s00246-017-1691-9. Epub 2017 Aug 3.


Background: There is controversy regarding the best echocardiographic diagnostic criteria for left ventricular noncompaction (LVNC). We assessed the diagnostic utility and reproducibility of the previously proposed echocardiographic diagnostic criteria in a pediatric population using a segmental approach.

Methods: Echocardiograms were matched for patients with and without a clinical diagnosis of LVNC. Blinded reviews of echocardiograms measured (1) depths of intertrabecular recesses (X/Y), (2) noncompaction-to-compaction ratio (NC/C), and (3) number of trabeculations, using a segmental approach. Measurements were analyzed for area under the receiver operating characteristic curves (AUC), sensitivity, and specificity.

Results: There were 30 echocardiograms in the initial cohort (15 LVNC cases, 15 controls). Median age was 1.7 years (IQR 0.2-6.9 years) and systolic function was decreased in 40%. Comparison of diagnostic criteria demonstrated the best interrater agreement and AUC with an X/Y ratio measured in end-diastole in the parasternal short axis in the apical anterolateral segment (κ 0.72, CI 0.43-1.00, p value <0.001), yielding 100% sensitivity and 70-86% specificity, among readers. The least predictive and reproducible method was the NC/C ratio. A validation cohort confirmed the superiority of the X/Y ratio, although the interrater agreement and AUC decreased.

Conclusion: Measurements according to existing LVNC diagnostic criteria vary by echocardiographic view and segment. Modification of the Chin et al. criteria (Circulation 82:507-513, 1990) using an X/Y ratio <0.5 had the greatest interrater reliability and predictive validity when measured in end-diastole in the parasternal short axis in the apical anterolateral segment. The NC/C ratio had the lowest reliability and predictive validity.

Keywords: Cardiomyopathy; Echocardiography; Left ventricular noncompaction; Pediatrics; Segment model.

MeSH terms

  • Area Under Curve
  • Case-Control Studies
  • Child
  • Child, Preschool
  • Echocardiography / methods*
  • Female
  • Humans
  • Infant
  • Isolated Noncompaction of the Ventricular Myocardium / diagnostic imaging*
  • Isolated Noncompaction of the Ventricular Myocardium / physiopathology
  • Male
  • ROC Curve
  • Reproducibility of Results
  • Retrospective Studies
  • Sensitivity and Specificity