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

Abstract

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 to 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, though 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 [1] 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.

Left Ventricular Noncompaction—A Systematic Review of Risk Factors in the Pediatric Population

“…Multiple diagnostic criteria have been outlined, with those according to Jenni et al [ 25 ] (presence of multiple trabeculations, deep intertrabecular recesses, and thickness of the noncompacted to compacted myocardium in systole (NC/C) > 2:1) are the most commonly used in the pediatric and adult populations; thus, it is of note that in the original criteria congenital heart diseases excluded the diagnosis of LVNC [ 25 , 37 ]. Some authors suggest that in children, the criteria according to Chin et al [ 30 ] (ratio between the epicardial surface to trabeculation base and epicardial surface to trabeculation peak in end-diastole [X/Y]) are more reliable [ 38 ]. Other diagnostic criteria that were found in the reviewed literature included those according to Stollberger et al [ 31 ] (presence of >3 trabeculations at end-diastole, which moved synchronously with the compacted myocardium and presence of perfusion on color Doppler of the intertrabecular spaces) and Ichida et al (NC/C > 2 measured at end-diastole, deep intertrabecular recesses with blood flow visualized on color Doppler)—the latter were mentioned in publications by Hirono et al [ 12 , 13 ].…”

Prevalence of Left Ventricular Noncompaction in Newborns

“…A ratio between noncompact and compact (NC:C) myocardium ≥2 has been suggested as a diagnostic criterion. 8,9 Several diagnostic imaging criteria for isolated LVNC have been proposed but none are considered gold standard. 3,8,[10][11][12][13][14][15] Finally, the prevalence of LVNC by echocardiography in an unselected population is uncertain.…”

Left Ventricular Non-Compaction: Review of the Current Diagnostic Challenges and Consequences in Athletes

“…The first criterion was developed by Chin et al and defines LVNC as an epicardial compacted myocardium layer (Χ) to endocardial non-compacted layer ratio (Y) ≤0.5 in the end-diastole [ 14 ]. Although this criterion has been shown to have the greatest sensitivity and specificity amongst the four 2D-echo criteria, it is not widely used in clinical practice [ 22 ]. The next criterion was developed by Jenni et al and defines LVNC as a non-compacted to compacted myocardial ratio >2.0 on short-axis images taken in end-systole [ 15 ].…”