Cardiac Segmental Strain Analysis in Pediatric Left Ventricular Noncompaction Cardiomyopathy

Abstract

Background: Left ventricular noncompaction cardiomyopathy (LVNC) is characterized by prominent left ventricular trabeculations, often leading to myocardial dysfunction and death or heart transplantation. Although diagnostic echocardiographic criteria exist for LVNC, segmental speckle-tracking strain patterns have not been described in pediatric LVNC. The objectives of this study were to characterize segmental noncompaction, evaluate segmental speckle-tracking strain, and characterize peak strain values in children with LVNC with adverse clinical outcomes. Methods: Echocardiographic noncompaction/compaction ratios and segmental radial, circumferential, and longitudinal strain were measured retrospectively in children with LVNC (January 2000 to June 2013). Segmental strain of the cohort was compared with that in control subjects matched by age and body surface area. Results: One hundred one pediatric patients with LVNC were included (median age 2.8 years; range, 0-19.4 years); 71 patients survived (benign group) and 30 patients did not survive or required heart transplantation (adverse group). The adverse group was younger at diagnosis (P = .03), with lower ejection fractions (P < .0001) compared with the benign group. Both groups demonstrated increased noncompaction/compaction ratios within apical segments, with significantly increased involvement in antero-and inferolateral midpapillary segments in the adverse group (P < .005). Radial, circumferential, and longitudinal strain of nearly all left ventricular segments were significantly decreased in the adverse compared with the benign group and in pediatric LVNC patients compared with control subjects.

Changes in strain parameters at different deterioration levels of left ventricular function: A cardiac magnetic resonance feature-tracking study of patients with left ventricular noncompaction

“…In studying the mean segmental strain values of the apical, mid and basal regions, we found an increase in segmental strain values from base to apex, which has been previously described in healthy adults; however, our results were slightly different, namely, the mid part had the best longitudinal strain value, which may be due to the small number of included patients [34]. Previous CMR and speckle-tracking echocardiography studies have mentioned that these regional strain differences disappear in patients with DCM, and in accordance with this, we also observed that the decrease in segmental strains affected all three parts of the LV in LVNC patients with a reduced EF [27,35]. Furthermore, an opposite pattern was exhibited in this LVNC patient group; namely, the apical longitudinal strain became the worst of the three parts, which was previously described in different DCM and LVNC populations with a severely reduced EF [11,12].…”

“…In our study, we found that the GLS, GCS and GRS gradually decreased as LV function worsened. The decreased strains in all three directions suggest that LVNC is a complex structural disease that affects not only the noncompacted but the compacted layer as well [27,28].…”

Left ventricular mechanics and cardiovascular outcomes in non-compaction phenotype

“…Recent studies showed that longitudinal strain of the apical region was impaired in LVNC even when compared to dilated and hypertrophic cardiomyopathies [13][14][15][16]23,24]. Such impairment of longitudinal strain was noticed in compacted and non-compacted segments of LVNC ventricles [13,23,24]. Similar results were obtained in a paediatric population including reduced circumferential and radial strain [23].…”

“…Regional assessment of strain by segmental analysis can be a promising tool to study systolic function in cardiomyopathies such as LVNC. Recent studies showed that longitudinal strain of the apical region was impaired in LVNC even when compared to dilated and hypertrophic cardiomyopathies [13][14][15][16]23,24]. Such impairment of longitudinal strain was noticed in compacted and non-compacted segments of LVNC ventricles [13,23,24].…”

Noncompaction Cardiomyopathy—History and Current Knowledge for Clinical Practice

“…Strain measurements were lowest in those with adverse compared with favorable outcomes. In NCCM, deformation was affected in all regions, including compacted myocardial segments [ 79 ].…”