P843Left ventricular twist for the diagnosis of left ventricular non-compaction in children and young adults

Abstract

Background: The incidence of left ventricular non-compaction (LV-NC) in children and young patient is significant. Remarkably, ventricular arrhythmias and sudden cardiac death are more frequent in infants than in adults with LV-NC. Recent studies demonstrated, in adults patients (pts) with LV-NC, impaired left ventricular (LV) twist and the presence of rigid body rotation (RBR), characterized by LV base and apex rotating in the same direction. However, cardiac mechanics and LV twist have not been evaluated in paediatric pts with LV-NC. Purpose(s): To assess LV cardiac mechanics and the prevalence of RBR, by using 2-D speckle tracking echocardiography (STE), in children and young pts with LV-NC and LV hypertrabeculation (HTr), as diagnosed by cardiac magnetic resonance (CMR). Method(s): The study population included 47 children and young adults (age 11.1+/-5 yrs, age range: 0-17 yrs) referred for suspected LV-NC. All pts underwent CMR at 1.5-T. 23 patients fulfilled the CMR diagnostic criteria of LV-NC (LVNC group), while 24 pts with HTr did not meet LV-NC diagnostic criteria (LV-HTr group). 47 age- and sex-matched healthy volunteers were included as control group (CTRL). Complete transthoracic echocardiographic studies and 2-D STE were performed. Global longitudinal (LS), radial (RS), circumferential strains (CS) and LV Twist were assessed by using TomTec (Image Arena4). Result(s): Global and regional LS, CS and RS were all reduced in LV-NC and LV-HTr groups compared to CTRLs (p<0.05), but only global CS (p=0.021) and apical CS (p=0.004) were reduced in LV-NC compared to LV-HTr. The average LV twist of LV-NC was significantly reduced compared to CTRL (p<0.001) and LV-HT (p<0.001). RBR was recognized in 13 (56%) children with LVNC and in 1 (4%) child with LV-HTr. A normal pattern of rotation was observed among all CTRLs. Interestingly, a good correlation was observed between LV Twist and the non-compacted (NC)/compacted (C) ratio of the myocardium (r=0.659; p=0.001). Multivariate analysis demonstrated that LV twist is an independent predictor of LV-NC (P=0.006; coeff.=0.462; standard error=0.168). Finally, the ROC curves, generated to assess the capability of LV-Twist, LVEF, BNP, GLS and GCS to discriminate pts with and without LV-NC, showed the best predictive value for LVTwist (AUC=0.914). Conclusion(s): LV twist is a useful parameter able to support, with good predictive value, the diagnosis of LV-NC in children and young adults.