The current echocardiographic diagnostic criteria for noncompaction cardiomyopathy (NCC) have variable sensitivity and low specificity. Moreover, there are limited data on the use of myocardial deformation imaging for early detection of myocardial dysfunction in children with NCC. We describe left ventricular (LV) deformation patterns in children with NCC, with the goal of identifying a potential diagnostic pattern. We prospectively enrolled 30 children with NCC (47% male; mean age 7.2 years) and 30 age- and gender-matched controls. Extent and severity of non compaction in each segment were evaluated in LV 16-segment model. Regional (base, mid and apex) and segmental (16 segments) longitudinal strain (LS), circumferential strain (CS) and radial strain (RS) were measured using speckle tracking echocardiography. In all patients with NCC, regional and segmental CS and RS at the apex were significantly decreased compared to controls (CS apex: - 19.2 ± 5.4% vs. - 30.2 ± 6.9%, p 50% and controls (sensitivity: 87%, specificity 79%, AUC 0.88, p
The use of untwisting rate as a novel index of LV diastolic function in clinical practice has been limited due to its tedious and time-consuming analysis. Therefore, we simplify the untwist measurement by only measuring the LV apex's recoil rate and validating and applying peak apical recoil rate (PARR) as an index of diastolic dysfunction (DD) in pediatric subjects during increased and decreased lusitropic states. We recruited 153 healthy subjects (mean age 13.8 ± 2.9 years), of whom 48 performed straight leg raising exercise and an additional 46 patients (mean 8.4 ± 5.6 years) with documented pulmonary capillary wedge pressures (PCWP) (validation cohort). In addition, we studied 16 dilated cardiomyopathy patients (mean age 9.5 ± 6.3 years) (application cohort). PARR and isovolumic relaxation time (IVRT) were compared to PCWP. Both PARR and PARR normalized by heart rate (nPARR) were excellent in detecting patients with PCWP ≥ 12 mmHg and greatly superior to IVRT in this respect (AUC: 0.98, 95% CI [0.96, 1.0] vs. AUC: 0.7 95%CI [0.54,0.86]). In DCM patients, PARR and nPARR were greatly decreased compared to controls (- 38.6 ± 18.6º/s vs - 63.1 ± 16.3º /s, p