Search

Background: Children with single-right ventricle anomalies such as hypoplastic left heart syndrome (HLHS) have left ventricles of variable size and function. The impact of the left ventricle on the performance of the right ventricle and on survival remains unclear. The aim of this study was to identify whether left ventricular (LV) size and function influence right ventricular (RV) function and clinical outcome after staged palliation for single-right ventricle anomalies., Methods: In the Single Ventricle Reconstruction trial, echocardiography-derived measures of LV size and function were compared with measures of RV systolic and diastolic function, tricuspid regurgitation, and outcomes (death and/or heart transplantation) at baseline (preoperatively), early after Norwood palliation, before stage 2 palliation, and at 14 months of age., Results: Of the 522 subjects who met the study inclusion criteria, 381 (73%) had measurable left ventricles. The HLHS subtype of aortic atresia/mitral atresia was significantly less likely to have a measurable left ventricle (41%) compared with the other HLHS subtypes: aortic stenosis/mitral stenosis (100%), aortic atresia/mitral stenosis (96%), and those without HLHS (83%). RV end-diastolic and end-systolic volumes were significantly larger, while diastolic indices suggested better diastolic properties in those subjects with no left ventricles compared with those with measurable left ventricles. However, RV ejection fraction was not different on the basis of LV size and function after staged palliation. Moreover, there was no difference in transplantation-free survival to Norwood discharge, through the interstage period, or at 14 months of age between those subjects who had measurable left ventricles compared with those who did not., Conclusions: LV size varies by anatomic subtype in infants with single-right ventricle anomalies. Although indices of RV size and diastolic function were influenced by the presence of a left ventricle, there was no difference in RV systolic function or transplantation-free survival on the basis of LV measures., (Crown Copyright © 2018. Published by Elsevier Inc. All rights reserved.)

Background: Pediatric heart transplant recipients are at risk for increased left ventricular (LV) diastolic stiffness. However, the noninvasive evaluation of LV stiffness has remained elusive in this population. The objective of this study was to compare novel echocardiographic measures of LV diastolic stiffness versus gold-standard measures derived from pressure-volume loop (PVL) analysis in pediatric heart transplant recipients., Methods: Patients undergoing left heart catheterization were prospectively enrolled. PVLs were obtained via conductance. The end-diastolic pressure-volume relationship was obtained via balloon occlusion. The stiffness constant, β, was calculated. Echocardiographic measures of diastolic function were derived from spectral and tissue Doppler and two-dimensional speckle-tracking. Ventricular volumes were measured using three-dimensional echocardiography. The novel echocardiographic estimates of ventricular stiffness included E:e'/end-diastolic volume (EDV) and E:early diastolic strain rate/EDV., Results: Of 24 children, 18 were heart transplant recipients. Six control patients had hemodynamically insignificant patent ductus arteriosus or coronary fistula. The mean age was 9.1 ± 5.6 years. Median end-diastolic pressure was 9 mm Hg (interquartile range, 8-13 mm Hg). Lateral E:e'/EDV (r = 0.59, P < .01), septal E:e'/EDV (r = 0.57, P < .01), and (E:circumferential early diastolic strain rate)/EDV (r = 0.54, P < .01) correlated with β. Lateral E:e'/EDV displayed a C statistic of 0.93 in detecting patients with abnormal LV stiffness (β > 0.015 mL -1 ). A lateral E:e'/EDV of >0.15 mL -1 had 89% sensitivity and 93% specificity in detecting an abnormal β., Conclusions: Echocardiographic estimates of ventricular stiffness may be accurate compared with the gold standard in pediatric heart transplant recipients. The clinical usefulness of these noninvasive measures in assessing LV stiffness merits further study in children., (Copyright © 2017 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Background: Multiple echocardiographic methods are used to measure left ventricular size and function. Clinical management is based on individual evaluations and longitudinal trends. The Pediatric Heart Network VVV study (Ventricular Volume Variability) in pediatric patients with dilated cardiomyopathy has reported reproducibility of several of these measures, and how disease state and number of beats impact their reproducibility. In this study, we investigated the impact of observer and sonographer variation on reproducibility of dimension, area, and volume methods to determine the best method for both individual and sequential evaluations., Methods and Results: In 8 centers, echocardiograms were obtained on 169 patients prospectively. During the same visit, 2 different sonographers acquired the same imaging protocol on each patient. Each acquisition was analyzed by 2 different observers; first observer analyzed the first acquisition twice. Intraobserver, interobserver, interacquisition, and interobserver-acquisition (different observers and different acquisition) reproducibility were assessed on measurements of left ventricular end-diastolic dimension, area, and volume. Left ventricular shortening fraction, ejection fraction, mass, and fractional area change were calculated. Percent difference was calculated as (interobservation difference/mean)×100. Interobserver reproducibility for both acquisitions was better for both volume and dimension measurements ( P ≤0.002) compared with area measurements, whereas intraobserver, interacquisition (for both observers), and interobserver-acquisition reproducibilities (for both observer-acquisition sets) were best for volume measurements ( P ≤0.01). Overall, interobserver-acquisition percent differences were significantly higher than interobserver and interacquisition percent differences ( P <0.001)., Conclusions: In pediatric patients with dilated cardiomyopathy, compared with dimension and area methods, left ventricular measurements by volume method have the best reproducibility in settings where assessment is not performed by the same personnel., Clinical Trial Registration: URL: https://www.clinicaltrials.gov. Unique identifier: NCT00123071., (© 2017 American Heart Association, Inc.)

Introduction: Single-ventricle patients with elevated pulmonary vascular resistance (PVR) or end-diastolic pressure (EDP) are excluded from undergoing total cavopulmonary connection (TCPC). However, a subset of patients deemed to be at acceptable risk experience prolonged length of stay (LOS) after TCPC. Routine assessment of ventricular function has been inadequate in identifying these high-risk patients. Speckle-tracking echocardiography (STE) is a novel method for assessment of myocardial deformation that may be useful in single-ventricle patients. The aim of this study was to perform a contemporary preoperative risk assessment for prolonged LOS to determine whether STE improves risk stratification before TCPC., Methods: Our single institution's perioperative data were retrospectively collected. The primary outcome was postoperative LOS >14 days. Longitudinal and circumferential STE deformation measures were analyzed on echocardiograms obtained during preoperative catheterization. Patient-specific, echocardiographic, and catheterization data were included in multivariable logistic regression. Receiver operating characteristic area under the curves (AUC) were analyzed., Results: From 2007 to 2014, 135 patients who underwent TCPC were included in the analysis. The median LOS was 11 (IQR 9-14) days. The PVR (P < .01) and circumferential strain rate (CSR) (P < .01) were the only variables independently associated with LOS >14 days. For every 0.1 s -1 CSR increased, there was a 20% increased odds of prolonged LOS. The AUC for CSR was 0.70. The AUC for PVR and EDP combined was 0.68. The AUC for PVR, EDP, and CSR combined was 0.73., Conclusion: Preoperative CSR is independently associated with LOS >14 days and improves preoperative risk stratification in patients undergoing TCPC., (Copyright © 2017 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

The accuracy of echocardiographic measures of right ventricular (RV) diastolic function has been sparsely studied. Our objective was to evaluate the correlation between echocardiographic and reference standard measures of RV diastolic function derived from micromanometer pressure analysis before and after preload alteration in children. Echocardiograms and micromanometer pressure analyses were prospectively performed before and after fluid bolus in children undergoing right heart catheterization. The isovolumic relaxation time constant (τ) and end-diastolic pressure (EDP) were measured. Conventional and speckle-tracking echocardiographic (STE) parameters of RV systolic and diastolic function were assessed. Normal saline bolus was given to increase RV EDP by 20 %. Twenty-eight studies were performed in 22 patients with congenital heart disease or postheart transplantation. Mean age was 8.7 ± 6.1 years. RV longitudinal early diastolic strain rate (EDSR) correlated with τ before (r = 0.57, p = 0.001) and after fluid bolus (r = 0.48, p = 0.008). No conventional echocardiographic measures correlated with τ both before and after fluid bolus. Multiple regression analysis revealed RV EDSR and LV circumferential EDSR as independent predictors of RV τ. There were no independent predictors of EDP. RV EDSR appears to correlate with the reference standard measure of early active ventricular relaxation in children at baseline and after changes in preload. Conventional echocardiographic measures of diastolic function were not predictive of diastolic function after preload alteration. Future studies should assess the prognostic significance of STE measures of diastolic function in this population.

Echocardiography is frequently performed under anesthesia during procedures such as cardiac catheterization with EMB in pediatric HTx recipients. Anesthetic agents may depress ventricular function, resulting in concern for rejection. The aim of this study was to compare ventricular function as measured by echocardiography before and during GA in 17 pediatric HTx recipients. Nearly all markers of ventricular systolic function were significantly decreased under GA, including EF (-4.2% ±1.2, P < .01) and RV FAC (-0.05 ± 0.02, P = .04). Subjects in the first post-transplant year (n = 9) trended toward a more significant decrease in EF vs those beyond the first post-transplant year (n = 8; -6.0% ±1.2 vs -2.1 ± 2.0, P = .1). This information quantifies a decline in biventricular function that should be expected in pediatric HTx recipients while under GA and can assist the transplant clinician in avoiding unnecessary treatment of transient GA-induced ventricular dysfunction., (© 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background: The accuracy of echocardiography in evaluating left ventricular contractility has not been validated in children. The objective of this study was to compare echocardiographic measures of contractility with those derived from pressure-volume loop (PVL) analysis in children., Methods: Patients with relatively normal loading conditions undergoing routine left heart catheterization were prospectively enrolled. PVLs were obtained via conductance catheters. The gold-standard measure of contractility, end-systolic elastance (Ees), was obtained via balloon occlusion of one or both vena cavae. Echocardiograms were performed immediately after PVL analysis under the same anesthetic conditions. Single-beat estimations of echocardiographic Ees were calculated using four different methods. These estimates were calculated using a combination of noninvasive blood pressure readings, ventricular volumes derived from three-dimensional echocardiography, and Doppler time intervals., Results: Of 24 patients, 18 patients were heart transplant recipients, and six patients had small patent ductus arteriosus or small coronary fistulae. The mean age was 9.1 ± 5.6 years. The average invasive Ees was 3.04 ± 1.65 mm Hg/mL. Invasive Ees correlated best with echocardiographic Ees by the method of Tanoue (r = 0.85, P < .01), with a mean difference of -0.07 mm Hg/mL (95% limits of agreement, -2.0 to 1.4 mm Hg/mL)., Conclusions: Echocardiographic estimates of Ees correlate well with gold-standard measures obtained via conductance catheters in children with relatively normal loading conditions. The use of these noninvasive measures in accurately assessing left ventricular contractility appears promising and merits further study in children., (Copyright © 2016 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)