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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: No data exist regarding the temporal trends in utilization of transthoracic echocardiography (TTE) in an outpatient pediatric cardiology setting. This study evaluates the trends in utilization of TTE for common diagnoses known to have low diagnostic yield and the factors influencing these trends., Methods: Patients evaluated at our pediatric cardiology clinics from January 2000 to December 2014 and discharged with final diagnoses of innocent murmur, noncardiac chest pain, benign syncope, and palpitations were included. Variables collected retrospectively included patient age, sex, insurance type, distance from clinic, and ordering physician's years of experience since fellowship., Results: Of the 74,881 patients seen by 35 physicians, 36,053 (48.1%) had a TTE. The TTE rates increased from the beginning of 2000 to the end of 2004 (5.2% per year; P < .001) and then steadily declined until the end of 2014 (1.6% per year; P < .001). Utilization for noncardiac chest pain remained the highest, and use in infants increased significantly during the study period (P < .001). After adjusting for all other factors, the following variables were associated with higher TTE utilization: younger age, males, Medicaid insurance, increased distance from clinic, and being seen by less experienced physicians. Temporal trends persisted after adjusting for all these factors., Conclusions: After an initial surge in TTE utilization from 2000 to 2004, there was a steady decline. This study identifies some important factors influencing these trends. This information could help design quality interventions, but additional factors need to be explored since the trends persist despite adjusting for these factors., (Copyright © 2016 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Background: Qualitative assessment of ventricular septal flattening is commonly used in pediatric patients with pulmonary hypertension (PH) who lack adequate tricuspid regurgitation (TR) Doppler signal. We sought to determine the relation between quantitative measures of septal flattening including the eccentricity index (EIs) and a novel marker, the septal flattening angle (SFA) with right ventricular systolic pressure (RVSP)., Methods: Subjects (≤18 years) with an anatomically normal heart, an adequate TR signal to obtain a peak velocity, and a simultaneous systemic systolic blood pressure (SBP) was included. RVSP was derived using TR gradient. Eccentricity index (EIs) and the SFA in systole were measured offline and correlated with RVSP/SBP., Results: Of the 108 subjects, RVSP/SBP was < 50% in 77 and ≥ 50% in 31. In those with RVSP/SBP ≥50%, the median SFA was significantly lower (7.4° vs. 22°, p < 0.0001), and the median EIs was higher (1.61 vs. 1.07, p < 0.0001). SFA and EIs had a significant correlation with RVSP/SBP (rs = -0.70 and 0.61, respectively). Area under the curve was higher for SFA compared to EIs (0.92 and 0.85, respectively). The sensitivity and specificity of SFA for predicting an RVSP/SBP ≥ 50% using a cut point of 16° was 84% and 95% and for an EIs cut point of 1.35 was 74.2% and 96.1%, respectively., Conclusion: Septal flattening angle and EIs are quantitative measures of ventricular septal flattening that correlate well with RVSP/SBP and should be considered more routinely in clinical practice, especially in patients with inadequate TR Doppler signal., (© 2015, Wiley Periodicals, Inc.)

Background: Left ventricular mass index (LVMI) is a surrogate of left ventricular hypertrophy and a predictor of cardiac morbidity and mortality in adults with hypertension. LVMI has not been linked to cardiovascular endpoints in children. The aim of this study was to identify an association between elevated LVMI and echocardiographic markers of systolic and diastolic function. Methods: The study was a retrospective review of chronic dialysis patients from June 1995 to December 2009 at a single tertiary care children's hospital. The upper limit cutoffs for LVMI were set at >38.6 g/m, >51 g/m, and by age and sex-based normative values. Sixty-three patients (mean age 14.1 years, 56% males) were enrolled in the study, with a total of 287 echocardiograms. Results: Post-dialysis hypertension was associated with elevated LVMI in both the >51 g/m [odds ratio (OR) 2.9, 95% confidence interval (CI) 1.5-5.5] and normative (OR 3.4, 95% CI 1.5-7.7) models. Elevated LVMI, when defined by the >51 g/m and normative models, was significantly associated with decreased shortening fraction (OR 4.1, 95% CI 1.7-9.8 and OR 5.4, 95% CI 1.3-22.9, respectively) and increased mitral E wave to lateral mitral tissue Doppler e′ wave velocity ratio (E/e′; OR 3.5, 95% CI 1.1-11.2 and OR 4.5, 95% CI 1.0-21.6, respectively). Conclusions: Elevated LVMI is associated with decreased systolic and diastolic cardiac function, justifying its use as a surrogate of hypertensive cardiomyopathy in children undergoing chronic dialysis. [ABSTRACT FROM AUTHOR]

Supplemental Digital Content is available in the text. Background: Heart size and function in children with single right ventricle (RV) anomalies may be influenced by shunt type at the Norwood procedure. We sought to identify shunt-related differences during early childhood after staged surgical palliations using echocardiography. Methods: We compared echocardiographic indices of RV, neoaortic, and tricuspid valve size and function at 14 months, pre-Fontan, and 6 years in 241 subjects randomized to a Norwood procedure using either the modified Blalock-Taussig shunt or RV-to-pulmonary-artery shunt. Results: At 6 years, the shunt groups did not differ significantly in any measure except for increased indexed neoaortic area in the modified Blalock-Taussig shunt. RV ejection fraction improved between pre-Fontan and 6 years in the RV-to-pulmonary artery shunt group but was stable in the modified Blalock-Taussig shunt group. For the entire cohort, RV diastolic and systolic size and functional indices were improved at 6 years compared with earlier measurements, and indexed tricuspid and neoaortic annular area decreased from 14 months to 6 years. The prevalence of ≥moderate tricuspid and neoaortic regurgitation was uncommon and did not vary by group or time period. Diminished RV ejection fraction at the 14-month study was predictive of late death/transplant; the hazard of late death/transplant when RV ejection fraction was <40% was tripled (hazard ratio, 3.18; 95% CI, 1.41–7.17). Conclusions: By 6 years after staged palliation, shunt type has not impacted RV size and function, and RV and valvar size and function show beneficial remodeling. Poor RV systolic function at 14 months predicts worse late survival independent of the initial shunt type. Clinical Trial Registration: URL: https://www.clinicaltrials.gov. Unique identifier: NCT00115934. [ABSTRACT FROM AUTHOR]

The article describes a continuing medical education (CME) activity in achocardiography.

Left ventricular noncompaction cardiomyopathy (LVNC) is characterized by prominent trabeculations in the left ventricle and has been associated with adverse outcomes; yet, there is difficulty in discerning pathologic trabeculations from normal variation. This study sought to test the hypothesis that a ratio of noncompacted to compacted (NC:C) myocardium of >2.3 in diastole by cardiac magnetic resonance imaging (CMR) is associated with adverse clinical outcome and imaging findings in children and young adults referred for CMR to assess for presence of LVNC cardiomyopathy. A retrospective cohort study was performed of patients who were referred for CMR at our center for suspected LVNC between 1/1/11 and 12/31/21. An NC:C ratio was calculated for each CMR, and patients were classified as CMR+ or CMR− using a NC:C cutoff of >2.3. Demographic, electrocardiographic, echocardiographic, CMR, and clinical outcome data were compared. Fifty-one patients were identified, with 30 (59 %) being CMR+. Patients were 12.9 ± 4.7 years old at the time of CMR and followed for a median of 1.7 (IQR 0.1–4.4) years. Baseline characteristics were similar between the CMR+ and CMR− groups. Of the cohort, 1/51 (2 %) who was CMR−, had a stroke, and 5/51 (10 %) experienced significant arrhythmia or ectopy with no significant difference between the groups. There was no mortality, transplant, or mechanical support. LV systolic dysfunction was noted in 8/51 (16 %) of patients by CMR, with no significant difference between the CMR+ and CMR− groups. There was no correlation between the NC:C ratio and left or right ventricular systolic function by CMR. NC:C ratio, as measured by CMR, alone may be an inadequate marker of pathologic left ventricular trabeculations in children and young adults. • Intermediate outcomes good in children referred for evaluation of LV noncompaction • No differences in outcomes or cardiac testing using a NC:C ratio of 2.3 by CMR • Degree of LV trabeculation may be limited in ability to predict adverse outcomes. [ABSTRACT FROM AUTHOR]