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No method of evaluating transthoracic echocardiograms (TTE) image quality (IQ) has been validated. Furthermore, structural echo lab elements impacting IQ are unknown. We sought to develop and validate a TTE IQ grading tool and determine patient and echo lab features associated with IQ. Ten pediatric echo labs each submitted 50 consecutive new patient TTEs without complex heart disease. For each study, 3 sonographers independently rated IQ and performed routine measurements. IQ scoring used the American College of Cardiology Quality Network (ACC QNet) tool plus a novel Likert scale component. IQ scores were validated against the ability to make measurements and, when all measurements were made, variation between sonographers. After validation, patient and lab factors, from a previously published survey, associated with improved TTE IQ were determined by linear regression. Total IQ score (R 2  = 0.64), ACC QNet (R 2  = 0.29) and the Likert component (R 2  = 0.52) correlated with the total number of measurements made (p < 0.001). For the 236 (52%) TTEs with all measurements, the Likert scale component (OR 0.9, 95% CI 0.82-0.99, p = 0.037) was associated with lower measurement variability. Factors significantly associated with worse IQ scores were higher patient weight, performance by a traveling sonographer, lower annual and inpatient TTE volume, less dedicated teaching/administrative sonographer time, and full-day sessions for reading physicians. A novel TTE IQ scoring tool was created and validated for pediatric TTE. Modifiable structural components of echo labs associated with TTE IQ were identified. This novel IQ tool should guide IQ beyond the ACC QNet score., Competing Interests: Declarations. Conflict of interest: Garick Hill served as a consultant for Ultragenyx. Ethical Approval: Not applicable. Consent to Participate: Not applicable., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Cardiomyopathy is the leading cause of death in boys with Duchenne muscular dystrophy (DMD). While cardiac magnetic resonance (CMR) is routinely used to assess fibrosis and left ventricular (LV) ejection fraction, CMR measures of LV filling and ejection in DMD have not been reported., Methods: Patients with DMD (n=179) and healthy controls (n=96) were prospectively enrolled and underwent CMR. The DMD cohort was followed clinically at multiple institutions, and clinical data were recorded. Standard volumes and functions were calculated, and LV filling and ejection curves were measured from baseline CMR. Multivariable linear regressions were used to compare ventricular filling and ejection measures between groups, adjusting for baseline differences. Cox regressions were used to evaluate the relationship between diastolic function measures and mortality in the DMD cohort., Results: Patients with DMD had significantly smaller stature and ventricular volumes than healthy control patients ( P <0.001). They had lower baseline LV ejection fraction ( P <0.001), though most had normal systolic function. When adjusted for age, sex, heart rate, body surface area, and LV end-diastolic volume, patients with DMD had slower peak filling rates ( P <0.001) and peak ejection rates ( P <0.001), as well as slower time to peak ventricular ejection rate ( P =0.011). When adjusted for heart rate, a lower peak ventricular ejection rate ( P =0.007) and peak filling rate ( P =0.033), normalized to LV end-diastolic volume, were associated with mortality in patients with DMD., Conclusions: Patients with DMD have significantly different baseline CMR filling and ejection indices compared with controls. Some filling indices are associated with mortality and may be useful prognostic measures. Further research is needed in larger cohorts to determine the prognostic value of these differences., Competing Interests: None.

Background: Longitudinal evaluation of allograft diastolic function in paediatric heart transplant recipients is important for early detection of acute rejection, cardiac allograft vasculopathy, and graft dysfunction. Mean diastolic right atrial and pulmonary capillary wedge pressures obtained at catheterisation are the reference standards for assessment. Echocardiography is non-invasive and more suitable for serial surveillance, but individual parameters have lacked accuracy. This study aimed to identify covariates of post-transplant mean right atrial and pulmonary capillary wedge pressures, including B-type natriuretic peptide and certain echocardiographic parameters., Methods: A retrospective review of 143 scheduled cardiac catheterisations and echocardiograms from 56 paediatric recipients transplanted from 2007 to 2011 was performed. Samples with rejection were excluded. Univariate and multivariate linear regression models using backward selection were applied to a database consisting of B-type natriuretic peptide, haemodynamic, and echocardiographic data., Results: Ln B-type natriuretic peptide, heart rate z-score, left ventricular end-diastolic dimension z-score, mitral E/e', and percent interventricular septal thickening in systole were independently associated with mean right atrial pressure. Ln B-type natriuretic peptide, heart rate z-score, left ventricular end-diastolic dimension z-score, left ventricular mass (observed/predicted), and mitral E/e' were independently associated with mean pulmonary capillary wedge pressure. Covariates of B-type natriuretic peptide included mean pulmonary artery and pulmonary capillary wedge pressures, height, haemoglobin, fractional shortening, percent interventricular septal thickening in systole, and pulmonary vascular resistance index., Conclusions: B-type natriuretic peptide and echocardiographic indices of diastolic function were independently related to post-transplant mean right atrial and pulmonary capillary wedge pressures in paediatric heart transplant recipients without rejection.

Background: There are few data on practice patterns and trends for cardiovascular magnetic resonance (CMR) in pediatric and congenital heart disease. The Society for Cardiovascular Magnetic Resonance (SCMR) sought to address this deficiency by performing an international survey of CMR centers., Methods: Surveys consisting of 31 (2014) and 33 (2018) items were designed to collect data on the use of CMR for the evaluation of pediatric and congenital heart disease patients. They were sent to all SCMR members in 2014 and 2018. One response per center was collected., Results: There were 93 centers that responded in 2014 and 83 in 2018. The results that follow show data from 2014 and 2018 separated by a dash. The median annual number of pediatric/congenital CMR cases per center was 183-209. The median number of scanners for CMR was 2-2 (range, 1-8) with 58-63% using only 1.5T scanners and 4-4% using only 3T scanners. The mean number of attending/staff reading CMRs was 3.7-2.6; among them, 52-61% were pediatric or adult cardiologists and 47-38% were pediatric or adult radiologists. The median annual case volume per attending was 54-86. The median number of technologists per center doing CMRs was 4-5. The median scanner time allocated for a non-sedated examination was 75-75 min (range, 45-120). Among the 21 centers responding to both surveys, the mean annual case volume increased from 320 in 2014 to 445 in 2018; 17 (81%) of the centers had an increase in annual case volume. For this subgroup, the median attending/staff per center was 4 in both 2014 and 2018. The median scanner time allotted per study was unchanged at 90 min. The mean time for an attending/staff physician to perform a typical CMR examination including reporting was 143-141 min., Conclusion: These survey data provide a novel comprehensive view of CMR practice in pediatric and congenital heart disease. This information is useful for internal benchmarking, resource allocation, addressing practice variation, quality improvement initiatives, and identifying unmet needs., (© 2021. The Author(s).)