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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 Longitudinal clinical surveillance by transthoracic echocardiography (TTE) is an established practice in children with repaired tetralogy of Fallot (TOF). Non-Invasive Imaging Guidelines recommends a list of reporting elements that should be addressed during routine TTE in this population. In this study, we assessed the adherence to these recommendations. Methods This was a multi-center (n = 8) retrospective review of TTE reports in children ≤11 years of age who have had complete TOF repair. We included 10 patients from each participating center (n = 80) and scored 2 outpatient follow-up TTE reports on each patient. The adherence rate was based on completeness of TTE reporting elements derived from the guidelines. Results We reviewed 160 TTE reports on 80 patients. Median age was 4.4 months (IQR 1.5-6.6) and 3.6 years (IQR 1.3-6.4) at the time of complete surgical repair and first TTE report, respectively. The median adherence rate to recommended reporting elements was 61% (IQR 53–70). Of the 160 reports, 9 (7%) were ≥80% adherent and 40 (25%) were ≥70% adherent. Quantitative measurements of right ventricular outflow tract (RVOT), right ventricular (RV) size and function, and branch pulmonary arteries were least likely to be reported. Conclusions Overall adherence to the most recent published imaging guidelines for surveillance of children with repaired TOF patients was suboptimal, especially for reporting of RVOT, RV size and function, and branch pulmonary arteries. Further studies are needed to explore the barriers to adherence to guidelines and most importantly, whether adherence is associated with clinical outcomes.

Background:Ventriculo-ventricular interactions are known to exist, though not well quantified. We hypothesised that the ventricular–vascular coupling ratio assessed by cardiovascular MRI would provide insight into this relationship. We also sought to compare MRI-derived ventricular–vascular coupling ratio to echocardiography and patient outcomes.Methods:Children with cardiac disease and biventricular physiology were included. Sanz’s and Bullet methods were used to calculate ventricular–vascular coupling ratio by MRI and echocardiography, respectively. Subgroup analysis was performed for right and left heart diseases. Univariate and multivariate regressions were performed to determine associations with outcomes.Results:A total of 55 patients (age 14.3 ± 2.5 years) were included. Biventricular ventricular–vascular coupling ratio by MRI correlated with each other (r = 0.41; p = 0.003), with respect to ventricle’s ejection fraction (r = −0.76 to −0.88; p < 0.001) and other ventricle’s ejection fraction (r = −0.42 to −0.47; p < 0.01). However, biventricular ejection fraction had only weak correlation with each other (r = 0.31; p = 0.02). Echo underestimated ventricular–vascular coupling ratio for the left ventricle (p < 0.001) with modest correlation to MRI-derived ventricular–vascular coupling ratio (r = 0.43; p = 0.002). There seems to be a weak correlation between uncoupled right ventricular–vascular coupling ratio with the need for intervention and performance on exercise testing (r = 0.33; p = 0.02).Conclusion:MRI-derived biventricular ventricular–vascular coupling ratio provides a better estimate of ventriculo-ventricular interaction in children and adolescents with CHD. These associations are stronger than traditional parameters and applicable to right and left heart conditions.

Introduction Understanding congenital heart disease (CHD) is vital for medical personnel and parents of affected children. While traditional 2D schematics serve as the typical approach used, several studies have shown these models to be limiting in understanding complex structures. Recent world-emphasis has shifted to 3D printed models as a complement to 2D imaging to bridge knowledge and create new opportunities for experiential learning. We sought to systematically compare 3D digital and physical models for medical personnel and parent education compared to traditional methods. Methods 3D printed and digital models were made out of MRI and CT data for 20 common CHD. Fellows and nurse practitioners used these models to explore intra-cardiac pathologies following traditional teaching. The models were also used for parent education in outpatient settings after traditional education. The participants were then asked to fill out a Likert scale questionnaire to assess their understanding and satisfaction with different teaching techniques. These ratings were compared using paired t-tests and Pearson’s correlation. Results Twenty-five medical personnel (18 fellows; 2 nurses; 4 nurse practitioners and one attending) and twenty parents participated in the study. The diagnosis varied from simple mitral valve pathology to complex single ventricle palliation. Parent and medical personnel perceived understanding with digital models was significantly higher than traditional (p = 0.01). Subjects also felt that physical models were overall more useful than digital ones (p = 0.001). Physicians using models for parent education also perceived the models to be useful, not significantly impacting their clinical workflow. Conclusions 3D models, both digital and printed, enhance medical personnel and parental perceived understanding of CHD.

Introduction Appropriate use criteria (AUC) guide initial transthoracic echocardiogram (TTE) use in outpatient pediatrics. We sought to improve pediatric cardiologist TTE ordering appropriateness (mean AUC score) with a quality improvement initiative. Methods The outcome of interest was the prospective AUC score for all initial outpatient TTEs ordered between November 2016 and August 2017, categorized per the AUC: "appropriate" (score 7-9), "may be appropriate" (4-6), "rarely appropriate" (1-3). Interventions included a didactic review of 2014 AUC and participant documentation of AUC criteria for each TTE. Participants met quarterly to evaluate outcome, process, and balancing measures, intervention effectiveness, and to identify and mitigate barriers. Results Twenty-two pediatric cardiologists participated. TTE appropriateness level before (n = 216) and after (n = 557) intervention was high. There was no significant difference in mean baseline and post-intervention AUC score (7.42 ± 1.87 versus 7.16 ± 2.87, P = 0.1), nor in TTE sensitivity (27% versus 25%, P > 0.1) as a balancing measure. Among baseline studies, 81% were "appropriate," and 6% "rarely appropriate." Among post-intervention studies, 76% were "appropriate," and 11% "rarely appropriate." Barriers identified to implementing AUC include TTE indications not specified by current AUC, expectations of referring provider or parent to perform TTE, consistent provider application of AUC, and ability of AUC to capture comprehensive clinical judgment. Conclusions Although the mean AUC appropriateness level was high, we were able to identify significant barriers to the implementation of AUC. Future efforts should focus on the reduction of "rarely appropriate" TTE ordering.

Objectives To report the results of the Nit‐Occlud PDA prospective postapproval study (PAS) along with a comparison to the results of the pivotal and continued access trials. Background The Nit‐Occlud PDA (PFM Medical, Cologne, Germany), a nitinol coil patent ductus arteriosus (PDA) occluder, was approved by the Food and Drug Administration in 2013. Methods The PAS enrolled a total of 184 subjects greater than 6 months of age, weighing at least 5 kg, with PDAs less than 4 mm by angiography at 11 centers. Patients were followed prospectively at 2 months, 12 months, and 24 months postprocedure. These outcomes were compared to the 357 subjects enrolled in the pivotal and continued access protocols. Efficacy and safety data were reported. Results Among 184 subjects enrolled for the PAS between 2014 and 2017, 180 (97.8%) had successful device implantation. After 12 months, 98.7% (150/152) had trivial or no residual shunt by echocardiography and two subjects had only small residual shunts. There were three device embolizations that were all retrieved by snare without clinical consequence. Together with the pivotal and continued access study, 97.4% (449/461) had complete echocardiographic closure at 12 months in 541 enrolled subjects. The composite success was 94.4%. There were no mortalities and no serious device‐related adverse events. Conclusions The Nit‐Occlud PDA is a safe and effective device for closure of a small to moderate sized PDA. There were no serious device‐related adverse events in a large cohort of three clinical trials.

Background The American Society of Echocardiography Committee on Pediatric Echocardiography Laboratory Productivity was formed in 2011 to study institutional factors that could influence the clinical productivity of physicians and sonographers in academic pediatric echocardiography laboratories. In the previous two surveys, staff clinical productivity remained stable while total echocardiography volumes increased. This third survey was designed to assess how clinical productivity is associated with laboratory infrastructure elements such as training, administrative tasks, quality improvement, research, and use of focused cardiac ultrasound (FCU). Methods Survey questions were sent by e-mail to North American laboratories. The aims were to assess (1) educational and training obligations, (2) academic productivity and research, (3) laboratory medical director satisfaction, (4) quality improvement, (5) laboratory leadership roles, and (6) impact and use of FCU. Survey responses were compared with clinical productivity metrics defined in the first two surveys. Results There were 38 responses. Academic productivity was higher at institutions with more dedicated imaging personnel, personnel with dedicated protected academic time, and advanced imaging fellows. Academic productivity did not correlate with clinical productivity and was not significantly affected by the presence of dedicated research sonographers. The satisfaction level of laboratory medical directors was related to dedicated administrative time and an administrative stipend. The majority of administrative roles were tasked to the laboratory medical director with support of the technical director. FCU was listed as a hospital privilege at four institutions (13%). Twenty-two (58%) were training FCU providers in one or more subspecialties. FCU was not associated with clinical or academic productivity. Conclusion This third survey gathered supplemental data to complement the clinical productivity data collected from the first two surveys. Together, the results of these surveys further describe the range of factors that can affect North American academic pediatric echocardiography laboratories.

We present an unusual case of concordant ventriculoarterial connections, subpulmonary infundibulum, and parallel arterial trunks. This case was complicated by extreme pulmonary artery tortuosity and low arching aorta causing severe tracheal compression. We discuss the difficulty in prenatal diagnosis, necessity for advanced imaging postnatally, and associated airway complications.

Background The American Society of Echocardiography Committee on Pediatric Echocardiography Laboratory Productivity aimed to study factors that could influence the clinical productivity of physicians and sonographers and assess longitudinal trends for the same. The first survey results indicated that productivity correlated with the total volume of echocardiograms. Methods Survey questions were designed to assess productivity for (1) physician full-time equivalent (FTE) allocated to echocardiography reading (echocardiograms per physician FTE per day), (2) sonographer FTE (echocardiograms per sonographer FTE per year), and (3) machine utilization (echocardiograms per machine per year). Questions were also posed to assess work flow and workforce. Results For fiscal year 2013 or academic year 2012–2013, the mean number of total echocardiograms—including outreach, transthoracic, fetal, and transesophageal echocardiograms—per physician FTE per day was 14.3 ± 5.9, the mean number of echocardiograms per sonographer FTE per year was 1,056 ± 441, and the mean number of echocardiograms per machine per year was 778 ± 303. Both physician and sonographer productivity was higher at high-volume surgical centers and with echocardiography slots scheduled concordantly with clinic visits. Having an advanced imaging fellow and outpatient sedation correlated negatively with clinical laboratory productivity. Machine utilization was greater in laboratories with higher sonographer and physician productivity and lower for machines obtained before 2009. Conclusion Measures of pediatric echocardiography laboratory staff productivity and machine utilization were shown to correlate positively with surgical volume, total echocardiography volumes, and concordant echocardiography scheduling; the same measures correlated negatively with having an advanced imaging fellow and outpatient sedation. There has been no significant change in staff productivity noted over two Committee on Pediatric Echocardiography Laboratory Productivity survey cycles, suggesting that hiring practices have matched laboratory volume increases.

Background Echocardiogram has limitations in effectively assessing right ventricular (RV) function in children post tetralogy of Fallot (TOF) repair. We evaluated the utility of speckle tracking echocardiography (STE)-based RV global longitudinal strain (GLS) for the assessment of RV systolic function. Methods All patients with repaired TOF who had both echocardiograms and cardiac MRI (CMR) within a 6-month interval were included. RV volumes and ejection fraction (EF) were obtained by CMRs. Traditional echocardiographic function parameters and RV GLS were compared to CMR-derived RV EF. Subjects were divided into two groups based on CMR RV EF (group I: RV EF ≥45%; and group II: RV EF

OBJECTIVES: Patients with Kawasaki disease can develop life-altering coronary arterial abnormalities, particularly in those resistant to intravenous immunoglobulin (IVIg) therapy. We tested the tumor necrosis factor α receptor antagonist etanercept for reducing both IVIg resistance and coronary artery (CA) disease progression. METHODS: In a double-blind multicenter trial, patients with Kawasaki disease received either etanercept (0.8 mg/kg; n = 100) or placebo (n = 101) subcutaneously starting immediately after IVIg infusion. IVIg resistance was the primary outcome with prespecified subgroup analyses according to age, sex, and race. Secondary outcomes included echocardiographic CA measures within subgroups defined by coronary dilation (z score >2.5) at baseline. We used generalized estimating equations to analyze z score change and a prespecified algorithm for change in absolute diameters. RESULTS: IVIg resistance occurred in 22% (placebo) and 13% (etanercept) of patients (P = .10). Etanercept reduced IVIg resistance in patients >1 year of age (P = .03). In the entire population, 46 (23%) had a coronary z score >2.5 at baseline. Etanercept reduced coronary z score change in those with and without baseline dilation (P = .04 and P = .001); no improvement occurred in the analogous placebo groups. Etanercept (n = 22) reduced dilation progression compared with placebo (n = 24) by algorithm in those with baseline dilation (P = .03). No difference in the safety profile occurred between etanercept and placebo. CONCLUSIONS: Etanercept showed no significant benefit in IVIg resistance in the entire population. However, preplanned analyses showed benefit in patients >1 year. Importantly, etanercept appeared to ameliorate CA dilation, particularly in patients with baseline abnormalities.

BACKGROUND: The reliability of LV systolic functional indices calculated from blinded echocardiographic measurements of LV size has not been tested in a large cohort of healthy children. The objective of this study was to estimate interobserver variability in standard measurements of left ventricular (LV) size and systolic function in children with normal cardiac anatomy and qualitatively normal function. METHODS: The Pediatric Heart Network Normal Echocardiogram Database collected normal echocardiograms from healthy children ≤18 years old distributed equally by age, gender, and race. A core lab used 2-dimensional echocardiograms to measure LV dimensions from which a separate data coordinating center calculated LV volumes and systolic functional indices. To evaluate interobserver variability, two independent expert pediatric echocardiographic observers re-measured LV dimensions on a subset of studies, while blinded to calculated volumes and functional indices. RESULTS: Of 3215 subjects with measurable images, 552 (17%) had a calculated LV shortening fraction (SF)

Right ventricular dilation is a common complication after tetralogy of Fallot (TOF) repair. Traditional echocardiographic assessments are imprecise due to the RV's location and complex geometry. We propose a novel echocardiographic measurement: RV apical flattening (RVAF) as a screening tool to help identify subjects with severe RV dilation. Patients with repaired TOF who had both echocardiograms and CMR's within 6-month interval at our institution were included in the study. The RVAF was measured in the four-chamber echocardiographic view as the minor length of RV cavity at the level of RV apical endocardium. Subjects were divided into two groups (group I: RVEDVi ≥ 150 ml/m(2) and group II; RVEDVi < 150 ml/m(2)). Echocardiogram and CMR data were compared between groups. A total of 75 subjects were included in the study. Mean age was 12.8 ± 3.6 years. Group I had 36 subjects, and group II had 39 subjects. The mean RVAF was significantly higher in group I (2.7 ± 0.5 cm) compared with group II (1.7 ± 0.4 cm; p < 0.001). There was significant correlation between RVAF and RVEDVi (r = 0.81; p < 0.001). By ROC analysis, an RVAF cutoff value of 2.0 cm had 94 % sensitivity and 77 % specificity in identifying severe RV dilation (area under the curve 0.95). RVAF is a simple and effective echocardiographic screening tool to help identify severe RV dilation. In conjunction with other 2D echocardiographic parameters, this technique would help further refine echocardiography-guided patient selection for timing of CMR and pulmonary valve replacement.

Stainless steel embolization coils (SSEC) have been used for over four decades for vascular occlusion. Recently, the safety of these coils in a magnetic resonance environment has been called into question, with important ramifications for thousands of patients with existing coils in place. We performed a retrospective chart review at five tertiary care pediatric centers evaluating all children and young adults with implanted SSEC who underwent magnetic resonance imaging (MRI). Data reviewed included demographics, coil implantation, MRI studies, and follow-up evaluations. Complications such as heating, discomfort, or device migration were specifically sought. Two hundred and ninety-seven patients with implanted SSEC underwent 539 MRI examinations. The median age at SSEC implantation was 2.3 years (1 week-23.2 years). The MRI studies were performed a median of 7.4 years (4 days-23.1 years) after implantation. No patients experienced any reported complications associated with their MRI examinations during the study or at median follow-up post-MRI of 4.8 years (1 day-23 years). In this large, retrospective review of patients with implanted SSEC undergoing MRI, there were no reported adverse events. These findings support the recent change by Cook Medical Inc. of their standard embolization coils from a designation of magnetic resonance unsafe to conditional.

Background— Published nomograms of pediatric echocardiographic measurements are limited by insufficient sample size to assess the effects of age, sex, race, and ethnicity. Variable methodologies have resulted in a wide range of Z scores for a single measurement. This multicenter study sought to determine Z scores for common measurements adjusted for body surface area (BSA) and stratified by age, sex, race, and ethnicity. Methods and Results— Data collected from healthy nonobese children ≤18 years of age at 19 centers with a normal echocardiogram included age, sex, race, ethnicity, height, weight, echocardiographic images, and measurements performed at the Core Laboratory. Z score models involved indexed parameters (X/BSA α ) that were normally distributed without residual dependence on BSA. The models were tested for the effects of age, sex, race, and ethnicity. Raw measurements from models with and without these effects were compared, and α ) were selected for each measurement. Multivariable regression revealed statistically significant effects by age, sex, race, and ethnicity for all outcomes, but all effects were clinically insignificant based on comparisons of models with and without the effects, resulting in Z scores independent of age, sex, race, and ethnicity for each measurement. Conclusions— Echocardiographic Z scores based on BSA were derived from a large, diverse, and healthy North American population. Age, sex, race, and ethnicity have small effects on the Z scores that are statistically significant but not clinically important.

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BackgroundIn CHD, three-dimensional strain analysis may overcome limitations of Doppler and two-dimensional strain of the left ventricle. The aims of this study were to evaluate feasibility and reproducibility of three-dimensional longitudinal, circumferential, and radial systolic strain by three-dimensional speckle-tracking echocardiography compared with two-dimensional echocardiography.MethodsPatients with CHD, biventricular circulation with a systemic left ventricle, and who had two- and three-dimensional imaging performed on the same day from 2010 to 2014 were included. Quantitative two- and three-dimensional strain analyses were performed (two-dimensional cardiac performance analysis version 1.2 and four-dimensional left ventricular analysis version 3.1). Intra- and inter-observer variabilities were calculated on 25 studies.ResultsA total of 30 patients, including 19 (61%) males, with a median age of 3.6 years (0.1–22 years) were included. The mean fractional shortening was 34.6±5.3%, and the mean ejection fraction was 62.0±6.4%. Measurement of two- and three-dimensional strain was feasible in >95% of segments. Good correlation was observed between longitudinal and circumferential strain (r=0.92, p⩽0.001 and r=0.87, p⩽0.001), but not radial strain (r=0.29, p=0.2). Intra- and inter-observer agreements were better for three-dimensional compared with two-dimensional strain, and better for both two- and three-dimensional longitudinal and circumferential strains compared with radial strain.ConclusionLeft ventricular three-dimensional strain analysis is feasible in children with CHD. The reproducibility of longitudinal and circumferential strain by three-dimensional analyses is better. Further longitudinal studies are warranted for the potential clinical application of this new technology.

Cardiac magnetic resonance imaging has demonstrated that the aortic root may be dilated in a dimension that two-dimensional echocardiography (2DE) is not able to interrogate. In the standard parasternal long-axis view, only a portion of the aortic root in the anteroposterior (AP) dimension can be visualized, as opposed to three-dimensional (3D) echocardiography (3DE), which can capture the entire root in an infinite number of planes. The purposes of the present study were to compare measurements of dilated aortic roots between 3DE and 2DE and to evaluate interobserver variability on 3DE.Thirty-one patients (median age, 13 years) with aortic root dilation were identified. Two-dimensional echocardiographic images and full-volume electrocardiographically gated 3D echocardiographic (3DE) images were obtained. Two blinded observers measured six dimensions of the aortic root in the short-axis view: three in the AP dimension and three in the transverse dimensions. Two-dimensional echocardiographic measurements were made by a third blinded observer. The largest AP 3DE measurement was compared with two-dimensional echocardiographic measurements. Interobserver 3DE measurements were also compared.The median aortic root Z score was +2.63. Maximum 3DE measurement in any plane of the root size was significantly greater than on 2DE (P.0001). The largest AP dimension by 3DE was significantly greater than on 2DE (P = .001). There was no significant interobserver variability for the largest dimension or those in the AP dimension, but a difference was found in the transverse dimension (P = .02).Three-dimensional echocardiography compares favorably with 2DE in the evaluation of aortic root dilation in patients with known histories of aortic root disease. Three-dimensional echocardiography found that the largest diameter measured was significantly larger than on 2DE. The interobserver variability of 3DE is low, particularly in the AP dimension and in identifying the largest diameter. Three-dimensional echocardiography can be a useful technique in the periodic surveillance of patients with aortic root dilation.

Cardiac MRI can predict the likely tumor type in the majority of children with a cardiac mass. A comprehensive imaging protocol is essential for accurate diagnosis. However, histologic diagnosis remains the gold standard, and in some cases malignancy cannot be definitively excluded on the basis of cardiac MRI images alone.

Background Kawasaki disease (KD) is a multisystem vasculitis affecting coronary arteries in children. Patients, refractory to standard treatment with intravenous immunoglobulin and aspirin, show higher rates of coronary artery dilation. Early tumor necrosis factor α receptor antagonism in KD may provide effective adjunctive therapy. Study Design The EATAK trial is a multicenter, double-blind, randomized, and placebo-controlled trial intended to assess the efficacy of etanercept in reducing the intravenous immunoglobulin refractory rate during treatment of acute KD. Each arm will enroll 110 patients who will receive 3 doses of study drug over 2 weeks in conjunction with standard therapy. Coronary artery dilation parameters will serve as secondary end points. Discussion The EATAK trial will test a new paradigm for treatment of acute KD involving tumor necrosis factor α antagonism by etanercept.

At an early morning hour at the start of a busy work day, a dozen individuals position themselves at conference room tables in front of a projector and screen. After the lights dim, a series of scenarios are presented, each one selected to review a diagnostic error. In the ensuing discussion, the group attempts to identify root causes. For those who had been directly involved in the error, personal feelings are set aside with the recognition that a dispassionate evaluation is the best means for identification and prevention of future errors. A nonblameful conference environment allows free discussion, leading to the formulation of an action plan. This type of conference likely takes place in many industries, whether it be manufacturing, aerospace, or health care. In clinical cardiology, the same kind of multidisciplinary approach is increasingly being used to assess and address diagnostic errors in echocardiography. The goal is to improve patient care. In this issue of JASE, Benavidez et al. 1 strive to better define the sources of diagnostic errors in congenital echocardiography, while at the same time inserting examples and suggesting strategies to mitigate error risks. Their work highlights and acknowledges a new focus in 21st-century medicine. In clinical echocardiography, we may know how to do it. 2 The next questions form the crux of Benavidez et al.’s present work: How well are we doing it? 3 How do we manage error risks if we don’t measure them? How dowe integrate these important goals within the quality domain, recognizing that additional resources will be required for effective implementation? Like health care itself, the concept of quality is evolving. The Institute ofMedicine,anarmofthe NationalAcademyof Sciences,listssix quality dimensions: safety, effectiveness, timeliness, equity, efficiency, and patient-centeredness. 4 We hear of ‘‘quality assurance’’ activities, implying that achievement of a preset standard represents ‘‘quality.’’ Once achieved, the process need go no further. Those words have been frequently replaced by the phrase ‘‘quality improvement’’ or the more explicit ‘‘continuous quality improvement.’’ The evolving phrase becomes its own illustration that just like any biologic system, a standard, an established process, or a condition is ephemeral. Ensuring quality demands meticulous attention and ongoing awareness of situations and resources. Success requires both the realization that however good a quality assurance process or echocardiogram may seem to be, it can always be better, and that the review process be free of blame or retaliation. Quality improvement initiatives involve not just the echocardiography laboratory but also extend broadly throughout other arenas, such as health care administration and physician and sonographer credentialing. In 2000, the American Board of Medical Specialties, composed of 24 different specialty boards, including pediatrics and internal medicine, chose to shift the idea of physician credentialing from recertification to the trademarked ‘‘maintenance of certification.’’ Even in the face of controversy, maintenance of certification has become an expected activity for those wishing to remain board certified. For both pediatrics and internal medicine, ongoing certification is now predicated on successfully completing activities that are considered to be important in quality improvement. More recently, the self-rebranded Intersocietal Accreditation Commission, formerly called the Intersocietal Commission for the Accreditation of Echocardiography Laboratories, created guidelines to incorporate quality improvement measures in echocardiography laboratories. 5 To come full circle, at the time of this writing, the commission offered its own program for pediatric cardiologists to accrue maintenance-ofcertification credit. 6 The mission and directives of creating quality improvement metrics are taking form, but questions remain. What metrics do we measure, and how do we measure them? Cardiologists have the opportunity to have a direct impact on what parameters should be tracked and assessed, while at the same time receiving credit toward certification.

Background— Quantitative assessment of ventricular volumes and mass in pediatric patients with single-ventricle physiology would aid clinical management, but it is difficult to obtain with 2-dimensional echocardiography. The purpose of the present study was to compare matrix-array 3-dimensional echocardiography (3DE) measurements of single-ventricle volumes, mass, and ejection fraction with those measured by cardiac magnetic resonance (CMR) in young patients. Methods and Results— Twenty-nine patients (median age, 7 months) with a functional single ventricle undergoing CMR under general anesthesia were prospectively enrolled. The 3DE images were acquired at the conclusion of the CMR. Twenty-seven of 29 3DE data sets (93%) were optimal for 3DE assessment. Two blinded and independent observers performed 3DE measurements of volume, mass, and ejection fraction. The 3DE end-diastolic volume correlated well ( r =0.96) but was smaller than CMR by 9% ( P P P Conclusions— In young pediatric patients with a functional single ventricle, matrix-array 3DE measurements of mass and volumes compare well with those obtained by CMR. 3DE will provide an important modality for the serial analysis of ventricular size and performance in young patients with functional single ventricles.

Background Myocarditis presenting as isolated acute chest pain with elevated troponins but normal systolic function by echocardiogram is rare in previously healthy children. Diagnosis is challenging in this situation with significant drawbacks even for the gold standard endomyocardial biopsy. Our study aim is to evaluate the diagnostic role of cardiac MRI in comparison with echocardiography in these patients.

Cardiomyopathy in Duchenne muscular dystrophy (DMD) is associated with death in approximately 40% of patients. Echocardiography is routinely used to assess left ventricular (LV) function; however, it has limitations in these patients. We compared echocardiographic measures of cardiac function assessment to cardiac MRI. We included children and young adults with DMD who had MRI performed between January 2010 and July 2015. We measured echocardiographic and MRI parameters of function assessment, including strain. Presence of late gadolinium enhancement (LGE) was assessed by MRI. Subjects were divided into two groups based on MRI left ventricular ejection fraction (LVEF): group I, LVEF ≥55% and group II, LVEF

Background Accurate and reproducible assessments of right ventricular (RV) size and function are important for clinical decisions. RV volume assessment by CMR has been traditionally performed using a short axis orientation (SAX). This projection, however, renders en-face views of the mitral and tricuspid valves. Distinction between atriums and ventricles can be difficult, which can complicate contour tracings for ventricular function. To improve reproducibility, alternative orientations for RV assessment have been proposed. This investigation sought to compare right horizontal long axis (RHLA) and SAX reproducibility in a pediatric cohort. Methods The RHLA method is an oblique-transverse cine stack where the planes are oriented parallel to the diaphragmatic aspect of the right ventricle (Figure 1). We performed a retrospective evaluation of 20 CMR patients born with right heart disease (age range 7 years - 17 years), in which RHLA and SAX steady-state free precession cine stacks were originally obtained on all patients. Siemens’ Leonardo software was utilized for contour tracing and volumetric calculations. Guidelines for contour tracing were reviewed by all readers to ensure comparable technique. Four separate readers participated in the study, with a wide range of experience. One of the 4 readers had no prior CMR experience, and practiced on 4 non-study patients while being trained by an attending. No further feedback was given until after study completion. The 4 readers, blinded to each others’ results, measured RV end diastolic (EDV) and end systolic (ESV) volumes for every RHLA and SAX data set. In order to minimize confounders, no cross referencing with additional views was performed. All RHLA and SAX volumes were then re-measured by all readers, to determine inter-rater and intra-rater associations. Differences between mean volumes obtained with each orientation were also assessed.

50 The article by Sachdeva et al. in this issue of the Journal of the American Society of Echocardiography describes the echocardiographic findings in pediatric patients with heart failure supported with the Berlin Heart EXCOR ventricular assist device (VAD) (Berlin Heart AG, Berlin, Germany). Chronic heart failure as a result of cardiomyopathy or congenital heart disease is diagnosed with increasing frequency in pediatric patients. As in the adult population, heart transplantation is the principal treatment for childrenwith end-stage heart failure. However, the limited availability of size-matched pediatric organ donors, particularly in neonates and infants, is associated with a prolonged median waiting time from listing to transplantation of 69 days. Currently, mortality for pediatric patients awaiting heart transplantation is 20%. Mechanical support of the severely failing heart may be used as a bridge to transplantation when pharmacologic therapy is no longer adequate. The resulting improvement in cardiac output stabilizes end-organ function, reduces mortality risk, and optimizes the patient’s overall condition before transplantation. A wide variety of devices are available for adult patients, but options for thepediatric population, particularly infants and small children, remain limited. Extracorporeal membrane oxygenation (ECMO) is the most frequently used form of mechanical assist in children. Advantages include widespread availability, ease of cannulation (via the right internal jugular vein and right common carotid artery), and applicability in neonates, infants, and older children. The primary disadvantage of ECMO is limited durability. Complications including infection, surgical site bleeding, and intracranial bleeding generally preclude support for longer than 3 to 4 weeks, severely reducing the utility of ECMO as a bridge to transplantation.

The aim of this study was to report the results of an international multicenter experience of cardiac magnetic resonance imaging (MRI) evaluation of cardiac tumors in children, each with histology correlation or a diagnosis of tuberous sclerosis, and to determine which characteristics are predictive of tumor type.Individual centers have relatively little experience with diagnostic imaging of cardiac tumors in children, because of their low prevalence. The accuracy of cardiac MRI diagnosis on the basis of a pre-defined set of criteria has not been tested.An international group of pediatric cardiac imaging centers was solicited for case contribution. Inclusion criteria comprised: 1) age at diagnosis ≤18 years; 2) cardiac MRI evaluation of cardiac tumor; and 3) histologic diagnosis or diagnosis of tuberous sclerosis. Data from the cardiac MRI images were analyzed for mass characteristics. On the basis of pre-defined cardiac MRI criteria derived from published data, 3 blinded investigators determined tumor type, and their consensus diagnoses were compared with histologic diagnoses.Cases (n = 78) submitted from 15 centers in 4 countries had the following diagnoses: fibroma (n = 30), rhabdomyoma (n = 14), malignant tumor (n = 12), hemangioma (n = 9), thrombus (n = 4), myxoma (n = 3), teratoma (n = 2), and paraganglioma, pericardial cyst, Purkinje cell tumor, and papillary fibroelastoma (n = 1, each). Reviewers who were blinded to the histologic diagnoses correctly diagnosed 97% of the cases but included a differential diagnosis in 42%. Better image quality grade and more complete examination were associated with higher diagnostic accuracy.Cardiac MRI can predict the likely tumor type in the majority of children with a cardiac mass. A comprehensive imaging protocol is essential for accurate diagnosis. However, histologic diagnosis remains the gold standard, and in some cases malignancy cannot be definitively excluded on the basis of cardiac MRI images alone.

Background— Quantitative assessment and validation of left ventricular (LV) volumes and mass in neonates and infants with complex congenital heart disease (CHD) is important for clinical management but has not been undertaken. We compared matrix-array 3D echocardiography (3D echo) measurements of volumes, mass, and ejection fraction (EF) with those measured by cardiac MRI in young patients with CHD and small LVs because of either young age or LV hypoplasia. Methods and Results— Thirty-five patients aged P =0.01; intraclass correlation coefficient [ICC], 0.96) and end-systolic volume (12.3±8.6 versus 9.6±6.8 mL; P P =0.6] and 2.7 mL [ P =0.001], respectively). Three-dimensional echo EF was smaller than MRI by 9.3% ( P P P =0.28). There was good intra- and interobserver reliability for all measurements. Conclusions— In neonates and infants with CHD and small LVs (age appropriate or hypoplastic), matrix-array 3D echo measurements of mass and volumes compare well with MRI, providing an important modality for ventricular size and performance analysis in these patients, particularly in those with left-side heart obstructive lesions.

Background Preliminary experience with matrix-array real-time (RT) 3-dimensional echocardiography (3DE) in pediatric patients has demonstrated consistently lower right ventricular volume (RVV) compared with magnetic resonance imaging. Our hypothesis was that variables in RT 3DE acquisition and offline analysis, including gain settings, thickness, and orientation of disks, could alter RVV measurements. Methods Displacements of water from latex models derived from excised lamb hearts were used for comparison. RT 3DE volume acquisitions were performed using a matrix-array probe (2-4 MHz); RVVs were calculated offline using summation of disks method. Results No significant difference and excellent agreement was found for comparison of RT 3DE with displacement of water using 5-mm cut planes, optimal gain settings, and short-axis tracings. Different gain settings and long-axis tracings significantly affected RVV. A slice thickness of 13 mm or greater affected volume measurements. Conclusions RT 3DE can accurately measure RVV. Specific variables will alter volumes measurements and must be considered in clinical studies.

Purpose Hemodynamic assessment by catheterization (CATH) remains the cornerstone of cardiac performance evaluation. However, it is expensive and invasive. At the opposite end of the spectrum is BNP followed by echocardiography (ECHO). We sought to examine the correlation of BNP with ECHO and CATH parameters. Methods and Materials Single-center, retrospective review of recipients transplanted at Results The cohort median age at transplant was 3.4 yrs (IQR 0.38-10) with 55% male and 45% cardiomyopathy. There were 225 annual datasets (3.2/patient) with a median followup of 3.4 years (IQR 2.0-6.1). The median BNP was 54 pg/mL (IQR 26-96). BNP was correlated with HCT (R=-0.29, p 2.7 (R=0.265, p >0.001). Evaluating the hemodynamic data, BNP correlated with right sided filling pressures: RAP (R=0.341, p=0.001), RVEDP (R=.344, p 100 pg/mL. Conclusions BNP as measured at annual catheterization was correlated with left and right side filling pressures and some ECHO parameters. In multivariate analysis, a BNP cutoff of >100 pg/mL was associated with RAP, vasculopathy, HCT, and possibly with LV mass. This simple test may have utility in the monitoring of cardiac performance with the caveat that a low HCT can also affect its levels.

We present the case of a woman born with transposition of the great arteries, who was surgically repaired with the Mustard technique. Because of interatrial shunting, she was brought to the cardiac catheterization laboratory for device closure. Matrix-array 3D transesophageal echocardiography enabled visualization of both baffle leaks and demonstrated its orientation in a fashion superior to 2D imaging, had it been used alone. The leaks were successfully closed with a single transcatheter device.