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Vascular rings are increasingly identified onfetal echocardiography. The purpose of this study is to analyze clinical outcomes and patterns of diagnostic testing in fetuses with vascular rings diagnosed by fetal echocardiography. A retrospective cohort study was performed of fetuses with postnatally confirmed vascular rings from 2017– 2022. Clinical outcomes included type and timing of symptoms, and timing of surgical intervention. Freedom from symptoms and/or surgery was assessed by Kaplan-Meier analysis. Frequency of genetic and diagnostic testing (barium esophagogram, CT/MRI angiogram, and bronchoscopy) was also assessed. Overall, 46 patients were evaluated (91% with a right aortic arch/left ductus and 4% with a double aortic arch). A vascular ring was isolated in 59%, associated with structural heart lesions in 33%, and associated with noncardiac anomalies in 8%. Prenatal diagnoses increased over time. Symptoms developed in 24%; 81% had respiratory and 45% had gastroesophageal complaints. Surgery was performed in 17%. Symptoms presented bimodally, prior to 100 or after 400 days of life. There was no difference in the type of symptoms for early (400 days) presenters. Symptomatic patients received more diagnostic testing. Genetic testing was obtained in 46% and positive in 33%, with 22q11 deletion and Trisomy 21 being identified. Prenatal diagnoses of vascular rings increased over time, with 24% developing symptoms and 17% requiring surgery. Subjects developed symptoms bimodally in early or late infancy. The frequency of genetic testing was suboptimal for the frequency of genetic abnormalities seen.

OBJECTIVE While the surgical stages of single ventricle (SV) palliation serve to separate pulmonary venous and systemic venous return, and to volume-unload the SV, staged palliation also results in transition from parallel to series circulation, increasing total vascular resistance. How this transition affects pressure loading of the SV is as yet unreported. METHODS We performed a retrospective chart review of Stage I, II, and III cardiac catheterization (CC) and echocardiographic data from 2001-2017 in all SV pts, with focus on systemic, pulmonary, and total vascular resistance (SVR, PVR, TVR respectively). Longitudinal analyses were performed with log-transformed variables. Effects of SVR-lowering medications were analyzed using Wilcoxon rank-sum testing. RESULTS There were 372 total patients who underwent CC at a Stage I (median age of 4.4 months, n=310), Stage II (median age 2.7 years, n = 244), and Stage III (median age 7.3 years, n = 113). Total volume loading decreases with progression to Stage III (P< 0.001). While PVR gradually increases from Stage II to Stage III, and SVR increases from Stage I to Stage III, TVR dramatically increases with progress towards series circulation. TVR was not affected by use of systemic vasodilator therapy. TVR, PVR, SVR, and CI did not correlate with indices of SV function at Stage III. CONCLUSIONS TVR steadily increases with an increasing contribution from SVR over progressive stages. TVR was not affected by systemic vasodilator agents. TVR did not correlate with echo-based indices of SV function. Further studies are needed to see if modulating TVR can improve exercise tolerance and outcomes.

Normalizing cardiovascular measurements for body size allows for comparison among children of different ages and for distinguishing pathologic changes from normal physiologic growth. Because of growing interest to use height for normalization, the aim of this study was to develop height-based normalization models and compare them to body surface area (BSA)-based normalization for aortic and left ventricular (LV) measurements. The study population consisted of healthy, non-obese children between 2 and 18 years of age enrolled in the Pediatric Heart Network Echo Z-Score Project. The echocardiographic study parameters included proximal aortic diameters at 3 locations, LV end-diastolic volume, and LV mass. Using the statistical methodology described in the original project, Z-scores based on height and BSA were determined for the study parameters and tested for any clinically significant relationships with age, sex, race, ethnicity, and body mass index (BMI). Normalization models based on height versus BSA were compared among underweight, normal weight, and overweight (but not obese) children in the study population. Z-scores based on height and BSA were calculated for the 5 study parameters and revealed no clinically significant relationships with age, sex, race, and ethnicity. Normalization based on height resulted in lower Z-scores in the underweight group compared to the overweight group, whereas normalization based on BSA resulted in higher Z-scores in the underweight group compared to the overweight group. In other words, increasing BMI had an opposite effect on height-based Z-scores compared to BSA-based Z-scores. Allometric normalization based on height and BSA for aortic and LV sizes is feasible. However, height-based normalization results in higher cardiovascular Z-scores in heavier children, and BSA-based normalization results in higher cardiovascular Z-scores in lighter children. Further studies are needed to assess the performance of these approaches in obese children with or without cardiac disease.

Background:Although cardiac catheterisation (cath) is the diagnostic test for pulmonary hypertension, it is an invasive procedure. Echocardiography (echo) is commonly used for the non-invasive diagnosis of pulmonary hypertension but maybe limited by lack of adequate signals. Therefore, emphasis has been placed on biomarkers as a potential diagnostic tool. No prior paediatric studies have simultaneously compared N-terminal pro-B-type-natriuretic peptide (NTproBNP) with cath/echo as a potential diagnostic tool. The aim of this study was to determine if NTproBNP was a reliable diagnostic tool for pulmonary hypertension in this population.Methods:Patients were divided into Study (echo evidence/established diagnosis of pulmonary hypertension undergoing cath) and Control (cath for small atrial septal defect/patent ductus arteriosus and endomyocardial biopsy post cardiac transplant) groups. NTproBNP, cath/echo data were obtained.Results:Thirty-one patients met inclusion criteria (10 Study, 21 Control). Median NTproBNP was significantly higher in the Study group. Echo parameters including transannular plane systolic excursion z scores, pulmonary artery acceleration time and right ventricular fractional area change were lower in the Study group and correlated negatively with NTproBNP. Receiver operation characteristic curve analysis demonstrated NTproBNP > 389 pg/ml was 87% specific for the diagnosis of pulmonary hypertension with the addition of pulmonary artery acceleration time improving the specificity.Conclusions:NTproBNP may be a valuable adjunctive diagnostic tool for pulmonary hypertension in the paediatric population. Echo measures of transannular plane systolic excursion z score, pulmonary artery acceleration time and right ventricular fractional area change had negative correlations with NTproBNP. The utility of NTproBNP as a screening tool for pulmonary hypertension requires validation in a population with unknown pulmonary hypertension status.

BACKGROUND: Retrospective multicenter research using echocardiograms obtained for routine clinical care can be hampered by issues of individual center quality, thus limiting the application of post-hoc research analytics. We sought to evaluate imaging and patient characteristics associated with poorer quality of archived echocardiograms from a cohort of childhood cancer survivors. METHODS: A single blinded reviewer at a central core lab graded quality of clinical echocardiograms from 5 contributing centers focusing on images to derive 2D and M-mode fractional shortening (FS), biplane Simpson ejection fraction (EF), myocardial performance index (MPI), tissue Doppler imaging (TDI) derived velocities and global longitudinal strain (GLS). RESULTS: Of 535 studies analyzed in 102 subjects from 2004 to 2017, all measures of cardiac function could be assessed in only 7%. While FS by 2D or M-mode, MPI and septal E/E’ could be measured in >80% studies, mitral E/E’ was less consistent (69%), but better than EF (52%) and GLS (10%). At least one quality issue was identified in 66% studies, with technical issues (ex. lung artifact, poor endocardial definition) being the most common (33%). Lack of 2- and 3-chamber views was associated with the performing center. Patients < 5 years had a higher chance of apex cut-off in 4-chamber views compared to 16–35 year old (RR 1.99 (1.07–3.72), p = 0.03). Overall, for any quality issue, earlier era of echo and center were the only significant risk factors. CONCLUSION: Assessment of cardiac function using pooled multicenter archived echocardiograms was significantly limited. Efforts to standardize clinical echocardiographic protocols to include apical 2- and 3-chamber views and TDI will improve the ability to quantitate LV function.

Objective:Operators are mindful of the balloon-to-aortic annulus ratio when performing balloon aortic valvuloplasty. The method of measurement of the aortic valve annulus has not been standardised.Methods and results:Patients who underwent aortic valvuloplasty at two paediatric centres between 2007 and 2014 were included. The valve annulus measured by echocardiography and angiography was used to calculate the balloon-to-aortic annulus ratio and measurements were compared. The primary endpoint was an increase in aortic insufficiency by ≥2 degrees. Ninety-eight patients with a median age at valvuloplasty of 2.1 months (Interquartile range (IQR): 0.2–105.5) were included. The angiographic-based annulus was 8.2 mm (IQR: 6.8–16.0), which was greater than echocardiogram-based annulus of 7.5 mm (IQR: 6.1–14.8) (p < 0.001). This corresponded to a significantly lower angiographic balloon-to-aortic annulus ratio of 0.9 (IQR: 0.9–1.0), compared to an echocardiographic ratio of 1.1 (IQR: 1.0–1.1) (p < 0.001). The degree of discrepancy in measured diameter increased with smaller valve diameters (p = 0.041) and in neonates (p = 0.044). There was significant disagreement between angiographic and echocardiographic balloon-to-aortic annulus ratio measures regarding “High” ratio of >1.2, with angiographic ratio flagging only 2/12 (16.7%) of patients flagged by echocardiographic ratio as “High” (p = 0.012). Patients who had an increase in the degree of aortic insufficiency post valvuloplasty, only 3 (5.5%) had angiographic ratio > 1.1, while 21 (38%) had echocardiographic ratio >1.1 (p < 0.001). Patients with resultant ≥ moderate insufficiency more often had an echocardiographic ratio of >1.1 than angiographic ratio of >1.1 There was no association between increase in balloon-to-aortic annulus ratio and gradient reduction.Conclusions:Angiographic measurement is associated with a greater measured aortic valve annulus and the development of aortic insufficiency. Operators should use caution when relying solely on angiographic measurement when performing balloon aortic valvuloplasty.

Background The pediatric Appropriate Use Criteria (AUC) for outpatient transthoracic echocardiography (TTE) aim to reduce practice variation. Little is known on variation in TTE use between physicians. Understanding this variation will help identify areas for improvement in standardization of TTE use. Methods and results This is a retrospective review of initial outpatient visits at 6 pediatric cardiology centers in the United States prior to AUC release. Variation in TTE use was examined using multilevel generalized mixed effects models. Forward selection identified combinations of variables that explained the most variance in TTE use between physicians. Due to collinearity, physician compensation model and center were analyzed separately. Of 2883 encounters, the most common indication was murmur (36%), followed by chest pain (15.2%). Overall TTE use was 41.9%, and varied widely between centers (22.9%-52.6%), and between physicians within centers. Center alone explained 29% of this physician variance. Adding physician characteristics increased the variance explained to 57%, which only minimally improved by adding patient characteristics. The variance explained was driven by subspecialty. The center-based multivariable model explained more variance over compensation model. Conclusions Center was the single largest determinant of physician variance in TTE use, followed by physician subspecialty. Efforts to reduce practice variation, such as the AUC, should be employed across centers and all pediatric cardiac providers. Center appears to have a stronger impact on variance than compensation model, though in this dataset the effect of center and compensation are hard to separate from each other and deserve further evaluation.

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.

Objectives: The purpose of this study was to assess the timing of changes in serial echocardiographic parameters in pediatric cancer survivors and to evaluate their associations with cardiomyopathy development. Background: Pediatric cancer survivors undergo serial echocardiograms to screen for cardiotoxicity. It is not clear whether small longitudinal changes in functional or structural parameters over time have clinical significance. Methods: This is a multicenter, retrospective, case-control study of ≥1-year survivors following the end of cancer therapy. Cardiomyopathy cases (fractional shortening [FS] ≤28% or ejection fraction [EF] ≤50% on ≥2 occasions) were matched to control subjects (FS ≥30%, EF ≥55%, not on cardiac medications) by cumulative anthracycline and chest radiation dose, follow-up duration, and age at diagnosis. Digitally archived clinical surveillance echocardiograms were quantified in a central core laboratory, blinded to patient characteristics. Using mixed models with interaction terms between time and case status, we estimated the least square mean differences of 2-dimensional, M-mode, pulsed wave Doppler, and tissue Doppler imaging–derived parameters over time between cases and control subjects. Results: We identified 50 matched case-control pairs from 5 centers. Analysis of 412 echocardiograms (cases, n = 181; control subjects, n = 231) determined that indices of left ventricular systolic function (FS, biplane EF), diastolic function (mitral E/A ratio), and left ventricular size (end-diastolic dimension z-scores) were significantly different between cases and control subjects, even 4 years prior to the development of cardiomyopathy. Conclusions: Longitudinal changes in cardiac functional parameters can occur relatively early in pediatric cancer survivors and are associated with the development of cardiomyopathy.

Introduction: To promote the rational use of cardiovascular imaging in the delivery of high-quality and cost-effective care to patients with congenital heart disease (CHD), the American College of Cardiology developed Appropriate Use Criteria (AUC). We aimed to evaluate the appropriateness of indications for cardiac CT and MRI in patients with conotruncal defects and to identify factors associated with Maybe or Rarely Appropriate (M/R) indications. Methods: Twelve US centers contributed a median of 147 studies performed prior to AUC publication (01/2020) on patients with conotruncal defects. Studies were excluded if the patient did not receive primary cardiac care at the center or were not seen > 5 years prior to study date. To account for the nesting of physicians within center, a hierarchical generalized linear mixed model was used to account for center-level variation with physicians nested within center. Results: Of the 1,753 studies (20% CT, 80% MRI) from 12 centers, 16% (range 4-39%) were rated M/R and 2.4% were unclassifiable (Figure 1). Patients age Conclusions: Most cardiac CTs and MRIs ordered for the follow-up care of patients with conotruncal defects were rated Appropriate and nearly all were able to be classified. However, there was significant center-level variation and higher annual MRI volume was independently associated with lower odds of M/R rating. These findings inform future quality improvement initiatives.

The COVID pandemic necessitated an altered approach to transthoracic echocardiography, especially in COVID cases. Whether this has effected echocardiography lab quality is unknown.We sought to determine whether echocardiography lab quality measures during the COVID pandemic were different from those prior to the pandemic and whether quality and comprehensiveness of echocardiograms performed during the pandemic was different between COVID and non-COVID patients.The four quality measures (diagnostic errors, appropriateness of echocardiogram, American College of Cardiology Image Quality metric and Comprehensive Exam metric in structurally normal hearts) reported quarterly in our lab were compared between two quarters during COVID (2020) and pre-COVID (2019). Each component of these metrics was also assessed in randomly selected echocardiograms in COVID patients and compared to non-COVID echocardiograms.For non-COVID echocardiograms, the image quality metric did not change between 2019 and 2020 and the comprehensive exam metric improved. Diagnostic error rate did not change, and appropriateness of echocardiogram indications improved. When COVID and non-COVID echocardiograms were compared, the image quality metric and comprehensiveness exam metric were lower for COVID cases (image quality mean 21.3/23 for non-COVID, 18.6/23 for COVID, p 0.001 and comprehensive exam mean 29.5/30 for non-COVID, 27.7/39 for COVID, p 0.001). In particular, systemic and pulmonary veins, pulmonary arteries and aortic arch were not adequately imaged in COVID patients. For studies in which a follow-up echocardiogram was available, no new pathology was found.At our center, though diagnostic error rate did not change during the pandemic and the proportion of echocardiograms ordered for appropriate indications increased, imaging quality in COVID patients was compromised, especially for systemic and pulmonary veins, pulmonary arteries and arch. Though no new pathology was noted on the small number of patients who had follow-up studies, we are paying careful attention to these structures to avoid diagnostic errors going forward.

Introduction Right ventricular pacing is associated with pacemaker induced cardiomyopathy and lesser degrees of pacing-induced LV dysfunction (PIVD) manifested by a reduction in left ventricular ejection fraction (LVEF). Our objective was to determine whether apical 4 chamber strain (A4C) by echocardiography can identify patients at risk of PIVD before LVEF declines. Methods and results A retrospective chart review of patients (0-21 years) who had a pacemaker with a ventricular lead placed between 2011 and 2017 was performed. Patients were divided into group A (LVEF 10% decline in LVEF within 12 months of pacemaker placement) and group B. Data have collected before and 1 and 12 months postpacemaker implantation. There were 30 patients in the group A and 60 in group B. At 1 and 12 months postpacemaker implantation, the LVEF was significantly lower while the A4C and QRS duration on electrocardiogram were significantly higher in the group A. While the LVEF and A4C became markedly abnormal in group A as early as 1 month, the A4C did not seem to demonstrate such marked abnormalities in group B. However, a sub-analysis of patients in the group A with preserved LVEF at 1 month demonstrated significant worsening in their A4C at that time. Conclusion Myocardial deformation imaging may be a clinically useful tool for the prediction of a decline in LV systolic function following pacemaker implantation. Abnormalities in A4C seem to appear before LVEF decline and as soon as 1-month postpacemaker implantation.

The Web pages are growing in a design of terabytes or even petabytes day by day. In the case of the small Web, it is an easy task to answer a query, whereas robust modus operandi for storage, searching, or spamming is needed in case of large volumes of data. This study gives a novel approach for the detection of malicious URLs and fast query retrieval. The proposed focused crawler checks URL before entering in the search engine database. It discards malicious URLs but allows benign URLs to enter in search engine database. The detection of malicious URLs is done via the proposed feature set of URL which is created by selecting those attributes of URL features which are susceptible to spammers. Thus, a non-malicious database is created. The searching process performed through this search engine database by triggering a query. Search time taken by the proposed crawler is less as compared to the base crawler. The reason behind it is that the proposed focused crawler uses a trie data structure for storing fetched results in Web repository instead of HashSet data structure as used by the base crawler. On behalf of computed average search time (for ten queries), it is observed that proposed focused crawler is 12% faster than base crawler. To check the performance of proposed focused crawler, quality parameters, i.e., precision and recall, are computed which are found to be 92.3% and 94.73%. Detection accuracy is found to be 90% with an error rate of 10%.

Introduction: Adverse outcomes in hypertrophic cardiomyopathy (HCM) includes arrhythmias, myocardial fibrosis, heart failure, and sudden cardiac arrest (SCA). These are well documented in children. Exercise stress echocardiography (ESE) can identify the severity and mechanism of left ventricular outflow tract (LVOT) obstruction that may not be present at rest. Our aim was to compare clinical outcomes in pediatric patients with varying severity of LVOT obstruction. Methods: We reviewed records of patients < 22 years with HCM who underwent staged ESE Jan 2009 - Dec 2019 at our center. Patients were divided into 3 groups: no resting or exercise LVOT gradient (Group 1), no resting gradient with LVOT gradient > 30 mmHg at peak exercise (Group 2), and resting LVOT gradient > 30 mmHg (Group 3). We assessed echocardiographic indices, advanced imaging, and clinical data. Statistical analysis performed with p-value Results: 93 patients met inclusion criteria. 6 SCA events occurred, though not during ESE.. Patients in Group 3 were classified less often as NYHA Class 1 compared to Group 1 or Group 2, (p < 0.01). Group 3 had the highest rate of myectomy (p=0.005) and ICD placement (p=0.003). There was no significant difference in exercise symptoms, peak oxygen consumption, or ischemic changes during ESE between the groups. Peak heart rate was also higher in Group 2 with no difference in blood pressure response. Groups were not associated with family history of disease, positive gene status, SCA, or the presence of fibrosis (Table). LVOT gradients differed as early as Stage 1 in Groups 1 & 2. Inducible LVOT obstruction was most commonly midcavitary in Group 2. Conclusion: The presence of resting versus inducible LVOT obstruction in pediatric HCM does not appear associated with adverse outcomes. This may be hampered by the rarity of events in pediatrics. The predominant mechanism of inducible LVOT obstruction was midcavitary, which has a link to adverse long-term outcomes in adult HCM.

Background: Elevated left ventricular outflow tract (LVOT) gradients during exercise can occur in patients with hypertrophic cardiomyopathy (HCM) as well as in athletes and normal controls. The authors’ staged exercise protocol calls for imaging at rest and during each stage of exercise to evaluate the mechanism of LVOT obstruction at each stage. They investigated whether this staged approach helps differentiate HCM from athletes and normal controls. Methods: They reviewed pediatric exercise stress echocardiograms completed between January 2009 and October 2017 at their center and identified those with gene-positive HCM, athlete’s heart, and normal controls. Children with inducible obstruction (those with no LVOT gradient at rest who developed a LVOT peak gradient > 25 mm Hg during exercise) were included. LVOT peak gradient, velocity time integral, acceleration time, and deceleration time were measured at rest, submaximal stages, and peak exercise. Results: Compared with athletes, HCM patients had significantly higher LVOT peak gradients at rest (P = .019), stage 1 of exercise (P = .002), and peak exercise (P = .051), as well as a significantly higher change in LVOT peak gradient from rest to stage 1 (P = .016) and from rest to peak (P = .038). The acceleration time/deceleration time ratio of the LVOT Doppler was significantly lower in HCM patients compared with normal controls at peak exercise. Conclusions: The HCM patients who develop elevated LVOT gradients at peak exercise typically manifest early obstruction in the submaximal stages of exercise, which helps to differentiate them from athletes and normal controls.

Background Different methods have resulted in variable Z scores for echocardiographic measurements. Using the measurements from 3,215 healthy North American children in the Pediatric Heart Network (PHN) echocardiographic Z score database, the authors compared the PHN model with previously published Z score models. Methods Z scores were derived for cardiovascular measurements using four models (PHN, Boston, Italy, and Detroit). Model comparisons were performed by evaluating (1) overlaid graphs of measurement versus body surface area with curves at Z = −2, 0, and +2; (2) scatterplots of PHN versus other Z scores with correlation coefficients; (3) Bland-Altman plots of PHN versus other Z scores; and (4) comparison of median Z scores for each model. Results For most measurements, PHN Z score curves were similar to Boston and Italian curves but diverged from Detroit curves at high body surface areas. Correlation coefficients were high when comparing the PHN model with the others, highest with Boston (mean, 0.99) and lowest with Detroit (mean, 0.90). Scatterplots suggested systematic differences despite high correlations. Bland-Altman plots also revealed poor agreement at both extremes of size and a systematic bias for most when comparing PHN against Italian and Detroit Z scores. There were statistically significant differences when comparing median Z scores between the PHN and other models. Conclusions Z scores from the multicenter PHN model correlated well with previous single-center models, especially the Boston model, which also had a large sample size and similar methodology. The Detroit Z scores diverged from the PHN Z scores at high body surface area, possibly because there were more subjects in this category in the PHN database. Despite excellent correlation, significant differences in Z scores between the PHN model and others were seen for many measurements. This is important when comparing publications using different models and for clinical care, particularly when Z score thresholds are used to guide diagnosis and management.

Background Physicians are exposed to workplace factors that may result in acute or chronic stress resulting in burnout. This may impact the productivity and result in suboptimal patient care practices. Methods We surveyed pediatric cardiology attending physicians at our institution to assess their perception of burnout and work-life balance using the Maslach Burnout Inventory and the Areas of Work-Life Survey. Results Forty-five out of the 50 pediatric cardiology attendings responded to the survey. They were divided into 4 groups: Interventional/Electrophysiology [n = 3], Cardiac Intensive Care/Inpatient [n = 8], Non-Invasive Imaging [n = 6], and Outpatient [n = 28]. The Maslach Burnout Inventory demonstrated group-specific scores in the areas of emotional exhaustion, depersonalization, and personal accomplishment that were all significantly better than the general population. However, group-specific Areas of Work-Life Survey results demonstrated concerning findings with respect to the perception of work-life balance. Conclusions Although the Maslach Burnout Inventory did not demonstrate significant burnout among the attending physicians, the Areas of Work-Life Survey results demonstrated reduced work engagement, which can impact patient care and lead to burnout in the future. Based on these results, we plan to implement strategies to help increase work engagement and improve overall organizational effectiveness.

In the pediatric Appropriate Use Criteria (AUC), abnormal electrocardiogram (ECG) in an asymptomatic patient has been rated as an "Appropriate" indication for transthoracic echocardiogram (TTE). We hypothesized that the yield of abnormal findings on TTE for this indication will be low.All asymptomatic patients (≤ 18 years) from January 1, 2015 to December 31, 2017 who underwent initial outpatient evaluation at our center and had a TTE ordered for an abnormal ECG, were included. Clinic records were reviewed to obtain ECG and TTE findings.Of the 199 study patients, 13 (6.5%) had abnormal findings. Incomplete right bundle branch block (IRBBB) had the highest yield of abnormal TTE findings (7/28), with secundum atrial septal defect being the most common (5/7); (Odds ratio (OR) compared to other ECG findings 9.2, 95% CI (2.8-29.9), P .001). OR further increased to 14.6, 95% CI (3.1-68.0), P .001 when either IRBBB, right axis deviation, or right ventricular hypertrophy were present. Left ventricular hypertrophy on ECG had only one incidental abnormality on TTE, while ST segment changes, left axis deviation, right/left atrial enlargement, premature atrial/ventricular contractions, ectopic atrial rhythm, sinus bradycardia/pause, preexcitation, low-grade atrioventricular block, and junctional rhythm did not yield abnormal TTEs.The yield of abnormal findings on TTE when performed for the AUC indication for an abnormal ECG in asymptomatic pediatric patients is low except when performed for ECG abnormalities suggestive of right heart disease such as IRBBB, right axis deviation, or right ventricular hypertrophy. Future revisions of the AUC document could consider further stratification of this indication and corresponding appropriateness ratings based on ECG findings rather than combining into one broad category.

OBJECTIVE The educational intervention (EI) through the Pediatric Appropriate Use of Echocardiography (PAUSE) multicenter study resulted in improved appropriateness of transthoracic echocardiogram (TTE) orders at our center. The current study evaluated if this pattern persisted after cessation of EI and the potential physician characteristics influencing appropriateness. DESIGN Outpatients (≤18 years old) seen for initial evaluation during the EI (July to October, 2015) and 6-month post-EI (May to August, 2016) phases were included. Comparison was made between TTE rates and appropriateness ratings during EI and post-EI phase. Association between TTE rate and appropriateness with physician characteristics (age, experience, patient volume, and area of practice) was determined using odds ratio. RESULTS The study included 7781 patients (EI: N = 4016; post-EI: N = 3765) seen by 31 physicians. Comparison of appropriateness ratings in a randomized sample (EI: N = 1270; post-EI: N = 1325 patients) showed no significant differences between the two phases (appropriate: 75.2% vs 74.9%, P = .960; rarely appropriate 4.1% vs 6.5%, P = .065). Though there was significant variability among physicians for TTE order appropriateness (P = .044) and ordering rate (P

BACKGROUND In patients with pulmonary atresia, intact ventricular septum (PA/IVS) following right ventricular (RV) decompression, RV size and morphology drive clinical outcome. Our objectives were to (1) identify baseline and postdecompression echocardiographic parameters associated with 2V circulation, (2) identify echocardiographic parameters associated with RV growth and (3) describe changes in measures of RV size and changes in RV loading conditions. METHODS We performed a retrospective analysis of patients who underwent RV decompression for PA/IVS at four centers. We analyzed echocardiograms at baseline, postdecompression, and at follow up (closest to 1-year or prior to Glenn circulation). RESULTS Eighty-one patients were included. At last follow-up, 70 (86%) patients had 2V circulations, 7 (9%) had 1.5 ventricle circulations, and 4 (5%) had single ventricle circulations. Follow-up echocardiograms were available in 43 (53%) patients. The majority of patients had improved RV systolic function, less tricuspid regurgitation (TR), and more left-to-right atrial shunting at a median of 350 days after decompression. Multivariable analysis demonstrated that larger baseline tricuspid valve (TV) z-score (P = .017), ≥ moderate baseline TR (P = .045) and smaller baseline RV area (P

ObjectiveMore patients with Fontan physiology are reaching adulthood. The purpose of this meta-analysis was to evaluate the late outcomes of patients palliated with Fontan procedure and to assess the risk factors for mortality.MethodsPubMed, Embase and Web of Science were queried to retrieve observational studies of survival in patients following the Fontan procedure with ≥5 years of follow-up. A random-effects model was used to determine pooled survival estimates at 5, 10 and 15 years. Meta-regression was used to assess potential moderators for death.ResultsNineteen articles with a total of 5859 patients were included. The weighted mean follow-up time was 8.94±2.64 years with overall 8.3% deaths and 1.5% transplants. Pooled survival estimates at 5, 10 and 15 years were 90.7%, 87.2% and 87.5%, respectively; and 88.4%, 85.7% and 84.1%, respectively, for studies that included all three time intervals (n=4). Earliest surgical year included in the study, proportion of atriopulmonary connections versus extracardiac conduit or lateral tunnel, and older age at Fontan were associated with higher rates of death, but ventricular morphology was not. Protein-losing enteropathy, reoperation and pacemaker insertion were reported in 2.1%, 5.6% and 6.8% patients, respectively.ConclusionsSurvival following the Fontan procedure has improved with time and is influenced by Fontan type and age at the time of Fontan. At a mean follow-up of 8.9 years, there was no significant association between survival and ventricular morphology, not taking into account the mortality prior to Fontan.

Objective It is unclear whether neonates with interrupted aortic arch (IAA) and a smaller left ventricular outflow tract may have improved outcomes with a Yasui operation (ventricular outflow bypass procedure) over a primary complete repair. This study sought to identify preoperative echocardiographic parameters to differentiate which neonates may have improved outcomes with a primary vs Yasui operation. Design Patient demographics, cardiac surgery type, complications, need for reoperation and/or interventional catheterization, and date of last follow-up were collected on neonates who underwent a biventricular repair for IAA from 2003 to 2014. Preoperative echocardiograms were analyzed for: IAA type, valve annulus size, aortic valve morphology, ventricular size and aortic arch anatomy. Results Seventy-seven neonates underwent IAA repair between 2003 and 2013. 60 neonates had a primary repair and 17 a Yasui operation. Neonates that underwent a Yasui operation had significantly smaller mitral and aortic valves with aortic arch hypoplasia. Within the primary repair group, a decreasing aortic root z-score on univariate analysis increased the odds of reoperation by twofold [OR = 1.98, 95% CI: (1.15-3.42), P = .014]. A significant interaction between repair type and aortic root z-score was identified on multivariable analysis (P = .039), for neonates with aortic root z-scores less than −2.5, the probability of reoperation during the follow up time period [mean 4.5 years (3.3 months-10 year)] was significantly higher in the primary repair group compared to the Yasui group (64.3% vs 37.5%). Conclusions Neonates with IAA and an aortic root z-score less than −2.5 have lower odds of subsequent reoperations with a Yasui operation compared to a primary repair over the follow up period. These findings suggest a Yasui operation should be considered if the preoperative aortic root z-score is less than −2.5. Careful evaluation of these morphologic predictors on preoperative echocardiograms can be helpful in surgical planning in neonates with IAA.

Although pediatric appropriate use criteria (AUC) for outpatient transthoracic echocardiography (TTE) are available, little is known about TTE utilization patterns before their release. The aims of this study were to determine the relation between AUC and TTE utilization and to identify patient and physician factors associated with discordance between the AUC and clinical practice.A retrospective review of 3,000 initial outpatient pediatric cardiology encounters at six centers was performed. Investigator-determined indications were classified using AUC definitions. Concordance between AUC and TTE utilization was determined. Multivariate analysis was performed to identify patient and physician factors associated with TTE's being performed for rarely appropriate and TTE's not being performed for appropriate indications.Concordance between AUC and TTE utilization was 88%. TTE was performed for rarely appropriate indications in 9% and was associated with patient age3 months, indications of murmur, noninvasive imaging physician subspecialty, and physician volume. No TTE was ordered for appropriate indications in 3% and was associated with indications including prior test result (primarily abnormal electrocardiographic findings), older patients, and physician subspecialty other than generalist or imaging. There was high variability in TTE utilization among centers.There was a reasonable degree of concordance between AUC and clinical practice before AUC publication. Several patient and physician factors were associated with discordance with the AUC. These findings should be considered in efforts to disseminate the AUC and in the development of future iterations. The causes for variation among centers deserve further exploration.

The aim of this study was to describe serial changes in echocardiographic Doppler pulmonary vein flow (PVF) patterns in infants with single right ventricle (RV) anomalies enrolled in the Single Ventricle Reconstruction trial. Measurement of PVF peak systolic (S) and diastolic (D) velocities, velocity time integrals (VTI), S/D peak velocity and VTI ratios, and frequency of atrial reversal (Ar) waves were made at three postoperative time points in 261 infants: early post-Norwood, pre-stage II surgery, and 14 months. Indices were compared over time, between initial shunt type [modified Blalock–Taussig shunt (MBTS) and right ventricle-to-pulmonary artery shunt (RVPAS)] and in relation to clinical outcomes. S velocities and VTI increased over time while D wave was stable, resulting in increasing S/D peak velocity and VTI ratios, with a median post-Norwood S/D VTI ratio of 1.14 versus 1.38 at pre-stage II and 1.89 at 14 months (P

Objectives To characterize the subgroup of outpatient pediatric patients presenting with chest pain and to determine the effectiveness of published pediatric appropriate use criteria (PAUC) to detect pathology. Study design The Pediatric Appropriate Use of Echocardiography study evaluated the use and yield of transthoracic echocardiography (TTE) before and after PAUC release. Data were reviewed on patients ≤18 years of age who underwent TTE for chest pain. Indications were classified as appropriate (A), may be appropriate (M), and rarely appropriate (R) based on PAUC ratings, and findings were normal, incidental, or abnormal. Results Chest pain was the primary indication in 772 of 4562 outpatient TTE studies (17%) (median age 14 years, IQR 10-16) ordered during the study period: 458 of 772 before (59%) and 314 of 772 after (41 %) the release of PAUC with no change in appropriateness. In A indications (n = 654), 642 (98%) were normal, 5 (1%) had incidental findings, and 7 (1%) were abnormal. A and M detected 100% of all abnormal findings (A: n = 7; M: n = 6; R: n = 0), with an association between ratings and findings ( P .001). There was no association between R rating and any pathology. Conclusions There was no change in ordering patterns with publication of the PAUC. Despite the high rate of TTEs ordered for indications rated A, most studies were normal. Studies that detected pathology were performed for indications rated A or M, but not R. This study supports PAUC as a useful tool in pediatric chest pain evaluation that may subsequently improve the use of TTE.

Background Concern exists over exponential growth in cardiac imaging in adults, but there is paucity of such data for cardiac imaging trends in pediatric patients. The aims of this study were to determine temporal trends in the use of noninvasive cardiac imaging and compare these with trends in the use of noncardiac imaging and to identify factors influencing those trends using the Pediatric Health Information Service database. Methods Pediatric inpatient encounter data from January 2004 to December 2017 at 35 pediatric hospitals were extracted from the Pediatric Health Information Service database. Temporal imaging utilization trends in cardiac and noncardiac ultrasound or echocardiography, magnetic resonance imaging (MRI), and computed tomography (CT) were assessed using linear mixed-effects models. Models were adjusted for case-mix index, complex chronic conditions, patient age, length of stay, payer source, and cardiac surgical volume. Results A total of 5,869,335 encounters over 14 years were analyzed (median encounters per center per year, 11,411; median patient age, 4 years; median length of stay, 3 days). From 2004 to 2017, the rates of pediatric inpatient cardiac and noncardiac ultrasound and MRI increased, whereas the rate of noncardiac CT decreased. Cardiac CT use increased beginning in 2014 (+0.264 cardiac CT encounters per 1,000 encounters per year), surpassing the rate of rise of cardiac MRI. Case-mix index, cardiac surgical volume, and payer source affected the largest number of imaging trends. Conclusions Among pediatric inpatients, utilization of cardiac and noncardiac ultrasound and MRI has steadily increased. Noncardiac CT use declined and cardiac CT use increased after 2014. Factors influencing imaging trends include case-mix index, cardiac surgical volume, and payer source. This study lays a foundation for investigations of imaging-related resource utilization and outcomes among pediatric inpatients.