Search

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.

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: 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.

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: 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 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

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.

TDI-MPI has been shown to predict cardiovascular mortality in adults; there are a paucity of data on its use in children. We sought to determine the prognostic significance of TDI-MPI at time of DCM diagnosis in children. Patients aged ≤18 years diagnosed with DCM were included along with age- and sex-matched controls. Echo at diagnosis was analyzed to obtain standard measures of LV function, PW-MPI, and septal and LV free wall TDI-MPI. Survival analysis was used to assess the time to composite outcome of death, VAD, or transplant, stratified by TDI-MPI z-score. The study included 79 patients with DCM and 79 controls. During a median follow-up of 182 days (IQR 41–815 days), 16 underwent VAD placement, 21 underwent cardiac transplant, 6 died, and 36 had event-free survival. The median septal TDI-MPI for cases was 0.70 for patients with DCM vs 0.45 for controls (P < .001). Those with septal TDI-MPI z-scores ≥2 develop events significantly earlier than those with z-score

The optimal treatment for congenital aortic stenosis (AS) has been debated over the past three decades of experience with both balloon aortic valvuloplasty (BAV) and surgical aortic valvotomy (SAV). While BAV has been the mainstay of therapy for children with AS in most centers, recent single-center reports suggest superior results following SAV.We queried Medline, EMBASE and Web of Science for eligible studies.A total of 18 studies were included in our meta-analysis: SAV alone (n = 3), BAV alone (n = 10), and both (n = 5). The mean follow-up duration of BAV patients was 6.5 years, while the mean follow-up duration for SAV patients was 7.2 years. Mortality rates following BAV and SAV were 11% (95% CI, 8-14) and 10% (95% CI, 7-15), respectively. Reintervention following initial procedure for treatment of AS was higher following BAV (37% [95% CI, 30-44]) compared with SAV (25% [95% CI, 20-31]). The predominant reintervention for both the BAV and SAV groups was surgery (SAV or aortic valve replacement [AVR]); the surgical reintervention rate was 59% for BAV (95% CI, 51-66) and 75% for SAV (95% CI, 48-91). Mean time to reintervention was shorter for BAV (2.7 years [95% CI, 1.4-4.1]) compared with SAV (6.9 years [95% CI, 4.4-9.4]). AVR following BAV was 20% (95% CI, 17-23) and following SAV was 17% (95% CI, 12-25). Long-term and mid-term follow-up in these studies showed moderate to severe aortic insufficiency (AI) was present in 28% (95% CI, 20-37) and 19% (95% CI, 12-27) in BAV and SAV patients, respectively.The rate of reintervention following BAV is higher than following SAV. However, survival rates, AVR, and development of late AI following BAV and SAV are equivalent. The costs associated with the two therapies in terms of hospital days and other morbidities should be considered in future comparative studies.

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)

Background Aortopulmonary window (APW) is a rare congenital defect that is often associated with other cardiac lesions. We analyzed our operative strategy to determine whether this had any relationship with outcomes. Methods Early and late outcomes of 40 children who underwent APW repair at our institution during a 20-year period (1994 to 2013) were analyzed. Results Median age at time of the operation was 22 days (interquartile range, 9 to 63 days), and median weight was 3.2 kg (interquartile range, 2.5 to 3.8 kg). Eleven patients (28%) were born prematurely at or before 36 weeks' gestation, and 10 (25%) had genetic/extracardiac malformations. Mean APW size was 0.84 ± 0.28 cm, and by the Mori classification was type I in 17 patients (43%), type II in 18 (45%), and type III in 5 (13%). Twenty-five patients (63%) had simple APW, with no associated cardiac lesions other than atrial septal defect or patent ductus arteriosus, whereas 15 (38%) had complex APW with one or more associated lesions, including interrupted aortic arch (n = 6), ventricular septal defect (n = 6), or other (n = 5). There were no hospital or late deaths. Four patients required cardiac reoperations, 3 of whom had interrupted aortic arch. The 10-year freedom from cardiac reoperation was 100% for simple APW vs 73% for complex APW ( p = 0.008), with 75% of reoperations related to aortic obstruction. Age, weight, prematurity, extracardiac anomalies, APW size and type, and APW repair technique were not associated with reoperation risk. Conclusions Current outcomes of early repair of APW are excellent, including infants with complex associated cardiac lesions. Compared with historic results, contemporary outcomes are favorable, supporting early and complete repair of APW and associated lesions. Cardiac reoperation can be required in complex APW, mainly with concomitant arch repair, and is usually related to aortic obstruction.

Objective To determine the appropriateness and yield of transthoracic echocardiograms (TTE) for murmur evaluation based on the pediatric Appropriate Use Criteria (AUC) and study the influence of patient age and physician experience on TTE appropriateness, yield, and ordering frequency. Design Retrospective review of medical records of patients referred to our practice for murmur evaluation from April to September 2014. Data collected included indication for TTE, patient age, physician experience since fellowship, TTE findings and exit diagnosis. Appropriateness was assigned based on the AUC document. Setting Pediatric cardiology clinics affiliated with a large pediatric cardiology practice. Patients. One thousand seven hundred one consecutive patients (≤ 18 years) referred to our practice for murmur evaluation. Interventions Not applicable Outcome Measures The primary outcome was appropriateness of TTE orders. The secondary outcomes were the yield of abnormal TTE findings and the influence of patient age and physician experience on appropriateness, yield, and frequency of ordering TTEs. Results Of the 1701 patients referred for a murmur, 526 (30.9%) had a TTE [441/526 (83.8%) Appropriate; 85/526 (16.2%) Rarely Appropriate]. Abnormal findings were present in 130/441 rated Appropriate and none rated Rarely Appropriate. Infants 20 years of experience not only had the lowest TTE ordering rate but also the lowest appropriateness rate with no difference in the yield of abnormal findings. Conclusions Most TTEs ordered for murmur were for indications rated Appropriate. Abnormal findings were present in one-fourth and only those rated Appropriate. Patient age and physician experience can significantly influence TTE utilization. This information is helpful in designing quality initiatives to optimize TTE utilization for murmur evaluation.

BACKGROUND Left ventricular outflow tract obstruction (LVOTO) is a common complication following surgical repair of atrioventricular septal defect (AVSD). OBJECTIVE We sought to determine predictors of LVOTO based on echocardiograms performed prior to initial repair of AVSD. METHODS Of the 415 children that had repair of AVSD from 2003 to 2012, 17 children were identified with LVOTO that required surgical intervention. Thirty-four patients with repaired AVSD and no LVOTO served as controls. Patient demographics, cardiac surgery type, and echocardiogram results at last follow-up were collected. Off-line analysis of the echocardiogram prior to AVSD repair was done to obtain: left ventricular outflow tract (LVOT) and interventricular septal diameter, chordae across LVOT, aortoseptal angle, left ventricular (LV) inflow/outflow length ratio, inferior displacement of the atrioventricular (AV) valve, atrioventricular valve index (AVVI) and presence of coarctation of the aorta. RESULTS The LVOTO group had significantly smaller indexed LVOT diameters (P

Background: Before the integration of the pediatric appropriate use criteria (AUC) for initial transthoracic echocardiography (TTE) in the outpatient setting with our electronic medical record (EMR), there was a high proportion of “rarely appropriate” TTEs (17.2%) ordered for palpitations/arrhythmias. We studied appropriateness ratings and applicability of pediatric AUC on the initial outpatient evaluation of children with palpitations/arrhythmias after EMR integration and the yield of abnormal TTEs for these indications. Methods: We obtained data after the EMR integration of the AUC at our institution. The TTE ordering physician assigned the AUC indication and the corresponding appropriateness ratings autopopulated as: appropriate (A), may be appropriate (M), and rarely appropriate (R). We recorded the abnormal TTE findings. Results: A total of 463 TTEs were ordered for palpitations/arrhythmias. Overall, 142 (30.7%) were for A, 263 (56.8%) for M, 41 (8.8%) for R, and 17 (3.7%) for “unclassifiable” indications. Only 14 (3.0%) had abnormal TTE findings, of which none were for indications rated R. A TTE ordered for premature ventricular contractions in a 17-year-old revealed a significant abnormality (moderate atrial septal defect). Conclusions: The integration of AUC with the EMR significantly improved the appropriate utilization of TTE for palpitations and arrhythmias with a decrease in the proportion of TTEs for R indications (17.2%–8.8%). Although the yield of abnormal findings on TTE performed for palpitations/arrhythmias is quite low, the AUC ratings successfully stratified the indication with no abnormalities for indications rated R. These findings can guide future revisions of AUC indications and ratings to optimize resource utilization.

This document is the second of 2 companion appropriate use criteria (AUC) documents developed by the American College of Cardiology, American Association for Thoracic Surgery, American Heart Association, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Thoracic Surgeons. The first document

Objective To determine whether implementation of a standardized clinical practice guideline (CPG) for the evaluation of syncope would decrease practice variability and resource utilization. Design A retrospective review of medical records of patients presenting to our practice for outpatient evaluation of syncope before and after implementation of the CPG. The guideline included elements of history, physical exam, electrocardiogram, and “red flags” for further testing. Setting Outpatient pediatric cardiology offices of a large pediatric cardiology practice. Patients. All new patients between 3 and 21 years old, who presented to cardiology clinic with a chief complaint of syncope. Interventions The CPG for the evaluation of pediatric syncope was presented to the providers. Outcome Measures Resource utilization was determined by the tests ordered by individual physicians before and after initiation of the CPG. Patient final diagnoses were recorded and the medical records were subsequently reviewed to determine if any patients, who presented again to the system, were ultimately diagnosed with cardiac disease. Results Of the 1496 patients with an initial visit for syncope, there was no significant difference in the diagnosis of cardiac disease before or after initiation of the CPG: (0.6% vs. 0.4%, P = .55). Electrocardiography provides the highest yield in the evaluation of pediatric syncope. Despite high compliance (86.9%), there were no overall changes in costs ($346.31 vs. $348.53, P = .85) or in resource utilization. There was, however, a decrease in the variability of ordering of echocardiograms among physicians, particularly among those at the extremes of utilization. Conclusions Although the CPG did not decrease already low costs, it did decrease the wide variability in echo utilization. Evaluation beyond detailed history, physical exam, and electrocardiography provides no additional benefit in the evaluations of pediatric patients presenting with syncope.

Newborns with hypoplastic left heart syndrome and other single right ventricular variants require substantial health care resources. Weekend acute care has been associated with worse outcomes and increased resource use in other populations but has not been studied in patients with single ventricle. Subjects of the Single Ventricle Reconstruction trial were classified by whether they had a weekend admission and by day of the week of Norwood procedure. The primary outcome was hospital length of stay (LOS); secondary outcomes included transplant-free survival, intensive care unit (ICU) LOS, and days of mechanical ventilation. The Student's t test with log transformation and the Wilcoxon rank-sum test were used to analyze associations. Admission day was categorized for 533 of 549 subjects (13% weekend). The day of the Norwood was Thursday/Friday in 39%. There was no difference in median hospital LOS, transplant-free survival, ICU LOS, or days ventilated for weekend versus non-weekend admissions. Day of the Norwood procedure was not associated with a difference in hospital LOS, transplant-free survival, ICU LOS, or days ventilated. Prenatally diagnosed infants born on the weekend had lower mean birth weight, younger gestational age, and were more likely to be intubated but did not have a difference in measured outcomes. In conclusion, in this cohort of patients with single right ventricle, neither weekend admission nor end-of-the-week Norwood procedure was associated with increased use of hospital resources or poorer outcomes. We speculate that the complex postoperative course following the Norwood procedure outweighs any impact that day of admission or operation may have on these outcomes.

Background Recently published appropriate use criteria (AUC) for initial pediatric outpatient transthoracic echocardiography (TTE) have not yet been evaluated for clinical applicability. Objectives This study sought to determine the appropriateness of TTE as currently performed in pediatric cardiology clinics, diagnostic yield of TTE for various AUC indications, and any gaps in the AUC document. Methods Data were prospectively collected from patients undergoing initial outpatient TTE in 6 centers. TTE indications (appropriate [A], may be appropriate [M], or rarely appropriate [R]) and findings (normal, incidental, or abnormal) were recorded. Results Of the 2,655 studies ordered by 102 physicians, indications rated A, M, and R were found in 1,876 (71%), 316 (12%), and 319 studies (12%), respectively, and 144 studies (5%) were unclassifiable. Twenty-four of 113 indications (21%) were not used. Innocent murmur and syncope or palpitations with no other indications of cardiovascular disease, a benign family history, and normal electrocardiogram accounted for 75% of indications rated R. Pathologic murmur had the highest yield of abnormal findings (40%). Odds of an abnormal finding in an A or M TTE were 6 times that of R (95% confidence interval [CI]: [2.8 to 12.8]). Abnormal findings were more common in patients 10 years of age (odds ratio: 6.4; 95% CI: 4.7 to 8.7). Age was a significant predictor of an abnormal finding after adjusting for indication and site (p Conclusions Most TTEs ordered in pediatric cardiology clinics were for indications rated A. AUC ratings successfully stratified indications based on the yield of abnormal findings. This study identified differences in the yield of TTE based on patient age and most common indications rated R. These findings should inform quality improvement efforts and future revisions of the AUC document.

We sought to review current-era experience with multistage palliation of neonates with hypoplastic left heart syndrome (HLHS) and to examine the patients' anatomic, and surgical risk factors influencing outcomes.A retrospective review of 219 HLHS patients who underwent the Norwood operation from 2002 to 2012 was performed. Competing risks analyses modeled events after the Norwood operation (death/transplantation, progression to Glenn operation) and after the Glenn operation (death/transplantation progression to Fontan operation), and examined the risk factors affecting outcomes.Competing risks analysis showed that 1 year after the Norwood operation, 25% of patients had died and 75% had undergone the Glenn operation. Three years after the Glenn operation, 11% of patients had died or received transplantation, 48% had undergone the Fontan procedure, and 41% were alive awaiting the Fontan. The overall 8-year survival after the Norwood operation was 66%. The risk factors for mortality on multivariable models were postoperative requirement for extracorporeal membrane oxygenation (hazard ratio [HR]: 3.1 [1.8-5.4], p0.001), genetic/major extracardiac anomalies (HR: 2.7 [1.4-5.2], p = 0.002), unplanned cardiac reoperation (HR: 2.5 [1.3-5.0], p = 0.007), modified Blalock-Taussig shunt use (HR: 2.4 [1.4-3.8], p0.001), and prematurity (HR: 1.9 [1.1-3.5], p = 0.030). Of note, anatomic factors such as HLHS subtype, ascending aorta diameter, restrictive atrial septum, and associated cardiac lesions did not affect outcomes (p0.10 each).This current single-institution experience demonstrates that patients' factors (ie, prematurity, low weight, genetic/extracardiac anomalies) continue to adversely affect survival. Conversely, surgical and perioperative management advances, along with the use of the right ventricle to pulmonary artery shunt, might have neutralized the effects of anatomic factors on survival. Technical imperfections requiring reoperations are associated with failure to progress through palliation stages and diminished survival.

Objective This study aims to determine echocardiographic parameters associated with spontaneous patent ductus arteriosus (PDA) closure in extremely low-birth-weight (ELBW) infants. Study Design Retrospective demographic review and analysis of echocardiograms from 189 ELBW infants with suspected and confirmed hemodynamically significant PDA identified on an initial echocardiogram was performed. Comparison of echocardiographic parameters was made between infants with spontaneous closure versus those who received treatment. Results The mean birth weight (787 ± 142 vs. 724 ± 141 g, p = 0.04) and gestational age (27.4 ± 2.8 vs. 26.2 ± 1.6 weeks, p = 0.03) were higher in the spontaneous closure versus the treatment group. Antegrade pulmonary artery (PA) diastolic velocity was lower in infants with spontaneous PDA closure versus those who received treatment (0.15 ± 0.06 vs. 0.22 ± 0.12 m/s, p = 0.009). Conclusion Heavier and more mature ELBW infants with a lower antegrade PA diastolic velocity were likely to have spontaneous closure of the PDA.

We sought to evaluate the prevalence of delayed puberty and abnormal anthropometry and its association with quality of life (QoL) in young Fontan survivors.This was a cross-sectional study at 11 Pediatric Heart Network centers. Demographic and clinical data, anthropomety, and Tanner stage were collected. Anthropometric measurements and pubertal stage were compared to US norms. QoL was assessed using Pediatric Quality of Life inventory (PedsQL). Mixed effects regression modeling adjusting for clustering by center was used to evaluate factors associated with abnormal anthropometry and delayed puberty and associations with QoL.Of the 299 subjects, 42% were female. The median enrollment age was 13.9 years, and the median age at Fontan was 3 years. Fontan survivors had a higher prevalence of short stature relative to normative data (20% vs 5%, P .0001) and an increased prevalence of abnormal BMI (16% vs 10%, P .0001) (low [43%] and high [57%]). Fontan subjects, both males (58%) and females (58%), had a delay of 1.5-2 years in ≥1 Tanner stage parameter compared to normal population. There was no association between delayed puberty and QoL. Abnormal anthropometry was associated with lower overall (62.3 ± 17.3 vs 72.5 ± 16.6; P .001) and physical appearance scores (72.2 ± 27.4 vs 79.8 ± 21.5; P .01). Lower exercise capacity was associated with abnormal anthropometry and2 surgeries before Fontan was associated with delayed puberty. Lower family income ($25 000) and hypoplastic left heart syndrome were associated with lower QoL.Compared to the normal population, Fontan survivors have high prevalence of short stature, abnormal BMI and delayed puberty. Abnormal anthropometry, but not delayed puberty, was associated with lower overall QoL and perceived physical appearance scores. Routine screening for abnormal anthropometry, especially in HLHS and in lower socioeconomic status families, should be considered to allow interventions, which might ameliorate the negative psychosocial impact.

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.

Background— Outcomes after right ventricle (RV) decompression in infants with pulmonary atresia with intact ventricular septum vary widely. Descriptions of outcomes are limited to small single-center studies. Methods and Results— Neonates undergoing RV decompression for pulmonary atresia with intact ventricular septum were included from 4 pediatric centers. Primary end point was reintervention post-RV decompression; secondary end points included circulation type at latest follow-up. Ninety-nine patients (71 with pulmonary atresia with intact ventricular septum and 28 with virtual atresia) underwent RV decompression at median 3 (25th–75th, 2–5) days of age. Seventy-one patients (72%) underwent at least 1 reintervention after decompression. Median duration of follow-up was 3 years (range, 1–10). Freedom from reintervention was 51% at 1 month and 23% at 3 years. In multivariable analysis, reintervention was associated with virtual atresia (hazard ratio [HR], 0.51; 95% confidence interval [CI], 0.28–091; P =0.027), smaller RV length (HR, 0.94; 95% CI, 0.89–0.99; P =0.027), and ≤mild tricuspid regurgitation (TR; HR, 3.58; 95% CI, 2.04–6.30; P P =0.02) and more likely to have higher RV end-diastolic pressure (HR, 1.07; 95% CI, 1.00–1.15; P =0.057) and ≤mild TR (HR, 3.50; 95% CI, 1.75–7.0; P P =0.0037). Multivariable analysis indicated that P P Conclusions— Patients with pulmonary atresia with intact ventricular septum deemed suitable for RV decompression have a high reintervention burden although most achieve 2-ventricle circulation. TR ≤mild at baseline is strongly associated with reintervention and

Robert M. Campbell, MD, FACC, FAHA, FAAP, FHRS, Chair [∗][1] Pamela S. Douglas, MD, MACC, FAHA, FASE, Moderator [∗][1] Louis I. Bezold, MD, FACC, FAAP, FASE[†][2] William B. Blanchard, MD, FACC, FAHA, FAAP[∗][1] Jeffrey R. Boris, MD, FACC[∗][1] Bryan Cannon, MD[‡][3] Gregory J.

Since 2005, the American College of Cardiology Foundation, in conjunction with other societies, has released Appropriate Use Criteria (AUC) for various procedures and imaging modalities. The AUC are being increasingly recognized as an important link in the chain of quality improvement processes in cardiovascular patient care. Article see p 7 Implementation studies evaluating AUC in usual clinical care have been instrumental in benchmarking the appropriateness and inappropriateness rates. A recent meta-analysis summarizing ≈60 published reports in >100 000 imaging tests found the rate of both appropriateness and rarely or inappropriate imaging test to vary widely by modality, as well as by iteration of the AUC document (Table).1 These data provide important information on how testing is used in usual care, thereby creating the framework for designing quality improvement initiatives. Such initiatives require physicians to change their ordering practices—not an easy task. A broad variety of methods have been proposed to change behavior from passive education to active interventions, with the latter being more effective and harder to implement.2 View this table: Table. Published Data on Appropriateness of Noninvasive Testing by Imaging Modality (After Fonseca et al1) In this issue of Circulation: Cardiovascular Quality and Outcomes , Chaudhuri et al3 provide a systematic review of studies using quality improvement initiatives to reduce the proportion of rarely appropriate cardiovascular imaging based on AUC.3 The meta-analysis used stringent criteria and included just 7 studies with 6 observational studies and 1 randomized control trial, assessing the use of noninvasive imaging. These studies varied significantly in terms of the number of patients, type of imaging modalities, target population for intervention, and the type and practice location of providers. Not surprisingly, the strategies used also varied, ranging from passive interventions, such as lectures, webinars, and pocket cards to more active ones, such as …

The objective of this study was to evaluate effectiveness of educational intervention (EI) in the Pediatric Appropriate Use of Echocardiography (PAUSE) study to improve appropriateness of transthoracic echocardiograms (TTEs) ordered in pediatric cardiology clinics.Data were prospectively collected after the publication of the Appropriate Use Criteria (AUC) document during 2 phases: the pre-EI phase (1/1/15 to 4/30/15) and the post-EI phase (7/1/15 to 10/30/15). Pre-EI, site-investigators (SI) determined AUC indications, by reviewing the clinic records. Post-EI, providers assigned indications prior to obtaining TTE.Pediatric cardiology clinics at six centers.Those ≤18 years old, receiving initial outpatient TTE.EI included (i) sharing the pre-EI appropriateness ratings with providers, (ii) lecture on AUC, (iii) providers self-assigning indications, and (iv) monthly e-mail feedback by SI to individual providers.The primary outcome measure was a change in the proportion of studies for indications rated R following EI.Of the 4542 TTEs (1907 pre-EI, 2635 post-EI) ordered by 90 physicians, overall comparison of appropriateness ratings before and after EI showed an increase in Appropriate (72.5%-76.2%, P = .004), no change in May Be Appropriate, and a decline in Rarely Appropriate (R) from 9.6% to 7.4%, P = .008. Following EI, a significant decline in R was observed only in three centers and EI did not affect the variation in TTEs ordered for R indications among physicians (P = .467). Physicians with the highest proportion of TTEs ordered for R before EI, showed the most significant decline in R.Appropriateness of pediatric outpatient TTE varies substantially by center. A customized EI resulted in modest improvement in the appropriateness of TTEs in the PAUSE study, with an increase in Appropriate and a decrease in R TTEs. Multifaceted EIs are required to improve adherence to national standards such as AUC.

We sought to determine the relationship between aortic valve morphology and left ventricular (LV) systolic function in children with aortic stenosis (AS) prior to balloon aortic valvuloplasty (BAV).Both aortic valve morphology and LV systolic function have been linked with outcomes in children with congenital AS undergoing BAV. The relationship between aortic valve morphology and LV function is poorly defined despite their importance in regard to outcomes.We performed a retrospective multicenter cohort study of 89 AS patients who underwent BAV between 2007-2013. Pre-BAV echocardiograms were analyzed for: aortic valve opening (AVO); aortic valve type (true bicuspid, functionally bicuspid, or unicuspid); maximal raphe length; aortic valve leaflet symmetry; and valve angle of excursion. The primary endpoint was low function, defined as LV shortening fraction (LVSF)28%.Median patient age was 0.17 years (interquartile range [IQR], 0.10-10.74 years) and the median aortic valve mean gradient was 47.00 mm Hg (IQR, 36.75-56.00 mm Hg). Multivariate analysis demonstrated that low AVO (P=.03) was associated with reduced LV function, independent of age or aortic valve gradient (R² = .652). Bicuspid aortic valve (P=.07) was associated with improved LV function compared with functionally unicuspid aortic valve. Low AVO0.10 was associated with higher adverse outcome.LV systolic function is most significantly influenced by degree of valve stenosis. Qualitative aspects such as valve type may also affect LV systolic function. Further study may elucidate whether aortic valve morphology or LV function is the principal predictor of response to BAV and of late outcomes after BAV.