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Background:Intraoperative imaging determines the integrity of surgical repairs. Transoesophageal echocardiography represents standard care for intraoperative imaging in CHD. However, some conditions preclude its use, and epicardial echocardiography is used alternatively. Minimal literature exists on the impact of epicardial echocardiography versus transoesophageal echocardiography. We aimed to evaluate accuracy between the two modalities and hypothesised higher imaging error rates for epicardial echocardiography.Methods:We retrospectively reviewed all epicardial echocardiograms performed over 16 years and compared them to an age- and procedure-matched, randomly selected transoesophageal echocardiography cohort. We detected un- or misidentified cardiac lesions during the intraoperative imaging and evaluated patient outcomes. Data are presented as a median with a range, or a number with percentages, with comparisons by Wilcoxon two-sample test and Fisher’s exact test.Results:Totally, 413 patients comprised the epicardial echocardiography group with 295 transoesophageal echocardiography matches. Rates of imaging discrepancies, re-operation, and incision infection were similar. About 13% of epicardial echocardiography patients had imaging discrepancies versus 16% for transoesophageal (p = 0.2352), the former also had smaller body sizes (p < 0.0001) and more genetic abnormalities (33% versus 19%, p < 0.0001). Death/mechanical support occurred more frequently in epicardial echocardiography patients (16% versus 6%, p < 0.0001), while hospitalisations were longer (25 versus 19 days, p = 0.0003).Conclusions:Diagnostic accuracy was similar between patients undergoing epicardial echocardiography and transoesophageal echocardiography, while rates of death and mechanical support were increased in this inherently higher risk patient population. Epicardial echocardiography provides a reasonable alternative when transoesophageal echocardiography is not feasible.

Targeted neonatal echocardiography (TNE) has been increasingly used at the bedside in neonatal care to provide an enhanced understanding of physiology, affecting management in hemodynamically unstable patients. Traditional methods of bedside assessment, including blood pressure, heart rate monitoring, and capillary refill are unable to provide a complete picture of tissue perfusion and oxygenation. TNE allows for precision medicine, providing a tool for identifying pathophysiology and to continually reassess rapid changes in hemodynamics. A relationship with cardiology is integral both in training as well as quality assurance. It is imperative that congenital heart disease is ruled out when utilizing TNE for hemodynamic management, as pathophysiology varies substantially in the assessment and management of patients with congenital heart disease. Utilizing TNE for longitudinal hemodynamic assessment requires extensive training. As the field continues to grow, guidelines and protocols for training and indications are essential for ensuring optimal use and providing a platform for quality assurance.

Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11). The most recent publication of the IPCCC was in 2017. This manuscript provides an updated 2021 version of the IPCCC.The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature.The members of ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.

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.

In both truncus arteriosus communis (TAC) and tetralogy of Fallot (TOF), there is a rare phenotype that includes a single branch pulmonary artery (PA) arising from a solitary great artery and major aortopulmonary collaterals (MAPCAs) supplying the contralateral lung. We describe the intracardiac and great vessel anatomy of infants with this phenotype, consider rationale for classifying patients as TOF vs. TAC, and describe surgical outcomes. Our institution's surgical database was reviewed for patients with a single branch PA from a solitary arterial trunk and contralateral MAPCAs from 2007 to 2019. Demographic, imaging, and surgical data were collected and described. All 11 patients underwent complete repair with a median right ventricular to aortic pressure ratio of 0.36 (range 0.26-0.50). At 0.1-9.1 years after repair (median 0.8 years) there was approximately balanced left-right lung perfusion (median 52% to the right lung, range 34-74%). The MAPCA lungs exemplified the full spectrum of PA and MAPCA anatomy, from absent intrapericardial PAs with all single supply MAPCAs to a normally arborizing PA with all dual supply MAPCAs and present pulmonary valve leaflet tissue. All patients had a systemic semilunar valve with 3 thin and similarly sized leaflets and fibrous continuity with the tricuspid valve, and all had coronary origins and outflow tract morphology more consistent with TOF. It is appropriate to classify all patients with a single anomalous PA from a solitary arterial trunk and MAPCAs to the contralateral lung as TOF rather than TAC Type A3. All variants were amenable to surgical repair.

Although the Affordable Care Act requires nonprofit hospital organizations to report how they identify and invest in community health needs, the utility of mandated reporting documents for tracking investments in the social determinants of health has been questioned. Using public reporting documents and focusing on housing as a social determinant of health, we describe how nonprofit hospital organizations in 5 communities with the highest rates of homelessness document needs and investments related to housing on their Community Health Needs Assessments, Implementation Strategies, and Schedule H (990H) tax forms. Of 47 organizations, 55% identified housing as a health need, 36% described housing-related implementation strategies, and 26% reported relevant 990H spending. Overall concordance among identified needs, strategies, and spending was low, with only 15% of organizations addressing housing across all 3 documents. Regulatory reform could help promote accountability and transparency in organizations' efforts to address housing and other health-related social needs.

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.

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.

Introduction: Echocardiographic assessment of left ventricular (LV) systolic function by ejection fraction (EF) is crucial for diagnosis and monitoring in pediatric patients. EF requires manual tracing of the LV but measurement reproducibility is affected by diverse phenotypes, image quality, and operator skill. We sought to train a deep learning model (EchoNet-Peds) on a pediatric-specific video dataset to automatically segment the LV and estimate EF by 5/6 Area Length (AL) using two common views, the apical 4 chamber (A4C) and parasternal short axis (PSAX). Methods: EchoNet-Peds evaluated raw A4C and PSAX videos to segment the LV and predict the EF from the A4C and PSAX views. Eighty percent of the dataset was used for algorithm training, 10% for validation, and 10% for testing. We used the training set to predict the EF by AL, determined hyperparameters from the validation set, and reported results using 10-fold cross-validation on the test set. Results: The dataset included 1923 children (43% female) with 4400 echocardiogram studies. EFs ranged from 10% to 81% (61% ± 10.1) and ages from 0 to 18 years (10 ± 5.4 years). EchoNet-Peds achieved Dice Similarity Coefficients of 0.901 (0.895 - 0.907) and 0.887 (0.879 - 0.895) for segmenting the LV in A4C and PSAX views respectively. The A4C view predicts an EF by AL with an R2 of 0.73 (0.67 - 0.77) and the PSAX view predicts an R2 of 0.74 (0.70 - 0.77). Combining both predictions results in an overall R2 of 0.78 (0.74 - 0.81). The model performs well when stratified by sex, age, and body surface area (BSA), and improves in younger patients and lower BSA (Figure 1 b-d). Conclusions: EchoNet-Peds is the first demonstration that AI trained on a pediatric-specific dataset utilizing multiple views can accurately segment the LV and predict EF. The automated functionality of EchoNet-Peds can be used for real-time assessment of cardiac function despite pediatric heterogeneity.

Leftward posterior deviation of the atrial septum primum (LDSP) has been reported in up to 64% of patients with hypoplastic left heart syndrome (HLHS) but there are no published data on its impact on neonatal outcomes. We reviewed the prevalence of LDSP and its correlation with neonatal outcomes in our institution. This was a single-center retrospective study of neonates with HLHS from 2001 to 2019. Echocardiograms were reviewed and the presence or absence of LDSP was noted. To quantify the degree of deviation in patients with LDSP, a new measurement, the deviation index (DI) was calculated using both the subcostal long and short-axis views. Of ninety-four patients with HLHS, fifty-seven (61%) patients were noted to have LDSP. There was no statistically significant difference in gestational age (GA), birth weight (BW), or mortality between patients with and without LDSP. Patients with LDSP had an increased incidence of unplanned reoperation (p 0.01), post-operative cardiac catheterization (p 0.05), and post-operative infection (p 0.05). After correction for GA, BW, HLHS subtype, and type of surgery, LDSP predicted reoperation (OR = 3.6, p 0.01), catheterization (OR = 2.7, p = 0.05), and infection (OR = 3.4, p 0.05). Higher degree of deviation predicted reoperation (DI 0.17), catheterization (DI 0.07), and infection (DI 0.12). There was excellent inter-observer reproducibility of the DI (ICCabsolute-agreement = 0.82, ICCconsistency = 0.90). Patients with LDSP have a higher prevalence of post-operative morbidity. The degree of deviation was found to be predictive of post-operative complications. Pre-operative echocardiographic evaluation of LDSP in patients with HLHS may be helpful in risk stratification and counseling.

Heterotaxy is a complex, multisystem disorder associated with single ventricle heart disease and decreased survival. Ciliary dysfunction is common in heterotaxy and other situs abnormalities (H/SA) and may increase post-operative complications. We hypothesized that patients with H/SA have increased respiratory and renal morbidities and increased in-hospital mortality after Fontan procedure. We queried the Pediatric Health Information System database for hospitalizations with ICD-9/10 codes for Fontan procedure in patients aged 1 through 11 years from 2004 to 2019. H/SA was identified by codes for dextrocardia, situs inversus, asplenia/polysplenia, or atrial isomerism and compared to non-H/SA controls. Outcomes were in-hospital mortality or heart transplantation, ECMO, hemodialysis, length of stay (LOS), and mechanical ventilation or vasoactive medication use ≥ 4 days. We adjusted estimates with multivariable logistic regression. Of 7897 patients at 50 centers, 1366 (17%) met criteria for H/SA. H/SA had worse outcomes for all study measures: death/transplantation (1.9 vs 1.1%, OR 1.74 (95% CI 1.01-3.03); p = 0.047), ECMO (3.7 vs 2.3%, OR 1.74 (1.28-2.35); p 0.001), hemodialysis (2.1 vs 1.2%, OR 1.66 (1.06-2.59); p = 0.026), prolonged mechanical ventilation (13.2% vs 7.6%, OR 1.85 (1.53-2.25); p 0.001) and vasoactive medication use (29.4 vs 19.7%, OR 1.65 (1.43-1.90), and longer LOS (11 (8-17) vs 9 (7-14) days; p 0.001). H/SA is associated with increased cardiovascular, renal, and respiratory morbidity, as well as in-hospital mortality after Fontan procedure. Attention to renal and respiratory needs may improve outcomes in this difficult population. The relationship between ciliary dysfunction and lung and renal morbidity should be explored further.

This cross-sectional study examines the proportion of US nonprofit hospitals with community health needs assessments and implementation strategies as required by the Patient Protection and Affordable Care Act.

Biykem Bozkurt, MD, FACC, FAHA, Chair Hani Jneid, MD, FACC, FAHA, Chair-Elect Sana M. Al-Khatib, MD, FACC, FAHA H. Vernon Anderson, MD, FACC, FAHA Lauren Gilstrap, MD[∗][1] Grant N. Graham, MD[∗][1] Gail K. Jones, MD[∗][1] David Kao, MD[∗][1] Leo Lopez, MD, FACC, FAHA, FASE[∗][1

Graphical abstract, Highlights • The authors report successful palliation of APVS in DORV and right-dominant AVCD. • Fetal echocardiography assists in the prenatal diagnosis. • Prenatal cardiac MRI can measure lung volumes to assess bronchial compression.

Background: Heterotaxy is a multisystem disorder of situs often associated with cardiac, immune, and gastrointestinal abnormalities. Heterotaxy and other situs abnormalities (H/SA) may share ciliary dysfunction as an embryologic origin. Extracardiac manifestations of H/SA may contribute to increased morbidity and mortality after total cavopulmonary connection (Fontan) procedure. We hypothesized that patients with H/SA have worse postoperative outcomes after Fontan than patients without H/SA. Methods: We queried the Pediatric Health Information System (PHIS) database for hospitalizations with ICD-9/10 codes for Fontan procedure in patients aged 1 to 12 years from 2004 to 2019. Those coded for dextrocardia, situs inversus, asplenia/polysplenia, and atrial isomerism were defined as H/SA. Outcomes were in-hospital all-cause mortality or heart transplantation, post-operative ECMO, dialysis, postsurgical length of stay (LOS), and mechanical ventilation >4 days. Outcomes were compared between H/SA and non-H/SA controls with chi-squared or rank-sum test (reported as median (IQR)), and statistically adjusted for surgical center, age, sex, race, genetic anomaly, and pre-operative support with multivariate logistic regression (reported as adjusted odds-ratio (95% CI)). Results: Of 7,897 patients who underwent Fontan at 50 PHIS centers, 1,366 (17%) met criteria for H/SA. Patients with H/SA versus non-H/SA were older (3.7 (2.9-4.8) vs 3.4 (2.7-4.2) years; p Conclusions: We observed increased mortality and postoperative complications in this largest report to date on patients with H/SA after Fontan. We postulate that ciliary dysfunction may contribute to increased lung and renal morbidity, and should be explored further. Adjustment for anatomic complexity and hemodynamics are required for further study .

Introduction: The transition from residency to subspecialty fellowship in pediatric cardiology is challenging, with the daunting requirement to rapidly acquire a broad scope of knowledge and skill. In 2015, a pediatric cardiology boot camp was designed as an educational tool to help prepare trainees for this transition. Hypothesis: A national pediatric cardiology boot camp consistently improves knowledge and decreases anxiety for prospective fellows. Methods: In late spring each year (2015-2019), a 2.5-day intensive program was provided for trainees prior to beginning fellowship in July. Hands-on, simulation-based experiences were provided on topics including anatomy, auscultation, echocardiography, catheterization, cardiovascular intensive care, electrophysiology, heart failure, pulmonary hypertension, and cardiac surgery. Knowledge based exams and surveys were completed by each participant pre- and post-training. Pre- and post-training exam results were compared via paired t-tests and survey results were compared via Wilcoxon rank sum. Results: Over 5 years 144 participants (72 female, 50%) completed the course, representing 40 fellowship programs in the United States and Canada. In aggregate, significant improvement was seen in participants’ knowledge assessment (pre 45 ± 11% vs. post 71 ± 9%; p Conclusions: The Pediatric Cardiology Boot Camp provides a significant and reproducible educational benefit to participants nationwide. This intensive program simultaneously improves learners’ knowledge and alleviates anxiety as they transition to subspecialty training.

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.

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.

PURPOSE OF REVIEW Left-to-right shunts are commonly seen in congenital heart diseases. This review aims to review the anatomy, physiology, and imaging of the four most common left-to-right lesions in adults. RECENT FINDINGS Reported late complications of left-to-right lesions provide guidance in follow-up imaging. Use of three-dimensional echocardiography helps not only in the diagnosis of these defects but also in assistance during device closure. SUMMARY Noninvasive imaging plays a significant role in the diagnosis and management of left-to-right lesions.

Biykem Bozkurt, MD, PhD, FACC, FAHA, Chair H. Vernon Anderson, MD, FACC, FAHA Garth N. Graham, MD, FACC Hani Jneid, MD, FACC, FAHA Gail K. Jones, MD David Kao, MD, FAHA Michael Kutcher, MD, FACC Leo Lopez, MD, FACC Gregory Marcus, MD, FACC, FAHA Jennifer Rymer, MD James E. Tcheng, MD

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.

The Intersocietal Accreditation Commission (IAC) began accrediting echocardiography laboratories in 1996 to improve quality in diagnostic imaging facilities. With no existing data linking accreditation to improved outcomes, the aim of this study was to examine the perceived value of accreditation among individuals who have successfully achieved IAC echocardiography accreditation. An electronic survey was sent to accredited facilities soliciting demographic data along with questions regarding the perceived value of accreditation related to 15 quality indicators; 10.455 emails were sent with 999 responses (9.6%), and 63% of respondents reported improvement in results due to accreditation. Of the 15 quality indicators, the process was perceived as leading to improvement by a majority for 10 of the quality indicators. Nonphysicians tended to report more improvement compared with physicians (64% vs. 54%, P = .056). The perceptions from hospital-based respondents were more favorable than nonhospital-based respondents (67% vs. 59%, P < .001). More than 90% of respondents reported that maintaining accreditation was important for improved quality and better reimbursement. The study showed that IAC echocardiography facility accreditation is perceived by most facilities to improve operations for most quality indicators, particularly regarding study quality and reporting.

In infants with a single right ventricle (RV), stage I palliation involves aortic reconstruction, systemic-to-pulmonary shunt placement, and atrial septectomy. Many require re-intervention for residual coarctation of the aorta (CoA). Doppler echocardiography can detect residual CoA in most infants, but its ability to predict severity has not been studied. This study compares gradients from Doppler interrogation to those from cardiac catheterization in infants with residual CoA. We performed a retrospective study of infants after stage I palliation from 2000 to 2014. Infants with an echocardiogram and catheterization before the second-stage palliative surgery were included. Infants with an echocardiogram >30 days before catheterization were excluded. Doppler-derived gradients were compared to catheterization-derived gradients. Echocardiographic assessment of tricuspid valve (TV) and RV function were recorded. The cohort included 95 infants, and thirty-three (35%) had CoA. Doppler-derived and catheterization-derived gradients correlated weakly in infants with CoA (r = 0.37, p = 0.036) and without CoA (r = 0.35, p = 0.005). Among infants with CoA, 17/33 had none or trivial tricuspid regurgitation (TR) and normal RV function, and Doppler-derived gradients correlated with catheterization gradients in this group (r = 0.71, p = 0.001). In 16/33 infants with ≥moderate TR or RV dysfunction, gradients did not correlate (r = −0.003, p = 0.992). After a stage I palliation in infants with single RV and CoA, Doppler-derived gradients poorly predicted the severity of CoA. Infants with normal TV or RV function had Doppler-derived gradients more predictive of catheterization-derived gradients. Doppler-derived gradients have limited utility in determining the severity of CoA after a stage I palliation.

Pediatric appropriate use criteria (AUC) were recently published for initial outpatient transthoracic echocardiography (TTE). The purpose of this study was to determine the effect of AUC publication on TTE ordering patterns of pediatric cardiologists. Data were prospectively collected on patients who had initial outpatient TTE ordered before (phase I, April to September 2014) and 3 months after (phase II, January to April 2015) AUC document publication at 6 centers. Site investigators assessed each study's indication and assigned AUC appropriateness as "appropriate" (A), "may be appropriate" (M), "rarely appropriate" (R), or "unclassifiable." One hundred three physicians ordered 4,562 TTEs (2,655 phase I and 1,907 phase II). Overall, there was no statistically significant change in the proportion of A, M, or unclassifiable, but R decreased (12.0% to 9.6%, p = 0.01). There was significant variability among the centers in the percentage of studies for indications rated R (4.9% to 34.8%). There was no significant change in any of the appropriateness ratings at 4 centers, a decrease in R and an increase in A at 1 and a decrease in R and increase in unclassifiable at another. The first pediatric AUC document had only a small impact on physician ordering behavior for initial TTEs, including a small decrease in R. There was a significant variability in appropriateness of studies among centers. These data suggest that active educational interventions are required to substantially improve the appropriate use of pediatric TTE in the outpatient setting.

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

BACKGROUND: 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)

Girls and women with Turner syndrome face a lifelong struggle with both congenital heart disease and acquired cardiovascular conditions. Bicuspid aortic valve is common, and many have left-sided heart obstructive disease of varying severity, from hypoplastic left-sided heart syndrome to minimal aortic stenosis or coarctation of the aorta. Significant enlargement of the thoracic aorta may progress to catastrophic aortic dissection and rupture. It is becoming increasingly apparent that a variety of other cardiovascular conditions, including early-onset hypertension, ischemic heart disease, and stroke, are the major factors reducing the life span of those with Turner syndrome. The presentations and management of cardiovascular conditions in Turner syndrome differ significantly from the general population. Therefore, an international working group reviewed the available evidence regarding the diagnosis and treatment of cardiovascular diseases in Turner syndrome. It is recognized that the suggestions for clinical practice stated here are only the beginning of a process that must also involve the establishment of quality indicators, structures and processes for implementation, and outcome studies.