Tricuspid Atresia with Absent Pulmonary Valve with Nearly Discontinuous Branch Pulmonary Arteries.

Absent pulmonary valve with tricuspid atresia or tricuspid stenosis (APV-TA/TS) is an extremely rare congenital heart defect associated with significant morbidity and mortality. Compared to Tetralogy of Fallot with Absent Pulmonary Valve Syndrome, branch pulmonary arteries are not typically significantly dilated. We present the case of a newborn male prenatally diagnosed APV-TA with intact ventricular septum (IVS) and nearly discontinuous branch pulmonary arteries, the surgical strategy employed, and the salient hemodynamic factors considered in the medical decision-making.

Cardiovascular Manifestations of Turner Syndrome: Phenotypic Differences Between Karyotype Subtypes.

Turner syndrome (TS) is a genetic disorder presenting in phenotypic females with total or partial monosomy of the X chromosome. Cardiovascular abnormalities are common, including congenital heart defects (CHD) and aortic dilation. Although mosaic TS is suspected to have less severe phenotype as compared to non-mosaic TS, differences in cardiovascular manifestations between karyotypes are not well studied. This is a single-center retrospective cohort study including patients with TS seen from 2000 to 2022. Demographic data, chromosomal analysis, and imaging were reviewed. Karyotypes were categorized as monosomy X (45X), 45X mosaicism, isochromosome Xq, partial X deletions, ring X (r(X)), TS with Y material, and others. Prevalence of CHD and aortic dilation were compared between monosomy X and other subtypes using Pearson's chi-square test and Welch two-sample t-test. We included 182 TS patients with median age 18 (range 4-33) years. CHD was more common in monosomy X as compared with others (61.4% vs. 26.8%, p < 0.001), including bicuspid aortic valve (44.3% vs. 16.1%, p < 0.001), partial anomalous pulmonary venous return (12.9% vs. 2.7%, p = 0.023), persistent left superior vena cava (12.9% vs. 1.8%, p = 0.008), and coarctation of the aorta (20.0% vs. 4.5%, p = 0.003). Cardiac surgery (24.3% vs. 8.9%, p = 0.017) was more prevalent in the monosomy X group. There was no statistically significant difference for presence of aortic dilation (7.1% vs 1.8%, p = 0.187). Although CHD and need for cardiac surgery are more common in TS with monosomy X as compared to others, all TS subtypes may have similar risk of developing aortic dilation. All TS patients should have similar cardiovascular surveillance testing to monitor for aortic dilation.

Assessment of the quality of the vital registration system for under-5 mortality in Yucatan, Mexico.

INTRODUCTION: Vital registration is an important element in health information systems which can inform policy and strengthen health systems. Mexico has a well-functioning vital registration system; however, there is still room for improvement, especially for deaths of children under 5. This study assesses the quality of the vital registration system in capturing deaths and evaluates the quality of cause of death certification in under-5 deaths in Yucatan, Mexico. METHODS: We collected information on under-5 deaths that occurred in 2015 and 2016 in Yucatan, Mexico. We calculated the Vital Statistics Performance Index (VSPI) to have a general assessment of the vital registration performance. We examined the agreement between vital registration records and medical records at the individual and population levels using the chance-corrected concordance (CCC) and cause-specific mortality fraction (CSMF) accuracy as quality metrics. RESULTS: We identified 966 records from the vital registry for all under-5 deaths, and 390 were linked to medical records of deaths occurring at public hospitals. The Yucatan vital registration system captured 94.8% of the expected under-5 deaths, with an overall VSPI score of 87.2%. Concordance between underlying cause of death listed in the vital registry and the cause determined by the medical record review varied substantially across causes, with a mean overall chance-corrected concordance across causes of 6.9% for neonates and 46.9% for children. Children had the highest concordance for digestive diseases, and neonates had the highest concordance for meningitis/sepsis. At the population level, the CSMF accuracy for identifying the underlying cause listed was 35.3% for neonates and 67.7% for children. CONCLUSIONS: Although the vital registration system has overall good performance, there are still problems in information about causes of death for children under 5 that are related mostly to certification of the causes of death. The accuracy of information can vary substantially across age groups and causes, with causes reported for neonates being generally less reliable than those for older children. Results highlight the need to implement strategies to improve the certification of causes of death in this population.

Impact of the Salud Mesoamerica Initiative on delivery care choices in Guatemala, Honduras, and Nicaragua.

BACKGROUND: The Salud Mesoamérica Initiative (SMI) is a public-private collaboration aimed to improve maternal and child health conditions in the poorest populations of Mesoamerica through a results-based aid mechanism. We assess the impact of SMI on the staffing and availability of equipment and supplies for delivery care, the proportion of institutional deliveries, and the proportion of women who choose a facility other than the one closest to their locality of residence for delivery. METHODS: We used a quasi-experimental design, including baseline and follow-up measurements between 2013 and 2018 in intervention and comparison areas of Guatemala, Nicaragua, and Honduras. We collected information on 8754 births linked to the health facility closest to the mother's locality of residence and the facility where the delivery took place (if attended in a health facility). We fit difference-in-difference models, adjusting for women's characteristics (age, parity, education), household characteristics, exposure to health promotion interventions, health facility level, and country. RESULTS: Equipment, inputs, and staffing of facilities improved after the Initiative in both intervention and comparison areas. After adjustment for covariates, institutional delivery increased between baseline and follow-up by 3.1 percentage points (ß = 0.031, 95% CI -0.03, 0.09) more in intervention areas than in comparison areas. The proportion of women in intervention areas who chose a facility other than their closest one to attend the delivery decreased between baseline and follow-up by 13 percentage points (ß = - 0.130, 95% CI -0.23, - 0.03) more than in the comparison group. CONCLUSIONS: Results indicate that women in intervention areas of SMI are more likely to go to their closest facility to attend delivery after the Initiative has improved facilities' capacity, suggesting that results-based aid initiatives targeting poor populations, like SMI, can increase the use of facilities closest to the place of residence for delivery care services. This should be considered in the design of interventions after the COVID-19 pandemic may have changed health and social conditions.

Height Versus Body Surface Area to Normalize Cardiovascular Measurements in Children Using the Pediatric Heart Network Echocardiographic Z-Score Database.

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.

Factors associated with delays in the search for care in under-5 deaths in Yucatán, Mexico.

OBJECTIVE: We examined delays during the search for care and associations with mother, child, or health services characteristics, and with symptoms reported prior to death. MATERIALS AND METHODS: Cross-sectional study compris-ing household interviews with 252 caregivers of children under-5 who died in the state of Yucatán, Mexico, during 2015-2016. We evaluated the three main delays: 1) time to identify symptoms and start search for care, 2) transport time to health facility, and 3) wait time at health facility. RESULTS: Children faced important delays including a mean time to start the search for care of 4.1 days. The mean transport time to the first facility was longer for children enrolled in Seguro Popular and there were longer wait times at public facilities, especially among children who also experienced longer travel time. CONCLUSIONS: Providing resources to enable caregiv-ers to access health services in a timely manner may reduce delays in seeking care.

Challenges and lessons learned from the Pediatric Heart Network Normal Echocardiogram Database study.

BACKGROUND: The Pediatric Heart Network Normal Echocardiogram Database Study had unanticipated challenges. We sought to describe these challenges and lessons learned to improve the design of future studies. METHODS: Challenges were divided into three categories: enrolment, echocardiographic imaging, and protocol violations. Memoranda, Core Lab reports, and adjudication logs were reviewed. A centre-level questionnaire provided information regarding local processes for data collection. Descriptive statistics were used, and chi-square tests determined differences in imaging quality. RESULTS: For the 19 participating centres, challenges with enrolment included variations in Institutional Review Board definitions of "retrospective" eligibility, overestimation of non-White participants, centre categorisation of Hispanic participants that differed from National Institutes of Health definitions, and exclusion of potential participants due to missing demographic data. Institutional Review Board amendments resolved many of these challenges. There was an unanticipated burden imposed on centres due to high numbers of echocardiograms that were reviewed but failed to meet submission criteria. Additionally, image transfer software malfunctions delayed Core Lab image review and feedback. Between the early and late study periods, the proportion of unacceptable echocardiograms submitted to the Core Lab decreased (14 versus 7%, p < 0.01). Most protocol violations were from eligibility violations and inadvertent protected health information disclosure (overall 2.5%). Adjudication committee reviews led to protocol changes. CONCLUSIONS: Numerous challenges encountered during the Normal Echocardiogram Database Study prolonged study enrolment. The retrospective design and flaws in image transfer software were key impediments to study completion and should be considered when designing future studies collecting echocardiographic images as a primary outcome.

Practice Variations in Pediatric Echocardiography Laboratories.

Quantification guidelines for pediatric echocardiograms were published in 2010 establishing consensus regarding standard measurements. However, a standard protocol for performance and analysis of pediatric echocardiograms was not defined. This study aims to identify practice variations among pediatric laboratories. A survey was sent to 85 North American pediatric laboratory directors. The survey included 29 questions assessing: demographics, methods of image acquisition, parameters routinely evaluated and reported, and methods used to assess chamber sizes, valves, and ventricular function. There were 47/85 (55%) responses; 83% were academic centers and 77% in an urban setting. Wide variations exist in acquisition method (clips versus sweeps) and color scale settings. The most commonly used methods for left ventricular (LV) function are M-mode shortening fraction, qualitative assessment, and Doppler Tissue Imaging. The most commonly used parameter for right ventricular function is qualitative. LV mass is routinely measured by the majority of centers with variations in methods of calculation. Conversely, while a minority measure left atrial volume, there is consensus regarding the preferred method. While multiple techniques exist for assessing valves, qualitative assessment is reported to be the preferred method. Despite quantification guidelines, there is a lack of uniformity in performance and analysis of pediatric echocardiograms. Further studies are needed to determine why variations exist and whether development of consensus guidelines might improve interpretation, consistency and quality of reports, patient care, and provide a standardized system allowing for comparative research among centers.

Consensus-Based Development of a Pediatric Echocardiography Complexity Score: Design, Rationale, and Results of a Quality Improvement Collaborative.

BACKGROUND: The complexity of congenital heart disease has been primarily stratified on the basis of surgical technical difficulty, specific diagnoses, and associated outcomes. We report on the refinement and validation of a pediatric echocardiography complexity (PEC) score. METHODS AND RESULTS: The American College of Cardiology Quality Network assembled a panel from 12 centers to refine a previously published PEC score developed in a single institution. The panel refined complexity categories and included study modifiers to account for complexity related to performance of the echocardiogram. Each center submitted data using the PEC scoring tool on 15 consecutive inpatient and outpatient echocardiograms. Univariate and multivariate analyses were performed to assess for independent predictors of longer study duration. Among the 174 echocardiograms analyzed, 68.9% had underlying congenital heart disease; 44.8% were outpatient; 34.5% were performed in an intensive care setting; 61.5% were follow-up; 46.6% were initial or preoperative; and 9.8% were sedated. All studies had an assigned PEC score. In univariate analysis, longer study duration was associated with several patient and study variables (age <2 years, PEC 4 or 5, initial study, preoperative study, junior or trainee scanner, and need for additional imaging). In multivariable analysis, a higher PEC score of 4 or 5 was independently associated with longer study duration after controlling for study variables and center variation. CONCLUSIONS: The PEC scoring tool is feasible and applicable in a variety of clinical settings and can be used for correlation with diagnostic errors, allocation of resources, and assessment of physician and sonographer effort in performing, interpreting, and training in pediatric echocardiography.

MRI validated echocardiographic technique to measure total cardiac volume: a tool for donor-recipient size matching in pediatric heart transplantation.

Our aim is to develop and validate an accurate method for estimating TCV using standard echocardiographic imaging that can be easily employed to aid in donor-recipient size matching in pediatric heart transplantation. Thirty patients who underwent Echo and cardiac magnetic resonance imaging (cMRI) were identified. TCV was measured on cMRI. TCV was determined echocardiographically by two methods: a volume measurement using the modified Simpson's method on a four-chamber view of the heart; and a calculated volume measurement which assumed a true-elliptical shape of the heart. These two methods where compared with the value obtained by cMRI using the concordance correlation coefficient (CCC). TCV by method 1 correlated well with cMRI (CCC = 0.98%, CI = 0.97, 0.99). TCV by method 2 had a CCC = 0.90 (CI = 0.9464, 0.9716) when compared to cMRI. Left ventricular end-diastolic volume (LVEDV) also correlated as a predictor of TCV in patients with structurally normal hearts and could be described by the equation: TCV = 6.6 (LVEDV) + 12 (R(2)  = 0.97). Echocardiographic assessment of TCV for recipients and their potential donors is a simple process and can be prospectively applied as part of donor evaluation.

Abnormal mitral valve anatomy in d-transposition of the great arteries: anatomic characterization and surgical outcomes.

Mitral valve anomalies can occur with S,D,D-transposition of the great arteries (d-TGA). Their influence on surgical technique and outcome after an arterial switch operation (ASO) has not been well described. Patients with d-TGA who underwent ASO from February 1990 to January 2011 were identified. Echocardiograms, operative reports, hospital course, and latest follow-up evaluation were reviewed. A total of 218 infants underwent ASO at a median age of 15.8 days. Survival was 95 % during a mean follow-up period of 60 months. Nine patients (4 %) were found to have similar mitral valve anomalies including anterior malalignment conoventricular septal defect, anterior displacement of the mitral valve toward the left ventricular outflow tract (LVOT), malpositioning of the posteromedial papillary muscle, unusual rotation of the mitral valve leaflets orienting the commissure toward the anterior ventricular septum, and redundant mitral valve tissue extending into the LVOT. Coarctation was more frequent in this subgroup (33 vs. 10 %; p = 0.05). Preoperative echocardiography consistently indicated suspicion of a cleft mitral valve with chordal attachments to the ventricular septum causing potential LVOT obstruction. Operative inspection did not identify a cleft or anomalous attachments in any patient, and no valvuloplasty or chordal manipulation was performed. The average hospital length of stay were similar (30.7 vs. 25.3 days; p = 0.54). One patient died late due to progressive LVOT obstruction, and one required heart transplantation. No patient had significant mitral valve regurgitation. We conclude that mitral valve anomalies associated with d-TGA are rare but present with consistent anatomic features and higher risk of coarctation. Unusual mitral valve apparatus positioning and chordal redundancy can suggest the need for valvuloplasty and chordal resection preoperatively, but this is rarely needed.

Assessing the impact of community-based interventions on hypertension and diabetes management in three Minnesota communities: Findings from the prospective evaluation of US HealthRise programs.

BACKGROUND: Community-based health interventions are increasingly viewed as models of care that can bridge healthcare gaps experienced by underserved communities in the United States (US). With this study, we sought to assess the impact of such interventions, as implemented through the US HealthRise program, on hypertension and diabetes among underserved communities in Hennepin, Ramsey, and Rice Counties, Minnesota. METHODS AND FINDINGS: HealthRise patient data from June 2016 to October 2018 were assessed relative to comparison patients in a difference-in-difference analysis, quantifying program impact on reducing systolic blood pressure (SBP) and hemoglobin A1c, as well as meeting clinical targets (< 140 mmHg for hypertension, < 8% Al1c for diabetes), beyond routine care. For hypertension, HealthRise participation was associated with SBP reductions in Rice (6.9 mmHg [95% confidence interval: 0.9-12.9]) and higher clinical target achievement in Hennepin (27.3 percentage-points [9.8-44.9]) and Rice (17.1 percentage-points [0.9 to 33.3]). For diabetes, HealthRise was associated with A1c decreases in Ramsey (1.3 [0.4-2.2]). Qualitative data showed the value of home visits alongside clinic-based services; however, challenges remained, including community health worker retention and program sustainability. CONCLUSIONS: HealthRise participation had positive effects on improving hypertension and diabetes outcomes at some sites. While community-based health programs can help bridge healthcare gaps, they alone cannot fully address structural inequalities experienced by many underserved communities.

Coronary artery abnormalities and sudden cardiac death.

Anomalous origin of a coronary artery (AOCA) can be associated with sudden cardiac death (SCD), particularly in young athletes. The diagnosis usually can be made by transthoracic echocardiography. In the case of patients for whom this method is not diagnostic, other methods are available including transesophageal echocardiography, cardiac magnetic resonance imaging (CMRI), and computed tomographic (CT) angiography. The decision to intervene is dependent on the type of lesion, the course of the coronary artery, its known association with SCD, and any symptoms present at the time of diagnosis. For patients without symptoms who have lesions less clearly associated with SCD [e.g., anomalous origin of the right coronary artery (AORCA)], the decision to intervene is more controversial. Further prospective studies hopefully will elucidate the optimum treatment pathway for such patients.

A Pilot Study Characterizing Flow Patterns in the Thoracic Aorta of Patients With Connective Tissue Disease: Comparison to Age- and Gender-Matched Controls via Fluid Structure Interaction.

Prior computational and imaging studies described changes in flow patterns for patients with Marfan syndrome, but studies are lacking for related populations. This pilot study addresses this void by characterizing wall shear stress (WSS) indices for patients with Loeys-Dietz and undifferentiated connective tissue diseases. Using aortic valve-based velocity profiles from magnetic resonance imaging as input to patient-specific fluid structure interaction (FSI) models, we determined local flow patterns throughout the aorta for four patients with various connective tissue diseases (Loeys-Dietz with the native aorta, connective tissue disease of unclear etiology with native aorta in female and male patients, and an untreated patient with Marfan syndrome, as well as twin patients with Marfan syndrome who underwent valve-sparing root replacement). FSI simulations used physiological boundary conditions and material properties to replicate available measurements. Time-averaged WSS (TAWSS) and oscillatory shear index (OSI) results are presented with localized comparison to age- and gender-matched control participants. Ascending aortic dimensions were greater in almost all patients with connective tissue diseases relative to their respective control. Differences in TAWSS and OSI were driven by local morphological differences and cardiac output. For example, the model for one twin had a more pronounced proximal descending aorta in the vicinity of the ductus ligamentum that impacted WSS indices relative to the other. We are optimistic that the results of this study can serve as a foundation for larger future studies on the connective tissue disorders presented in this article.

Applicability and Durability of Valve-Sparing Tetralogy of Fallot Repair.

BACKGROUND: Although valve-sparing repair remains ideal for patients with tetralogy of Fallot, the durability of valve-sparing repair and which patients may have been better served with a transannular patch remain unclear. METHODS: Retrospective review was performed of tetralogy of Fallot operations at our institution from January 2008 to December 2018. Standard demographic data were collected, including echocardiographic parameters, operative details, and clinical outcomes. Statistical analysis was performed comparing the transannular patch and valve-sparing repair groups. RESULTS: Sixty-seven patients underwent tetralogy of Fallot repair with a median age of 4.5 (3.2-6.0) months and weight of 5.8 (5.2, 6.7) kg. Seventeen (25%) patients underwent transannular patch repair and 50 (75%) patients underwent valve-sparing repair. There was no difference in age or weight between patients who underwent a transannular patch repair and those who underwent a valve-sparing repair. At last follow-up (median 42 months), there was a trend of a higher peak pulmonary valve/right ventricular outflow tract gradient (P = .06) in the valve-sparing group, but no difference in the pulmonary valve annulus z-scores. Additionally, the pulmonary valve z-scores in the valve-sparing group decreased from -2.3 ± 1.0 on predischarge echocardiogram of to -1.2 ± 1.6 on last follow-up, with the peak gradient on predischarge 23 (0-37) mm Hg remaining stable on last follow-up at 18 (0-29) mm Hg. There was one reoperation: pulmonary valve replacement six years after a transannular patch. CONCLUSIONS: Obtaining a postrepair pulmonary valve z-score of -2 yields satisfactory, stable valve-sparing repair with pulmonary valve growth, acceptable gradients, minimal regurgitation, and high freedom from reintervention during follow-up.

Community-based interventions for detection and management of diabetes and hypertension in underserved communities: a mixed-methods evaluation in Brazil, India, South Africa and the USA.

INTRODUCTION: As non-communicable disease (NCD) burden rises worldwide, community-based programmes are a promising strategy to bridge gaps in NCD care. The HealthRise programme sought to improve hypertension and diabetes management for underserved communities in nine sites across Brazil, India, South Africa and the USA between 2016 and 2018. This study presents findings from the programme's endline evaluation. METHODS: The evaluation utilises a mixed-methods quasi-experimental design. Process indicators assess programme implementation; quantitative data examine patients' biometric measures and qualitative data characterise programme successes and challenges. Programme impact was assessed using the percentage of patients meeting blood pressure and A1c treatment targets and tracking changes in these measures over time. RESULTS: Almost 60 000 screenings, most of them in India, resulted in 1464 new hypertension and 295 new diabetes cases across sites. In Brazil, patients exhibited statistically significant reductions in blood pressure and A1c. In Shimla, India, and in South Africa, country with the shortest implementation period, there were no differences between patients served by facilities in HealthRise areas relative to comparison areas. Among participating patients with diabetes in Hennepin and Ramsey counties and hypertension patients in Hennepin County, the percentage of HealthRise patients meeting treatment targets at endline was significantly higher relative to comparison group patients. Qualitative analysis identified linking different providers, services, communities and information systems as positive HealthRise attributes. Gaps in health system capacities and sociodemographic factors, including poverty, low levels of health education and limited access to nutritious food, are remaining challenges. CONCLUSIONS: Findings from Brazil and the USA indicate that the HealthRise model has the potential to improve patient outcomes. Short implementation periods and strong emphasis on screening may have contributed to the lack of detectable differences in other sites. Community-based care cannot deliver its full potential if sociodemographic and health system barriers are not addressed in tandem.

Challenges With Left Ventricular Functional Parameters: The Pediatric Heart Network Normal Echocardiogram Database.

BACKGROUND: The reliability of left ventricular (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 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 two-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 remeasured LV dimensions on a subset of studies, while blinded to calculated volumes and functional indices. RESULTS: Of 3,215 subjects with measurable images, 552 (17%) had a calculated LV shortening fraction (SF) < 25% and/or LV ejection fraction (EF) < 50%; the subjects were significantly younger and smaller than those with normal values. When the core lab and independent observer measurements were compared, individual LV size parameter intraclass correlation coefficients were high (0.81-0.99), indicating high reproducibility. The intraclass correlation coefficients were lower for SF (0.24) and EF (0.56). Comparing reviewers, 40/56 (71%) of those with an abnormal SF and 36/104 (35%) of those with a normal SF based on core lab measurements were calculated as abnormal from at least one independent observer. In contrast, an abnormal EF was less commonly calculated from the independent observers' repeat measures; only 9/47 (19%) of those with an abnormal EF and 8/113 (7%) of those with a normal EF based on core lab measurements were calculated as abnormal by at least one independent observer. CONCLUSIONS: Although blinded measurements of LV size show good reproducibility in healthy children, subsequently calculated LV functional indices reveal significant variability despite qualitatively normal systolic function. This suggests that, in clinical practice, abnormal SF/EF values may result in repeat measures of LV size to match the subjective assessment of function. Abnormal LV functional indices were more prevalent in younger, smaller children.

Relationship of Echocardiographic Z Scores Adjusted for Body Surface Area to Age, Sex, Race, and Ethnicity: The Pediatric Heart Network Normal Echocardiogram Database.

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/BSAα) 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 <5% difference was considered clinically insignificant because interobserver variability for echocardiographic measurements are reported as ≥5% difference. Of the 3566 subjects, 90% had measurable images. Appropriate BSA transformations (BSAα) 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.

Prenatal diagnosis of the rare association of common arterial trunk and double aortic arch.

Common arterial trunk with associated double aortic arch is a very rare constellation of congenital heart disease. Prenatal diagnosis allows for surgical repair prior to development of respiratory morbidity, which is otherwise described in all cases with this association.