Impaired myocardial deformation persists at 2 years in offspring of mothers with diabetes mellitus.

BACKGROUND: A diabetic intrauterine environment has been proposed as a potential etiological mechanism for in utero programming of cardiac disease, and is associated with impaired fetal cardiac function. We aimed to assess cardiac function in offspring of mothers with diabetes mellitus (ODM) and determine whether fetal cardiac abnormalities persist during follow-up. METHODS: Longitudinal observational study to evaluate and compare myocardial function in 40 ODM to age-matched control offspring (CO). Myocardial deformation was measured using speckle-tracking echocardiography (STE). RESULTS: Significant differences were detected in global longitudinal strain (-20.9 ± 3.1 vs. -23.6 ± 2.2%; p = 0.001), global circumferential strain (-24.4 ± 3.9 vs. -26.9 ± 2.7%; p = 0.017), average radial strain (29.0 ± 9.8 vs. 37.1 ± 7.2%; p = 0.003), average longitudinal systolic strain rate (-1.24 ± 0.25/s vs. -1.47 ± 0.30/s; p = 0.011) and average circumferential systolic strain rate (-1.56 ± 0.37/s vs. -1.84 ± 0.37/s; p = 0.013) in comparison to CO up to 2 years of follow-up. Minimal differences were observed within ODM over the 2-year period. CONCLUSION: Impaired cardiac function in ODM persists during 2 years follow-up. Functional cardiac assessment might therefore be useful to detect these unfavorable changes, independent of screening for congenital heart disease or hypertrophic cardiomyopathy in this population. IMPACT: We demonstrate persistence of subclinical myocardial deformation abnormalities in offspring of mothers with diabetes mellitus from fetal life to early childhood years. These results extend the cellular observations in basic and translational research of developmental programming into the clinical realm. Persistence of subclinical myocardial deformation abnormalities may shed light on the known incidence of early cardiovascular disease in offspring of mother with diabetes. Cardiac myocardial strain assessment can be useful to detect these abnormalities, independent of screening for congenital heart disease or hypertrophic cardiomyopathy in this population.

Renal injury and recovery in pediatric patients after ventricular assist device implantation and cardiac transplant.

BACKGROUND: The use of ventricular assist devices (VADs) in children with heart failure may be of particular benefit to those with accompanying renal failure, as improved renal function is seen in some, but not all recipients. We hypothesized that persistent renal dysfunction at 7 days and/or 1 month after VAD implantation would predict chronic kidney disease (CKD) 1 year after heart transplantation (HT). METHODS: Linkage analysis of all VAD patients enrolled in both the PEDIMACS and PHTS registries between 2012 and 2016. Persistent acute kidney injury (P-AKI), defined as a serum creatinine ≥1.5× baseline, was assessed at post-implant day 7. Estimated glomerular filtration rate (eGFR) was determined at implant, 30 days thereafter, and 12 months post-HT. Pre-implant eGFR, eGFR normalization (to ≥90 mL/min/1.73 m2 ), and P-AKI were used to predict post-HT CKD (eGFR <90 mL/min/1.73 m2 ). RESULTS: The mean implant eGFR was 85.4 ± 46.5 mL/min/1.73 m2 . P-AKI was present in 19/188 (10%). Mean eGFR at 1 month post-VAD implant was 131.1 ± 62.1 mL/min/1.73 m2 , significantly increased above baseline (P < 0.001). At 1 year post-HT (n = 133), 60 (45%) had CKD. Lower pre-implant eGFR was associated with post-HT CKD (OR 0.99, CI: 0.97-0.99, P = 0.005); P-AKI was not (OR 0.96, CI: 0.3-3.0, P = 0.9). Failure to normalize renal function 30 days after implant was highly associated with CKD at 1 year post-transplant (OR 12.5, CI 2.8-55, P = 0.003). CONCLUSIONS: Renal function improves after VAD implantation. Lower pre-implant eGFR and failure to normalize renal function during the support period are risk factors for CKD development after HT.

HeartWare Ventricular Assist Device Implantation for Pediatric Heart Failure-A Single Center Approach.

While pediatric HeartWare HVAD application has increased, determining candidacy and timing for initiation of pediatric VAD support has remained a challenge. We present our experience with a systematic approach to HVAD implantation as a bridge to pediatric heart transplantation. We performed a retrospective, single center review of pediatric patients (n = 11) who underwent HVAD implantation between September 2014 and January 2018. Primary endpoints evaluated were survival to heart transplantation, need for right ventricular assist device (RVAD) at any point, ongoing HVAD support, or death. Median patient age was 11 years (range: 3-16). Median BSA was 1.25 m2 (range: 0.56-2.1). Heart failure etiologies requiring support were dilated cardiomyopathy (n = 8), myocarditis (n = 1), congenital mitral valve disease (n = 1), and single ventricle heart failure (n = 1). Median time from cardiac ICU admission for heart failure to HVAD placement was 15 days (range 3-55), based on standardized VAD implantation criteria involving imaging assessment and noncardiac organ evaluation. The majority of patients (91%) were INTERMACS Level 2 at time of implant. Three patients (27%) had CentriMag RVAD placement at time of HVAD implantation. Two of these three patients had successful RVAD explanation within 2 weeks. Median length of HVAD support was 60 days (range 6-405 days). Among the 11 patients, survival during HVAD therapy to date is 91% (10/11) with 9 (82%) bridged to heart transplantation and one (9%) continuing to receive support. Posttransplant survival has been 100%, with median follow-up of 573 days (range 152-1126). A systematic approach to HVAD implantation can provide excellent results in pediatric heart failure management for a variety of etiologies and broad BSA range.

Genetic aetiologies should be considered in paediatric cases of acute heart failure presumed to be myocarditis.

There are a variety of causes of acute heart failure in children including myocarditis, genetic/metabolic conditions, and congenital heart defects. In cases with a structurally normal heart and a negative personal and family history, myocarditis is often presumed to be the cause, but we hypothesise that genetic disorders contribute to a significant portion of these cases. We reviewed our cases of children who presented with acute heart failure and underwent genetic testing from 2008 to 2017. Eighty-seven percent of these individuals were found to have either a genetic syndrome or pathogenic or likely pathogenic variant in a cardiac-related gene. None of these individuals had a personal or family history of cardiomyopathy that was suggestive of a genetic aetiology prior to presentation. All of these individuals either passed away or were listed for cardiac transplantation indicating genetic testing may provide important information regarding prognosis in addition to providing information critical to assessment of family members.

Study design and rationale for ELPIS: A phase I/IIb randomized pilot study of allogeneic human mesenchymal stem cell injection in patients with hypoplastic left heart syndrome.

Despite advances in surgical technique and postoperative care, long-term survival of children born with hypoplastic left heart syndrome (HLHS) remains limited, with cardiac transplantation as the only alternative for patients with failing single ventricle circulations. Maintenance of systemic right ventricular function is crucial for long-term survival, and interventions that improve ventricular function and avoid or defer transplantation in patients with HLHS are urgently needed. We hypothesize that the young myocardium of the HLHS patient is responsive to the biological cues delivered by bone marrow-derived mesenchymal stem cells (MSCs) to improve and preserve right ventricle function. The ELPIS trial (Allogeneic Human MEsenchymal Stem Cell Injection in Patients with Hypoplastic Left Heart Syndrome: An Open Label Pilot Study) is a phase I/IIb trial designed to test whether MSC injection will be both safe and feasible by monitoring the first 10 HLHS patients for new major adverse cardiac events. If our toxicity stopping rule is not activated, we will proceed to the phase IIb component of our study where we will test our efficacy hypothesis that MSC injection improves cardiac function compared with surgery alone. Twenty patients will be enrolled in a randomized phase II trial with a uniform allocation to MSC injection versus standard surgical care (no injection). The 2 trial arms will be compared with respect to improvement of right ventricular function, tricuspid valve annulus size, and regurgitation determined by cardiac magnetic resonance and reduced mortality, morbidity, and need for transplantation. This study will establish the safety and feasibility of allogeneic mesenchymal stem cell injection in HLHS patients and provide important insights in the emerging field of stem cell-based therapy for congenital heart disease patients.

Right Ventricular Outflow Tract (RVOT) Changes in Children with an Atrial Septal Defect: Focus on RVOT Velocity Time Integral, RVOT Diameter, and RVOT Systolic Excursion.

OBJECTIVE: Aim of the study was to determine the influence of right heart volume overload in children with atrial septal defect (ASD) on right ventricular outflow tract (RVOT) variables. METHODS: A prospective study was conducted in 115 children (age range: 2 days-18.1 years) with a moderate to large ASD. We determined effects of age, body length (BL), body weight (BW), and body surface area (BSA) on the variables RVOT diameter, RVOT velocity time integral (VTI), and RVOT systolic excursion (SE), and tested the predictive value of published normal values for age, BW, BL, and BSA in our ASD patients. RESULTS: In our pediatric ASD patients, the age-specific RVOT diameter (z-score: +2.2, 95% CI: 2.0-2.4, P < 0.001) was significantly increased compared to normal values with 54% of our ASD patients having a z-score >2.0. The age-specific RVOT VTI z-score (z-score: +3.6, 95% CI: 3.2-3.9, P < 0.001) was significantly increased compared to normal values with 81% of our ASD patients having a z-score >2.0. The age-specific RVOT SE z-score was not increased but slightly lower compared to normal values (z-score: -0.5, 95% CI: -0.7 to -0.3, P < 0.001) with 3% of our ASD patients having a z-score >2.0 while 12% of the patients had a z-score <-2. CONCLUSION: In our study population, we show the RVOT VTI and diameter to be relevant predictors in identifying an enlarged RVOT size and flow in children with moderate to large ASD.

Echocardiographic Reference Values for Right Atrial Size in Children with and without Atrial Septal Defects or Pulmonary Hypertension.

Right atrial (RA) size may become a very useful, easily obtainable, echocardiographic variable in patients with congenital heart disease (CHD) with right-heart dysfunction; however, according studies in children are lacking. We investigated growth-related changes of RA dimensions in healthy children. Moreover, we determined the predictive value of RA variables in both children with secundum atrial septal defect (ASD) and children with pulmonary hypertension (PH) secondary to CHD (PH-CHD). This is a prospective study in 516 healthy children, in 80 children with a secundum ASD (>7 mm superior-inferior dimension), and in 42 children with PH-CHD. We determined three RA variables, i.e., end-systolic major-axis length, end-systolic minor-axis length, and end-systolic area, stratified by age, body weight, length, and surface area. RA end-systolic length and area z scores were increased in children with ASD and PH-CHD when compared to those variables in the healthy control population. Using the Youden Index to determine the best cutoff scores in sex- and age-specific RA dimensions, we observed a sensitivity and specificity up to 94 and 91 %, respectively, in ASD children and 98 and 94 %, respectively, in PH-CHD children. We provide normal values (z scores -2 to +2) for RA size and area in a representative, large pediatric cohort. Enlarged RA variables with scores >+2 were predictive of secundum ASD and PH-CHD. Two-dimensional determination of RA size can identify enlarged RAs in the setting of high volume load (ASD) or pressure load (PH-CHD).

The past, present, and future of paediatric cardiology training at the Johns Hopkins Hospital, in the tradition of Dr Helen Taussig.

Johns Hopkins has been a leader in paediatric cardiology for over 85 years. In the 1940s, Dr Helen Taussig began training fellows in paediatric cardiology at Johns Hopkins at a time when the diagnosis and treatment of CHD were in the earliest stage. Under her leadership, the fellowship developed a strong foundation that has continued to evolve to meet the current needs of learners and educators. In the current era, the Johns Hopkins programme implements the current theories of adult education and actively engages our fellows in learning as well as teaching. The programme uses techniques such as flipped classroom, structured case-based small-group learning, observed and structured clinical examination, simulations, and innovative educational technology. These strategies combined with our faculty and rich history give our fellows a unique educational experience.

Longitudinal systolic left ventricular function in preterm and term neonates: reference values of the mitral annular plane systolic excursion (MAPSE) and calculation of z-scores.

The mitral annular plane systolic excursion (MAPSE) is a quick and reliable echocardiographic tool for assessing longitudinal left ventricular (LV) systolic function in children and adults. Because this parameter is affected by the LV longitudinal dimension, pediatric and adult normal values are not suitable for preterm and term neonates. A prospective study investigated a large group of preterm and term neonates [gestational age (GA), 26/0-6 to 40/0-6; birth weight (BW), 670-4,140 g]. The growth- and BW-related changes in MAPSE were determined to establish normal z-score values for preterm and term neonates. The MAPSE ranged from a mean of 0.36 ± 0.05 cm in preterm neonates with a GA of 26/0-6 to 0.56 ± 0.08 cm in term neonates with a GA of 40/0-6. The findings showed MAPSE, GA, and BW to be moderately correlated. Pearson's correlation coefficient was 0.56 for GA (MAPSE; p < 0.001) and 0.58 for BW (MAPSE; p < 0.001). The normal MAPSE values did not differ significantly between females and males (p = 0.946). The absolute values and z-scores of normal MAPSE values in healthy preterm and term neonates within the first 48 h of life were calculated, and percentile charts were established. Determination of LV function using MAPSE might be useful for vulnerable infants for whom a prolonged examination is inappropriate and for neonates with suboptimal visualization of the endocardium.

Right Ventricular Outflow Tract Velocity Time Integral Determination in 570 Healthy Children and in 52 Pediatric Atrial Septal Defect Patients.

Determination of the right ventricular outflow tract velocity time integral (RVOT VTI) is an important part of the noninvasive investigation of pulmonary blood flow in adults; however, age-related pediatric reference data are lacking. We examined growth-related changes of RVOT VTI values in children and the predictive value of RVOT VTI values in identifying enhanced pulmonary blood flow in children with secundum type atrial septal defect (ASD). A prospective study was conducted in a group of 570 healthy children and 52 children with a moderate-sized to large ASD. We determined the effects of age, body length (BL), body weight (BW), and body surface area (BSA) on RVOT VTI values. The predictive value of normal values stratified for age, BW, BL, and BSA was tested in our 52 ASD children. RVOT VTI values ranged from mean 9.7 ± 1.2 cm in neonates to 23.3 ± 2.7 cm in children with 18 years of age and showed a positive correlation with age, BL, BSA, and BW. In our population, RVOT VTI z-scores showed a high specificity for detecting ASD patients (>97 %) with sensitivity up to 71 %. We provide normal ranges and calculated z-scores of pediatric RVOT VTI values. Normal RVOT VTI z-scores might be additional predictors in identifying increased pulmonary blood flow in patients with ASD.

Reference values of the mitral annular peak systolic velocity (Sm) in 690 healthy pediatric patients, calculation of Z-score values, and comparison to the mitral annular Plane systolic excursion (MAPSE).

BACKGROUND: The mitral annular peak systolic velocity (Sm) is an echocardiographic measurement using tissue Doppler imaging to assess longitudinal left ventricular (LV) systolic function in children and adults. We determined growth-related changes in Sm to establish reference values for the entire pediatric age group. METHODS AND RESULTS: A prospective study was conducted in a group of 690 healthy pediatric patients (age: 1 day-18 years). We determined the effects of age, sex, and body surface area (BSA) on the Sm values. Regression analysis was used to estimate Sm from age, BSA, and sex. In addition, a correlation of normal Sm with normal age-matched values of the M-mode parameter mitral annular plane systolic excursion (MAPSE) was measured. The Sm ranged from a mean of 5.8 cm/sec (Z-score ±2: 3.6-8.0 cm/sec) in the newborn to 11.8 cm/sec (Z-score ±2: 8.5-15.1 cm/sec) in the 18-year-old adolescent. The Sm values showed a positive correlation with age and BSA with a nonlinear course. There was no significant difference in Sm values between females and males. A significant correlation was found between Sm and MAPSE values. CONCLUSION: Z-scores of Sm values were calculated and percentile charts were established to serve as reference data in patients with congenital heart diseases.

Sacubitril-valsartan vs ACE/ARB in pediatric heart failure: A retrospective cohort study.

BACKGROUND: The first angiotensin receptor/neprilysin inhibitor on the market, sacubitril-valsartan, has shown marked improvements in death and hospitalization for heart failure among adults, and is now approved for use in pediatric heart failure. While the ongoing PANORAMA-HF trial is evaluating the effectiveness of sacubitril-valsartan for pediatric patients with a failing systemic left ventricle, the enrollment criteria do not include the majority of pediatric heart failure patients. Additional studies are needed. METHODS: Using the TriNetX database, we performed a propensity score matched, retrospective cohort study to assess the incidence of a composite of all-cause mortality or heart transplant within 1 year. The 519 patients who received sacubitril-valsartan were compared to 519 matched controls who received an angiotensin converting enzyme inhibitor (ACE) or angiotensin II receptor blocker (ARB). RESULTS: There was no significant difference in the incidence of the composite outcome with sacubitril-valsartan over an ACE/ARB (13.3% vs 13.2%, p = 0.95), or among the components of mortality (5.0% vs 5.8%, p = 0.58) or heart transplantation (8.7% vs 7.5%, p = 0.50). Patients who were receiving full goal-directed medical therapy (14.4% vs 16.0%, p = 0.55) also showed no difference in the composite outcome. We observed a significantly increased incidence of hypotension (10% vs 5.2%, p = 0.006) and a trend toward reduced number of hospitalizations per year (mean (SD) 1.3 (4.4) vs 2.0 (9.1), p = 0.09). CONCLUSIONS: Sacubitril-valsartan is not associated with a decrease in the composite of all-cause mortality or heart transplantation within 1 year. Future studies should evaluate the possible reduction in hospitalizations and optimal dosing to minimize hypotension.

Neoaortic Regurgitation Detected by Echocardiography After Arterial Switch Operation: A Systematic Review and Meta-Analysis.

Background: Neoaortic root dilatation (NeoARD) and neoaortic regurgitation (NeoAR) are common sequelae following the arterial switch operation (ASO) for transposition of the great arteries. Objectives: The authors aimed to estimate the cumulative incidence of NeoAR, assess whether larger neoaortic root dimensions were associated with NeoAR, and evaluate factors associated with the development of NeoAR during long-term follow-up. Methods: Electronic databases were systematically searched for articles that assessed NeoAR and NeoARD after ASO, published before November 2022. The primary outcome was NeoAR, classified based on severity categories (trace, mild, moderate, and severe). Cumulative incidence was estimated from Kaplan-Meier curves, neoaortic root dimensions using Z-scores, and risk factors were evaluated using random-effects meta-analysis. Results: Thirty publications, comprising a total of 6,169 patients, were included in this review. Pooled estimated cumulative incidence of ≥mild NeoAR and ≥moderate NeoAR at 30-year follow-up were 67.5% and 21.4%, respectively. At last follow-up, neoaortic Z-scores were larger at the annulus (mean difference [MD]: 1.17, 95% CI: 0.52-1.82, P < 0.001; MD: 1.38, 95% CI: 0.46-2.30, P = 0.003) and root (MD: 1.83, 95% CI: 1.16-2.49, P < 0.001; MD: 1.84, 95% CI: 1.07-2.60, P < 0.001) in patients with ≥mild and ≥moderate NeoAR, respectively, compared to those without NeoAR. Risk factors for the development of any NeoAR included prior pulmonary artery banding, presence of a ventricular septal defect, aorto-pulmonary mismatch, a bicuspid pulmonary valve, and NeoAR at discharge. Conclusions: The risks of NeoARD and NeoAR increase over time following ASO surgery. Identified risk factors for NeoAR may alert the clinician that closer follow-up is needed. (Risk factors for neoaortic valve regurgitation after arterial switch operation: a meta-analysis; CRD42022373214).

Impact of prolonged ischemic time on pediatric heart transplantation outcomes: Improved outcomes in the most recent era.

BACKGROUND: Impacts of ischemic time (IT) on pediatric heart transplant outcomes are multifactorial. We aimed to analyze the effect of prolonged IT on graft loss after pediatric heart transplantation. We hypothesized that graft survival with prolonged IT has improved across eras. METHODS: Patients <18 years old in the Pediatric Heart Transplant Society database were included (N=6,765) and stratified by diagnosis and era (1993-2004, 2005-2009, and 2010-2019). Severe graft failure (SGF) was defined as death, retransplant, or need for mechanical circulatory support in the first 7 days post-transplant. Descriptive statistical methods were used to compare differences between patient characteristics and IT. Kaplan-Meier survival analysis compared freedom from graft loss, rejection, and infection. Multivariable analysis was performed for graft loss and SGF (hazard and logistic regression modeling, respectively). RESULTS: Diagnoses were cardiomyopathy (N = 3,246) and congenital heart disease (CHD; N = 3,305). CHD were younger, more likely to have an IT ≥4.5 hours, and more likely to require extracorporeal membrane oxygenation or mechanical ventilation at transplant (all p < 0.001). Median IT was 3.6 hours (interquartile range 2.98-4.31; range 0-10.5). IT was associated with early graft loss (HR 1.012, 95% CI 1.005-1.019), but not when analyzed only in the most recent era. IT was associated with SGF (OR 1.016 95%CI 1.003-1.030). CONCLUSIONS: Donor IT was independently associated with an increased risk of graft loss, albeit with a small effect relative to other risk factors. Graft survival with prolonged IT has improved in the most recent era but the risk of SGF persists.

GenTAC registry report: gender differences among individuals with genetically triggered thoracic aortic aneurysm and dissection.

Previous data suggest women are at increased risk of death from aortic dissection. Therefore, we analyzed data from the GenTAC registry, the NIH-sponsored program that collects information about individuals with genetically triggered thoracic aortic aneurysms and cardiovascular conditions. We performed cross-sectional analyses in adults with Marfan syndrome (MFS), familial thoracic aortic aneurysm or dissection (FTAAD), bicuspid aortic valve (BAV) with thoracic aortic aneurysm or dissection, and subjects under 50 years of age with thoracic aortic aneurysm or dissection (TAAD <50 years). Women comprised 32% of 1,449 subjects and were 21% of subjects with BAV, 34% with FTAAD, 22% with TAAD <50 years, and 47% with MFS. Thoracic aortic dissections occurred with equal gender frequency yet women with BAV had more extensive dissections. Aortic size was smaller in women but was similar after controlling for BSA. Age at operation for aortic valve dysfunction, aneurysm or dissection did not differ by gender. Multivariate analysis (adjusting for age, BSA, hypertension, study site, diabetes, and subgroup diagnoses) showed that women had fewer total aortic surgeries (OR = 0.65, P < 0.01) and were less likely to receive angiotensin converting enzyme inhibitors (ACEi; OR = 0.68, P < 0.05). As in BAV, other genetically triggered aortic diseases such as FTAAD and TAAD <50 are more common in males. In women, decreased prevalence of aortic operations and less treatment with ACEi may be due to their smaller absolute aortic diameters. Longitudinal studies are needed to determine if women are at higher risk for adverse events.

Tetralogy of Fallot and aortic root dilation: a long-term outlook.

Dilation of the sinus of Valsalva (SoV) has been increasingly observed after repaired tetralogy of Fallot (TOF). We estimate the prevalence of SoV dilation in adults with repaired TOF and analyze possible factors related to aortic disease. Adults with TOF [n = 109, median age 33.2 years (range 18.1 to 69.5)] evaluated at Johns Hopkins Hospital from 2001 to 2009 were reviewed in an observational retrospective cohort study. Median follow-up was 27.3 (range 0.1-48.8) years. SoV dilation was defined as >95 % confidence interval adjusted for age and body surface area (z-score > 2). The prevalence of SoV dilation was 51 % compared with that of a normal population with a mean z-score of 2.03. Maximal aortic diameters were ≥ 4 cm in 39 % (42 of 109), ≥ 4.5 cm in 21 % (23 of 109), ≥ 5 cm in 8 % (9 of 109), and ≥ 5.5 cm in 2 % (2 of 109). There was no aortic dissection or death due contributable to aortic disease. Aortic valve replacement was performed in 1.8 % and aortic root or ascending aorta (AA) replacement surgery in 2.8 % of patients. By multivariate logistic regression analysis, aortic regurgitation (AR) [odds ratio (OR) = 3.09, p = 0.005], residual ventricular septal defect (VSD) (OR = 4.14, p < 0.02), and TOF with pulmonary atresia (TOF/PA) (OR = 6.75, p = 0.03) were associated with increased odds of dilated aortic root. SoV dilation after TOF repair is common and persists with aging. AR, residual VSD, and TOF/PA are associated with increased odds of dilation. AA evaluation beyond the SoV is important. Indexed values are imperative to avoid bias on the basis of age and body surface area.

Impact of race and health coverage on listing and waitlist mortality in pediatric cardiac transplantation.

BACKGROUND: Social factors like race and insurance affect transplant outcomes. However, little is known in pediatric heart transplantation. We hypothesized that race and insurance coverage impact listing and waitlist outcomes across eras. METHODS: Data from the Pediatric Heart Transplant Society multi-center registry prospectively collected between January 1, 2000-December 31, 2019 were analyzed. Patients were divided by race as Black, White and other and by insurance coverage at listing (US governmental, US private and non-US single payer systems (UK, Canada). Clinical condition at listing and waitlist outcomes were compared across races and insurance coverages. Categorical variables were compared using a chi-square test and continuous variables using the Wilcoxon rank sum test. Risk factors for waitlist mortality were examined using multiphase parametric hazard modeling. A sensitivity analysis using parametric hazard explored the interaction between race and insurance. RESULTS: At listing, compared to Whites (n = 5391) and others (n = 1167), Black patients (n = 1428) were older, more likely on US governmental insurance and had cardiomyopathy as the predominant diagnosis (p < 0.0001). Black patients were more likely to be higher status at listing, in hospital, on inotropes or a ventricular assist device (p < 0.0001). Black patients had significantly shorter time on the waitlist compared to other races (p < 0.0001) but had higher waitlist mortality (p = 0.0091), driven by the earlier era (2000-2009) (p = 0.0005), most prominently within the US private insurance cohort (p = 0.015). Outcomes were not different in other insurance cohorts or in the recent era (2010-2019). CONCLUSION: Black children are older and sicker at the time of listing, deteriorate more often and face a higher wait list mortality, despite a shorter waitlist period and favorable clinical factors, with improvement in the recent era associated with the recent US healthcare reforms. The social construct of race appears to disadvantage Black children by limiting referral, consideration or access to pediatric cardiac transplantation.

Recommendations for Centers Performing Pediatric Heart Surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Recommendations for centers performing pediatric heart surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Recommendations for Centers Performing Pediatric Heart Surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.