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A letter to the editor is presented in response to the article "Disparity in right vs left ventricular recovery during follow-up after ventricular septal defect correction in children" by L.M. Klitsie and colleagues in a 2013 issue.

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. 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. 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). 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. [ABSTRACT FROM AUTHOR]

Background: The GenTAC (Genetically Triggered Thoracic Aortic Aneurysm and Cardiovascular Conditions) Registry enrolled patients with genetic aortopathies between 2007 and 2016.Objectives: The purpose of this study was to compare age distribution and probability of elective surgery for proximal aortic aneurysm, any dissection surgery, and cardiovascular mortality among aortopathy etiologies.Methods: The GenTAC study had a retrospective/prospective design. Participants with bicuspid aortic valve (BAV) with aneurysm (n = 879), Marfan syndrome (MFS) (n = 861), nonsyndromic heritable thoracic aortic disease (nsHTAD) (n = 378), Turner syndrome (TS) (n = 298), vascular Ehlers-Danlos syndrome (vEDS) (n = 149), and Loeys-Dietz syndrome (LDS) (n = 121) were analyzed.Results: The 25% probability of elective proximal aortic aneurysm surgery was 30 years for LDS (95% CI: 18-37 years), followed by MFS (34 years; 95% CI: 32-36 years), nsHTAD (52 years; 95% CI: 48-56 years), and BAV (55 years; 95% CI: 53-58 years). Any dissection surgery 25% probability was highest in LDS (38 years; 95% CI: 33-53 years) followed by MFS (51 years; 95% CI: 46-57 years) and nsHTAD (54 years; 95% CI: 51-61 years). BAV experienced the largest relative frequency of elective surgery to any dissection surgery (254/33 = 7.7), compared with MFS (273/112 = 2.4), LDS (35/16 = 2.2), or nsHTAD (82/76 = 1.1). With MFS as the reference population, risk of any dissection surgery or cardiovascular mortality was lowest in BAV patients (HR: 0.13; 95% CI: 0.08-0.18; HR: 0.13; 95%: CI: 0.06-0.27, respectively). The greatest risk of mortality was seen in patients with vEDS.Conclusions: Marfan and LDS cohorts demonstrate age and event profiles congruent with the current understanding of syndromic aortopathies. BAV events weigh toward elective replacement with relatively few dissection surgeries. Nonsyndromic HTAD patients experience near equal probability of dissection vs prophylactic surgery, possibly because of failure of early diagnosis. [ABSTRACT FROM AUTHOR]

Abstract Background The Faces Pain Scale-revised (FPS-r) has been developed as an interval scale. For other pain measurement instruments, several studies found evidence for and against an interval level of measurement. Objectives The primary aim of the current study was to evaluate the scale properties of the FPS-r using an item response theory approach. Design Secondary analysis of published data. Setting Three studies; Study 1 and study 2: One university hospital; Study 3: international pain registry. Participants Study 1: n = 246, female: 41%, age: 11–18 years, 3 pain items; Study 2: n = 240, female: 43%, age: 11–18 years, 9 pain items; Study 3: n = 2266, female: 41%, age: 4–18 years, 3 pain items. Methods The rating scale model (interval scale), the graded response model (no interval scale, ordered response categories) and the partial credit model (no interval scale) were used to scale the data. Results In all three studies, the rating scale model was outperformed by the graded response model or the partial credit model in terms of model fit. Overlapping response categories were found in items associated with less pain. Response category widths were wider for categories associated with low pain intensity and smaller for categories associated with high pain intensities. Smallest response categories were 1%–67% smaller compared to the widest response category of the same item. Conclusion According to these findings, the interval scale properties of the FPS-r may be questioned. Item response theory methods may help to solve the problem of missing linearity in pain intensity ratings using FPS-r. [ABSTRACT FROM AUTHOR]

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., (Copyright © 2024 International Society for the Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

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., Competing Interests: Disclosure statement Z.E.H., L.P., S.J., and W.R. report no conflicts of interest for this work. This work was not financially supported. This work has been accepted for abstract presentation at the American Academy of Pediatrics and the World Congress of Pediatric Cardiology and Cardiac Surgery, (Copyright © 2024 International Society for the Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

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)., Competing Interests: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

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., (Copyright © 2023 [Author/Employing Institution]. Published by Elsevier Inc. All rights reserved.)

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., (© 2023. The Author(s), under exclusive licence to the International Pediatric Research Foundation, Inc.)

Background: Previous single-center studies of diaphragm paralysis (DP) after pediatric cardiac surgery report incidence of 0.3% to 12.8% and associate DP with respiratory complications, prolonged ventilation and length of stay, and mortality. To better define incidence and associations between DP and various procedures and outcomes, we performed a multicenter study., Methods: The Society of Thoracic Surgeons Congenital Heart Surgery Database was queried to identify children who experienced DP after cardiac surgery (2010-2018; 126 centers). Baseline characteristics and postoperative outcomes were compared between patients with and without DP as well as between patients who underwent plication and those who did not. Associations between center volume and center rates of DP and use of plication were also explored., Results: A total of 2214 of 191,463 (1.2%) patients experienced DP. Postoperative DP portended worse outcomes, including mortality (5.6% vs 3.5%; P < .001), major morbidity (37.2% vs 10.7%; P < .001), tracheostomy (7.1% vs 0.9%; P < .001), prolonged mechanical ventilation (38.0% vs 7.8%; P < .001), and 30-day readmission (22.0% vs 10.6%; P < .001). A total of 1105 of 2214 (49.9%) patients with DP underwent plication. Patients who underwent plication were younger, were smaller, had more risk factors, and underwent more complex surgeries. Plication rates varied widely across centers. There was no correlation between center volume and center risk-adjusted rates of DP (r = .05, P = .5), nor frequency of plication (r = .08, P = .4)., Conclusions: DP complicating pediatric heart surgery is rare but portends significantly worse outcomes. One-half of patients underwent plication. Center-level risk-adjusted rates of DP and plication are not associated with case volume. Significant variability in plication practices suggests a target for quality improvement., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Our study was to apply the 2015 American Heart Association/American College of Cardiology Athletic Participation Guidelines to a group of otherwise healthy school age children and young adults with bicuspid aortic valve (BAV) and describe the potential competitive sports restriction as they age. We performed a retrospective chart review of children and young adults aged 5 to 22 years with isolated BAV with at least two echocardiograms between 2000 and 2013. Using task force guidelines, exercise restriction was recommended for any of the following: (1) any dilation of the aortic root, (2) any dilation of the ascending aorta, (3) moderate aortic stenosis, (4) severe aortic regurgitation; (5) left ventricular dilation or (6) reduced shortening fraction. Of the 345 patients with isolated BAV, 202 were considered restricted at study entry. The final cohort included 123 children and young adults. Over the course of follow up, 36% (44 of 123) met restriction criteria. The most likely cause for restriction was aortic dilation (34%). Progression of aortic valve disease occurred in a minority of patients (3%). There were no reports of death, dissection or catheter or surgical based intervention. In conclusion, we found that strict adherence to current guidelines would result in restriction of more than 1/3 of school age children and young adults with BAV from some form of competitive athletics during school age years. Strict application of the current guidelines in this age group may lead to over-restriction of youths from competitive sports., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: Quantitative determination of right ventricular (RV) function has gained more interest over the last years. The RV outflow tract systolic excursion (RVOT SE) has been recently proposed as an echocardiographic tool to assess RV systolic function in adults. We aimed to determine growth-related changes of RVOT SE in children and to establish references values., Study Design: A prospective study was conducted in a group of 711 healthy paediatric patients (age: 1 day to 18 years). We determined the effects of age and body surface area (BSA) on RVOT SE values. RVOT SE values were further correlated with the established RV systolic function parameters tricuspid annular plane systolic excursion (TAPSE) and tricuspid annular peak systolic velocity (S')., Results: The RVOT SE ranged from a mean of 3.4 mm in neonates to 9.5 mm in 18-year-old adolescents. The RVOT SE values showed a positive correlation with age (r = 0.90, P < 0.001) and BSA (r = 0.91, P < 0.001). A significant positive correlation was seen between RVOT SE and TAPSE (r = 0.93, P < 0.001) and also between RVOT SE and S' (r = 0.86, P < 0.001) in our patients., Conclusion: RVOT SE provides a simple measure and, in combination with long-axis excursion parameters TAPSE and S', a comprehensive assessment of RV systolic function. Z-scores of RVOT SE values were calculated, and percentile charts were established to serve as reference data., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2014. For permissions please email: journals.permissions@oup.com.)

Transthoracic echocardiography (TTE) is an important tool for diagnosis and follow-up of patients with congenital heart disease (CHD). Appropriate use of TTE can reduce the need for more invasive and complex modalities, such as cardiac catheterization and cardiac magnetic resonance imaging. New echocardiographic techniques have emerged for the assessment of ventricular systolic and diastolic function: Tissue Doppler imaging, tissue tracking, strain and strain rate imaging, vector velocity imaging (VVI), myocardial performance index, myocardial acceleration during isovolumic contraction (IVA), the ratio of systolic to diastolic duration (S/D ratio), and other measurements of systolic right ventricular (RV) function like tricuspid annular plane systolic excursion (TAPSE). These modalities may become valuable indicators of ventricular performance, compliance and disease progression, with the caveat of preload-dependency of the variables measured. In addition, three-dimensional (3D) echocardiography for the assessment of cardiac anatomy, valvular function, device position, ventricular volumes and ejection fraction is integrated into routine clinical care. In this review, we discuss the potential use and limitations of these new echocardiographic techniques in patients with CHD. A particular focus is on the echocardiographic assessment of right ventricular (RV) function by means of tissue Doppler imaging, tissue tracking, and three-dimensional imaging, in conditions associated with increased right ventricular volume or pressure load.