BACKGROUND:Sensitive fetal prediction of acute neonatal compromise at birth following a prenatal diagnosis of dextro-transposition of the great arteries/intact ventricular septum (dTGA/IVS) remains challenging. Using a large multicenter prospectively collected cohort, we aimed to determine whether fetal echocardiogram findings could sensitively and specifically predict neonatal hypoxia. METHODS:Twenty-three centers enrolled maternal-infant dyads. Demographics and clinical information were collected. Previously reported fetal predictors were evaluated from the third trimester by a core lab blinded to outcome. Primary outcome was preoperative neonatal hypoxia (PaO2 < 30 mm Hg or SpO2 < 75% if PaO2 was unavailable). Univariate and multivariate analyses of predictors with the primary outcome were performed. Sensitivity analyses were explored. Incidences of balloon atrial septostomy (BAS) and mortality were calculated. RESULTS:Of the 179 maternal-infant pairs with a fetal diagnosis of dTGA/IVS and postnatal data, 132 had data at all time points and were included in the final cohort, 50 (38%) had neonatal hypoxia, and 38/50 (76%) underwent BAS. Univariate and multivariate analyses showed no significant anatomic or physiologic fetal echocardiography predictors of neonatal hypoxia. Sensitivity analysis showed that an increasing Foramen ovale:total septal length ratio and increasing atrial septal excursion ratio decreased the likelihood of combined outcome of hypoxia and BAS but still did not provide useful discrimination to predict outcome. Survival to initial hospital discharge occurred in 131/132 (99%) of cases. CONCLUSIONS:Our multicenter prospective study did not identify any sensitive prenatal predictors of neonatal hypoxia in infants prenatally diagnosed with transposition of the great arteries (TGA)/IVS. High-risk delivery planning for the possibility of neonatal hypoxia and urgent BAS is needed for all infants with prenatally diagnosed TGA/IVS.

Objective: To evaluate the frequency and diagnostic yield of transthoracic echocardiography (TTE) in a noncardiac pediatric intensive care unit (PICU). Study design: This was a single-center retrospective observational study of a 48-bed quaternary-care PICU. Patients younger than 18 years who had a TTE performed during their PICU stay from February 3, 2015, to December 8, 2022, were included, and patients on extracorporeal membrane oxygenation support were excluded. Results: We analyzed 2633 TTEs from 1445 patients, with only the first study included for patients with multiple TTEs. The median age of all patients was 4.4 years (IQR 1-12.4 years). Low ventricular function, defined as moderate or severe systolic dysfunction, was found in 6% of all TTEs, whereas 94% showed hyperdynamic, normal, or mildly diminished ventricular function. TTEs were classified into 25 categories on the basis of indications. Low ventricular function was present in 10% of shock cases, 17% of cardiac arrests, and 2% of respiratory failures. Among cases of respiratory failure, 88% showed no pulmonary hypertension. A low yield group, comprising 16% of total first TTEs, included acute respiratory failure, persistent tachycardia, abnormal electrocardiograms, and systemic hypertension. Among those evaluated for pulmonary hypertension, 62% showed no evidence of it, whereas 4% exhibited severe pulmonary hypertension, all with a pre-existing history. For pericardial effusion evaluation, 82% had no or trivial-small effusion. Conclusions: TTE frequently is used in the noncardiac PICU, but its diagnostic yield varies widely on the basis of clinical indications. This study emphasizes the need for careful use of TTE in the PICU, with consideration of pretest probability and clinical context to enhance diagnostic effectiveness in critically ill pediatric patients.

Background: Staged palliation of hypoplastic left heart syndrome (HLHS) and variants typically begins with the Norwood operation or the hybrid procedure. Hybrid palliation is used in 13% of patients nationally and is often reserved for high-risk patients. A stented hybrid (SH) consists of bilateral pulmonary artery bands (bPAB) and a ductal stent while a ‘medical hybrid’ (MH) consists of bPAB and prostaglandins. MH use and outcomes have not been well described. Hypothesis: MH is used in higher-risk patients and therefore has lower survival than other stage 1 strategies. Aims: Compare use and outcomes of MH, SH, and surgical stage 1 (SS1) using a multicenter database. Methods: Patients from the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) database were categorized by initial intervention: MH, SH, or SS1. Other/unclear procedures were excluded. Results: The study included 2423 patients from 65 centers: 277 (11%) MH, 133 (5%) SH, 2013 (83%) SS1. MH had the lowest birth weight (BW) and gestational age (GA), most non-cardiac anomalies, and most preoperative risk factors (PRF). Most centers performed MH in a minority of cases, though use ranged from 0% to 82% at centers with ≥10 patients. Transplant-free survival at 1 year was 56% for MH, 66% for SH, and 81% for SS1 (p<0.0001). Using multivariable logistic regression, predictors of MH vs SS1 were lower BW (p<0.001), lower GA (p<0.001), genetic syndrome (p=0.010), non-cardiac anomaly (p=0.031), and ≥4/12 PRF (p<0.001). Predictors of MH vs SH were HLHS (p=0.044) and ≥4/12 PRF (p=0.045). Cox proportional hazards regression was used to compare risk-adjusted outcomes, excluding one center that performed >50% SH: MH had higher risk of one-year mortality/transplant compared to SS1 (HR = 1.89, 95% CI 1.47-2.42) and no difference compared to SH (HR = 1.16, 95% CI 0.76-1.79). Conclusions: Survival after MH is similar to SH and worse than SS1. This may be due to patient risk factors not controlled for in this study.

BACKGROUND:Case series report high mortality after fetal diagnosis of moderate to severe mitral regurgitation in combination with left atrial dilation, hydrops, or restrictive atrial septum. However, overall outcomes and specific high-risk features remain unclear. METHODS:We performed a multicenter retrospective cohort study of fetuses with ≥moderate mitral regurgitation and normal cardiac connections evaluated January 1, 2005 to January 31, 2016. Fetuses undergoing fetal cardiac intervention (FCI) were described separately. We assessed associations between fetal echocardiographic features and discharge mortality using mixed logistic regression and classification and regression tree modeling, stratified by studies performed <28 and ≥28 weeks gestational age (GA). RESULTS:Of 67 fetuses, 96% had aortic valve stenosis or atresia. Among the non-FCI group, fetal and discharge mortality was 10% (5/51) and 55% (28/51), respectively. For echocardiograms performed <28 weeks GA, only restrictive atrial septum (odds ratio [OR], 49.6 [95% CI, 5.6-437.5]) and earlier GA at referral (OR 0.72 per increasing week GA [95% CI 0.54-0.96]) remained associated in multivariable analysis. For those ≥28 weeks GA, in multivariable analysis, left atrial dilation (OR, 12.41 [95% CI, 2.19-70.33]) and lower ascending aortic Z score (OR per 1 unit increase, 0.61 [95% CI, 0.39-0.94]) remained in the model. By classification and regression tree modeling, left atrial dilation, restrictive atrial septum, and ascending aortic Z score <-1.3 were the key discriminators between survivors and nonsurvivors. Most FCIs were aortic valvuloplasty (13/16). Discharge mortality among FCI fetuses was 38%, not significantly different from non-FCI once adjusting for mitral regurgitation and LA dilation. CONCLUSIONS:Fetal ≥moderate MsR is associated with high mortality. Left atrial dilation, restrictive atrial septum, and smaller ascending aorta confer highest risk for mortality.

BACKGROUND:Disparities in clinical outcomes exist among children with CHD, and social determinants of health are a significant contributor. Follow-up care plays an important role in long-term health and may be impacted by social determinants of health. We aimed to describe the population with lapses in care to outpatient, general paediatric cardiology from a social determinants of health standpoint and compare them to our primary service area. METHODS:A retrospective chart review was completed for children who had lapses in care within outpatient, general cardiology. Data were compared to the Cincinnati Children's Hospital Medical Center's primary service area population. RESULTS:A higher proportion of patients who had lapses in care were noted to identify as Hispanic/Latino, utilised some form of public insurance, and lived in communities with more social deprivation. CONCLUSION:Patients who identify as a minority race/ethnicity, utilise public insurance, or live in an area with a higher social deprivation may be at a higher risk for lapses in outpatient cardiology care, including both follow-up care and initial evaluations.

OBJECTIVE:To assess the variables associated with incomplete and unscheduled cardiology clinic visits among referred children with a focus on equity gaps.STUDY DESIGN:We conducted a retrospective chart review for patients less than 18 years of age who were referred to cardiology clinics at a single quaternary referral centre from 2017 to 2019. We collected patient demographic data including race, an index of neighbourhood socio-economic deprivation linked to a patient's geocoded address, referral information, and cardiology clinic information. The primary outcome was an incomplete clinic visit. The secondary outcome was an unscheduled appointment. Independent associations were identified using multivariable logistic regression.RESULTS:There were 10,610 new referrals; 6954 (66%) completed new cardiology clinic visits. Black race (OR 1.41; 95% CI 1.22-1.63), public insurance (OR 1.29; 95% CI 1.14-1.46), and a higher deprivation index (OR 1.32; 95% CI 1.08-1.61) were associated with higher odds of incomplete visit compared to the respective reference groups of White race, private insurance, and a lower deprivation index. The findings for unscheduled visit were similar. A shorter time elapsed from the initial referral to when the appointment was made was associated with lower odds of incomplete visit (OR 0.62; 95% CI 0.52-0.74).CONCLUSION:Race, insurance type, neighbourhood deprivation, and time from referral date to appointment made were each associated with incomplete referrals to paediatric cardiology. Interventions directed to understand such associations and respond accordingly could help to equitably improve referral completion.

Genetic counseling is an important component of pediatric cardiac care. The financial costs and benefits of this care have yet to be defined in the literature. Downstream revenue (DSR) analysis can be used to assess the economic impact of genetic counselors (GCs) at their institution beyond the initial patient contact. Previous literature has reported DSR generated by oncology GCs, but there is no published DSR data from a cardiac GC setting. This study measured the DSR generated at a private hospital following a cardiac GC appointment. A chart review identified patients seen by a cardiac GC between 2018 and 2022. The study population included patients and their pediatric relatives who had not previously seen a cardiologist. Patients were included if they were affected with or at-risk for long QT syndrome, hypertrophic cardiomyopathy, dilated cardiomyopathy, or familial thoracic aortic aneurysm at the time of the GC visit. We recorded the frequency of common cardiac services and calculated the reimbursement for all cardiology services for 1 year following the initial GC appointment. The cohort included 121 participants from 61 families. Most individuals were at-risk for (n = 114, 94.3%) rather than affected by an inherited cardiac condition and presented for screening. The total DSR was $247,592.27, with an annual median of $1819.50 per patient (IQR $0, $3761.33). Revenue was similar among individuals who had undergone genetic testing and those who had not. Among participants, 72 (59.5%) had subsequent cardiology services. Most frequently, a patient who presented for subsequent care had an EKG, an echocardiogram, and a cardiology appointment. While the economic contributions of GC services do not speak to the broader value of GC involvement in patient care, they are important metrics for sustainability. This study outlines an approach to evaluating DSR and establishes a baseline understanding of DSR related to cardiac GC services.

OBJECTIVES: Sepsis-associated myocardial dysfunction is common in pediatric septic shock and negatively impacts outcomes. Early estimation of sepsis-associated myocardial dysfunction risk has the potential to inform clinical care and improve clinical trial design. The Pediatric Sepsis Biomarker Risk Model II is validated as a biomarker-based enrichment algorithm to discriminate children with septic shock with high baseline mortality probability. The objectives were to determine if Pediatric Sepsis Biomarker Risk Model biomarkers are associated with risk for sepsis-associated myocardial dysfunction in pediatric septic shock and to develop a biomarker-based model to reliably estimate sepsis-associated myocardial dysfunction risk. DESIGN: Secondary analysis of prospective cohort study. SETTING: Single-center, quaternary-care PICU. PATIENTS: Children less than 18 years old admitted to the PICU from 2003 to 2018 who had Pediatric Sepsis Biomarker Risk Model biomarkers measured for determination of Pediatric Sepsis Biomarker Risk Model II mortality probability and an echocardiogram performed within 48 hours of septic shock identification. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Pediatric Sepsis Biomarker Risk Model II mortality probability was calculated from serum biomarker concentrations and admission platelet count. Echocardiograms were reread by a single cardiologist blinded to Pediatric Sepsis Biomarker Risk Model II data, and sepsis-associated myocardial dysfunction was defined as left ventricular ejection fraction less than 45% for primary analyses. Multivariable logistic regression analyzed the association of Pediatric Sepsis Biomarker Risk Model II mortality probability with sepsis-associated myocardial dysfunction. Classification and regression tree methodology was employed to derive a Pediatric Sepsis Biomarker Risk Model biomarker-based model for sepsis-associated myocardial dysfunction. Thirty-two of 181 children with septic shock demonstrated sepsis-associated myocardial dysfunction. Pediatric Sepsis Biomarker Risk Model II mortality probability was independently associated with sepsis-associated myocardial dysfunction (odds ratio, 1.45; 95% CI, 1.17–1.81; p = 0.001). Modeling with Pediatric Sepsis Biomarker Risk Model biomarkers estimated sepsis-associated myocardial dysfunction risk with an area under the receiver operating characteristic curve of 0.90 (95% CI, 0.85–0.95). Upon 10-fold cross-validation, the derived model had a summary area under the receiver operating characteristic curve of 0.74. Model characteristics were similar when sepsis-associated myocardial dysfunction was defined by both low left ventricular ejection fraction and abnormal global longitudinal strain. CONCLUSIONS: A newly derived Pediatric Sepsis Biomarker Risk Model biomarker-based model reliably estimates risk of sepsis-associated myocardial dysfunction in pediatric septic shock, but independent prospective validation is needed.

Comunicación breve RESUMENNo es clara la carga de morbimortalidad de la lesión cardíaca clínicamente evidente secundaria a la enfermedad por coronavirus de 2019 (COVID-19) en los niños en edad escolar.A lo largo de 12 meses, en un importante hospital pediátrico académico en la región del medio oeste de Estados Unidos, hubo 1481 casos de COVID-19 sin hospitalización en niños en edad escolar por lo demás sanos, en quienes se hicieron 195 pruebas cardíacas.Si bien aparecieron hallazgos fortuitos, no se descubrió ninguna patología cardíaca relacionada con la COVID-19.Además, ~3 % de los niños solamente tuvieron síntomas cardíacos agudos que requirieron una evaluación por el área de cardiología pediátrica.Los niños que no fueron hospitalizados por COVID-19 tienen un riesgo muy bajo de desarrollar daño cardíaco clínicamente significativo y son más propensos a presentar hallazgos fortuitos.

The burden of clinically-apparent cardiac injury secondary to coronavirus disease 2019 (COVID-19) in school-age children is unclear. Over 12 months at a large academic pediatric hospital in the Midwestern portion of the United States, there were 1481 COVID-19 positive non-hospitalized otherwise healthy schoolaged children with 195 having cardiac testing performed. While incidental findings occurred, no definitive COVID-19 related cardiac pathology was discovered. Additionally, only ~3% of children had acute cardiac symptoms necessitating evaluation by pediatric cardiology. School-age children who were not hospitalized for COVID-19 have a very low risk of having clinically significant cardiac damage and are more likely to discover incidental findings.

INTRODUCTION:Local institutional echocardiogram protocols reflect standard measurements as per national guidelines, but adherence to measurements was inconsistent. This inconsistency led to variability in reporting and impacted the use of serial measurements for clinical decision-making. Therefore, we aimed to improve complete adherence to universal and protocol-specific measures for echocardiograms performed for first-time or cardiomyopathy studies from 60% to 90% from July 2019 to February 2020.METHODS:We included all sonographer-performed echocardiograms for first-time or cardiomyopathy protocol studies. We reviewed universal measures and protocol-specific measures for all included studies. We created a scoring system reflecting measurement completion. We used a control chart to measure compliance and established a baseline over 2 months. PDSA cycles over 5 months included interventions such as sonographer education, technical improvements to the measurement toolbar, and group and individual performance feedback.RESULTS:We reviewed over 4000 studies-the reporting of complete universal measures improved significantly from a median score of 60% to 93%. Protocol-specific measures for first-time studies also showed significant improvement from 62% to 90% adherence. Cardiomyopathy-specific measures demonstrated 87% adherence at baseline, which improved to 95% but then returned to baseline. Sonographer education and toolbar adjustment prompted special cause variation with further improvement following performance feedback. The universal and first-time protocol measures reached 90% adherence with sustained improvement for over 9 months.CONCLUSIONS:We employed quality improvement methodology to improve complete adherence to echocardiographic protocol measurements, thereby facilitating echocardiographic quality and reporting consistency. We plan to spread these interventions to improve adherence to other protocols.