Dyslipidemia is associated with increased risk for atherosclerosis and cardiovascular events in adulthood. Atherosclerosis begins in childhood and guidelines exist to screen for at-risk children. Children with congenital...Dyslipidemia is associated with increased risk for atherosclerosis and cardiovascular events in adulthood. Atherosclerosis begins in childhood and guidelines exist to screen for at-risk children. Children with congenital heart disease (CHD) have high lifetime cardiovascular risk but data are limited on their rates of dyslipidemia. Our study aims to detail rates of lipid screening and dyslipidemia in children with CHD. Children with CHD aged 9-18 years who followed at our institution from 2012 to 2019 were included. Subjects were classified as dyslipidemic if they met one of the following: total cholesterol ≥ 200 mg/dL, low-density lipoprotein-C ≥ 130 mg/dL, triglycerides ≥ 130 mg/dL, high-density lipoprotein cholesterol [HDL-C] < 40 mg/dL, non-HDL-C ≥ 145 mg/dL. Of 1579 patients, only 11.3% (N = 179/1579) had a documented lipid panel. Of those screened, 60% (N = 107/179) had dyslipidemia. Screened subjects had higher median BMI percentiles versus unscreened (84th vs. 64th ). Dyslipidemic subjects had higher median BMI percentiles compared to those without (92nd vs. 74th ). Black patients were a higher proportion of the screened population compared to unscreened (26% vs. 11%). Overall, there were similar rates of dyslipidemia across all races and CHD lesions. Only a small proportion of children with CHD had lipid screening performed despite universal screening guidelines. However, a sizable percentage of children screened had dyslipidemia. Screening favored Black patients and those with higher BMI. Higher BMI patients had increased rates of dyslipidemia. These findings emphasize the importance of universal screening for dyslipidemia in children with CHD.
Work-related musculoskeletal disorders (WRMD) is an ongoing concern for fetal cardiac sonographers (f-CS). Decreased grasp and pincher strength have been associated with an increased risk of WRMD. This report aimed to de...Work-related musculoskeletal disorders (WRMD) is an ongoing concern for fetal cardiac sonographers (f-CS). Decreased grasp and pincher strength have been associated with an increased risk of WRMD. This report aimed to determine if certain variables associated with fetal echocardiographic (f-echo) studies correlated with grasp and pincer strength. Grasp and pincer strength values for the f-CS's scanning hand were obtained immediately pre- and post-f-echo. Maternal, f-echo, and fetal characteristics were recorded. Values are presented as mean and standard deviation or percentage. Univariable linear mixed-effects regression models were performed to determine possible associations with grasp and pincher value changes versus f-echo variables with secondary analyses adjusting for additional clinical factors via multivariable models. Total of 304 f-echo were performed with 292 paired pre- and post-grasp and pre- and post-pincher values recorded. Maternal body mass index (BMI) was 33 (9) kg/m, scan length was 33 (15) minutes, and f-CS reported image quality was rated 7 out of 10 (6, 8). 96% of f-echo were performed on singleton pregnancies, 71% of f-echo were reported as normal, placental position was posterior in 54%, fluid status was normal in 97%, fetus was vertex 67%, and spine position was predominantly either right (33%) or left (30%) side up. Pre-grip was 26.2 (3.9) kg and post-grip was 25.9 (4.1) kg. Pre-pincher was 6.0 (1.1) kg and post-pincher was 5.9 (1.3) kg. There was a significant negative correlation between grip strength change and maternal BMI (β = -0.05, 95% CI = -0.08, -0.01, p = 0.013) and with twin scans (β = -2.00, 95% CI = -3.8, -0.26, p = 0.03). No other significant correlations existed between grip or pincher strength changes and recorded f-echo variables. Increasing maternal BMI and twin status significantly decreased grip strength in f-CS after f-echo. Future studies are needed to determine how to minimize the risk of WRMD when performing f-echo on mothers with these characteristics.
Peer review remains a cornerstone of scientific publishing, ensuring the integrity, rigor, and relevance of the biomedical literature. However, structured training in peer review is rarely provided, particularly for earl...Peer review remains a cornerstone of scientific publishing, ensuring the integrity, rigor, and relevance of the biomedical literature. However, structured training in peer review is rarely provided, particularly for early-career clinicians and researchers who are increasingly invited to contribute. This article presents ten practical considerations aimed at supporting first-time reviewers in delivering high-quality, fair, and constructive evaluations. These include selecting appropriate review invitations, recognising the importance of mentorship, minimising bias, and maintaining ethical standards. By combining experiential insights with established principles, this guide seeks to demystify the peer-review process and promote best practices among emerging reviewers.
The optimal timing of aortic valve intervention in children and young adults with chronic aortic regurgitation (AR) remains poorly defined. Surgical thresholds derived from adult populations may not reliably predict left...The optimal timing of aortic valve intervention in children and young adults with chronic aortic regurgitation (AR) remains poorly defined. Surgical thresholds derived from adult populations may not reliably predict left ventricular (LV) reverse remodeling or functional recovery in younger patients, whose myocardium and adaptive responses differ substantially. The objective of this study was to identify preoperative echocardiogram and cardiac magnetic resonance (CMR) parameters associated with postoperative LV recovery in young patients with AR. We conducted a retrospective study of patients younger than 30 years with moderate-to-severe AR who underwent aortic valve surgery. LV recovery was defined as normalization of LV volumes and systolic function within 6-12 months following surgery. Preoperative imaging variables were compared between patients with and without LV recovery. Logistic regression analysis was used to identify predictors of postoperative LV recovery. Twenty-two patients (mean age 16 years) were included. Postoperative improvement in LV dimensions was observed in 91% of patients. Complete LV recovery occurred in only 77%. Larger echocardiographic LV end-systolic volume (LVESV) was associated with reduced odds of LV recovery. Impaired CMR derived global circumferential strain (GCS) was significantly associated with failure to achieve LV recovery. In our cohort with chronic AR, preoperative echocardiographic LVESV and CMR-derived GCS were associated with postoperative LV recovery, whereas adult-derived volumetric thresholds were less predictive. This underscores the limitations of applying adult surgical criteria to younger patients and suggests advanced imaging markers may better inform optimal timing of intervention. Larger prospective studies are needed to validate these findings.
Postural orthostatic tachycardia syndrome (POTS) is associated with autonomic dysregulation, but ambulatory markers that capture autonomic phenotype remain incompletely defined. To compare Holter-derived heart rate varia...Postural orthostatic tachycardia syndrome (POTS) is associated with autonomic dysregulation, but ambulatory markers that capture autonomic phenotype remain incompletely defined. To compare Holter-derived heart rate variability (HRV) metrics between adolescents with POTS and healthy controls and to derive candidate discriminatory thresholds. Retrospective case-control analysis of an existing Holter dataset (POTS n = 19, controls n = 44). Heart rate variability was processed in Kubios HRV Premium. Between-group comparisons used Welch's t-test with Benjamini-Hochberg false discovery rate adjustment. Nonparametric sensitivity analyses yielded similar results. Discrimination was assessed using receiver operating characteristic analysis; optimal thresholds were defined by the Youden index. An exploratory penalized logistic regression model (L1 regularization) was fit with standardized candidate HRV predictors and internal cross-validation. Among 19 POTS patients and 44 control subjects, POTS demonstrated lower time- and frequency-based HRV, higher rate-related and sympathetic composite indices, and lower parasympathetic composite measures. The strongest univariate discriminator was PNSi (Parasympathetic Nervous System index; AUROC 0.874; threshold ≤ -1.10; sensitivity 77.8%; specificity 88.6%). Other high-performing discriminators included SNSi, mean RR, mean heart rate, and SDNNi. Holter-derived HRV metrics demonstrate a reproducible pattern consistent with reduced parasympathetic modulation and relative sympathetic predominance in POTS. Several variables yield candidate discriminatory thresholds that may support physiologic phenotyping and warrant validation in independent cohorts.
Left ventricle (LV) systolic dysfunction, which has been observed in pediatric patients with cancer even prior to chemotherapy, is associated with adverse cardiac events on short-term follow-up. Left atrium (LA) function...Left ventricle (LV) systolic dysfunction, which has been observed in pediatric patients with cancer even prior to chemotherapy, is associated with adverse cardiac events on short-term follow-up. Left atrium (LA) function is pivotal in LV filling and output. Our aim was to evaluate LA function and dimensions before starting chemotherapy in children with cancer, compared to controls. This retrospective cross-sectional study assessed echocardiographic LA function in asymptomatic children with cancer before starting chemotherapy and normal controls. LA function was assessed during the reservoir phase (LASr) denoting LA filling, the passive emptying conduit phase (LAScd) and the active atrial contraction booster phase (LASbo) of the LA strain curve. LV diastolic functional parameters, mitral valve early filling maximum velocity (E), atrial filling maximum velocity (A) and tissue Doppler imaging (E' and A') were concurrently measured. The pre-chemotherapy (n = 79) and control (n = 80) groups had comparable gender and age at echocardiogram and similar LASr (39.0% ± 9.2% vs. 39.0% ± 9.1%, p = 0.99). However, LAScd was significantly lower (23.1% ± 11.3% vs. 28.6% ± 8.6%, p < 0.05) and was compensated by increased LASbo function (15.9% ± 11.2% vs. 10.4% ± 7.5%, p < 0.05) in the pre-chemotherapy group, compared to controls. Our data demonstrating abnormalities in LA functional strain parameters in children with cancer, prior to chemotherapy, are novel and perplexing. These LA strain abnormalities may be associated with cancer related systemic effects or inflammatory cytokine release, though the exact mechanisms require further investigation.
de Souza LV, de Araújo Almeida Muniz ME, de Souza DRC
… +6 more, de Meneck F, da Silva Teixeira R, de Freitas Dantas Gomes EL, de Almeida LB, Chagas PSC, do Carmo Pinho Franco M
Physical activity is known to influence cardiovascular regulation in children. However, evidence regarding cardiac autonomic responses to recreational physical training (RPT) in healthy pediatric populations remains limi...Physical activity is known to influence cardiovascular regulation in children. However, evidence regarding cardiac autonomic responses to recreational physical training (RPT) in healthy pediatric populations remains limited. Thus, this study aimed to investigate changes in cardiac autonomic modulation associated with a RPT program in healthy children. We conducted a quasi-experimental pre-post intervention study including healthy children who participated in a 10-week RPT. Cardiac autonomic modulation was assessed at rest using short-term heart rate variability (HRV) analysis. HRV measurements (time- and frequency-domain), anthropometric, hemodynamic, and cardiorespiratory variables were evaluated before and after the intervention. Effect sizes were calculated to estimate the magnitude of observed changes. Seventy-four participants (31 girls and 43 boys; mean age 8.6 ± 0.9 years) were included. Following the intervention, significant increases were observed in time-domain HRV measurements, including SDNN (standard deviation of normal RR intervals; pre- 49.1 ± 2.1, post- 61.9 ± 2.1 ms; p < 0.01) and RMSSD (root mean square of successive differences between normal RR intervals; pre- 52.1 ± 2.7, post- 65.2 ± 2.7 ms; p < 0.01), with large effect sizes. No significant changes were detected in blood pressure, estimated peak oxygen uptake, or frequency-domain HRV. Waist circumference decreased (pre- 66.2 ± 2.0, post- 65.2 ± 2.0 cm; p < 0.01), whereas body mass index percentile remained unchanged. Therefore, participation in a RPT was associated with favorable changes in cardiac autonomic modulation in healthy children, particularly in measurements reflecting global variability and parasympathetic activity. These findings suggest that RPT may elicit early autonomic adaptations during childhood, even in the absence of measurable changes in traditional cardiovascular risk markers.
Body mass index (BMI) incompletely captures body composition and functional reserve in congenital heart disease. We assessed whether skeletal muscle mass (SMM) relates more closely than BMI to stroke volume (SV), strengt...Body mass index (BMI) incompletely captures body composition and functional reserve in congenital heart disease. We assessed whether skeletal muscle mass (SMM) relates more closely than BMI to stroke volume (SV), strength, and peak oxygen uptake (VO₂max) in Fontan physiology versus healthy controls. Cross-sectional assessment included multifrequency bioimpedance SMM, noninvasive SV (NICaS), handgrip dynamometry, and cardiopulmonary exercise testing. Associations of SMM and BMI with SV, grip strength, and VO₂max were evaluated within cohorts using Pearson correlations, and impedance SMM was compared with Lee-equation SMM. Forty-two participants were studied: Fontan group (n = 21; mean age 28.3 ± 9.7 years; 8 women) and control group (n = 21; mean age 29.5 ± 5.9 years; 7 women). BMI was lower in the Fontan group than in the control group (24.0 ± 4.4 vs. 26.4 ± 3.4 kg/m²), as was SMM (27.8 ± 6.2 vs. 34.0 ± 7.7 kg). SMM correlated with SV in the control group (r = 0.770, p < 0.0001) and Fontan group (r = 0.573, p = 0.007), whereas BMI showed a weaker association in the control group (r = 0.465, p = 0.034) and no significant association in the Fontan group (r = 0.365, p = 0.104). SMM correlated with handgrip strength in both groups (control r = 0.754; Fontan r = 0.807), whereas BMI did not (controls r = 0.197; Fontan r = 0.242). SMM correlated with VO₂max in the control group (r = 0.503, p = 0.020), but not in the Fontan group (r = 0.106, p = 0.65). BMI correlated with SMM in the control group (r = 0.448, p = 0.042), but not in the Fontan group (r = 0.361), suggesting greater body composition heterogeneity in Fontan patients. Impedance-derived and Lee-estimated skeletal muscle mass showed strong agreement in both groups (control r = 0.903; Fontan r = 0.864). SMM outperformed BMI in reflecting SV and strength; in Fontan circulation, VO₂max appears more centrally constrained than muscle-mass limited.
Despite recommendations for early neurodevelopmental evaluation of infants who undergo complex congenital heart surgery, attendance at cardiac neurodevelopmental clinics (CNC) across the nation is low. Several studies ha...Despite recommendations for early neurodevelopmental evaluation of infants who undergo complex congenital heart surgery, attendance at cardiac neurodevelopmental clinics (CNC) across the nation is low. Several studies have identified factors associated with attendance, however geographical and language-based factors have not been explored. We examined the association between geographical and language-based factors and CNC attendance. We hypothesized that patients with increased travel distance, lower neighborhood affluence, and families who speak a language other than English had lower CNC attendance. We also hypothesized increased use of interpretation services improved attendance. We performed a single center cross-sectional study by linking data from cardiac-specific quality databases, interpretation services, and geocoding tools. We performed descriptive statistics, univariate, and multivariate analysis with a primary outcome of CNC attendance before 12 months of age. Among 755 infants, 22% attended CNC. We did not detect a significant difference across all neighborhood deprivation indices (p > 0.05) and all languages (p > 0.05). Of those who did not attend CNC, 51% received greater than 2 interpretation encounters per day in comparison to 62% in those who did attend CNC (p > 0.9). Patients who lived out of state were at decreased odds of CNC attendance (OR 0.17, 95%CI 0.08-0.33). Patients who live out-of-state attend CNC less, however we failed to find a significant difference in attendance based on preferred language, frequency of interpretation services, and community deprivation index. These findings may guide interventions and future research to optimize CNC attendance.
The Fontan procedure provides long-term palliation for patients with single-ventricle physiology, but long-term outcomes remain limited by progressive Fontan circulatory failure. Early identification of patients at risk...The Fontan procedure provides long-term palliation for patients with single-ventricle physiology, but long-term outcomes remain limited by progressive Fontan circulatory failure. Early identification of patients at risk for adverse outcomes is critical to guide surveillance and management strategies. We aimed to identify peri-Fontan factors associated with mortality or need for advanced cardiac therapies in Fontan patients. We conducted a retrospective matched case-control study of patients who underwent Fontan completion between 2000 and 2023 at a single tertiary congenital heart center. Cases were defined as patients who experienced death, ventricular assist device (VAD) implantation, or heart transplantation after Fontan completion. Each case was matched with three controls by year of Fontan surgery and duration of follow-up. Pre-Fontan hemodynamics, cardiac anatomy, and perioperative variables were analyzed using conditional logistic regression. A total of 53 cases who experienced death, VAD implantation, or heart transplantation were matched to 151 controls. The median age at Fontan failure was 11.5 years (IQR 6.9-16.1), occurring a median of 8.6 years (IQR 3.8-13.8) after Fontan completion. In multivariable analysis, the factors independently associated with death/transplant/VAD implantation were systemic right ventricular morphology (adjusted odds ratio [aOR] 6.87, 95% CI 2.07-22.8, p = 0.002), pre-Fontan Glenn/hemi-Fontan pressure > 12 mmHg (aOR 4.72, 95% CI 1.74-12.8, p = 0.002), and postoperative hospital length of stay > 12 days (aOR 8.57, 95% CI 2.78-26.4; p = 0.0002). Systemic right ventricular morphology, elevated pre-Fontan Glenn/hemi-Fontan pressure, and prolonged postoperative hospitalization are independently associated with long-term mortality or need for advanced cardiac therapies after Fontan completion. These findings add to the growing body of literature identifying peri-Fontan factors associated with adverse long-term outcomes and support the importance of perioperative hemodynamics and postoperative recovery in long-term Fontan risk assessment.
Cardiac index (CI) is used to assess graft function and calculate pulmonary vascular resistance (PVR) following orthotopic heart transplantation (OHT). The gold standard is thermodilution (TD), but in practice, the Fick...Cardiac index (CI) is used to assess graft function and calculate pulmonary vascular resistance (PVR) following orthotopic heart transplantation (OHT). The gold standard is thermodilution (TD), but in practice, the Fick method using the LaFarge VO estimation is commonly used. We hypothesize that the difference between cardiac index and PVR using the estimated Fick principle versus thermodilution decreases over time following OHT in pediatric patients. This is a single center prospective observational cohort study of patients who received an orthotopic heart transplant between 5 and 18 years of age. We performed serial pair-wise comparisons of cardiac index measured using the Fick method (LaFarge VO estimation) and thermodilution. PVR was calculated using both the Fick CI and the thermodilution CI. Descriptive statistics, multivariable model, linear mixed-effects model, intra-class correlation, absolute difference, and unpaired t-test statistical methods were utilized to analyze the data. In 48% of cases, cardiac index was normal by Fick but abnormal using thermodilution. In 27% of cases, PVR was normal by Fick but abnormal using thermodilution. The ICC between Fick CI and TDCI was 0.13, indicating low agreement between the two methods. The absolute difference between Fick and TD did not vary significantly by pre-transplant use of VAD, milrinone, inpatient hospitalization, or indication for transplant. There was not a significant relationship between days since transplantation and absolute difference between Fick and TDCI. Upon controlling for patient level differences, TDCI did not significantly change over time since OHT. There is poor agreement between the estimated Fick method, using the LaFarge equation, and thermodilution in determining cardiac index after pediatric heart transplantation. When providers only use the Fick method, they are under-estimating cardiac dysfunction and pulmonary hypertension, two very important measures of graft function and post-transplant health. These differences between Fick and thermodilution persist for at least the first year after heart transplant.
Furosemide resistance and fluid overload remain major challenges in postoperative pediatric cardiac patients. Although adult studies suggest synergistic effects of hypertonic saline with loop diuretics, pediatric postope...Furosemide resistance and fluid overload remain major challenges in postoperative pediatric cardiac patients. Although adult studies suggest synergistic effects of hypertonic saline with loop diuretics, pediatric postoperative physiology differs substantially. Data describing the relationship between serum sodium and diuretic responsiveness after congenital heart surgery remain limited. We conducted a retrospective observational cohort study of 264 pediatric patients (0-18 years) admitted to a tertiary cardiac intensive care unit following cardiac surgery (2021-2023) who received intravenous furosemide. The analytic window comprised postoperative days 1-2 (POD1-2), yielding 528 patient-day observations. The primary outcome was total urine output normalized to body weight (mL/kg/day); the primary exposure was mean daily serum sodium (mEq/L). Linear mixed-effects models with patient-level random intercepts were used. Serum sodium was modeled using natural cubic splines (3 degrees of freedom) to allow for nonlinearity, adjusting for furosemide dose, log(VIS + 1) as a marker of vasoactive support, albumin, creatinine, fluids administered, postoperative day, and infusion mode (continuous infusion vs. bolus). To complement the spline model with a clinically interpretable summary, we additionally fit a piecewise threshold model with a data-driven changepoint. To address temporal-direction concerns from same-window measurement, we performed prespecified lagged-exposure (POD1 sodium → POD2 urine output and POD2 → POD3) and within-patient first-difference sensitivity analyses. The strength of unmeasured confounding required to attenuate the primary association to the null was quantified via E-values for the point estimate and the lower bound of the 95% confidence interval. Multiple imputation by chained equations (m = 30) was used for modest missingness on laboratory covariates; the primary outcome and furosemide dose were essentially fully observed. Mean age was 2.7 ± 3.7 years (median 1.0 [IQR 3.6]). The sodium-urine-output association followed a steep-then-flat shape: predicted urine output rose with sodium across the hyponatremic range and plateaued near low-normal values; a data-driven changepoint analysis on a single imputed dataset estimated the slope transition at serum sodium ≈ 136.5 mEq/L (95% CI 133.5 to 139.5). The formal test for nonlinearity was not significant (χ²=3.24, df = 2, p = 0.198). Translated to a clinically standard 5 mEq/L contrast, correcting sodium from 135 to 140 mEq/L was associated with + 9.48 mL/kg/day urine output (95% CI 7.24 to 11.72), while no significant change was observed for 140 to 145 mEq/L (+ 0.90 mL/kg/day, 95% CI - 1.29 to 3.09). Daily fluids administered were positively associated with urine output (β = +0.27 mL/kg/day per 1 mL/kg/day intake; 95% CI 0.18 to 0.36; p < 0.001), the most precisely estimated covariate in the model. Vasoactive support, entered as log(VIS + 1), was independently associated with greater urine output (β = +11.11 mL/kg/day per unit log[VIS + 1]; 95% CI 7.13 to 15.10; p < 0.001). Continuous furosemide infusion was associated with + 24.76 mL/kg/day greater urine output than bolus dosing (95% CI 7.15 to 42.37; p = 0.006). Furosemide total daily dose was not independently associated with urine output after adjustment (β = -0.27 mL/kg/day per mg/kg/day; p = 0.85). Serum sodium did not modify furosemide responsiveness (interaction likelihood-ratio test χ²=2.26, df = 3, p = 0.520). The primary association was directionally consistent in lagged analyses (POD2 sodium → POD3 urine output: contrast + 9.22 mL/kg/day, 95% CI 7.45 to 11.00; POD1 → POD2: +6.83 mL/kg/day, 95% CI 5.41 to 8.25). The E-value for the lower 95% confidence bound of the primary contrast was 1.75. In postoperative pediatric cardiac patients receiving intravenous furosemide, higher serum sodium in the hyponatremic range was associated with substantially greater urine output, with the association plateauing near 136 mEq/L. Vasoactive support intensity was an additional independent positive correlate of urine output, while serum sodium and furosemide dose did not interact. These observational findings are most consistent with serum sodium serving as a marker of physiologic recovery and renal perfusion rather than a directly modifiable mediator of diuresis. Prospective studies are needed to test whether sodium-modifying or infusion-mode interventions improve diuretic responsiveness and clinical outcomes.
Caffeine consumption among children and adolescents has increased in recent years, largely through energy drinks. While caffeine is generally considerd as safe in low doses, current evidence suggests that high intake may...Caffeine consumption among children and adolescents has increased in recent years, largely through energy drinks. While caffeine is generally considerd as safe in low doses, current evidence suggests that high intake may provoke arrhythmias and electrocardiographic abnormalities in younger populations. This systematic review evaluates the arrhythmogenic effects of caffeine consumption in pediatric and adolescent patients. This review followed PRISMA 2020 guidelines and was registered in PROSPERO (ID 1180917). A systematic search of PubMed, EMBASE, and Cochrane CENTRAL identified studies evaluating caffeine-related arrhythmias in individuals under 19 years of age. Eligible publications included peer-reviewed studies, case reports, and case series reporting arrhythmogenic or electrocardiographic outcomes. Screening and data extraction were performed independently by two reviewers using Covidence. The search yielded 126 articles, of which 12 met the inclusion criteria. These comprised 9 case reports, 1 prospective study, 1 retrospective study, and 1 review including an unpublished case report. Across all eligible studies, a total of 39 adolescents (aged 13-18 years) were reported to have caffeine associated arrhythmias or ECG changes. Caffeine sources included energy drinks, caffeine containing tablets and powdered caffeine. Reported cardiac side effects ranged from sinus tachycardia and premature atrial or ventricular contractions to supraventricular tachycardia, ventricular tachycardia, QTc prolongation and ST-segment abnormalities. This systematic review demonstrates that high dose caffeine from sources like energy drinks, powders and tablets can induce a range of cardiac arrhythmias and electrophysiological abnormalities. Our findings underscore the need for increased awareness and improved public health education regarding the cardiovascular danger of high-dose caffeine in this population.
Sotatercept, a first-in-class activin signaling inhibitor, has demonstrated disease-modifying efficacy in adults with pulmonary arterial hypertension (PAH). Pediatric data remain limited to ongoing clinical trials. We co...Sotatercept, a first-in-class activin signaling inhibitor, has demonstrated disease-modifying efficacy in adults with pulmonary arterial hypertension (PAH). Pediatric data remain limited to ongoing clinical trials. We conducted an IRB-approved, single-center retrospective analysis of pediatric patients with WHO Group 1 or Group 3 PAH treated with off-label sotatercept at Rady Children's Hospital / UC San Diego. All patients had received maximal background triple therapy yet demonstrated persistent symptoms or adverse hemodynamics. Right heart catheterization was performed before sotatercept initiation and at approximately 24 weeks thereafter. Hemodynamic parameters were analyzed descriptively. Seven pediatric patients (median age 14 years; range 4-19) were included. Diagnoses comprised idiopathic PAH, heritable PAH, and PAH associated with congenital heart disease or congenital diaphragmatic hernia. Following sotatercept initiation, the majority of patients demonstrated reductions in pulmonary vascular resistance index (PVRI) and mean pulmonary artery pressure (mPAP), with heterogeneity in magnitude of response. Pulmonary capillary wedge pressure (PCWP) remained stable in all patients. In this small, highly selected pediatric cohort with advanced PAH refractory to triple therapy, off-label sotatercept was associated with favorable hemodynamic trends at 24 weeks. These findings support biologic plausibility suggested by adult randomized trials and underscore the urgent need for prospective pediatric studies.
This scoping review characterizes the relationship of blood brain biomarkers (BBM) to outcomes in pediatric congenital heart disease (CHD) and pediatric cardiac arrest (CA). PubMed, Embase, the Cochrane Library, and Web...This scoping review characterizes the relationship of blood brain biomarkers (BBM) to outcomes in pediatric congenital heart disease (CHD) and pediatric cardiac arrest (CA). PubMed, Embase, the Cochrane Library, and Web of Science were searched on 9/25/23 and again on 1/7/26 for literature on pediatric populations with brain injury and CHD and/or CA. Inclusion criteria were: (1) human pediatric subjects ≤ 18 years of age who have CHD and/or underwent CA, (2) BBM. Exclusion criteria were: (1) adult populations, (2) studies without BBM, (3) inflammatory or imaging biomarkers. This review is reported following the PRISMA extension for scoping reviews (PRISMA-ScR) and conducted following a study protocol developed in line with the PRISMA-P reporting guidelines. A total of 3687 articles were screened by a minimum of two reviewers; 87 were included in this review. All studies incorporated at least 1 of 13 included BBM with the most frequent being S100B (n = 53, 60.9%), GFAP (n = 27, 31.0%), and NSE (n = 32, 36.8%). 51.7% included neurologic imaging/monitoring (n = 45/87), and 49.4% specified neurodevelopmental/neurofunctional testing within neurologic outcome analysis (n = 43/87). 67.4% of studies reporting neurodevelopmental/functional testing observed a correlation between BBM and poor neurologic outcome (n = 29/43). BBM show promise in predicting neurologic outcomes in pediatric patients following cardiac arrest and in the setting of surgical procedures and other interventions for CHD patients. Due to the limited number of studies, further research is needed.
Implicit bias and socioeconomic factors may influence diagnostic testing in pediatrics, but their effects on echocardiogram ordering in outpatient pediatric cardiology are not well understood. We evaluated whether patien...Implicit bias and socioeconomic factors may influence diagnostic testing in pediatrics, but their effects on echocardiogram ordering in outpatient pediatric cardiology are not well understood. We evaluated whether patient demographics, insurance type, neighborhood Childhood Opportunity Index (COI), and provider characteristics (years of experience, degree attained, gender) were associated with guideline-concordant echocardiogram ordering for initial outpatient evaluation of pediatric chest pain. We conducted a retrospective study of 299 pediatric patients undergoing initial outpatient evaluation for chest pain between 2018 and 2024 at the University of Maryland Children's Hospital outpatient cardiology clinics. Echocardiogram appropriateness was determined using previously published appropriate use criteria (AUC) and structured clinical management and assessment plan (SCAMP) criteria. Associations between patient and provider factors and echocardiogram appropriateness were assessed. Echocardiogram ordering was concordant with AUC/SCAMP recommendations in 232 encounters (77.6%). White patients were more likely than non-White patients to receive guideline-concordant ordering (83.8% vs. 72.8%, p = .025). Patients from "Moderate", "Low", or "Very Low" COI neighborhoods were more likely to receive a non-indicated echocardiogram (27.5%, 31.3%, and 20%) compared to patients with "High" or "Very High" COI (16.4% and 14.4%) (p=.019). Providers with less than 10 years of experience were more likely to order echocardiograms in concordance with AUC/SCAMP criteria (86.9% vs. 71.2%, p < .001). Most echocardiogram ordering was guideline-concordant; however, deviations from guidelines were associated with patient race, neighborhood COI, and provider experience. Non-White patients and those from lower COI neighborhoods received more non-indicated echocardiograms than White patients or those from higher COI neighborhoods. These findings identify opportunities to improve equitable, guideline-concordant care in outpatient pediatric cardiology.
Paediatric cardiomyopathy is a rare, serious inherited cardiac condition and a leading indication for paediatric heart transplantation. Population-based data describing paediatric cardiomyopathy within a single healthcar...Paediatric cardiomyopathy is a rare, serious inherited cardiac condition and a leading indication for paediatric heart transplantation. Population-based data describing paediatric cardiomyopathy within a single healthcare system complements multinational registry studies by providing important local epidemiological context. To describe the incidence, temporal trends, phenotypic characteristics, genetic findings, and clinical outcomes of paediatric cardiomyopathy in Northern Ireland over a 22-year period. A national retrospective cohort study of children diagnosed with cardiomyopathy in Northern Ireland between 2003 and 2025 was conducted. Population-based incidence rates were calculated for children aged 0-15 years affected by Dilated and Hypertrophic Cardiomyopathy using annual population denominators (2003-2023), with exact Poisson 95% confidence intervals. Temporal trends were assessed using quasi-Poisson and negative binomial regression models with population offsets. Clinical characteristics, genetics and outcomes (implantable cardioverter-defibrillator implantation, transplantation, and death) were analysed descriptively. Kaplan-Meier survival analysis and Cox regression were performed using a composite outcome of death or transplantation. Eighty-four children were diagnosed during the study period, corresponding to a mean annual incidence of 0.9 per 100,000 children. Annual incidence varied from 0.26 to 3.11 per 100,000. There was no statistically significant temporal increase in incidence (p = 0.058), although higher incidence values were observed in more recent years. HCM (n = 38, 45%) and DCM (n = 24, 29%) were the most common subtypes. Overall, 61% of patients had a pathogenic or likely pathogenic genetic variant or phenocopy condition identified. There were seven deaths, six cardiac transplants, and eighteen implantable cardioverter-defibrillator insertions. Children diagnosed before one year of age accounted for 65% of deaths or transplants. Compared with HCM, DCM was associated with a significantly increased risk of death or transplantation (hazard ratio 6.25, 95% CI 1.30-33.33, p = 0.0094). Paediatric cardiomyopathy remains rare in Northern Ireland with a similar incidence to previous international reports but is associated with substantial morbidity and mortality, particularly among infants and those with DCM. Although higher annual estimates were observed in more recent years, no statistically significant temporal increase was identified. Ongoing population-based surveillance and integration with international registries are essential to improve understanding and outcomes in these rare conditions.
Complex congenital cardiac surgery demands precise three-dimensional (3D) spatial understanding, yet preoperative planning continues to rely predominantly on two-dimensional (2D) image interpretation. Contemporary cardia...Complex congenital cardiac surgery demands precise three-dimensional (3D) spatial understanding, yet preoperative planning continues to rely predominantly on two-dimensional (2D) image interpretation. Contemporary cardiac computed tomography (CT) and magnetic resonance imaging (MRI) are volumetric by design, but their value is constrained by how data are ultimately converted into shared spatial understanding. The growing availability of CT-based segmentation, virtual reality (VR) visualization, and three-dimensional printing offers a practical means to externalize patient-specific anatomy into manipulable, immersive representations. Current evidence, though modest and largely single-centre, consistently shows that 3D models can faithfully represent complex anatomy and that clinicians repeatedly report improved comprehension of spatial relationships through their use. In this perspective, we argue for a deliberate, case-selected approach to 3D image visualization in complex congenital cardiac surgery, identify the human and institutional factors that determine real-world utility beyond hardware and software alone, and propose criteria for systematic adoption. Prospective outcome data are needed; in their absence, thoughtful incorporation of these tools into surgical planning workflows represents a clinically rational approach.
Catheter ablation for supraventricular tachycardia (SVT) in children is technically challenging, and outcomes in children younger than 5 years and weighing less than 15 kg remain poorly characterised. No systematic revie...Catheter ablation for supraventricular tachycardia (SVT) in children is technically challenging, and outcomes in children younger than 5 years and weighing less than 15 kg remain poorly characterised. No systematic review has previously focused exclusively on this population. We searched PubMed, Embase, the Cochrane Library, and ScienceDirect from January 2000 to December 2025 for studies reporting catheter ablation outcomes for SVT in children aged < 5 years and weighing < 15 kg. Risk of bias was assessed using the ROBINS-I tool. A narrative synthesis was performed due to clinical and methodological heterogeneity. The protocol was registered on PROSPERO (CRD420251207901). Ten retrospective cohort studies comprising 128 patients undergoing 154 procedures were included. Acute procedural success was high across all studies, with an overall mean of approximately 94% (range 75-100%). Recurrence rates ranged from 0% to 20%, with most studies reporting low or no recurrence. Complication rates varied widely (0-32%); three studies reported no complications, while others documented events including vascular injury, transient conduction disturbances, and structural complications. In carefully selected children aged < 5 years and weighing < 15 kg, catheter ablation for SVT achieves high acute success rates comparable to older paediatric populations, with an acceptable but variable complication profile. These findings may support consideration of ablation in this high-risk group, challenging rigid weight-based thresholds that may delay definitive therapy. Prospective multicentre studies are needed to define optimal patient selection criteria and generate higher-quality evidence.