Beta-blockers (BB) are a core treatment in adult heart failure (HF) guideline directed medical therapy with evidence of reductions in mortality and HF hospitalizations. The optimal timing of BB initiation in children remains difficult to determine due to possible hemodynamic effects. We sought to analyze outcomes of pediatric HF readmission or death within 30 days of discharge after ambulatory vs. inpatient BB initiation. This retrospective analysis utilized the Advanced Cardiac Therapies Improving Outcomes Network HF registry to compare outcomes in patients with BB initiation during inpatient or outpatient encounters. Patients were excluded if BB initiation occurred prior to admission. In total, 94 patients were started on BB therapy with 69 (73.4
Pulmonary venous obstruction (PVO) is a major determinant of early morbidity and mortality in neonates with total anomalous pulmonary venous return (TAPVR). Accurate prenatal identification of obstructed TAPVR remains challenging due to low pulmonary blood flow in fetal circulation. Maternal hyperoxygenation (MHO) increases fetal pulmonary blood flow and may unmask clinically significant obstruction. We retrospectively reviewed three fetuses with prenatally diagnosed TAPVR who underwent late‑gestation fetal echocardiography with MHO testing. Doppler indices of pulmonary vasoreactivity and vertical vein (VV) flow were assessed at baseline and during MHO and correlated with postnatal clinical outcomes. All fetuses demonstrated preserved pulmonary vasoreactivity during MHO. One fetus had no Doppler evidence of PVO at rest or during MHO and had a stable postnatal course without emergent intervention. One fetus demonstrated elevated VV velocities at baseline that worsened during MHO and required emergent surgical repair shortly after birth. In the third fetus, VV Doppler findings were normal at baseline but became abnormal during MHO, accurately predicting postnatal clinical deterioration and the need for early catheter‑based intervention. These findings suggest that MHO testing can unmask occult pulmonary venous obstruction in fetal TAPVR and may improve prenatal risk stratification and perinatal planning.
Pre-operative valve assessment is critical for surgical planning prior to the Ross procedure. No pediatric studies have assessed preoperative sizing comparing different imaging modalities. Single center study including all patients undergoing the Ross procedure from January 2018 – December 2023. Demographics and measurements of the aortic and pulmonary valve dimensions using transthoracic echocardiography (TTE), cardiac CT (CCT), and direct surgical measurement were included. Bland Altman Plots and intraclass correlation coefficient (ICC) assessed agreement between direct surgical measurement, TTE, and CCT, and between investigators. Of the 71 Ross patients during the study period, 62 patients met surgical inclusion criteria and of those, 58 met TTE inclusion criteria, 39 met CCT inclusion criteria, and 35 met both TTE and CCT criteria. CCT overestimated aortic and pulmonic valve annular dimensions while TTE more closely approximated direct surgical measurement. ICC demonstrated poor agreement between direct surgical measurement and pulmonary valve CCT. Agreement for CCT evaluation of the aortic valve was fair (0.74) and for TTE was fair to good for both valve measurements (range 0.71 to 0.88). TTE evaluation of the aortic and pulmonary valve annular measurements had better agreement with surgical measurements when compared to CCT. While CCT may not be required for pre-operative valve sizing in these patients, CCT plays an important role in delineating coronary, sternal, and other anatomic variables. Both modalities provide valuable complementary pre-operative information.
Kawasaki disease (KD) and multisystem inflammatory syndrome in children (MIS-C) share overlapping clinical features, making diagnosis challenging. We compared patients with incomplete KD to those with non-severe MIS-C to identify distinguishing characteristics. Patients were enrolled in the International KD Registry (01/2020–10/2023) from 40 centers across eight countries. Among 2146 MIS-C patients, 769 met criteria for non-severe confirmed MIS-C (no shock or ICU admission). Among 1358 KD patients, 146 met American Heart Association (AHA) criteria for confirmed incomplete KD and 372 were classified as unconfirmed incomplete KD. Demographics, clinical and laboratory features, treatment, and cardiac outcomes were compared. Non-severe MIS-C patients were significantly older (median 7.4 years) than both confirmed (2.3 years) and unconfirmed (2.5 years; p < 0.001) incomplete KD patients, and had different ethnic distributions with more Black and fewer East or South Asian patients. MIS-C patients had shorter fever duration, fewer classic KD features, and more gastrointestinal and respiratory symptoms. They demonstrated lower white blood cell counts but higher inflammatory markers and markers of organ dysfunction. Cardiac involvement differed: MIS-C patients had more myocardial dysfunction, including lower ejection fraction and higher cardiac biomarkers. Coronary artery involvement in MIS-C (median maximum z-score 1.3) was similar to unconfirmed incomplete KD (median z-score 1.3) but less than confirmed incomplete KD (median z-score 2.0; p < 0.01). Older age, distinct ethnicity, prominent gastrointestinal and respiratory symptoms, and multisystem inflammation with ventricular dysfunction but relatively less coronary artery involvement help distinguish non-severe MIS-C from incomplete KD.
Thrombocytopenia following transcatheter aortic valve implantation has been described, but platelet count decline after self-expanding transcatheter pulmonary valve replacement (TPVR) has not been previously highlighted. This study aimed to describe the frequency, magnitude, and short-term clinical associations of platelet count decline after self-expanding TPVR. We retrospectively reviewed all consecutive self-expanding TPVRs performed at our institution between May 2022 and August 2025. Demographic, procedural, echocardiographic, and laboratory data were collected, including platelet and hemoglobin levels before and after TPVR. Descriptive statistics were used, and correlation analysis assessed the relationship between platelet and hemoglobin decreases. Forty-one patients were included, with a median age of 18.7 years [IQR 16.1–23.9]; 80
Inhaled nitric oxide (iNO) lowers pulmonary vascular resistance and improves pulmonary gas exchange. It has become a common therapy following pediatric cardiac surgery, but there are limited data on its current use or clinical impact in this population. This retrospective analysis reviewed all patients within the Pediatric Cardiac Critical Care Consortium (PC4) clinical registry, representing 63 North American cardiac intensive care units (CICU), who underwent cardiac surgery from August 2014 to August 2023, to describe contemporary iNO use and its relationship to patient characteristics and outcomes. We also investigated the relationship between timing of iNO therapy for postoperative pulmonary hypertension (PH) and mortality. Of 63,582 non-neonatal patients admitted to the CICU following cardiac surgery, 12.2
We report hybrid transaortic coronary intervention for severe stenosis of a left main coronary artery in a 3-month-old 2.9-kg female, following surgical repair of an anomalous left coronary artery from the pulmonary artery. The patient experienced myocardial ischaemia with severe left ventricular dysfunction, necessitating extracorporeal membrane oxygenation. Surgical reoperation was deemed of high-risk. After 2 failed attempts of percutaneous coronary interventions, hybrid transaortic access of the left main coronary artery was successfully performed. Because of immediate recoil after balloon angioplasty, a drug-eluting stent was implanted. The patient dramatically improved, with ECMO discontinuation, extubation, and recovery of an almost normal left-ventricular function. She was discharged home but experienced in-stent restenosis with suboptimal compliance to antiplatelet treatment. She remained clinically stable and awaiting bypass coronary graft. Such procedure with LMCA stenting in small infants is feasible and successful. It should be limited to life-saving situations, when no other treatment option is available.
The role of balloon atrial septostomy (BAS) in the contemporary management of neonates with dextra-transposition of the great arteries (d-TGA) undergoing arterial switch operation (ASO) remains controversial, with marked inter-institutional practice variation and conflicting outcome data. We evaluated the association between prior BAS and mortality, length of stay, pre-operative intubation rate, and neurological outcomes in neonates with d-TGA undergoing ASO, with pre-specified subgroup analyses stratified by TGA anatomical subtype. We conducted a systematic review and meta-analysis of studies reporting outcomes in neonates with d-TGA undergoing ASO with and without prior BAS. Databases were searched from inception to 2024. Risk of bias was assessed using ROBIN-I. Random-effects meta-analyses were performed for mortality, length of stay, and pre-operative intubation rate. Studies reporting only adjusted effect estimates without counts data were described narratively. The protocol was registered in PROSPERO (CRD420251041501). Twenty-eight studies (1983–2024) were included. Six contributed to mortality analysis (n=1151; 43 deaths). The pooled odds ratio (OR) for mortality was 0.73 (95
Pediatric cardiology has traditionally been organized around cardiac anatomy and physiology. Congenital and acquired cardiovascular disorders are classified according to structural and functional abnormalities, and clinical management is directed toward their identification and treatment. This approach has transformed survival and remains foundational to the discipline. As outcomes improve, however, an increasingly important question is why individuals exposed to similar cardiovascular constraints follow markedly different long-term trajectories. We propose adaptation under cardiovascular constraint as an integrative framework for studying this variation. Cardiovascular disorders establish physiological constraints within developing individuals, while trajectories of neurodevelopment, exercise capacity, growth, physiological reserve, and multisystem health emerge through interactions among those constraints, development, and adaptive responses. We propose the developmental trajectory as a fundamental unit through which adaptation can be observed and studied alongside anatomical diagnosis and conventional physiology. This perspective builds on developments already occurring within pediatric cardiology, including longitudinal and precision-health approaches, and integrates concepts from resilience theory, developmental biology, complexity science, network physiology, and physiological reserve. Pediatric cardiovascular disorders provide particularly informative models of developmental adaptation because physiological constraints may begin early, persist across development, and be characterized longitudinally. The framework reframes a central scientific question in pediatric cardiology: how do developing individuals maintain, modify, or lose function under sustained cardiovascular constraint? Its empirical value will depend on whether longitudinal measures of adaptation improve prediction, identify emerging vulnerability, or reveal potentially modifiable processes beyond established risk models.
Congenital heart disease (CHD) can lead to neurodevelopmental impairments and increased need for routine healthcare visits, potentially leading to deficits in academic performance. Children with CHD may also experience health-related discrimination in school and clinical settings. To determine the association of health-related discrimination (independent of bullying) with academic performance and school absenteeism among children with CHD. We analyzed data on schoolchildren with CHD from the 2022–2023 National Survey of Children’s Health (NSCH). Our primary exposure was health-related discrimination towards the child, based on caregiver report, with bullying included as one of the covariates. Our outcomes were children’s academic performance categorized as excellent, good and poor and school absenteeism dichotomized into 0–10 and 11 or more missed school days. Based on a sample of 1,058 children, approximately 10
Activation of cell stress-related pathways and systemic inflammation during cardiopulmonary bypass (CPB) are observed in both neonatal and adult cardiac surgery. Surgical CPB stress can impact on the long-term cardiac and neurodevelopmental outcomes of patients undergoing congenital cardiac surgery. Elevated HMGB1, an inflammatory, danger-associated molecular pattern (DAMP) molecule, is associated with adverse outcomes after surgery. Here, we investigated its utility as a biomarker for adverse surgical and neurodevelopmental outcomes in CHD patients undergoing surgery with CPB. We enrolled 29 CHD patients undergoing neonatal cardiac surgery with CPB. Blood was collected before surgery, immediately after surgery, and at 24 and 48 h after CPB. HMBG1 serum levels were determined using an ELISA assay. Death from index surgery was tracked, and neurodevelopmental outcome was determined between 4 months and 24 months using the Bayley instrument (BSID-III). Serum HMGB1 level was significantly increased at 24 and 48 h after CPB. The peak HMGB1 level was higher in patients who died post-surgically. Higher HMGB1 levels were associated with Bayley scores below 1 standard deviation for expressive speech and motor, especially in the gross motor domain. These findings support the utility of HMGB1 as a biomarker for prognosticating postsurgical cardiac and neurodevelopmental outcomes in CHD patients.
Nasal nitric oxide (nNO) is a non-invasive marker for predicting the risk of neurodevelopmental delays. We investigated whether nNO in infants with CHD undergoing congenital cardiac surgery could serve as a biomarker for assessing the risk of impaired neurodevelopment. Retrospective cohort study. Single center in a tertiary care children’s hospital. A retrospective study was conducted on 63 CHD patients, 0–12 months old, who had cardiac bypass surgery in the 1st year of life and for whom nNO levels had been collected before surgery using the chemiluminescent CLDsp88 NO analyzer. Neurodevelopmental assessments in patients older than 1 year were retained for analysis. NO levels were dichotomized as normal or low based on previously established age-stratified norms. Multivariable regression analysis was conducted to examine possible confounders. CHD patients with low nNO exhibited significant neurodevelopmental delays (P < 0.001), cognitive delays (P = 0.0021), language delays (P < 0.001), and a trend toward motor delays (P = 0.034). Multivariable regression analysis incorporating various confounders as covariates (stroke, cardiac arrest, arrhythmia, seizures, genetic anomalies) supports a significant association between low nNO and cognitive delay (P = 0.011), language delay (P = 0.001), and any delays (P = 0.001). Our findings show that low nNO in newborns with CHD is significantly associated with impaired neurodevelopmental outcomes. These findings suggest nNO may serve as a biomarker to identify newborns with CHD who are at increased risk for adverse neurodevelopment. This may create opportunities for early intervention targeting a common factor underlying both low NO and worse neurodevelopmental outcomes.
Traditional pediatric electrophysiology (EP) procedures rely on fixed laboratory infrastructure with multiple hardwired systems which can limit procedural availability. This is the first reported use of a fully mobile EP system integrated with mixed reality (MxR) virtual displays to perform catheter ablation in a pediatric patient. In this case, a 9-year-old safely underwent successful radiofrequency ablation for atrioventricular nodal reentry tachycardia with the fully mobile MxR system. This experience demonstrates the feasibility of mobile, MxR-enabled EP systems to expand procedural access while maintaining clinical performance.
Editors of pediatric cardiology journals have an important role at the intersection of clinical science, education, ethics, and academic leadership. They help shape research standards, scholarly discussion, and the dissemination of evidence. Relatively little attention has been given to the challenges they face or to how the editorial workforce in pediatric cardiology is developed and supported. This perspective considers current challenges in pediatric cardiology publishing and practical approaches to strengthening editorial practice and future leadership. Editors must navigate increasing methodological complexity, difficulties recruiting appropriately qualified reviewers, and growing expectations around transparency and equity. They must also respond to rapid technological change while maintaining editorial independence within evolving publishing models and performance metrics. The specialized nature of pediatric and congenital cardiology creates additional challenges. Patient populations are often small, conditions may be rare, and clinical presentations can be heterogeneous. Research methods are also becoming more complex. Together, these factors can make the assessment of scientific quality, methodological rigor, and clinical relevance more difficult. Several practical approaches may help strengthen editorial practice. These include structured training and onboarding, mentorship and succession pathways, and reviewer development. Broader and more internationally representative editorial and reviewer networks are also important. Greater recognition and support for editorial work are needed, alongside the responsible integration of emerging technologies with appropriate human oversight. The field now needs to move beyond identifying editorial challenges toward developing practical and adaptable solutions. These should promote scientific rigor, fairness, efficiency, accessibility, and innovation. Strengthening the systems used to recruit, train, support, and develop editors will be important for maintaining the integrity, sustainability, and global relevance of pediatric cardiology publishing.
The diagnosis of long QT syndrome (LQTS) in the fetus is rare and can be difficult to manage. Fetuses most commonly present with sinus bradycardia but can develop more significant arrhythmias such as pseudo-atrioventricular block and ventricular tachycardia. Here we report the case of a fetus presenting with "pulseless" ventricular tachycardia in the setting of LQTS, including diagnostic strategies as well as fetal and postnatal management.
Pediatric pulmonary vein stenosis (PVS) is associated with high morbidity and mortality. We describe a case series of pediatric patients with advanced multivessel PVS who underwent cardiac catheterization with veno-arterial extracorporeal life support (VA-ECLS) to prevent cardiac arrest around high-risk pulmonary vein angioplasty and/or stent placement. VA-ECLS provided hemodynamic and respiratory stabilization, allowing completion of technically complex interventions in critically-ill pediatric patients who may not have otherwise tolerated catheterization. Using VA-ECLS in this setting requires a thorough assessment of risks versus benefits and specific patient criteria for consideration. This series highlights the potential role of VA-ECLS as an adjunctive strategy during high-risk catheter-based interventions for pediatric PVS to improve quality of life and lengthen lifespan.
Atrial septal defect (ASD) is considered a benign condition and is usually closed electively in pre-school age. Early symptomatic presentation is uncommon and often associated with comorbidities such as chronic lung disease and recurrent respiratory infections. Although pulmonary hypertension is rare in ASD, infants with large defects may develop respiratory symptoms and failure to thrive even in its absence. In such conditions earlier intervention may be considered, particularly in those with right heart volume overload or growth impairment. However, limited evidence suggests potential benefit from early intervention, especially in infants with bronchopulmonary dysplasia. This study aims to systematically review the literature and perform a pooled analysis of clinical and hemodynamic characteristics of children undergoing early ASD closure (weight < 15 kg or age < 2 years). Additionally, it evaluates the safety and efficacy of both surgical and transcatheter closure in this population.
Accuracy and reliability are important in grading aortic stenosis (AS) by echocardiogram. There are no pediatric-specific consensus guidelines for grading AS by echo. Our objective was to adopt a consensus reference standard for AS and evaluate resulting interrater reliability in a pediatric echo laboratory. A reference standard for grading AS was derived from published adult guidelines. Measurements for mean gradient and aortic valve area as determined by the continuity equation were included. Comparisons were made between AS grades on 20 historic echo reports and AS grades using the new reference standard. Additionally, 6 readers used the reference standard to grade 10 studies prospectively and interrater reliability was evaluated for measurements required for grading AS and overall AS grade. Twenty echo studies were included (median subject age 7 IQR 4–16 years, median weight 32.2 IQR 13.8–44.8 kg). AS grade on historic echo reports and AS grade using new reference standard were in agreement 85
