Background Adenosine is used as a diagnostic tool during electrophysiology studies to transiently block atrioventricular node (AVN) conduction. Persistence of VA conduction after adenosine administration is often interpreted as evidence of accessory pathway conduction. Studies in adults have shown that adenosine effect on retrograde AVN conduction is heterogeneous and there may be differing responses in those with dual AVN physiology. Objectives Our objective was to evaluate these phenomena in pediatric patients. Specifically, our goal was to assess retrograde AVN response to adenosine in pediatric patients. Methods We evaluated retrograde AVN response to adenosine in pediatric patients undergoing an electrophysiology study for supraventricular tachycardia or ventricular pre-excitation. Patients aged 4–25 years received intravenous adenosine (0.2 mg/kg, maximum 18 mg) during ventricular overdrive pacing. Results Of 58 analyzed patients, 30 had dual AVN physiology. Adenosine administration resulted in complete retrograde AVN block in 50% of patients with dual AVN physiology compared to 82% of those without dual AVN physiology (p = 0.01). Conclusion In children, continued retrograde AVN conduction after adenosine does not reliably indicate accessory pathway presence and does not rule out dual AVN physiology as the mechanism of tachycardia.
Background Pediatric hypertension has emerged as an increasingly prevalent public health challenge driven by rising childhood obesity, adverse perinatal exposures, metabolic dysfunction, and improved recognition. Elevated blood pressure (BP) during childhood is associated with early hypertension-mediated organ damage (HMOD) and strongly predicts adult hypertension and premature cardiovascular (CV) disease, highlighting the need for timely diagnosis, standardized evaluation, and lifelong risk reduction. Aim of review To provide a contemporary, clinically oriented review of pediatric hypertension by integrating recent evidence with recommendations from major pediatric and adult hypertension guidelines. This review emphasizes practical approaches to diagnosis, ambulatory BP monitoring (ABPM), risk stratification, differences across age spectrum, evaluation for secondary causes, hypertension-mediated organ damage, pharmacologic and non-pharmacologic management, and the transition from pediatric to adult CV care. Key scientific concepts of review Current pediatric hypertension management continues to rely on age-, sex-, and height-specific BP percentiles in children younger than 13 years, whereas adolescents increasingly adopt fixed adult thresholds, facilitating continuity of care. ABPM has become the preferred method for confirming hypertension, identifying white-coat and masked hypertension, assessing circadian BP patterns, and improving cardiovascular risk stratification. Management should follow a stepwise, individualized approach beginning with lifestyle modification and progressing, when indicated, to guideline-directed pharmacotherapy using angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, long-acting calcium channel blockers, and/or thiazide diuretics. This review also presents practical diagnostic algorithms, summarizes current referral pathways and evaluation strategies, and discusses emerging advances, including telemedicine, wearable BP technologies, artificial intelligence, and precision medicine that are likely to reshape future pediatric hypertension care. Early recognition, harmonized management across the pediatric-adult continuum, and multidisciplinary care remain essential to reducing HMOD and preventing lifelong CV disease.
Background The co-occurrence of congenital diaphragmatic hernia (CDH) and pulmonary atresia with ventricular septal defect (PA-VSD) is exceptionally rare and creates a physiologically hostile neonatal condition characterized by pulmonary hypoplasia, persistent pulmonary hypertension of the newborn (PPHN), and ductal-dependent pulmonary blood flow. No prior published case of CDH combined with PA-VSD has been identified in a systematic literature review, and no management consensus exists, particularly for centers without extracorporeal membrane oxygenation (ECMO). Case presentation We report two neonates with left-sided CDH and PA-VSD admitted to a tertiary NICU in Ho Chi Minh City, Vietnam. Both had severe cardiorespiratory instability at birth and underwent early echocardiographic diagnosis, immediate prostaglandin E1 (PGE1) infusion, and multidisciplinary hemodynamic assessment. Case 1 (male, 38 + 1 weeks, 2400 g) underwent delayed CDH repair on day of life (DOL) 15 and elective PDA stenting on DOL 29, followed by prolonged ventilator dependence attributable to unrecognized tracheobronchial anomalies. Case 2 (female, 37 + 3 weeks, 2000 g) underwent CDH repair on DOL 8 and urgent PDA stenting on DOL 9 after post-operative ductal re-narrowing caused acute cyanotic decompensation. Outcomes Both infants survived to hospital discharge without ECMO. Case 1 was discharged on DOL 144 on supplemental oxygen and antiepileptic therapy following a fourth successful extubation attempt. Case 2 was discharged on DOL 61 breathing room air with adequate feeding and weight gain. Conclusion These cases, representing the first published documented survival of CDH combined with PA-VSD, support a staged management strategy: early echocardiographic recognition, PGE1-maintained ductal stabilization, delayed CDH repair after predefined hemodynamic targets, and timely PDA stenting when pulmonary blood flow becomes inadequate after repair. Tracheobronchial anomalies should be actively sought in CDH neonates with repeated extubation failure. Survival without ECMO is achievable in experienced tertiary centers with coordinated multidisciplinary management.
Background Fragmented QRS (fQRS) is a myocardial conduction abnormality reflecting fibrosis and has been investigated in adults as a predictor of adverse cardiovascular outcomes. However, its prognostic importance in children remains unclear. Aim of review This systematic review aimed to evaluate the association between fQRS and adverse outcomes in pediatric patients with heart diseases. Key scientific concepts of review A search of PubMed, Web of Science, EMBASE, Scopus, and the Cochrane Library was performed up to June 2024, using predefined keywords. Included studies were observational and enrolled participants younger than 20 years with isolated cardiac disease, assessing fQRS on electrocardiography in relation to outcomes including ventricular dysfunction, arrhythmias, or major adverse cardiovascular events (MACE). The risk of bias in the included studies was assessed using the Newcastle–Ottawa checklists, and the study was designed based on the PRISMA guidelines. (PROSPERO: CRD420250656353).From 5694 studies, nine comprising 625 pediatric patients were included. Populations consisted of repaired Tetralogy of Fallot (rTOF), dilated cardiomyopathy with or without left ventricular noncompaction, and congenitally corrected transposition of the great arteries. In rTOF, fQRS was consistently associated with right ventricular structural and functional impairment, including reduced RVEF and increased RVEDVi and RVESVi, whereas correlations with left ventricular parameters were inconsistent in pediatric patients with cardiomyopathy. Evidence for arrhythmias was variable, though two studies identified links between fQRS and ventricular arrhythmias in patients with left ventricular noncompaction. Available evidence also suggested a possible association between fQRS and MACE in pediatric DCM, although methodological heterogeneity and the limited number of studies warrant cautious interpretation.
Major aortopulmonary collateral arteries (MAPCAs) are commonly seen in cyanotic congenital heart disease as anomalous systemic vessels supplying pulmonary parenchyma. We describe a neonate with Trisomy 21 and a large inlet ventricular septal defect (VSD) who developed early tachypnea and heart failure. Airway evaluation and contrast-enhanced CT revealed an unsuspected large MAPCA arising from the celiac trunk to the right lower lobe that was not seen using standardized echocardiographic technique. Cardiac catheterization confirmed a hemodynamically significant dual-supplying MAPCA, prompting coil embolization to reduce pulmonary overcirculation and simplify surgical repair. This case highlights the potential value of cross-sectional angiography for identifying occult MAPCAs in acyanotic infants with congenital heart disease and refractory heart failure symptoms.
In behavioral economics, a “free roll” describes a decision with potential gain and no meaningful downside risk. In rare, extreme-risk congenital cardiac cases, families may perceive intervention as a clinical analog of a free roll because mortality is certain without treatment, while survival remains possible with it. For clinicians, however, attempting the same intervention is not without downside: publicly visible outcome reporting assigns procedural deaths to surgical programs, creating professional and institutional consequences even in near-certain-fatality cases. Differences in reporting visibility across procedural domains create asymmetric downside exposure: high-risk interventions may carry institutional penalty in the surgical domain but function as effective free rolls in the catheterization domain. These structural pressures interact with predictable features of human judgment in both families and clinicians, amplifying their influence at the margins. This Perspective examines how reporting architecture shapes decision environments in near-certain-fatality scenarios and proposes targeted structural refinements, such as compassionate-use designations, contextualized reporting, and parallel learning registries, to preserve transparency while supporting patient-centered care for the sickest children.
Background Gerbode defect is a rare cardiac anomaly characterized by abnormal communication between the left ventricle (LV) and right atrium (RA). Indirect Gerbode defects occur due to a perimembranous ventricular septal defect (VSD) associated with secondary tricuspid valve abnormalities, often leading to significant tricuspid regurgitation (TR). Surgical repair has traditionally been the standard treatment; however, transcatheter approaches have emerged as a less invasive alternative. Objectives To evaluate the safety, feasibility, and intermediate-term outcomes of transcatheter closure of indirect Gerbode defects using the Konar-Multifunctional Occluder (Konar-MFO). Methods This retrospective observational study included 18 patients with indirect Gerbode defects associated with significant TR who underwent transcatheter closure between January 2022 and December 2024. Patients were selected based on clinical and echocardiographic criteria, including moderate TR and right ventricular volume overload or standard indications for VSD closure according to Koneti criteria. All procedures were performed using an antegrade approach with deployment of the Konar-MFO device under fluoroscopic and echocardiographic guidance. Procedural success, complications, and echocardiographic outcomes were assessed during follow-up. Results Eighteen patients (median age 7.5 years; equal male-to-female distribution) underwent attempted device closure. Technical success was achieved in 100% of cases. Post-procedural echocardiography demonstrated complete or near-complete elimination of the shunt with significant reduction in TR in all patients. There were no major complications, including heart block, device embolization. One patient had new onset trivial aortic regurgitation. At a median follow-up of six months, all patients remained asymptomatic with sustained improvement in echocardiographic parameters. Conclusion Transcatheter closure of congenital indirect Gerbode defects with the Konar-MFO device is safe and effective, providing excellent hemodynamic outcomes.
Background: Thrombosis is a critical complication in children with congenital heart disease (CHD), contributing to significant perioperative morbidity and mortality. While the risk is recognized across CHD, the specific incidence and predictors of thrombosis in patients with transposition of the great arteries (TGA) remain poorly defined. Objectives: This study aimed to determine the incidence of perioperative thrombosis in children undergoing corrective cardiac surgery and to compare the risk between patients with and without TGA. Methods: We conducted a retrospective cohort study of 217 consecutive children who underwent corrective openheart surgery for biventricular repair using cardiopulmonary bypass (CPB) between January 2016 and December 2022. The cohort was divided into patients with TGA (n = 111, 51%) and a comparative group with other forms of CHD (non-TGA, n = 106, 49%). The primary outcome was the incidence of clinically diagnosed thrombosis in the perioperative period. Results: The overall incidence of thrombosis was 10% (22/217). The incidence was significantly higher in the TGA group at 16.2% (18/111) compared to 3.7% (4/106) in the non-TGA group (p = 0.003; relative risk = 4.3). Multivariable analysis identified TGA as a strong independent predictor of thrombosis (Odds Ratio: 11.71; p < 0.001). Thrombosis occurred in 14/72 (19%) patients with TGA and an intact interventricular septum compared to 4/39 (10%) TGA patients with a ventricular septal defect. Sixty-five (58%) patients with TGA underwent balloon atrial septostomy; 18 (27%) developed thrombosis, whereas none of those who did not undergo BAS developed thrombosis (p < 0.001). Conclusion: Children with TGA have a substantially increased risk of perioperative thrombosis compared to children with other forms of congenital heart disease undergoing biventricular repair. Balloon atrial septostomy could be a major contributing factor to this elevated risk, although it also reflects severe preoperative illness that independently predisposes to thrombosis. These findings highlight the need for heightened vigilance and suggest that prophylactic strategies may be warranted in this vulnerable population, particularly in the context of BAS.
Background: Despite the increased use of guideline directed medical therapy (GDMT) (defined as renin angiotensin aldosterone system inhibitor, beta blocker, mineralocorticoid receptor antagonist, and sodium-glucose cotransporter-2 inhibitors) in adult heart failure, there is no consensus regarding their use in adults with single ventricular failure, and pediatric data is scarce. Objectives: This was a single center observational study to evaluate the feasibility of GDMT in children with single ventricular dysfunction. Methods: Consecutive patients aged <= 19 years with single ventricular physiology at various stages of surgical palliation, with ejection fraction <= 50% and received at least three of the four pillars of GDMT for more than thirty consecutive days, were considered for inclusion in this study. Results: Ten consecutive children constituted the study cohort. The primary composite outcome of worsening heart failure, heart transplantation, and mortality was identified in 40% of patients at 1-year follow-up. The median ejection fraction improved from 32% to 50%, and the cardiothoracic ratio reduced from 59 to 55%, predominantly in those with left ventricular morphology. The NT-proBNP levels reduced from 5530 to 1400 pg/ ml. The patients demonstrated a mild decrease in systolic blood pressure on follow-up but remained above the 15th centile. The serum creatinine levels and estimated glomerular filtration rate showed a biphasic pattern with an initial worsening followed by normalization to baseline levels at 1-year follow-up with continued GDMT. Conclusions: The improvements in ejection fraction observed in children with single ventricle physiology and ventricular dysfunction with quadruple GDMT suggest the need for larger prospective trials in children.
Background: Prenatal diagnosis (PND) of congenital heart disease (CHD) optimizes perinatal management and improves preoperative status; with survival benefit demonstrated for select lesions. Despite advances in fetal imaging, PND rates remain variable, with persistent disparities linked to socioeconomic, demographic, and geographic factors. However, these variables and their combined impact within a regional care network encompassing a large area of the Northwest United States have not been previously examined. Objective: Evaluate the association between socioeconomic and geographic factors-including race, ethnicity, Area Deprivation Index (ADI), and distance from a regional surgical center-and PND of significant CHD, and to compare postoperative outcomes by PND status. Methods: This single-center cross-sectional study included all patients with significant CHD requiring surgery within the first year of life and diagnosed between 2014 and 2022 at a regional referral center in the Northwestern United States. Socioeconomic and geographic variables were extracted, including race, ethnicity, insurance status, and address, which were used to determine state and national ADI rankings and distance to the surgical center. Postoperative outcomes were obtained. Group comparisons used nonparametric and categorical analyses. Results: Among 929 patients, 736 (79.2%) were diagnosed prenatally. Higher state-level ADI (p < 0.001), non-White race (p = 0.003), Hispanic ethnicity (p = 0.03), and greater distance from the surgical center (p = 0.009) were independently associated with lower PND rates. Prenatally diagnosed patients had higher 30-day mortality (p = 0.003) and reintubation rates (p < 0.001), reflecting increased disease severity. Conclusion: Socioeconomic disadvantage, non-White race, Hispanic ethnicity, and greater geographic distance were associated with lower PND rates. Mapping PND alongside regional deprivation indices may help identify underserved areas and guide targeted interventions.
Interrupted aortic arch (IAA) is a rare, life-threatening congenital cardiac anomaly characterized by complete discontinuity between the ascending and descending aorta. Neonates depend on ductal flow for systemic perfusion and face high mortality without early intervention. Surgical repair is standard, but critically ill infants may not tolerate immediate surgery. We report a 2.9-kg term female neonate with IAA type A, ventricular septal defect, and duct-dependent systemic circulation who developed sepsis, necrotizing enterocolitis, pulmonary hemorrhage, and shock. On day 16 of life, percutaneous recanalization of the interrupted aortic segment was performed. A chronic total occlusion wire was used to cross the atretic segment, followed by deployment and dilation of a 4.8 × 16 mm coronary covered stent. Angiography confirmed restoration of antegrade flow to the descending aorta. The procedure was well tolerated and led to rapid clinical improvement, extubation, and discontinuation of inotropes.
Background Patent ductus arteriosus (PDA) is common in preterm infants and may lead to significant hemodynamic and respiratory complications. Despite the typical use of pharmacological treatments, invasive interventions such as surgical ligation or percutaneous closure are sometimes required. This study evaluated the role of lung ultrasound score (LUS) in monitoring hemodynamic changes following PDA closure. Objective To characterize peri-procedural cardiopulmonary changes following closure of hemodynamically significant patent ductus arteriosus (hsPDA), with particular focus on lung ultrasound score (LUS) dynamics, and to explore its association with post-ligation cardiac syndrome (PLCS). Methods In this prospective longitudinal study (2013–2024) at a tertiary referral center, preterm infants with hsPDA were assessed. Clinical and echocardiographic data were collected at predefined time points. Pulmonary congestion was quantified using LUS and analyzed alongside stroke volume (SV) and additional hemodynamic parameters. Results Twenty infants (median gestational age: 27 weeks) were included. LUS significantly decreased over time, indicating improved pulmonary congestion. Blood pressure and SV also changed significantly: mean arterial and systolic pressures increased 24 h post-closure, while SV decreased initially (first 6 h) before rising subsequently. In infants developing post-ligation cardiac syndrome (PLCS), echocardiographic parameters remained similar, but LUS variations were more pronounced, suggesting a potential association with early PLCS development. Conclusions LUS is a valuable tool for continuous monitoring of preterm infants after PDA closure, supporting its role as a bedside monitoring tool for peri-procedural cardiopulmonary changes.
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a very rare congenital defect with a high mortality rate (90%) that presents mostly during infancy with ischemia, arrhythmia, and ventricular dysfunction. However, extensive collateral circulation from the right coronary artery may lead to survival into adulthood (Blickenstaff et al., 2023). We report a case of an adult survivor of ALCAPA with a delay in this patient's diagnosis due to suboptimal imaging acquisition on initial Compute Tomography (CT) Coronary Angiogram (CA). We also focus on the concept of diagnostic error, a relevant but under-recognized area of medicine.
Background Cardiometabolic risk in children and adolescents is an increasing global concern, driven by rising rates of obesity, physical inactivity, and unhealthy lifestyle behaviors. Early alterations such as insulin resistance, dyslipidemia, elevated blood pressure, and low-grade inflammation may develop during childhood and persist into adulthood, contributing to premature cardiovascular disease. Identifying simple, accessible, and reliable markers for early risk detection is therefore a priority in pediatric clinical practice and prevention strategies, particularly in diverse healthcare settings. Aim of review To synthesize the available evidence on the clinical utility of handgrip strength (HGS) in relation to cardiometabolic risk in children and adolescents. Key scientific concepts of review Available evidence indicates that lower HGS is observed in relation to less favorable cardiometabolic profiles across different pediatric populations, including higher adiposity, elevated blood pressure, unfavorable lipid profiles, insulin resistance, and inflammatory markers. Relative HGS, adjusted for body size, is frequently reported as a more informative measure than absolute strength, although different normalization strategies have been used. Considerable heterogeneity is evident in measurement protocols, dynamometry procedures, normalization methods, and population characteristics, which limits comparability across studies and the interpretation of findings. The relationship between HGS and cardiometabolic variables appears to be influenced by body composition, particularly the balance between lean and fat mass, and may vary according to population characteristics. Most of the available evidence is based on observational designs, predominantly cross-sectional, which limits causal inference. Although HGS represents a simple, low-cost, and non-invasive measure, the absence of standardized protocols, normative values, and validated cut-off points currently limits its use in routine clinical practice. Overall, HGS may represent a potential complementary measure for early identification and risk stratification, although further standardization and longitudinal studies are needed to clarify its clinical utility.
Background Many US children have congenital heart defects (CHD), yet data on their long-term outcomes have limitations. The Congenital Heart Survey To Recognize Outcomes, Needs, and well-beinG of KIDS (CHSTRONG KIDS) addresses these gaps by surveying caregivers of children with CHD identified through birth defect surveillance systems [BDSS]. Objectives To describe the CHSTRONG KIDS project design, characteristics of the eligible population, and the percentage not up-to-date on recommended cardiology care. Methods Children born 2006–2021 with CHD were identified using active, population-based BDSS in Atlanta, Georgia, Massachusetts, and Minnesota and linked to vital records. Caregivers of eligible (living) children with CHD were invited to complete surveys in 2024–2025. Characteristics were compared by site and response status using χ2 tests. We also estimated percentages of children in CHSTRONG KIDS who had not seen a cardiologist within the guideline-recommended timeframe for their specific defects. Results Among 7239 identified children with CHD, 6240 were eligible for survey recruitment. Of those,1841 (30%) had caregiver-reported survey data. Several characteristics, including CHD severity, birth year, and Trisomy 21 diagnosis, varied by site (p < 0.05). Survey response rates differed by site, CHD severity, maternal race, maternal education, and rurality (p < 0.05), prompting development of post-stratification weights. A weighted 21.6% were not up-to-date on their cardiology care. Conclusion With data on >7200 CHD cases and >1800 caregiver-reported surveys, CHSTRONG KIDS provides a population-based view of long-term outcomes among children with CHD. Notably, one in five were not up-to-date on their cardiology care and therefore may not be represented in clinical cohorts.
Background Neutrophil-to-lymphocyte ratio (NLR) has been studied as a prognostic marker in adults with coronary artery disease following coronary artery bypass graft surgery. We sought to evaluate the utility of this metric in pediatric patients undergoing cardiac surgery. Objective The aim of this study was to evaluate the association between preoperative NLR value and adverse outcomes in infants with single ventricle physiology undergoing Glenn procedure. Methods A retrospective cohort study included infants who underwent Glenn procedure between 2019 and 2025. NLR within two weeks prior to the operation was recorded. A composite adverse clinical outcome was defined as an occurrence of the need for extracorporeal mechanical support, cardiac arrest, Glenn takedown, transplant, or death. Results A total of 99 patients (158 days, 67% male, 48% Caucasian) were reviewed. The composite adverse outcome occurred in 11 patients, including 6 deaths. Patients with adverse outcomes had a higher rate of moderate atrioventricular, sepsis, and chylous effusion (p < 0.05). The median NLR was1.01 (0.51–2.21) among patients with adverse outcomes and 0.68 (0.37–1.02) among patients without adverse outcomes (p = 0.06). The optimal cut point for NLR was 0.89 with sensitivity of 64% (95% CI, 30.8–89.1) and specificity of 70% (95%CI, 58.6–78.7). The ROC area under the curve was 0.66 (95%CI, 0.49–0.85). The association between preoperative NLR > 0.89 and adverse outcomes was statistically significant after controlling for moderate atrioventricular regurgitation, sepsis, and chylous effusion, adjusted odds ratio 2.47 (95% CI 1.11–5.49), p 0.026. Conclusion Elevated preoperative NLR value above 0.89 was associated with a higher risk of adverse outcomes. Further studies are warranted to determine whether treatment targeting inflammation can prevent such outcomes.
Patients with single ventricle heart disease require a series of palliative surgeries, culminating in the Fontan, which results in passive pulmonary arterial blood flow after direct connection of the venae cavae to the pulmonary arteries [1]. Fontan circulation leads to complications impacting multiple organ systems; however, cancers outside of hepatocellular carcinoma are not reported frequently [1].Anthracycline-based chemotherapeutic regimens, frequently used to treat pediatric cancers, carry a significant dose-dependent risk of cardiotoxicity. Protocols require monitoring of left ventricular ejection fraction to detect declines in cardiac function and guide treatment.Monitoring cardiac function in Fontan patients with systemic right ventricle presents unique challenges due to limitations of echocardiography to assess right ventricular systolic function, lack of established values for quantitative measures of systemic right ventricular function, and limited applicability of heart failure regimens for systemic right ventricles [1]. Cardiac magnetic resonance imaging and computed tomography can better quantitate right ventricular systolic function; however, these modalities are limited by ferromagnetic artifacts from prior interventions and contraindicated with renal dysfunction, respectively [2].We present a patient with Fontan physiology and systemic right ventricle, diagnosed with acute myeloid leukemia (AML) requiring multi-disciplinary management with a goal of curative treatment of AML while minimizing cardiac and nephrotoxicity.
