Pediatr Cardiol [JOURNAL]
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200 papers
Comparison of Near-Infrared Spectroscopy and Traditional Parameters for Monitoring Neonates with Aortic Coarctation.
Sharp DP, Kochanski J, Lee S … +4 more , Weigel N, Maskatia SA, Bhombal S, Chock VY
Pediatr Cardiol · 2026 Feb · PMID 41653324 · Publisher ↗
In neonates with aortic coarctation (CoA), near-infrared spectroscopy (NIRS) may be utilized to asses for impaired end-organ perfusion. To test the utility of Cerebral (CSat) and renal (RSat) oxygen saturations compared...
In neonates with aortic coarctation (CoA), near-infrared spectroscopy (NIRS) may be utilized to asses for impaired end-organ perfusion. To test the utility of Cerebral (CSat) and renal (RSat) oxygen saturations compared to traditional parameters in determining the need for surgical intervention in neonates with significant risk for CoA. This single-center, retrospective cohort study included newborns > 34 weeks gestation with fetal echocardiographic diagnosis of moderate or high-risk CoA admitted to the neonatal intensive care unit under an institutional arch watch protocol. NIRS and traditional monitoring values (pre- and post-ductal oxygen saturation, 4-extremity blood pressure, urine output, blood gas, and lactate) were measured for seven days. Fifty patients met the inclusion criteria, and 16 required surgical repair. Univariate analysis showed no significant difference between surgical and non-surgical patients for pre- and post-ductal saturations, arterial blood pH, or base deficit. CSat and RSat were significantly lower in surgical patients on days 2–6 and 1, 2, and 4, respectively. ROC analysis of NIRS measures on day 3 showed AUC of 0.77 for surgical repair. CSat and RSat values were significantly decreased in infants with CoA requiring surgery. The addition of NIRS monitoring may better identify patients who require surgical intervention than traditional monitoring alone.
Augmenting Venous Return in Fontan Circulation: The Role of the Skeletal Muscle Pump and Implications for Mechanical Compression Devices-A Review of the Literature.
Balaji A, Makam R, Balaji A … +6 more , Bocchetta N, Nadeem M, Azam A, Sherif M, Hussein N, Loubani M
Pediatr Cardiol · 2026 Feb · PMID 41649542 · Publisher ↗
Post-Fontan patients often face significant hemodynamic challenges, including systolic dysfunction and diminished cardiac output due to reduced preload and venous return. Long-term complications such as hepatic dysfuncti...
Post-Fontan patients often face significant hemodynamic challenges, including systolic dysfunction and diminished cardiac output due to reduced preload and venous return. Long-term complications such as hepatic dysfunction and protein-losing enteropathy arise from poor venous return, increasing morbidity and mortality. Enhancing venous return could potentially improve long-term outcomes for these patients. This literature review examines the role of the skeletal muscle pump in Fontan circulation and evaluates non-pharmacological strategies to augment venous return, with discussion of mechanical compression devices as a potential translational application. A comprehensive literature search identified 26 studies focusing on the relationship between lower limb muscle mass, venous return, and cardiac performance in post-Fontan patients, as well as the efficacy of peristaltic leg pumps in enhancing venous return. Across included studies, higher lower-limb skeletal muscle mass and structured exercise interventions were consistently associated with improved functional and cardiopulmonary performance metrics in Fontan patients. Additionally, the use of peristaltic leg pumps in other patient cohorts enhanced venous return across various patient positions, suggesting an avenue for implementation in the Fontan patient. Peristaltic leg pumps may serve as a valuable tool in managing the hemodynamic challenges of post-Fontan patients, potentially leading to better long-term outcomes. Further research, particularly randomized controlled trials, is necessary to establish the efficacy and optimal application of these devices in the paediatric Fontan population.
Ductal Stenting Versus Aorto-Pulmonary Shunt for Lesions with Duct-Dependent Pulmonary Blood Flow: A Systematic Review and Meta-analysis.
Elhedai H, Mohamedahmed AY, Mohamed S … +4 more , Mohammed SS, Shebani S, Bu'Lock F, Kantzis M
Pediatr Cardiol · 2026 Feb · PMID 41627405 · Publisher ↗
To compare clinical outcomes of ductal stenting (DS) with modified Blalock–Taussig–Thomas shunt (BTS) as initial palliation of neonates with duct-dependent pulmonary blood flow (PBF) lesions. This systematic review and m...
To compare clinical outcomes of ductal stenting (DS) with modified Blalock–Taussig–Thomas shunt (BTS) as initial palliation of neonates with duct-dependent pulmonary blood flow (PBF) lesions. This systematic review and meta-analysis was designed and carried out in accordance with the Cochrane Handbook for Systematic Reviews of Interventions and followed the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines for reporting. The trial strategy was registered in PROSPERO (CRD420251034196). Eleven studies had a total of 1348 patients, divided between 533 in the DS group and 815 in the BTS group. The DS group showed significantly lower early and overall mortality rate compared to the BTS group [[5.3% in the DS group versus 8.9% in the BTS group, OR 0.51 (0.28, 0.93) 95% CI, P = 0.03] and [7.6% in DS group versus 14.4% in BTS group, OR 0.50 (0.33, 0.76) 95% CI, P = 0.001], respectively. The DS group also had a significantly lower post-procedure complications rate [16.1% in the DS group versus 21.3% in the BTS group, OR 0.50 (0.35, 0.69) 95% CI, P = 0.0001]. Additionally, the DS group was superior in terms of post-procedure oxygen saturation [MD 1.89 (1.52, 2.27) 95% CI, P = 0.00001] and hospital and Intensive care unit (ICU) length of stay (LOS) [MD − 6.69 (− 9.09, − 4.28) 95% CI, P = 0.00001], [MD − 4.80 (− 6.01, − 3.18), P = 0.00001]. There was no significant difference between the two groups regarding early and late interventions, the need for extra-corporeal membrane oxygenation (ECMO) and post-procedure pulmonary artery (PA) growth and symmetry. DS is associated with a significant reduction in both early and late mortalities, shorter hospital and ICU LOS and fewer procedural complications compared to the BTS group. There is higher rate of late re-interventions in the DS group but no significant difference in need for ECMO or PA growth between the two groups.
Endovascular Occlusion of an External Iliac Artery Rupture as an Alternative to Open Vascular Surgery.
Parsons S, Mireskandari M, Fraisse A … +2 more , Spada M, Bautista-Rodriguez C
Pediatr Cardiol · 2026 Jan · PMID 41618996 · Publisher ↗
A 5-month-old (5.7 kg) infant with trisomy 21 suffered an external iliac artery (EIA) rupture following femoral arterial access for attempted percutaneous retrograde ventricular septal defect (VSD) closure. An endovascul...
A 5-month-old (5.7 kg) infant with trisomy 21 suffered an external iliac artery (EIA) rupture following femoral arterial access for attempted percutaneous retrograde ventricular septal defect (VSD) closure. An endovascular occlusion approach was employed as an alternative to high-risk open vascular surgery. The infant experienced a cardiac arrest during anaesthetic induction, with angiography revealing active EIA bleeding. The vessel was successfully occluded with an Amplatzer Piccolo device, with distal perfusion likely preserved with collateralisation via the internal iliac artery. Recovery was uneventful, with good perfusion of the right leg, though at 4-month follow-up, there is a mild limb-length discrepancy. To our knowledge, this is the first reported case of emergency endovascular EIA occlusion in an infant following cardiac catheterisation as an alternative to open surgery.
High BMI Associated with Lower Child Opportunity Index Negatively Impacts Exercise Capacity in Fontan Patients.
Thompson Z, Bhat DP, Williams S … +1 more , Sabati A
Pediatr Cardiol · 2026 Jan · PMID 41618995 · Publisher ↗
Cardiopulmonary exercise testing (CPET) is a valuable prognostic tool in patients with Fontan physiology, yet the impact of social determinants of health – such as the Childhood Opportunity Index (COI) – on exercise capa...
Cardiopulmonary exercise testing (CPET) is a valuable prognostic tool in patients with Fontan physiology, yet the impact of social determinants of health – such as the Childhood Opportunity Index (COI) – on exercise capacity (VO2/kg) remains unclear. We evaluate the relationship between COI and predicted VO2/kg in a pediatric Fontan cohort. We performed a retrospective review of 158 Fontan patients who completed a clinically indicated CPET. CPET parameters were expressed as a percent of predicted value using standardized equations. COI quintile (1 = lowest opportunity, 5 = highest) was assigned based on home address (diversitydatakids.org). The association between COI and VO2/kg was assessed using a Spearman rank correlation(ρ). Group comparisons were performed for lower performers (VO2/kg < 60% predicted) vs. higher performers (VO2/kg ≥ 60% predicted). Patients in COI groups were compared using univariate and multivariate analysis to explore the independent predictors of exercise capacity. Patients in higher opportunity groups (COI 4–5, n = 51) had higher VO2/kg compared to the lowest opportunity group (COI 1, n = 54) (57.7 ± 2.0 vs. 51.5 ± 1.5, p = 0.01). Across COI quintiles, there was an association with VO2/kg (Spearman ρ = 0.191, p = 0.016; bootstrap 95% CI 0.041–0.335). Lower performers (VO2/kg < 60% predicted) were older (14.7 vs. 13.0 years, p = < 0.001) and had higher BMI (22.4 vs. 17.8, p < 0.001). BMI had a inverse relationship with COI (Spearman ρ=−0.319, p < 0.001; bootstrap 95% CI − 0.45 to − 0.18). In multivariate analysis BMI > 20 (OR 6.5; 2.1–19.7) along with systemic right ventricle morphology (OR = 1.8, 1.1–2.9) were both independent predictors of low VO2/kg. In this single center cohort of 158 patients with Fontan physiology, higher BMI and systemic right ventricular morphology both independently predicted lower VO2/kg. Lower neighborhood opportunity, as measured by lower COI, was associated with reduced VO2/kg and higher BMI but was not an independent predictor after adjustment, suggesting an indirect effect of SDOH on VO2/kg possibly through BMI.
Neighborhood Socioeconomic & Geographic Determinants of Prenatal Congenital Heart Disease Diagnosis.
Tran MM, Schiff MD, Thoma FW … +3 more , Roberts FW, Mazzocco S, Dobson CP
Pediatr Cardiol · 2026 Jan · PMID 41609844 · Publisher ↗
Prenatal detection of congenital heart disease (CHD) reduces neonatal morbidity and mortality. Disparities in access to fetal echocardiography persist across socioeconomic status, rurality, insurance, race, and ethnicity...
Prenatal detection of congenital heart disease (CHD) reduces neonatal morbidity and mortality. Disparities in access to fetal echocardiography persist across socioeconomic status, rurality, insurance, race, and ethnicity. To assess whether neighborhood-level socioeconomic and environmental factors influence the likelihood of receiving a prenatal echocardiogram among infants with CHD in Western Pennsylvania. Retrospective, single-center cohort study (2010-2024) of mother-infant dyads where infants had CHD requiring surgical or catheter intervention within 6 months after birth. Exclusions included only minor anomalies, inability to link dyads, intervention after 6 months, or missing neighborhood data. Maternal variables included demographics, diabetes status, insurance type, and neighborhood-level characteristics (Area Deprivation Index, Rural-Urban Commuting Area codes, Walk Score). The primary outcome was receipt of a prenatal echocardiogram within 6 months before delivery. Among 1,092 dyads, 545 (49.9%) received a prenatal echocardiogram. In unadjusted analysis, those who received a prenatal echocardiogram were more likely to live in highly-deprived neighborhoods (38.7% vs. 28.7%), have Black mothers (15.8% vs. 8.2%), and have mothers with diabetes (9.7% vs. 3.9%). After adjusting for maternal diabetes, neighborhood deprivation was no longer significantly associated with receiving a prenatal echocardiogram. Maternal diabetes remained a strong independent predictor (OR = 2.60, 95% CI: 1.29-5.20). Rurality, Walk Score, and insurance type were not associated with odds of receiving a prenatal echocardiogram. Maternal diabetes, rather than neighborhood factors, predicted prenatal echocardiogram receipt. Only half of significant CHD cases had a prenatal echocardiogram, highlighting room for improvement. Integrating clinical and neighborhood data can help distinguish true disparities from differences driven by clinical risk.
Pediatric Cardiology Training and Certification in Latin America and the Caribbean: A Cross-Sectional Regional Survey of Current Practice.
de Campos CV, Pignatelli RH, Larrazabal A … +4 more , Srivastava S, Hasan B, Lai W, McMahon CJ
Pediatr Cardiol · 2026 Jan · PMID 41609843 · Publisher ↗
Developing high-quality congenital and pediatric cardiology training programs across Latin America and the Caribbean remains an ongoing challenge. Regional variations in resources, institutional infrastructure, and accre...
Developing high-quality congenital and pediatric cardiology training programs across Latin America and the Caribbean remains an ongoing challenge. Regional variations in resources, institutional infrastructure, and accreditation processes have not been systematically studied. Empowering Hearts Globally (EHG) is an international consortium supporting CHD training in low- and middle-income countries LMICs. A regional survey was distributed to clinicians involved in CHD care across Latin America and the Caribbean. Responses were analysed at the country level, using one data point per country to avoid clustering bias and included aggregated data on disease burden, urgency of intervention, and care resources. We also performed a secondary desk-based verification using publicly available government, university, and hospital sources to corroborate country-level training status. There were 32 respondents (44 invited, 73% response rate) from 17 countries. Formal accredited paediatric cardiology training programmes were reported by 11 of 17 countries (65%), while formal but non-accredited programmes were present in 2 (12%), and no formal training pathways were reported in 4 (23%). Most programmes trained a small number of fellows (median 2 per year) over a median 2.5 years (range 2–3.5 years). National recognition or certification of training existed in 13 of 17 countries (77%). CHD care was provided within public (82%), private (82%), and mixed (65%) institutional models, with children’s hospital cardiac units reported in 53% of countries. Paediatric cardiology training across Latin America and the Caribbean remains highly heterogeneous. Although over half of countries report formal accredited programmes, nearly one-third lack structured training opportunities. The EHG framework aims to provide a scalable pathway to standardize and strengthen pediatric cardiology education regionally.
Lifetime Earnings in Pediatric Cardiology: A Net Present Value Analysis of Academic and Private Practice Pathways.
Almasri M, Ehsan L, Masri AF … +4 more , Ayyad M, Zaghw A, Joshi K, Daily J
Pediatr Cardiol · 2026 Jan · PMID 41609842 · Publisher ↗
Pediatric cardiology requires extensive training, yet the long-term financial implications across academic subspecialties and private practice remain poorly characterized. A clearer understanding of these differences is...
Pediatric cardiology requires extensive training, yet the long-term financial implications across academic subspecialties and private practice remain poorly characterized. A clearer understanding of these differences is essential for informed career decision-making and institutional workforce planning. We used a net present value (NPV) framework to model lifetime earnings for pediatric cardiologists across three academic subspecialties (diagnostic, cardiac intensive care, and interventional) under five promotion trajectories and compared them with private practice. Compensation data were sourced from the Association of Academic Administrators in Pediatrics, the Association of American Medical Colleges, and the Medical Group Management Association. Monte Carlo simulations (10,000 iterations) and sensitivity analyses accounted for variation in salary percentile, discount rate, and career length. Lifetime earnings were substantial across all pediatric cardiology career pathways, with NPVs exceeding $7 million in nearly all modeled scenarios. Interventional cardiology yielded the highest NPV at the 50th percentile under a typical academic promotion trajectory ($7.99 million), followed by cardiac intensive care ($7.76 million) and diagnostic cardiology ($7.00 million). Private practice produced an NPV of $7.08 million at the 50th percentile, with a ramp-up model increasing this to $7.30 million; still below interventional and CICU academic tracks. Academic earnings increased by up to $2.44 million through early promotion compared to no promotion, and by up to $1.5 million through leadership roles, depending on subspecialty. Salary percentile was the most influential driver of NPV; interventional cardiology at the 90th percentile exceeded $10.4 million, and private practice reached $10.76 million. Private practice exhibited the widest range of lifetime earnings. Pediatric cardiologists, particularly those in interventional subspecialties or academic leadership; achieve substantial lifetime earnings. At the 50th percentile, academic and private practice careers offer comparable financial outcomes, but private practice shows greater variability. Optimizing academic career pathways through early promotion, high-percentile salaries, or leadership roles can match or exceed private practice earnings. These findings provide financial context that may inform trainee deliberation and institutional discussions regarding recruitment, retention, and compensation equity in pediatric cardiology.
The Evolving Role of Corticosteroids in Kawasaki Disease: Evidence, Mechanisms, and Clinical Implications.
Nguyen PS, Tran HMD
Pediatr Cardiol · 2026 Jan · PMID 41603901 · Publisher ↗
Kawasaki disease (KD) is an acute pediatric vasculitis and remains the leading cause of acquired heart disease in children in developed countries. Although timely administration of intravenous immunoglobulin (IVIG) signi...
Kawasaki disease (KD) is an acute pediatric vasculitis and remains the leading cause of acquired heart disease in children in developed countries. Although timely administration of intravenous immunoglobulin (IVIG) significantly reduces coronary complications, approximately 10-20% of patients are resistant to initial therapy and remain at increased risk for coronary artery abnormalities (CAA). Corticosteroids, previously avoided because of concerns regarding vascular healing, have re-emerged as an adjunctive anti-inflammatory strategy in selected patients. Over the past decade, randomized controlled trials and meta-analyses have clarified their role, particularly in high-risk populations. The RAISE trial demonstrated that early, multi-day prednisolone administered concomitantly with IVIG significantly reduced CAA incidence in high-risk children, whereas earlier single-pulse methylprednisolone regimens failed to show consistent benefit. The 2022 Cochrane review and subsequent observational studies from Asia and North America confirmed improved coronary outcomes without an increased risk of adverse events. Recent guidelines from the American Heart Association (2024) and the Japanese Circulation Society (2023) now recommend corticosteroids as part of initial therapy for patients at high risk of IVIG resistance. This review summarizes the evolving evidence, pathophysiologic rationale, and practical considerations regarding corticosteroid use in KD, highlighting their role as a risk-stratified adjunct to IVIG rather than universal first-line therapy.
Late Aortic Obstruction Post Patent Ductus Arteriosus Closure Managed with Renata Minima Stent.
Herron C, Iacono K, Nykanen D
Pediatr Cardiol · 2026 Jan · PMID 41603900 · Publisher ↗
Descending aorta obstruction is a known though uncommon event that can occur post patent ductus arteriosus device closure in premature infants. This adverse event has generally been managed with surgical repair or stent...
Descending aorta obstruction is a known though uncommon event that can occur post patent ductus arteriosus device closure in premature infants. This adverse event has generally been managed with surgical repair or stent placement if the child is large enough for the vascular access needed. We present two cases of late aortic obstruction associated with device closure of a patent ductus arteriosus managed with the Renata Minima stent. Key Words: PDA, Coarctation, Renata Minima Stent.
Hybrid vs. Norwood: An Analysis of the NPC-QIC Database to Determine Optimal Approach for High-Risk Single Ventricle Patients.
Callahan CP, Miller P, Carvajal H … +5 more , Wan F, Canter M, Mannie C, Hill GD, Eghtesady P
Pediatr Cardiol · 2026 Jan · PMID 41579199 · Publisher ↗
We sought to identify the optimal management strategy of infants with high-risk hypoplastic left heart syndrome and variants (HR-SV) and identify factors associated with futility. Patients with birth weight ≤ 2.5 kg or g...
We sought to identify the optimal management strategy of infants with high-risk hypoplastic left heart syndrome and variants (HR-SV) and identify factors associated with futility. Patients with birth weight ≤ 2.5 kg or gestation ≤ 35 weeks, and age ≤ 30 days at admission were included (N = 398). Norwood (n = 225), hybrid (pulmonary artery band (PAB) + ductal stent, n = 76), and PAB with prostaglandin (PAB/PGE, n = 77) were compared from the National Pediatric Cardiology Quality Improvement Collaborative database. Transplantation referral (n = 1) and comfort care (n = 19) occurred. Baseline factors, 1-year survival, and stage 2 completion were reviewed. Norwood had higher gestational age, birth weight, and younger age at intervention than hybrid and PAB/PGE; while more PAB/PGE and hybrid had chromosomal and other organ abnormalities. Norwood had higher survival (70% vs. 54% hybrid, 55% PAB/PGE, p = 0.03) and stage 2 completion (90% vs. 61% hybrid, 67% PAB/PGE, p < 0.001) than hybrid. In birthweight ≤ 2.12 kg, the survival advantage in Norwood was eliminated (59% for Norwood vs. 56% for hybrid and PAB/PGE, p = 0.61). Hybrid (HR 2.8, p < 0.001), genetic abnormality (HR 1.5, p = 0.03), and post stage 1 ECMO (HR: 7.2, p < 0.001) were associated with decreased survival and stage 2 completion, while higher birth weight (HR: 1.5, p < 0.001) was associated with increased stage 2 completion. Less than 25% of HR-SV with birthweight ≤ 2.12 kg and ≥ 1 genetic abnormality was alive with stage 2 at end of follow-up. HR-SV have better outcomes following Norwood than hybrid. In patients with birth weight ≤ 2.1 kg and genetic abnormalities, both strategies offer low survival.
Gastrointestinal Symptoms in Kawasaki Disease and Multisystem Inflammatory Syndrome in Children.
Fabi M, Harahsheh AS, Raghuveer G … +32 more , Wehrmann M, Portman MA, Dahdah N, Sabati AA, Misra N, Yamazaki-Nakashimada MA, Khoury M, Jain SS, Sundaram B, Orr W, Szmuszkovicz J, Dionne A, Thacker D, Prasad D, Sutton N, Hicar MD, Harris TH, Elganzoury M, Knutson S, Cruz J, Elias MD, Tierney S, Mondal T, Dallaire F, Lee S, Grcic M, Barnes B, Garrido LM, Manlhiot C, Farid P, McCrindle BW, International Kawasaki Disease Registry
Pediatr Cardiol · 2026 Jan · PMID 41579198 · Publisher ↗
Kawasaki disease (KD) and Multisystem Inflammatory Syndrome in Children (MIS-C) are systemic inflammatory diseases sharing clinical and laboratory features. Gastrointestinal (GI) symptoms are common presenting features o...
Kawasaki disease (KD) and Multisystem Inflammatory Syndrome in Children (MIS-C) are systemic inflammatory diseases sharing clinical and laboratory features. Gastrointestinal (GI) symptoms are common presenting features of both conditions. We aimed to determine the association of GI symptoms and clinical features and outcomes for KD and MIS-C. The International Kawasaki Disease Registry enrolled contemporaneous KD and MIS-C patients < 18 years of age from 42 sites from January 1, 2020, through October 31, 2023. The association of GI symptoms (abdominal pain, vomiting and diarrhea), demographics, clinical and laboratory features, and cardiac involvement were determined using ordinal logistic regression incorporating interaction terms with diagnosis. We included 883 KD and 1827 MIS-C patients with confirmed diagnostic criteria. The presence of at least one GI symptom (vomiting, abdominal pain, diarrhea) was more prevalent for MIS-C patients (88% versus 57%; p < 0.001). A greater number of GI symptoms was significantly associated with older age and higher adiposity, presentation with shock, intensive care unit admission, inotropic support, and greater immunomodulatory therapy. Greater number of GI symptoms was significantly associated with higher peak markers of inflammation and organ dysfunction, and higher peak NT-proBNP and lower left ventricular ejection fraction, but not with coronary artery involvement. There were few differences related to diagnosis group. A greater number of presenting GI features is associated with features that may indicate a more severe form of KD and MIS-C, including cardiac involvement, and may merit increased attention.
Genetic Burden in Congenital Anomalies of the Mitral and Tricuspid Valves: A Case-Control Study.
Campos-Garcia FJ, Castillo-Espinola AM, Medina-Escobedo CE … +8 more , Zenteno JC, Lara-Riegos JC, Chuc-Chan JA, Velazquez-Ibarra AI, Cauich-Pool PD, Favela-Perez EA, Pech-Gomez PY, Villasis-Keever MA
Pediatr Cardiol · 2026 Jan · PMID 41579197 · Publisher ↗
Congenital heart disease (CHD) is the most common congenital malformation, with most cases exhibiting a multifactorial etiology involving genetic and environmental factors. Congenital anomalies of the atrioventricular va...
Congenital heart disease (CHD) is the most common congenital malformation, with most cases exhibiting a multifactorial etiology involving genetic and environmental factors. Congenital anomalies of the atrioventricular valve or septum (CAAVAS) and functionally univentricular heart (FUH) are complex subtypes of CHD, where disruptions in key molecular pathways are implicated. This study investigates the genetic burden contributing to these anomalies. This case-control study included 48 participants: 24 patients diagnosed with CAAVAS or FUH and 24 healthy controls. Whole-exome sequencing (WES) was conducted to assess genetic burden by evaluating minor allele frequencies (MAF) using gnomAD and predicting functional impact of variants with REVEL scores. A secondary filtration was performed, focusing on 349 genes associated with abnormal heart valve morphology (HP:0001654) as defined by the Human Phenotype Ontology (HPO) database, to identify pathogenic variants exclusive to the case group. Genetic burden risk (GBR) analysis revealed a significantly higher median number of common variants in the case group compared to controls (p = 0.035). Genetic analysis identified variants in genes involved in contractile cardiac and cytoskeletal proteins (MYH3, ACTC1), extracellular matrix proteins (FBN1, FREM1, HSPG2), ciliary proteins (EVC2, PKD1L1), enzymes (POLG, DNASE1L3), cell-signaling proteins (TGFB2, CCDC22) and transcription factors (NKX2-5, NONO). This study highlights the significant role of genetic burden and gene variants associated with congenital mitral and tricuspid valve anomalies. Our findings reinforce the strong genetic predisposition underlying these malformations, as evidenced by the increased genetic burden in affected individuals compared to controls without CHD.
Can Real-Time Three-Dimensional (Four-Dimensional) and Speckle-Tracking Echocardiography Predict Cardiac Involvement Detected by Magnetic Resonance Imaging in Children with Duchenne Muscular Dystrophy?
Eroğlu AG, Kaynak E, Murt NU … +6 more , Durmaz EŞ, Kurukaya SS, Adaletli İ, Kaya AS, Karatoprak EY, Saltık S
Pediatr Cardiol · 2026 Jan · PMID 41579196 · Publisher ↗
This study aimed to evaluate usefulness of real-time three-dimensional (four-dimensional) and three-dimensional speckle-tracking echocardiography in detecting cardiac involvement identified by cardiac magnetic resonance...
This study aimed to evaluate usefulness of real-time three-dimensional (four-dimensional) and three-dimensional speckle-tracking echocardiography in detecting cardiac involvement identified by cardiac magnetic resonance imaging in children with Duchenne muscular dystrophy. This cross-sectional study included 18 boys with a median age of 11.9 years (10.5-13.2 years). Left ventricular ejection fraction, global and regional myocardial strains were assessed using real-time three-dimensional echocardiography and three-dimensional speckle-tracking echocardiography as well as magnetic resonance imaging. Late gadolinium enhancement was evaluated by cardiac magnetic resonance imaging. Moderately positive correlations were found for ejection fraction (r = 0.669, p = 0.003), global longitudinal strain (r = 0.618, p = 0.008), global circumferential strain (r = 0.513, p = 0.035), and global radial strain (r = 0.605, p = 0.01) measured by three-dimensional speckle-tracking echocardiography and cardiac magnetic resonance imaging. In addition, longitudinal and circumferential strain values measured by three-dimensional speckle-tracking echocardiography showed correlations with cardiac magnetic resonance imaging in the same segment and/or adjacent segments in 13 of 16 regions , with correlation coefficients between 0.487 and 0.776 (p < 0.05). Radial strain values demostrated correlations in 25 regions, with correlation coefficients between 0.487 and 0.690 (p < 0.05). Real-time three-dimensional echocardiography and three-dimensional speckle-tracking echocardiography provided valuable information about left ventricular function as well as global and regional myocardial strain compared with magnetic resonance imaging in our patients with Duchenne muscular dystrophy. Further studies including larger patient populations and a broader range of disease severity are needed to confirm these findings.
Seventeen Years of Pediatric Cardiology Fellow Echo Boot Camp: Evolution and Lessons Learned.
O'Meara D, Sachdeva R, Eric Ferguson M … +3 more , Wilson HC, Neal T, Border W
Pediatr Cardiol · 2026 Jan · PMID 41575501 · Publisher ↗
Success in pediatric cardiology fellowship requires a firm foundation in both the performance and interpretation of echocardiography. Recognizing this need, our institution introduced an “Echo Boot Camp” in 2009 to prepa...
Success in pediatric cardiology fellowship requires a firm foundation in both the performance and interpretation of echocardiography. Recognizing this need, our institution introduced an “Echo Boot Camp” in 2009 to prepare incoming pediatric cardiology fellows for their first on-call echocardiograms and clinical experiences managing congenital heart disease. In this article, we describe the evolution of our institution’s echo boot camp over the past 17 years. We detail lessons learned, key elements, and technologies incorporated along the way to improve the educational experience. By sharing the history and essential components of our echo boot camp, we aim to provide a framework for other pediatric cardiology fellowships to construct or add to their own boot camps with many possible points of entry.
Child Opportunity, Race, and Language in Congenital Cardiac Surgery Outcomes.
Coca KM, Fierstein JL, Manipadam J … +5 more , Ignjatovic V, Johnson J, Puchalski M, Morrison JM, Ng B
Pediatr Cardiol · 2026 Jan · PMID 41569376 · Publisher ↗
Racial and ethnic disparities and language barriers coexist with inequities in a child's educational, environmental, and economic opportunity. We evaluated the association between surgical outcomes and a composite child...
Racial and ethnic disparities and language barriers coexist with inequities in a child's educational, environmental, and economic opportunity. We evaluated the association between surgical outcomes and a composite child neighborhood opportunity index (COI), race and ethnicity, and language barriers among children undergoing congenital heart surgery. Utilizing the Society of Thoracic Surgeons database, we conducted a single-center retrospective cohort study of patients who underwent congenital cardiac surgery from 2010 to 2023. Patients were classified by quintile COI scores based on their reported address. Outcomes included in-hospital mortality, length of stay (LOS), and major complications. Adjusted analyses were performed using generalized linear mixed models. Among 1,568 patients, 51.2% were non-Hispanic White and 64.1% had public insurance. 57.4% lived in very low or low COI neighborhoods. Race, ethnicity, primary caregiver language, and insurance payer were significantly associated with COI (p < 0.0001). After adjustment, COI was not associated with mortality, LOS, or complications. Compared with non-Hispanic White patients, non-Hispanic Black patients had higher odds of in-hospital mortality (OR 2.19, 95% CI 1.07-5.30) and longer LOS (β = 0.25, 95% CI 0.09-0.42). Patients with non-English-speaking caregivers had longer LOS (β = 0.30, 95% CI 0.10-0.51). Neighborhood opportunity was not independently associated with surgical outcomes. However, disparities by race and ethnicity and language persisted, suggesting that COI may not fully capture structural racism or communication barriers in pediatric cardiac care.
A New Modification of Temporary Suture-Holding Technique for Transcatheter Repair of Sinus Venosus Atrial Septal Defect.
Elhalabi IA, Hijazi ZM
Pediatr Cardiol · 2026 Jan · PMID 41563434 · Publisher ↗
Transcatheter repair of superior sinus venosus atrial septal defect (SVASD) and associated partial anomalous pulmonary venous return (PAPVR) using a covered stent has evolved as a less invasive alternative to surgery. Pr...
Transcatheter repair of superior sinus venosus atrial septal defect (SVASD) and associated partial anomalous pulmonary venous return (PAPVR) using a covered stent has evolved as a less invasive alternative to surgery. Precise control of stent position remains a key technical challenge during the procedure. Previously, our group has reported on the suture technique with improved outcome. Here, we further report on an enhanced/modified suture technique. We report on a 14-year young boy with a superior SVASD associated with PAPVR of the right middle and upper pulmonary veins who underwent a successful transcatheter repair of the defect using a covered stent with a modification of the previously described novel temporary suture-holding technique by our group. The new modified technique (the holding-wire technique-HWT) uses a 150 cm, 0.018" Terumo® guidewire positioned in the outermost strut of the stent and both ends of the wire are exteriorized from the internal jugular vein to hold the stent temporarily instead of the suture. This allowed precise control and better grip of the attached stent, as well as visualizing the wire on fluoroscopy throughout the procedure. An 80-mm covered stent was deployed and anchored with a 28-mm bare-metal stent proximally, achieving complete exclusion of the atrial communication and rerouted the anomalous vein flow to the left atrium without any obstruction. This simple modification enhances procedural control and safety, and may broaden the clinical applicability of transcatheter SVASD closure.
Triple Pulmonary Venous Drainage in an Infant with a Hypertensive Left Atrium: A Rare Anatomic Finding.
Mohammad Nijres B, Ahmad Z, Al-Ata J
Pediatr Cardiol · 2026 Jan · PMID 41563433 · Publisher ↗
Triple drainage of a pulmonary vein is exceedingly rare. We report an unusual case of a left common pulmonary vein draining into the left atrium, coronary sinus, and innominate vein in an infant with complex congenital h...
Triple drainage of a pulmonary vein is exceedingly rare. We report an unusual case of a left common pulmonary vein draining into the left atrium, coronary sinus, and innominate vein in an infant with complex congenital heart disease.
Agreement Between Echocardiography and Cardiac MRI in Assessing Function, Strain, and Fibrosis in Pediatric Hypertrophic Cardiomyopathy: A Preliminary Study.
Şentürk NGK, Karakaş A, Ehlil SA … +4 more , Durmaz EŞM, Çulpan HC, Yücel İK, Dedeoğlu R
Pediatr Cardiol · 2026 Jan · PMID 41563432 · Publisher ↗
Hypertrophic cardiomyopathy (HCM) is the most common genetic heart disease in children and is associated with arrhythmia, heart failure, and sudden cardiac death. Early detection of myocardial dysfunction and fibrosis is...
Hypertrophic cardiomyopathy (HCM) is the most common genetic heart disease in children and is associated with arrhythmia, heart failure, and sudden cardiac death. Early detection of myocardial dysfunction and fibrosis is essential for risk stratification. Transthoracic echocardiography (TTE) is the primary diagnostic tool but has limitations, while cardiac magnetic resonance imaging (MRI) provides superior structural and fibrosis assessment. Speckle-tracking echocardiography (STE) offers sensitive markers of subclinical dysfunction, yet comparative pediatric data are limited. To investigate the concordance between TTE, STE, and MRI in pediatric HCM and to evaluate relationships between fibrosis, diastolic function, and strain parameters. Patients were categorized as sarcomeric (genetic/idiopathic) or phenocopy/non-sarcomeric (metabolic, syndromic, or endocrine) HCM according to guideline-based classification. Twenty-five HCM patients were included in this cross-sectional prospective study. All underwent clinical evaluation, TTE, and ECG; MRI was performed when eligible. Sarcomeric and phenocopy subgroups were analyzed separately. Septal thickness, LV mass, EF, and volumes were measured by TTE and MRI, while global and segmental strain values were obtained by 2D-STE. Diastolic function and fibrosis (LGE) were also assessed. Strong agreement was observed between TTE and MRI for septal thickness and LV mass, while EF differed. Complete concordance was found between 2D-STE and MRI for EF and volumes. Global strain values correlated highly, but segmental variability was noted, with strong agreement in the apical lateral segment. GLS was pathological in 77% of sarcomeric and 83% of non-sarcomeric patients. No significant difference in diastolic function was observed according to fibrosis. 2D-STE may serve as a reliable alternative to MRI for serial EF and volume monitoring in pediatric HCM. Despite segmental variability, strong global strain correlation supports complementary use. The observation that diastolic dysfunction may precede fibrosis highlights its role as an early clinical marker.
A Novel Artefact on the Newborn Electrocardiogram: A Tale of Two Hearts.
Dama M, Klimek J, Lawley CM
Pediatr Cardiol · 2026 Jan · PMID 41555068 · Publisher ↗
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