Pediatr Cardiol [JOURNAL]
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200 papers
Risk Factors for the Development of Prosthetic Valve Thrombosis After Systemic Atrioventricular Valve Replacement in Pediatric Patients with a Functional Single Ventricle.
Yamada Y, Yamamura K, Kuraoka A … +4 more , Go K, Ishikawa Y, Nakano T, Sagawa K
Pediatr Cardiol · 2026 May · PMID 42171700 · Publisher ↗
Risk factors for developing prosthetic valve thrombosis (PVT) after systemic atrioventricular valve replacement in patients with functional single ventricles remain poorly understood. Medical records of 39 operations in...
Risk factors for developing prosthetic valve thrombosis (PVT) after systemic atrioventricular valve replacement in patients with functional single ventricles remain poorly understood. Medical records of 39 operations in 28 patients with functional single ventricles who underwent systemic atrioventricular valve replacement between 1998 and 2021 were retrospectively reviewed. Clinical variables were compared between patients with and without PVT. Median age at operation was 20 (IQR: 10-57) months; body weight was 7.8 (4.9-11.4) kg. Valve size was 21 (19-22) mm, and ratio of valve size (mm) to weight (kg) was 2.4 (1.8-3.6). Valve replacement was performed before bidirectional Glenn procedure (BDG) in 8 cases, with BDG in 7 cases, between BDG and total cavopulmonary connection (TCPC) in 13 cases, with TCPC in 4 cases, and after TCPC in 7 cases. PVT occurred after 12 of 39 operations (31%). Significant differences existed in median age at operation (12 vs. 26 months, p = 0.003), median weight at operation (4.9 vs. 10.2 kg, p = 0.001), and median valve size/weight ratio (3.6 vs. 2.1, p = 0.002) between operations with and without PVT. PVT incidence was higher before TCPC completion: before BDG (4/8 operations, 50%), BDG (3/7, 43%), and between BDG and TCPC (5/13, 38%), compared to TCPC (0/4, 0%) and after TCPC (0/7, 0%) (p < 0.001). Patients who developed PVT were younger and underweight; in these patients, the use of a relatively oversized prosthesis is clinically inevitable. Close monitoring for PVT is warranted, especially before TCPC completion.
Temporal Changes in the Timing of Pulmonary Valve Replacement and Impact on Outcomes in Tetralogy of Fallot.
Gröning M, Smerup MH, Nissen H … +16 more , Nielsen DG, Andersen H, Munk K, Mortensen UM, Engholm M, Juul K, Bjerre J, Søndergaard EV, Thyregod HGH, Nerst D, De Backer O, Jensen AS, Jørgensen TH, Jøns C, Vejlstrup N, Schmidt MR
Pediatr Cardiol · 2026 May · PMID 42149215 · Publisher ↗
To assess temporal changes in the timing of pulmonary valve replacement (PVR) among patients with tetralogy of Fallot and to determine whether temporal shifts toward earlier PVR were accompanied by differences in ventric...
To assess temporal changes in the timing of pulmonary valve replacement (PVR) among patients with tetralogy of Fallot and to determine whether temporal shifts toward earlier PVR were accompanied by differences in ventricular volumes, ventricular function and exercise capacity after PVR. The timing of PVR was evaluated in a national, retrospective cohort using the Aalen-Johansen method to estimate cumulative incidence while accounting for death as competing risk. Trends in ventricular volumes, ventricular function and exercise capacity were assessed in patients with prior PVR, who underwent cardiac imaging and exercise testing in a cross-sectional study using robust linear regression. Among 463 potential candidates for PVR in the retrospective cohort, estimated time from repair to PVR was 34 years, 22 years and 16 years in patients born in 1940-1976, 1977-1999, and 2000-2021, respectively (p < 0.001). In total, 153 patients with prior PVR born between 1942 and 2009 were examined in the cross-sectional study. Estimated right ventricular end-systolic volume index was lower by 5.43 mL/m per decade (p = 0.02), estimated right ventricular ejection fraction increased by 2.08% per decade (p = 0.01). Left ventricular volumes, left ventricular ejection fraction and exercise capacity was not more favorable in later eras. The timing of PVR has shifted toward earlier intervention over time. The temporal shift toward earlier PVR coincided with lower right ventricular end-systolic volume index and higher right ventricular ejection fraction after PVR, whereas left ventricular parameters and exercise capacity showed no clear temporal change. These findings are compatible with, but do not prove, a beneficial effect of earlier intervention.
Single Center Retrospective Initiative to Improve Reporting Times of Echocardiograms.
Ramachandra R, Thorson K, Reddy CD … +2 more , Buddhe S, Tierney S
Pediatr Cardiol · 2026 May · PMID 42149214 · Publisher ↗
Timely completion and reporting of echocardiograms (echo) directly influence patient management decisions in pediatric cardiology. Tracking these measures is paramount to improving efficiency in pediatric echo laboratori...
Timely completion and reporting of echocardiograms (echo) directly influence patient management decisions in pediatric cardiology. Tracking these measures is paramount to improving efficiency in pediatric echo laboratories. Previous efforts in pediatric and adult echo laboratories have demonstrated a significant improvement in the timely reporting of studies by replacing dictation with online electronic reporting systems, increasing awareness of delays among physicians, and implementing a cardiologist-led triage system for echo requests. We sought to evaluate the impact of a modified sonographer workflow initiative on the time to completion of echo reports at our center. A modification to the sonographer workflow was introduced at our echo laboratory, requiring sonographers to complete preliminary measurements immediately after image acquisition for each study, rather than the previous stacked workflow of completing measurements after image acquisition for 2-3 studies. With this intervention, the average time from first image acquisition to completion of preliminary reporting by sonographers reduced by 27% from 1 h (h) 39 min (m) to 1 h 12 m (p < 0.001), while the average time to completion of reports by attending cardiologists reduced by 15% from 4 h 49 m to 4 h 4 m (p < 0.001). We demonstrated a significant improvement in the timely completion of echo reports by switching from a stacked workflow to a linear, sequential model. This intervention led to a 27% reduction in sonographer reporting time and a 15% reduction in attending cardiologist report completion time.
Outcomes After Tetralogy of Fallot Repair with Preservation of Intact Pulmonary Valve Structure.
Ryu SW, Jang DH, Kwon SJ … +5 more , Kim DH, Choi ES, Kwon BS, Park CS, Yun TJ
Pediatr Cardiol · 2026 May · PMID 42138732 · Publisher ↗
The optimal post-repair pulmonary valve annulus (PVA) diameter in tetralogy of Fallot (TOF) may be far smaller than anticipated. Therefore, selected patients can undergo repair without manipulating the PV structure. Of t...
The optimal post-repair pulmonary valve annulus (PVA) diameter in tetralogy of Fallot (TOF) may be far smaller than anticipated. Therefore, selected patients can undergo repair without manipulating the PV structure. Of the 347 patients who underwent TOF repair with PVA preservation (AP) from January 2016 to December 2023, 100 had AP while leaving the PV structure untouched. Median age, weight, and PVA (Z) at repair were 125 days (interquartile range [IQR], 96.5-167.5), 6.3 kg (IQR, 5.1-7.1), and -0.8 (IQR, -1.8 to 0.3), respectively. One non-cardiac late death occurred 9 months after repair. During a median 46.2 months follow-up, 14 patients (14%) required reinterventions for significant right ventricular outflow tract obstruction (RVOTO): balloon pulmonary valvuloplasty (BPV) in 6, surgical RVOTO relief in 4, and BPV followed by surgery in 4 (including 1 who received a right ventricle to pulmonary artery conduit to bypass a left anterior descending coronary artery crossing the RVOT). Except for the conduit recipient, only 2 patients showed significant RVOTO (n = 1) and significant pulmonary regurgitation (PR) (n = 1) at the last follow-up. On Cox regression, neonatal repair (hazard ratio [HR], 5.12, P = 0.02) and a higher P (post-repair pressure ratio of the right ventricle to the left ventricle) (HR 1.61 per 0.1 increase, P = 0.04) were risk factors for decreased time to reintervention. The post-repair P cutoff predicting reintervention was 0.52. Preserving an intact PV during TOF repair is feasible in a subset. Post-repair RVOTO, when it occurs, can be relieved by a timely reintervention without inducing significant PR.
Reduced Perfusion Pressure as a Predictor of Protein-Losing Enteropathy After Fontan Procedure: Beyond Central Venous Pressure.
Kadoya T, Kawasaki Y, Fujino M … +5 more , Sasaki T, Nakamura K, Ehara E, Murakami Y, Sugiyama H
Pediatr Cardiol · 2026 May · PMID 42133043 · Publisher ↗
Protein-losing enteropathy (PLE) is a significant complication that affects the prognosis of Fontan patients. Although elevated central venous pressure (CVP) is associated with PLE, some patients develop PLE without elev...
Protein-losing enteropathy (PLE) is a significant complication that affects the prognosis of Fontan patients. Although elevated central venous pressure (CVP) is associated with PLE, some patients develop PLE without elevated CVP. This study aimed to identify whether reduced perfusion pressure (PP), calculated as the mean systemic arterial pressure minus CVP, is a predictor of PLE and assess the impact of angiotensin-converting enzyme inhibitor (ACEI) use on PLE incidence. A retrospective analysis of 107 post-Fontan patients aged < 10 years who underwent catheterization between 1998 and 2021 was performed. The primary endpoint was freedom from PLE. Freedom from the endpoint and significant clinical and hemodynamic predictors, including PP and ACEI use, were analyzed. During a median follow-up of 11.4 years, seven patients (6.5%) developed PLE, with a median onset at 3.8 years. Patients with PP < 60 mmHg showed significantly shorter freedom from PLE, and PP remained a significant predictor in the multivariate analysis considering most of the other variables, including CVP. ACEI use was a significant predictor for PLE but did not remain significant after adjustment for fenestration at Fontan, brain natriuretic peptide, pulmonary artery index, ejection fraction, and end-diastolic ventricular volume of the single ventricle. PP was a significant predictor of PLE development in the post-Fontan hemodynamic evaluation, independent of CVP. PP warrants consideration alongside CVP in the hemodynamic assessment of Fontan circulation. Although ACEI use was associated with PLE development in the univariable analysis, the present data do not establish ACEI use as an independent risk factor, given the potential for indication bias in the ACEI-treated group.
Incidence and Predictors of Stent Thrombosis Following Transcatheter Sinus Venosus Defect Closure.
Sagar P, Thejaswi P, Athira M … +1 more , Sivakumar K
Pediatr Cardiol · 2026 May · PMID 42118178 · Publisher ↗
Following transcatheter closure (TCC) of sinus venosus defects (SVD), concerns exist about thrombus formation over the stents. Spatial and temporal resolution of Transesophageal echocardiography (TEE) is sensitive to det...
Following transcatheter closure (TCC) of sinus venosus defects (SVD), concerns exist about thrombus formation over the stents. Spatial and temporal resolution of Transesophageal echocardiography (TEE) is sensitive to detect even small thrombus. Following TCC of SVD between May 2014 to August 2025, all patients were imaged for thrombus after one-month and one-year. Thrombus were classified as major (> 5 mm thickness) or minor (layered ≤5 mm). Anatomical, procedural details and post-procedural thromboprophylaxis were compared between patients with and without thrombus detection. Among 153 patients following SVD closure, surveillance imaging involved TEE, computed tomography and magnetic resonance in 137, 7 and 2 cases respectively and transthoracic echocardiography alone in 7 patients. At a median follow-up period of one-month (range 0.5-16 months), thrombus was detected in eleven (7.2%) cases; major in three and minor layered in eight patients. All thrombi were asymptomatic, non-obstructive without any systemic or pulmonary thromboembolism. Bilateral superior venacava was associated with thrombus with an odds ratio of 4.5 (95% confidence interval 1.3-15.7; p = 0.01). Use of multiple overlapping stents (p = 0.02) and long procedural time (p = 0.04) were also identified as risk factors on univariate analysis. Occurrence of thrombus after dual antiplatelet therapy versus anticoagulation did not reach statistical significance (p-value 0.396). Artifacts in non-TEE imaging precluded high-quality pictures. Asymptomatic stent thrombosis is detected in 7.2% of patients after SVD closure during surveillance TEE. Alternative imaging might miss minor thrombus detection. Risk factors include bilateral SVC and use of multiple overlapping stents. Correct thromboprophylaxis following SVD closure needs prospective evaluation.
Associations of Overweight and Obesity Indicators with Cardiometabolic Risk Profile in Children and Adolescents with Congenital Heart Disease.
Honicky M, Cardoso SM, de Lima LRA … +3 more , Vieira FGK, Back IC, Moreno YMF
Pediatr Cardiol · 2026 May · PMID 42113240 · Publisher ↗
This study aimed to compare the cardiometabolic risk profile between overweight or obese and normal weight and to investigate the influence of overweight and obesity indicators on cardiometabolic risk profile in children...
This study aimed to compare the cardiometabolic risk profile between overweight or obese and normal weight and to investigate the influence of overweight and obesity indicators on cardiometabolic risk profile in children and adolescents with congenital heart disease (CHD). A cross-sectional study with 232 children and adolescents with CHD. Cardiometabolic risk profile were evaluated: high-density lipoprotein cholesterol (HDL-c), total cholesterol (TC), low-density lipoprotein cholesterol (LDL-c), non-HDL-c, fasting glucose, triglycerides, high-sensitivity C-reactive protein (hs-CRP), and systolic and diastolic blood pressure (SBP and DBP). Overweight and obesity indicators included body fat percentage (%BF) by air displacement plethysmography and waist circumference percentile (WC-p) and were categorized in "overweight or obese children and adolescents" versus "normal weight children and adolescents". Data were examined using linear regressions. In linear regression analysis, WC-p was inversely associated with HDL-c (ß: - 0.19, 95% CI - 0.16; - 0.03) and directly associated with hs-CRP (ß: 0.02, 95% CI 0.02; 0.03). %BF was directly associated with LDL-c (ß: 0.38, 95% CI 0.00;0.75), non-HDL-c (ß: 0.42, 95% CI 0.02;0.81), hs-CRP (ß: 0.02, 95% CI 0.01; 0.02), SBP (ß: 0.26, 95% CI 0.13;0.39) and DBP (ß: 0.15, 95% CI 0.07;0.23). When stratified by overweight or obese children and adolescents versus normal weight children and adolescents, WC-p and %BF were directly associated hs-CRP (ß:0.14, 95%CI 0.03;0.26 and ß:0.02, 95%CI 0.01; 0.04, respectively) and %BF was also directly associated with TC (ß:1.68, 95%CI 0.30;3.07), LDL-c (ß:1.64, 95%CI 0.32;2.95), non-HDL-c (ß:1.56, 95%CI 0.12;3.01) in overweight or obese children and adolescents. The increase in WC-p and %BF seems to increase the risk of altered cardiometabolic risk profile, especially in the overweight or obese children and adolescents. Early identification of overweight or obesity indicators may allow timely intervention to prevent adverse cardiovascular outcomes in adulthood.
Fetal Cardiology Prenatal Care Quality and Social Determinants of Health in Critical Congenital Heart Disease.
Trussell TM, A DeFranco E, Seo J … +3 more , Ollberding NJ, Cnota JF, A Divanovic A
Pediatr Cardiol · 2026 May · PMID 42113239 · Publisher ↗
Prenatal diagnosis of critical congenital heart disease (cCHD) and subsequent perinatal recommendations are vital components of prenatal care that affect infant morbidity and mortality. However, there is limited understa...
Prenatal diagnosis of critical congenital heart disease (cCHD) and subsequent perinatal recommendations are vital components of prenatal care that affect infant morbidity and mortality. However, there is limited understanding of the relationship between fetal cardiology prenatal care quality, infant outcomes, and maternal social determinants of health (SDoH). The aim of this study was to better understand this relationship, and to further characterize the continuum of prenatal to postnatal care in a population of maternal-infant dyads diagnosed with cCHD. This was a single-site, retrospective cohort study of 217 maternal-infant dyads with cCHD cared for between 2021 and 2022. A community deprivation index was used as a marker of maternal SDoH. 198 dyads had a prenatal cCHD diagnosis. Maternal deprivation was similar in those with prenatal versus postnatal diagnosis. The majority were identified on obstetric anatomy ultrasound, with median 15 days (IQR: 7, 28) from concern to fetal echocardiogram. Nearly all had perinatal delivery recommendations, while 73% had cardiothoracic surgery and 60% genetics referral. Only 76% of those diagnosed prenatally were alive at birth admission discharge. Multivariable analysis demonstrated an association between fetal cardiology visit cancelation and earlier gestational age at birth (β -1.5, 95% CI -2.9, -0.05); time to fetal echocardiogram and no-show visits were not associated with outcomes. There was no association between higher maternal deprivation and fetal cardiology care. In conclusion, cCHD represents a subset of lesions with high morbidity and mortality, with nearly 1 in 4 prenatally diagnosed patients not reaching birth admission discharge. Identifying areas for fetal cardiology care optimization has the potential to affect outcomes; however further research is needed to understand the relationship between visit cancelation and gestational age at birth, as well as the impact of care quality and SDoH on optimal care delivery.
Antidepressant Use in Children with Congenital Long QT Syndrome.
Rebhan NE, Zhang L, Yan K … +2 more , Singh AK, Kovach J
Pediatr Cardiol · 2026 May · PMID 42105111 · Publisher ↗
Congenital long QT syndrome (LQTS) conveys a significant risk of cardiac events. Primary management includes avoiding medications that prolong the QTc. Many antidepressant medications are known to be QT-prolonging, but t...
Congenital long QT syndrome (LQTS) conveys a significant risk of cardiac events. Primary management includes avoiding medications that prolong the QTc. Many antidepressant medications are known to be QT-prolonging, but there is scant literature on whether their use increases event rate or prolongs the QTc in LQTS. The objectives of this study are to determine the prevalence of depression and anxiety in patients with congenital LQTS, and to evaluate the effect of antidepressant treatment on frequency of LQTS-triggered events and QTc changes during antidepressant treatment. This was a single-center, retrospective study. Patients were divided based on whether they were prescribed antidepressants during the study period. Incidence of LQTS-triggered events and QTc measurements before and after antidepressant use were recorded for comparison. Ninety-eight subjects were identified for analysis. Most (92%) were prescribed beta-blockers as treatment for LQTS. 27% (n = 27) of the cohort were diagnosed with anxiety and/or depression. Of these, 15 received antidepressant therapy. We found no significant difference in the LQTS event rate or QTc measurements in patients prescribed antidepressants. Baseline median (IQR) QTc 467 (460, 479) ms vs. median (IQR) of first QTc post-antidepressant 482 (462, 487), p = 0.27. In this single-center study, the prevalence of anxiety and depression in patients with LQTS was consistent with the general population. Rates of treatment were also comparable. Our findings suggest that certain antidepressant therapy may be safe in patients with LQTS on beta-blockers. Further study across multiple centers and with a larger cohort may help clarify the safety of these medications.
Pulse Oximetry, Skin Pigmentation, and Occult Hypoxemia in Pediatric Cardiac Critical Care.
Savorgnan F, Prabhu J, Pilla P … +3 more , Flores S, Loomba RS, Acosta S
Pediatr Cardiol · 2026 May · PMID 42105110 · Publisher ↗
Pulse oximetry (SpO₂) is central to oxygenation assessment in pediatric and cardiac intensive care. Increasing evidence demonstrates that SpO₂ may systematically overestimate arterial oxygen saturation (SaO₂), particular...
Pulse oximetry (SpO₂) is central to oxygenation assessment in pediatric and cardiac intensive care. Increasing evidence demonstrates that SpO₂ may systematically overestimate arterial oxygen saturation (SaO₂), particularly in individuals with darker skin pigmentation, thereby increasing the risk of occult hypoxemia-arterial hypoxemia not detected by pulse oximetry. We sought to synthesize current evidence and define implications for pediatric and congenital heart disease populations. We performed a narrative review of experimental physiology studies; observational adult and pediatric cohorts; pediatric COVID-19, ICU, and cardiac ICU investigations; device-comparison analyses; systematic reviews; and regulatory evaluations reporting paired SpO₂-SaO₂ measurements or validated reference standards stratified by race, ethnicity, or objectively measured skin pigmentation. Data sources included PubMed, MEDLINE, and bibliographies of relevant studies. Experimental data demonstrate directional SpO₂ overestimation that increases during hypoxemia. Large adult cohorts confirm higher rates of occult hypoxemia in patients with darker skin pigmentation, findings subsequently replicated in hospitalized children. Emerging pediatric cardiac ICU data suggest that physiologic complexity may further amplify SpO₂-SaO₂ discordance. Device-comparison studies reveal variability across manufacturers, influenced by calibration datasets and signal-processing algorithms. Across populations, misclassification is most clinically relevant near commonly used escalation thresholds. SpO₂ frequently overestimates SaO₂ in darker skin pigmentation and during hypoxemia, increasing vulnerability to occult hypoxemia in hospitalized and critically ill children. In pediatric cardiac populations-where narrow saturation thresholds guide management-contextual, bias-aware interpretation of pulse oximetry is essential to support accurate decision-making and equitable care.
Fenestration Re-creation for Early Fontan Deterioration: Long-Term Hemodynamic and Clinical Outcomes.
Tran THY, Kurita Y, Kondo M … +8 more , Fukushima Y, Shigemitsu Y, Kawamoto Y, Hara M, Tsukahara H, Iwasaki T, Kasahara S, Baba K
Pediatr Cardiol · 2026 May · PMID 42105109 · Publisher ↗
Spontaneous closure of a Fontan fenestration may precipitate early hemodynamic deterioration in vulnerable patients. In such situations, fenestration re-creation may be considered as a rescue strategy when medical therap...
Spontaneous closure of a Fontan fenestration may precipitate early hemodynamic deterioration in vulnerable patients. In such situations, fenestration re-creation may be considered as a rescue strategy when medical therapy is insufficient. However, the long-term hemodynamic consequences of this intervention remain poorly defined. We retrospectively reviewed 62 patients who underwent the Fontan procedure between January 2011 and December 2020 and subsequently experienced spontaneous fenestration closure. Patients were divided into two groups according to management strategy: those who underwent fenestration re-creation (Group 1, n = 19) and those managed without re-creation (Group 2, n = 43). Longitudinal hemodynamic data were analyzed using linear mixed models over a median follow-up of 10.2 years. Fenestration closed spontaneously at a median of 12 days (IQR 2.5-26.5) and initial re-creation was performed at the median of 42 days (IQR 11-213) postoperatively. Early and mide-term hemodynamic trends were broadly similar between groups, however, clear divergence emerged in the late follow-up. fenestration re-created patients demonstrated persistently higher central venous pressure and pulmonary vascular resistance, lower systemic vascular resistance, progressive increases in cardiac index, and declining oxygen saturation. Survival in this group remained above 80% during the mid-term follow-up but declined substantially in the late phase compared with patients without re-creation (52% vs. 85%, p = 0.043). This was accompanied by higher incidences of protein-losing enteropathy/plastic bronchitis, thromboembolism, and severe cyanosis. Fenestration re-creation may provide temporary hemodynamic stabilization in early Fontan deterioration; however, its benefits appear to diminish in the late phase. These findings highlight the need for hemodynamically tailored management strategies and alternative therapeutic options for patients with deteriorating Fontan circulation.
Outcomes of Fontan Modifications for Isolated Hepatic Vein Drainage: 5 Years Comparative Analysis.
Benli O, Avşar MK
Pediatr Cardiol · 2026 May · PMID 42105108 · Publisher ↗
Isolated (IHV) drainage can prevent uniform delivery of hepatic venous effluent to the pulmonary circulation after Fontan completion, predisposing to pulmonary arteriovenous malformations (PAVMs), hypoxemia, and thrombos...
Isolated (IHV) drainage can prevent uniform delivery of hepatic venous effluent to the pulmonary circulation after Fontan completion, predisposing to pulmonary arteriovenous malformations (PAVMs), hypoxemia, and thrombosis. Comparative data across operative strategies remain limited. Accordingly, Fontan configurations that differ in how hepatic venous return is incorporated and distributed within the cavopulmonary pathway may plausibly influence oxygenation and PAVM risk, although direct flow quantification is required for definitive mechanistic confirmation. We conducted a multicenter retrospective cohort study. Among 988 Fontan procedures performed between 2014 and 2025, we identified 99 patients with IHV drainage who underwent Fontan completion between 2014 and 2020 and had ≥ 5 years of follow-up. Patients received one of three techniques: intra-extracardiac Fontan (IECF, n = 42), lateral tunnel (LTT, n = 17), or extracardiac Fontan with hepatic-vein graft incorporation (ECF-HV, n = 40). Prespecified 5-year primary outcomes were oxygen saturation, PAVM, and thrombosis. Secondary outcomes included mean pulmonary artery pressure (PAP), arrhythmia, protein-losing enteropathy (PLE), pleural effusion, NYHA class, and mortality. Group comparisons used nonparametric tests with Dunn post-hoc testing and exact tests; sensitivity analyses excluded fenestrated patients. Baseline demographics, pulmonary artery indices, and pre-Fontan hemodynamics were similar across groups. At 5 years, oxygen saturation was higher with IECF (mean [SD] 92 [4]%) than with LTT (85 [6]%; p = 0.002) and ECF-HV (89 [5]%; p = 0.001). Mean PAP was lower with IECF (12 [2] mmHg) than with LTT (14 [3] mmHg; p = 0.001) and ECF-HV (13 [2] mmHg; p = 0.003). PAVM incidence was 4.8% with IECF versus 29.4% with LTT and 7.5% with ECF-HV (overall p = 0.017). Thrombosis occurred in 2.4% with IECF versus 29.4% with LTT and 5.0% with ECF-HV (overall p = 0.002). NYHA class distribution favored IECF but did not reach statistical significance (overall p = 0.12). Kaplan-Meier analysis showed no statistically significant difference in 5-year survival (log-rank p = 0.11). Findings were consistent after excluding fenestrated patients. In patients with IHV drainage undergoing Fontan completion, IECF was associated with higher 5-year oxygen saturation, lower PAP, and lower rates of PAVMs and thrombosis compared with LTT and ECF-HV, without a statistically significant difference in survival by log-rank testing. These findings may reflect differences in hepatic venous incorporation and pathway geometry; however, direct flow-based assessment (e.g., 4D-flow MRI, MRI-based energy-loss analysis, angiographic flow mapping, or computational flow modeling) was not performed, and mechanistic interpretation should therefore remain speculative.
Organizational Factors to Support Collaborative Care in Adult Congenital Heart Disease Programs.
Ito S, Park IH, Carter TC … +1 more , Zaveri P
Pediatr Cardiol · 2026 May · PMID 42095919 · Publisher ↗
Collaboration among healthcare workers is critical for adult congenital heart disease (ACHD) programs to provide care to ACHD patients with complex needs. This study aimed to describe ACHD programs' organizational struct...
Collaboration among healthcare workers is critical for adult congenital heart disease (ACHD) programs to provide care to ACHD patients with complex needs. This study aimed to describe ACHD programs' organizational structure, environment, and indicators of collaboration. A cross-sectional survey among ACHD programs in the United States was conducted, including questions on characteristics, personnel, services, and ten indicators of collaboration using a model described by D'Amour. The survey was sent to 86 ACHD programs, and 36 programs (41.8%) responded. The types of ACHD programs' primary institution were combined adult and pediatric institution (61.1%, N = 22), pediatric institution (27.8%, N = 10), and adult institution (11.1%, N = 4), and most ACHD programs had 1 to 3 healthcare organization(s) participating. The median number and interquartile range (IQR) of ACHD providers, advanced practice providers (APP), clinical coordinators, and administrative coordinators or assistants were 4 (IQR 3-5), 1 (IQR 1-2), 1 (IQR, 1-3), and 1 (IQR 1-2) respectively. Among the ten indicators of collaboration, leadership and connectivity were rated higher, and goals, centrality, formalization tools, and information exchange were rated lower. When analyzed by subgroups based on the types of primary institution, statistically significant differences in responses were demonstrated in support for innovation, formalization tools, and information exchange, showing higher levels of collaboration in the combined adult and pediatric institution group compared with the pediatric institution group. This study provided descriptions of the organizational structure and environment of ACHD programs. It also described organizational factors and suggested unique challenges and opportunities ACHD programs face.
Transcatheter Superior Cavopulmonary Shunt Completion After Pulmonary Artery Septation.
McLean K, Desai M, Kanter JP
Pediatr Cardiol · 2026 May · PMID 42095918 · Publisher ↗
We present the case of a 16-month-old patient with tricuspid atresia and absent pulmonary valve who underwent staged single ventricle palliation, ultimately to a unidirectional superior cavopulmonary anastomosis (Glenn)...
We present the case of a 16-month-old patient with tricuspid atresia and absent pulmonary valve who underwent staged single ventricle palliation, ultimately to a unidirectional superior cavopulmonary anastomosis (Glenn) to the right pulmonary artery and a central shunt to a hypoplastic left pulmonary artery. The pulmonary arteries were septated with a thin patch of Gore-Tex at that surgery in anticipation of a transcatheter superior cavopulmonary shunt completion at a later date. She returned to the cardiac catheterization laboratory about 12 months later and underwent uncomplicated shunt completion. There are no other reports of transcatheter superior cavopulmonary anastomosis shunt completion in a pediatric patient in the literature.
A Novel, Interpretable Machine Learning Model Predicts Furosemide Dosing After Congenital Cardiac Surgery.
Ehrmann DE, Hodgman MW, Wittrup EM … +4 more , Charpie JR, Owens GE, Aiyagari R, Najarian K
Pediatr Cardiol · 2026 May · PMID 42095917 · Publisher ↗
Fluid overload is common after neonatal congenital cardiac surgery (CCS) and is frequently managed with continuous furosemide infusions requiring iterative dose titration. An interpretable prediction model could support...
Fluid overload is common after neonatal congenital cardiac surgery (CCS) and is frequently managed with continuous furosemide infusions requiring iterative dose titration. An interpretable prediction model could support more consistent early postoperative dosing decisions. We hypothesized that a novel, interpretable machine learning approach could accurately predict furosemide dosing decisions in neonates following CCS. We identified term neonates admitted to the Pediatric Cardiothoracic ICU at a large academic children's hospital between 8/1/2014 and 3/1/2023 following CCS with cardiopulmonary bypass. Demographic and clinical data from the first 48 postoperative hours were used to train, validate, and test a Tropical Geometry-Based Fuzzy Neural Network Regressor (TGFNN-R) tasked with predicting furosemide infusion dose changes after CCS. The TGFNN-R was primed with clinician heuristics and provides transparent explanations behind predictions. A held-out internal validation/testing cohort was drawn from the same single-center population. Data from 506 neonates were extracted; 398 received a continuous furosemide infusion. Mean age at surgery was 6.2 (± 5.1) days; 67.3% were White. The most common surgeries were Stage I (Norwood) (25.1%) and arterial switch operation (18.6%). There were 783 furosemide dose increases and 224 dose decreases. Test set performance was R²=0.515, mean absolute error = 0.119 mg/kg/hr, and false positive rate = 0.062. In this retrospective single-center cohort of neonates following CCS, an interpretable TGFNN-R model predicted and explained furosemide dose changes with good test performance. Next steps include external validation and nonclinical studies evaluating the model within clinical decision support and closed-loop paradigms to achieve prespecified fluid balance goals.
ASD Diameter Indexed to Total Septal Length Outperforms Expert Opinion in Predicting Future Candidacy for Percutaneous ASD Closure.
James L, O'Halloran CP, Lemley BA … +5 more , Cornicelli M, Fox J, Winlaw D, Nugent A, Tannous P
Pediatr Cardiol · 2026 May · PMID 42095916 · Publisher ↗
Percutaneous device closure is an attractive alternative to surgical closure of secundum atrial septal defects (ASDs) for those with suitable anatomy and size. Early surgery (< 2 years of age) allows for a more feasible...
Percutaneous device closure is an attractive alternative to surgical closure of secundum atrial septal defects (ASDs) for those with suitable anatomy and size. Early surgery (< 2 years of age) allows for a more feasible "mini sternotomy" technique, while device closure is more commonly performed at an older age. Thus, the ability to predict future device-closure candidacy before 2 years of age is needed. Secundum ASD closures after 3 years of age from 2012 to 2023 at a single center were reviewed (n = 54). "Early" echocardiograms (obtained at 12-24 months of age) and "pre-intervention" echocardiograms (the last echo obtained prior to closure and after 36 months of age) for each patient were independently reviewed by echocardiographers who performed standardized measurements. The catheterization team reviewed the same echocardiograms and made recommendations on device closure candidacy. Reviewers were blinded to the paired echocardiograms and eventual interventions. There was moderate agreement in perceived device candidacy by catheterization team consensus between early and pre-intervention echocardiogram (Cohen's Kappa = 0.56, p < 0.001). In a multivariable model, the ratio of maximum ASD diameter indexed to total septal length (ASD/TSL) between 1 and 2 years of age was strongly predictive of eventual perceived candidacy for device closure (Area Under the Curve = 0.92, sensitivity 89%, specificity 89% at the optimal threshold of 0.46) outperforming expert consensus (sensitivity 81%, specificity of 77%). Patients with ASD/TSL < 0.46 on early echocardiograms are likely to be considered device candidates later in life.
ECG Changes in Tetralogy of Fallot: Association with Right Ventricular Function, Early Age of Repair and Prematurity.
Bhat M, Malm T, Nordenstam F … +3 more , Hanséus K, Rosenkvist CJ, Liuba P
Pediatr Cardiol · 2026 May · PMID 42095915 · Publisher ↗
Tetralogy of Fallot (TOF) patients are at risk for long-term adverse events. Electrocardiogram (ECG) abnormalities may reflect ventricular maladaptation and predict complications. We investigated whether ECG changes corr...
Tetralogy of Fallot (TOF) patients are at risk for long-term adverse events. Electrocardiogram (ECG) abnormalities may reflect ventricular maladaptation and predict complications. We investigated whether ECG changes correlated with cardiac magnetic resonance imaging (CMR) measures of right ventricular (RV) function and their prevalence in less mature myocardium (prematurity or early repair). This retrospective study included 219 TOF patients operated between 2000-01-01 and 2018-12-31. Parameters analyzed included PQ interval, QRS duration, dispersion, fragmentation, QTc, JTc, and right bundle branch block (RBBB) at predefined time points. In patients undergoing pulmonary valve replacement (PVR) with available CMR, measures of RV size and function were compared with ECG variables. Early repair was defined as < 3 months and prematurity < 37 weeks' gestation. Median age at follow-up was 12.3 years (IQR, 8.4;17), and 4.9 months (IQR, 3.4;6.9) at primary repair. PQ interval correlated with RV end-diastolic, stroke and regurgitant volume, whereas RBBB correlated with larger RV. Forty-five patients (21%) underwent early repair (median 2.3 months [IQR 1.3;2.7]) with no significant ECG differences. Forty-one patients (20%) were premature. Premature patients were older at repair versus term (5.8 months [IQR 4.1;7.3] vs. 4.5 months [IQR 3.0;6.3], p = 0.001) but weighed less (5.6 kg [SD 1.2] vs. 6.5 kg [SD 1.7], p = 0.001). PQ interval was shorter in premature patients at multiple time points. Neither prematurity nor early repair were associated with increased reintervention or PVR. These findings support an association between PQ interval and RV volume load. Shorter PQ intervals with prematurity may reflect stiff myocardium, with unknown long-term implications.
Transaxillary Aortic Arch Stenting in a Critically Ill Neonate with a Near-Interrupted, Angulated Aortic Arch.
Razzuk A, Kedda M, Al Hutaimi M … +2 more , Fares M, Mohammad Nijres B
Pediatr Cardiol · 2026 May · PMID 42095914 · Publisher ↗
Neonatal near-interrupted aortic arch is a medical emergency that requires immediate attention, with primary surgical repair being the preferred treatment. This report describes a critically ill neonate with a complex, n...
Neonatal near-interrupted aortic arch is a medical emergency that requires immediate attention, with primary surgical repair being the preferred treatment. This report describes a critically ill neonate with a complex, near-interrupted aortic arch in whom surgical repair was not feasible. The patient was successfully stabilized with rescue stent placement via a less commonly used approach-left axillary artery access.
Entrustable Professional Activity Framework Implementation and Impressions from U.S. and Canadian Pediatric Cardiology Training Programs.
Kim ME, Neal A, Karnik R … +3 more , Frank LH, Turner D, McMahon CJ
Pediatr Cardiol · 2026 May · PMID 42095913 · Publisher ↗
INTRODUCTION: Medical training assessment frameworks have continued to evolve to effectively capture clinical competency and graduation readiness. Entrustable Professional Activities are being integrated into training pr...
INTRODUCTION: Medical training assessment frameworks have continued to evolve to effectively capture clinical competency and graduation readiness. Entrustable Professional Activities are being integrated into training programs and describe the activities expected in practice to guide education and assessment. U.S. Pediatric cardiology fellowship programs have been working on creating and implementing these since 2015. We sought to investigate the current landscape of EPA implementation and perceptions amongst fellowship training program directors. METHODS: This was a mixed-methods study with grounded theory employing both qualitative and quantitative assessments utilizing a survey instrument distributed to the Society of Pediatric Cardiology Training Program Directors from August 2023 to October 2023. Open-ended prompts were manually coded by two independent team members followed by theme creation. Demographics and descriptive statistics were summarized by frequencies with percentages and Likert-scale responses were tabulated. RESULTS: 24 of 62 pediatric cardiology program directors (39%) completed the study. >80% of respondents used a competency-based framework with/without milestones with ~ 40% utilizing EPA-based assessments. Programs utilizing EPA-based assessment report high satisfaction with their ability to capture trainee performance longitudinally. There is overall good familiarity with the EPA framework amongst all participants. Qualitative analysis revealed three themes: (1) EPA-based assessments provide objective, practical, goal-oriented assessments; (2) Significant effort and time are required from faculty and program directors assessing EPAs; (3) Major limiting factors assessing clinical fellows include a lack of comprehensive and consistent assessment from evaluators that may not necessarily be solved by the EPA framework alone. CONCLUSION: Near half of pediatric cardiology fellowship programs who responded in this study have begun to implement the EPA framework to facilitate trainee assessment. While there are perceived strengths with the addition of EPAs, early reservations also exist and continued follow-up should be done for both programs and training cohorts who were assessed under the EPA model.
Pulsed-Field Ablation for Paroxysmal Atrial Fibrillation in a Pediatric Patient.
Liu F, Hua Y, Li Y … +1 more , Zhou K
Pediatr Cardiol · 2026 May · PMID 42089929 · Publisher ↗
We report a case of a 12-year-old male patient presenting with paroxysmal atrial fibrillation (AF). Catheter ablation was indicated for symptomatic paroxysmal AF. The procedure was conducted utilizing a cardiac pulsed-fi...
We report a case of a 12-year-old male patient presenting with paroxysmal atrial fibrillation (AF). Catheter ablation was indicated for symptomatic paroxysmal AF. The procedure was conducted utilizing a cardiac pulsed-field ablation technology (LEAD-PFA, JJET). Lesion creation followed a systematic protocol comprising two sets of four consecutive applications at 1800 V targeting pulmonary veins, plus fifteen applications in inter-pulmonary vein regions and pulmonary venous vestibule. AF was induced during left inferior pulmonary vein ablation and terminated post-procedure. This case represents a documented successful ablation of paroxysmal AF utilizing PFA technology in a pediatric patient. These findings suggest that PFA may constitute a viable and potentially superior therapeutic modality for young patients with AF.
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