Pediatr Cardiol [JOURNAL]
Sun
200 papers
Transforming Pediatric Cardiology Fellowship Education: Multi-center Outcomes of the MACH-3 Virtual Reality Program for HLHS.
McCormick AD, Pritz BW, Yu S … +19 more , Lowery RE, Axelrod DM, Batsis M, Brown DW, Chau A, Diab KA, Dove ML, Hahn E, Handler SS, LaSalle EE, Mille FK, Parra DA, Patel SS, Pierick AR, Romans RA, Salavitabar A, Werho DK, Wilson HC, Owens ST
Pediatr Cardiol · 2026 Mar · PMID 41885933 · Publisher ↗
Previous work has shown that virtual reality (VR) curricula can improve basic understanding of congenital heart disease. The efficacy of using VR to teach more complex cardiac anatomy and physiology, such as hypoplastic...
Previous work has shown that virtual reality (VR) curricula can improve basic understanding of congenital heart disease. The efficacy of using VR to teach more complex cardiac anatomy and physiology, such as hypoplastic left heart syndrome (HLHS), is unknown. First-year fellows from thirteen pediatric cardiology fellowship programs participated in this study and were included in an intervention group or a control group. The intervention group completed the Michigan Anatomic Congenital Heart in 3D (MACH-3) curriculum. Both groups then completed a validated assessment tool. The total assessment score was calculated by summing the number of questions that participants answered correctly, ranging from 0 to 29. The difference in total assessment scores between the groups was examined using two-sample t-test and Cohen’s d effect size. A total of 56 fellows were included in the analysis. Participants in the intervention group achieved higher mean total assessment scores (n = 25, 23.8 ± 3.0 questions correct out of 29) than those in the control group (n = 31, 21.7 ± 3.9 questions correct) (p = 0.04), with a moderate effect size of 0.58. Most participants in the intervention group reported the curriculum was easy to use (96%) and enjoyable (92%), and all felt the curriculum improved their knowledge base (100%). In a multi-center educational study, a VR curriculum was shown to improve pediatric cardiology fellow knowledge of HLHS anatomy and physiology while receiving nearly universally positive feedback from participants. Future directions include expansion to include other complex cardiac lesions and investigation of incorporation of VR into fellowship curricula.
Medical Versus Surgical Therapy of Infected Percutaneous Pulmonary Valve.
ALJufan MB, Saleem M, Yansaneh AA … +7 more , Tsai FW, ALFadely FA, Alhazmi F, ALtuwariqi K, Abdelbaky N, Rezaiqi HA, AlAllaf A
Pediatr Cardiol · 2026 Mar · PMID 41885932 · Publisher ↗
This study compared medical therapy versus combined medical-surgical therapy of infective endocarditis (IE) after transcatheter pulmonary valve replacement (tPVR). Patients who underwent tPVR between January 2008 and May...
This study compared medical therapy versus combined medical-surgical therapy of infective endocarditis (IE) after transcatheter pulmonary valve replacement (tPVR). Patients who underwent tPVR between January 2008 and May 2022 were included in the study. Endocarditis was confirmed using the modified Duke Criteria. Medical therapy compared with medical-surgical therapy. A total of 387 patients underwent 404 tPVR procedures, 276 received Melody valves and 128 received Sapien valves. The cohort, 65% male with median age 19 years, was followed for 3,053 patient-years. Of these, 26 developed endocarditis. The annualized incidence rate of IE was 7.79 cases per 1000 patient-years (95% confidence interval (CI): 2.51–24.17) at 1 year and 6.96 cases per 1000 patient-years (95%CI: 3.75–12.94) at 5 years. Melody valve was associated with a significantly higher risk of IE than Sapien valve (11.40 vs. 2.90 per 1000 patient-years; incidence rate ratio (IRR) 3.94; p = 0.0058); this was confirmed in the reduced Cox proportional hazards model, HR 3.81 (95%CI: 1.12–12.91). Patients with IE were younger than those without IE (p = 0.017), and 77% of cases with IE received medical therapy whilst 23% received medical-surgical therapy. Among medically treated patients, 55% needed catheter re-intervention. The medical-surgical group experienced more severe symptoms, including increased pulmonary valve stenosis. Methicillin-resistant Staphylococcus aureus, Coxiella burnetii, and Streptococcus gordonii together accounted for 65% of cases. Medical therapy is effective for uncomplicated tPVR-endocarditis. The Melody valve has nearly fourfold higher endocarditis rate and increased mortality. Therefore, pre-implant assessment is recommended for younger high-risk patients.
Pulmonary Arterial Hypertension-Specific Therapy for Atrial Septal Defects with Severe Pulmonary Arterial Hypertension.
Nakayama S, Asano R, Tsuji A … +6 more , Ueda J, Hayashi H, Takano R, Fujisaki S, Hoshiga M, Ogo T
Pediatr Cardiol · 2026 Mar · PMID 41885931 · Publisher ↗
Pulmonary arterial hypertension (PAH) complicates a subset of patients with atrial septal defects (ASD). Although patients with ASD-PAH and high pulmonary vascular resistance (PVR) generally do not benefit, those who dem...
Pulmonary arterial hypertension (PAH) complicates a subset of patients with atrial septal defects (ASD). Although patients with ASD-PAH and high pulmonary vascular resistance (PVR) generally do not benefit, those who demonstrate a favorable response to PAH-specific therapy, particularly a PVR of < 5 Wood units (WU) post-treatment, may be considered for shunt closure. We aimed to examine the hemodynamic characteristics of patients with severe ASD-PAH who achieved a PVR of < 5 WU with PAH-specific therapy. This retrospective study evaluated 15 patients with severe ASD-PAH (PVR ≥ 5.0 WU) who underwent PAH-specific therapy. The effects of PAH-specific therapy and acute vasoreactivity testing (AVT) were examined. Patients who achieved PVR < 5 WU post-therapy were classified into the low-PVR group, and others into the high-PVR group. The PVR significantly decreased from 10.7 ± 5.5 to 7.0 ± 5.2 WU after oral PAH-specific therapy (p < 0.05). The maximum baseline PVR in the low-PVR group (n = 7, 47%) was 9.5 WU. The low-PVR group showed a greater change in PVR during AVT than the high-PVR group (44.9 ± 8.9% vs. 15.4 ± 15.4%, p < 0.01). The baseline PVR of 9.5 WU was the upper threshold for achieving PVR < 5.0 WU with oral PAH-specific therapy in severe ASD-PAH. Patients who exhibited a positive response to AVT achieved greater hemodynamic improvement.
What Limits Cardiorespiratory Fitness in Adolescents with Fontan Circulation: An Integrated Hemodynamic and Pulmonary Perspective.
Klungerbo V, Døhlen G, Holmstrøm H … +3 more , Edvardsen E, Matthews IL, Möller T
Pediatr Cardiol · 2026 Mar · PMID 41885930 · Publisher ↗
Cardiorespiratory fitness (CRF) is strongly associated with morbidity and mortality in patients with Fontan circulation. However, the factors influencing CRF during adolescence remain incompletely understood. The primary...
Cardiorespiratory fitness (CRF) is strongly associated with morbidity and mortality in patients with Fontan circulation. However, the factors influencing CRF during adolescence remain incompletely understood. The primary aim of this study was to investigate the associations between CRF and ventricular function represented by end-diastolic pressure (VEDP), the transpulmonary pressure gradient (TPG), somatic growth (height) and pulmonary diffusing capacity (DL/VA). The secondary aim was to assess the prevalence of exercise oscillatory ventilation (EOV) and its physiological associations. In this national, cross-sectional study, 44 adolescents (median age 16.6 years) with Fontan circulation underwent cardiopulmonary exercise testing, heart catheterization and pulmonary function assessment. Peak oxygen uptake (V̇O₂peak) was modelled as the dependent variable in a multiple linear regression with VEDP, TPG, height, and DL/VA as predictors. Logistic regression was used to identify physiological associations of EOV. The multiple regression model for absolute V̇O₂peak explained 61% of the overall variance. Lower VEDP and greater height were independently associated with higher V̇O₂peak. When V̇O₂peak was expressed relative to body mass, TPG also became associated with higher V̇O₂peak, while greater height and lower VEDP remained associated. This model explained 30% of the variance. DL/VA was not independently associated with V̇O₂peak. EOV was observed in 50% of patients but showed no association with V̇O₂peak, hemodynamic, or pulmonary variables. In adolescents with Fontan circulation, lower resting VEDP and somatic growth were associated with higher CRF, reflecting more efficient hemodynamics. Pulmonary diffusing impairment and EOV were common but not significantly associated with lower CRF.
Correction to: Prediction of Symptoms and Evaluation of Surgical Indications After Birth Based on Tracheal Morphology of Double Aortic Arch.
Ikegawa T, Kato A, Kawataki M … +3 more , Inagaki Y, Toyoshima K, Ueda H
Pediatr Cardiol · 2026 Mar · PMID 41879977 · Publisher ↗
Abstract loading — click title to view on PubMed.
Quantitative Electroencephalogram Patterns During Neonatal Antegrade Cerebral Perfusion and Deep Hypothermic Circulatory Arrest that Suggest Neurologic Injury.
Orie GA, Swartz MF, Seltzer L … +2 more , Yoshitake S, Alfieris GM
Pediatr Cardiol · 2026 Mar · PMID 41879976 · Publisher ↗
During cerebral ischemia, the quantitative Electroencephalogram (EEG) Alpha: Delta Ratio (ADR) decreases, and the asymmetry percentage increases. The utility of analyzing these metrics during neonatal Antegrade Cerebral...
During cerebral ischemia, the quantitative Electroencephalogram (EEG) Alpha: Delta Ratio (ADR) decreases, and the asymmetry percentage increases. The utility of analyzing these metrics during neonatal Antegrade Cerebral Perfusion (ACP) or Deep Hypothermic Circulatory Arrest (DHCA), and whether significant EEG changes precede neurologic injury after pediatric cardiac surgery, remains unknown. Neonates requiring DHCA or ACP with intraoperative EEG monitoring were retrospectively reviewed, and the left and right anterior, posterior, and hemispheric ADRs and asymmetry percentages were measured every 5 min throughout cardiopulmonary bypass. A paired right versus left cerebral ADR difference of > 25% or an asymmetry percentage of > 50% was considered significant for ischemia, and the cumulative duration of time was calculated. Neurologic injury was defined as postoperative stroke or seizure. Among 86 neonates, 14 (16.2%) underwent a period of DHCA and 72 (83.8%) ACP. The ADR remained similar during ACP but decreased significantly during DHCA. The asymmetry percentage remained similar during DHCA but increased significantly during ACP. From the 11 neonates (ACP = 7, DHCA = 4) that developed neurologic injury, although there were no significant differences in the duration of significant asymmetry, neonates with neurologic injury had longer anterior right vs. left ADR differences > 25% (25 [IQR 0-65] vs. 5 [IQR 0-15] minutes, p < 0.001), which was confirmed using multivariate analysis (OR 1.044; 95% CI 1.010-1.080; p = 0.012). The ADR decreased significantly during DHCA, the asymmetry percentage increased during ACP, and a prolonged anterior right vs. left ADR difference preceded neurologic injury.
The Incidence and Spectrum of Congenital Heart Defects in Patients with Anorectal Malformations: A 10-Year Retrospective Cohort Study.
Shalaby MM, Shalaby HK, Taha FA
Pediatr Cardiol · 2026 Mar · PMID 41879975 · Publisher ↗
Congenital heart defects (CHD) and anorectal malformations (ARM) frequently co-occur, but contemporary large-scale data are limited. This study aimed to define CHD incidence, spectrum, and risk factors in ARM patients. W...
Congenital heart defects (CHD) and anorectal malformations (ARM) frequently co-occur, but contemporary large-scale data are limited. This study aimed to define CHD incidence, spectrum, and risk factors in ARM patients. We conducted a retrospective cohort study of infants with ARM at a tertiary pediatric center (2013–2022). Demographic, clinical, and echocardiographic data were analyzed. Primary outcome was CHD incidence; secondary outcomes included CHD types and associations with ARM complexity and VACTERL features. Among 314 ARM patients, 92 had CHD (incidence 29.3%, 95% CI: 24.3–34.7%). Significant CHD requiring intervention/surveillance occurred in 48 patients (52.2% of CHD cohort). Common CHD types included atrial septal defects (ASD) (28.3%), ventricular septal defects (VSD) (26.1%), and tetralogy of Fallot (TOF) (10.9%). Complex ARM patients had significantly higher CHD incidence versus simple ARM (52.9% vs. 23.1%, p < 0.001). ≥2 VACTERL (Vertebral, Anorectal, Cardiac, Tracheo-Esophageal, Renal, Limb anomalies) features predicted CHD (OR: 4.8, 95% CI: 2.9–8.1). Nearly one-third of ARM patients had concomitant CHD, with over half being clinically significant. ARM alone confers increased CHD risk, with VACTERL features further elevating this risk. Universal echocardiographic screening is imperative in all ARM patients.
Hemodynamic Effects of Methylene Blue in Pediatric Cardiac Critical Care Patients: A Descriptive Study of Causal Inference and Cluster Analysis Using High-Fidelity Data.
Loomba RS, Akbasli IT, Flores S … +3 more , Farias J, Savorgnan F, Sourour W
Pediatr Cardiol · 2026 Mar · PMID 41874659 · Publisher ↗
Methylene blue inhibits the nitric oxide-soluble guanylyl cyclase-cyclic GMP pathway and is used to restore vascular tone in vasoplegic circulatory failure. Its hemodynamic and oxygenation effects in children, however, r...
Methylene blue inhibits the nitric oxide-soluble guanylyl cyclase-cyclic GMP pathway and is used to restore vascular tone in vasoplegic circulatory failure. Its hemodynamic and oxygenation effects in children, however, remain incompletely defined. A single-center retrospective, mechanistic, physiology-focused analysis of pediatric cardiac intensive care unit patients who received methylene blue for vasoplegia was conducted. High-fidelity physiologic data were extracted from Sickbay. Percent change in vasoactive score, mean arterial blood pressure, cerebral near infrared spectroscopy, and renal near infrared spectroscopy within four hours after administration were calculated relative pre-dose baseline. Unsupervised cluster analysis identified physiologic response phenotypes, and random forest regression models were used to determine which pre-administration variables predicted response magnitude. Independent variables included vasoactive medications, fluid balance, sedation, and patient characteristics. 19 administrations across 15 unique patients were analyzed. Cluster analysis revealed 3 distinct response phenotypes including a group with significant improvement in adequacy of oxygen delivery and a group without such increase. Random forest modeling identified methylene blue dose, vasopressin dose, and epinephrine dose as important response prediction features. Calcium chloride augmented response while vasopressin blunted it. Data from this descriptive study suggest that methylene blue may improve arterial blood pressure, and adequacy of oxygen delivery in critically ill pediatric patients with vasoplegia. The greatest response occurred in patients with higher catecholamine but lower vasopressin doses, consistent with a catecholamine-responsive, nitric oxide-mediated phenotype. Calcium appeared to augment methylene blue response while vasopressin blunted it. These findings provide a physiologic framework for targeted methylene blue use in pediatric circulatory failure.
Pulmonary Artery Growth Among Duct-Dependent Physiology After Ductal Stenting Compared to Modified Blalock-Taussig Shunt: Insights from a Systematic Review and Meta-Analysis.
Megantara HP, Utami JR, Addina CY
Pediatr Cardiol · 2026 Mar · PMID 41874658 · Publisher ↗
Early palliation of duct dependent pulmonary circulation aims to secure adequate pulmonary blood flow before definitive repair. Two common strategies are transcatheter ductal stenting and the surgical modified Blalock Ta...
Early palliation of duct dependent pulmonary circulation aims to secure adequate pulmonary blood flow before definitive repair. Two common strategies are transcatheter ductal stenting and the surgical modified Blalock Taussig shunt. Whether these approaches differ in pulmonary artery growth and branch symmetry remains uncertain. This meta-analysis compared pulmonary artery growth and symmetry after ductal stenting versus modified Blalock Taussig shunt in duct dependent pulmonary blood flow. Five electronic databases (PubMed, Scopus, Cochrane, ScienceDirect and Google Scholar) were searched up to December 2025. Comparative studies reporting quantitative pulmonary artery growth or symmetry (Nakata index, McGoon ratio, and pulmonary artery symmetry index [PASI]) were pooled using random effects meta-analysis and expressed as mean difference (MD) with 95% confidence intervals (CI). Heterogeneity was assessed using I2 and study quality using the Newcastle Ottawa Scale. Nine studies involving 1,460 patients met inclusion criteria. Ductal stenting was associated with a lower PASI (MD -0.16; 95% CI -0.26 to -0.05; p = 0.003; I2 = 94%), suggesting more symmetric branch pulmonary artery development. Pulmonary artery growth assessed by the Nakata index (MD 24.08; 95% CI -17.98 to 66.14; p = 0.26; I2 = 98%) and McGoon ratio (MD-0.02; 95% CI -0.44 to 0.39; p = 0.92; I2 = 90%) did not differ significantly between groups. Ductal stenting was associated with modestly improved pulmonary artery symmetry and similar global pulmonary artery growth compared with the modified Blalock Taussig shunt. Interpretation is limited by high heterogeneity and observational study design. Prospective multicenter studies are needed to clarify patient selection and long term outcomes.
Association of Music Therapy and Physiologic Indices in the Pediatric Cardiac Intensive Care Unit: Insights from Machine Learning and High-fidelity Physiologic Data.
Loomba RS, Hales G, Chauhan A … +2 more , Sourour W, Ward K
Pediatr Cardiol · 2026 Mar · PMID 41872485 · Publisher ↗
Music therapy is increasingly used in critically ill children, yet its real-time physiologic effects remain incompletely characterized. This study used high-fidelity physiology data to characterize the physiologic respon...
Music therapy is increasingly used in critically ill children, yet its real-time physiologic effects remain incompletely characterized. This study used high-fidelity physiology data to characterize the physiologic response to music therapy in pediatric cardiac intensive care unit patients. Physiologic data were extracted from Sickbay. Heart rate, mean arterial blood pressure, central venous pressure, arterial saturation, respiratory rate, end tidal carbon dioxide, and renal near infrared spectroscopy were analyzed from 1 h before through 4 h after the start of therapy. Percent change from baseline was modeled using multivariable regression adjusting for participation level, therapy technique, circulatory physiology, mechanical ventilation, mechanical circulatory support, sedation, and vasoactive inotrope score. Cluster analysis of session-level percent changes was conducted to identify physiologic response phenotypes. A total of 52 music therapy sessions across 33 unique patients were included in the final analyses. Music therapy was independent associated with changes in heart rate (+ 3%, p = 0.03), central venous pressure (+ 10%, p < 0.01), end tidal carbon dioxide (+ 11%, p < 0.01), and renal near infrared spectroscopy (+ 3%, p = 0.02). Cluster analysis revealed two physiologic response clusters: one demonstrating stable hemodynamics and improved adequacy of oxygen delivery and another demonstrating heightened respiratory activity but no improvement in the adequacy of oxygen delivery. Cluster 1, with improvement in the adequacy of oxygen delivery, was associated with active patient participation. Music therapy was independently associated with physiologic changes. In a majority of patients, the adequacy of oxygen delivery improved and was associated with active patient participation.
International Experience of Transcatheter Sinus Venosus Defect Closure in Children and Adolescents.
Thejaswi P, Sagar P, Sivakumar K … +15 more , Chattopadhyay A, Francis E, Surucu M, Demir IH, Celebi A, Rosenthal E, Bautista C, Kenny D, Hascoet S, Baruteau AE, Boudjemline Y, Hijazi ZM, AlMaskari S, Abdullah HA, Angtuaco MJ
Pediatr Cardiol · 2026 Mar · PMID 41863591 · Publisher ↗
Transcatheter closure of sinus venosus defects (SVD) is an emerging alternative to surgery, however, concerns remain regarding growth potential in paediatric patients treated with fixed-diameter stents. This retrospectiv...
Transcatheter closure of sinus venosus defects (SVD) is an emerging alternative to surgery, however, concerns remain regarding growth potential in paediatric patients treated with fixed-diameter stents. This retrospective study included individuals younger than 18 years from 12 centres. SVD closure was performed using covered stents after demonstration of right upper pulmonary vein redirection during balloon interrogation. Complete closure without significant residual flow was defined as procedural success. Major complications were those necessitating surgical intervention, whereas minor complications were managed intraprocedurally. Among 54 patients < 18 years, 17 had bilateral superior vena cava and six had a high-draining vein. The majority (37) underwent single-stent placement; others required multiple stents. Procedural success was 98.1%, with one embolization needing surgery. Seven needed extra stents due to migration or instability. Minor complications included arrhythmia and stent thrombus. Compared to global registry data, this cohort required smaller sheaths and fewer stents. Pulmonary vein protection and jugular-railroad techniques were frequently employed; transseptal puncture was less frequent. Over a follow-up period ranging 1-174 months, three cases of residual shunt necessitated reintervention. No late stent thrombosis was noted. One deep vein thrombosis and two instances of mild asymptomatic pulmonary vein gradients were managed conservatively. Transcatheter closure of SVD is both feasible and safe in paediatric populations. Smaller anatomical structures permitted use of single-stents through smaller sheaths, thereby streamlining the intervention. Continued long-term surveillance will be essential to further elucidate benefits in young patients. Future advancements in stent-technology and increased procedural expertise are expected to enhance outcomes.
Pulmonary Hypertension in Pediatric Patients with Noonan Syndrome Undergoing Cardiac Catheterization.
Collins SL, Critser PJ, Gauvreau K … +3 more , Porras D, Roberts AE, Mullen MP
Pediatr Cardiol · 2026 Mar · PMID 41863590 · Publisher ↗
Noonan syndrome (NS) is an autosomal dominant genetic disorder associated with a high incidence of cardiovascular disease. We investigated pulmonary hypertension (PH) in patients with NS, characterizing the prevalence, g...
Noonan syndrome (NS) is an autosomal dominant genetic disorder associated with a high incidence of cardiovascular disease. We investigated pulmonary hypertension (PH) in patients with NS, characterizing the prevalence, genetic, and clinical features including hemodynamics, cardiovascular interventions, and transplant-free survival. A retrospective, descriptive cohort study was conducted among pediatric patients with NS who underwent cardiac catheterization. Patient demographic, clinical, and hemodynamic data were collected for cohorts of patients with and without PH. We identified 87 patients with NS who had undergone cardiac catheterization including hemodynamics at Boston Children's Hospital between 2000 and 2020. Of them, 59 (68%) had a NS-associated genetic mutation in the RAS-MAPK signaling pathway, while 28 (32%) had a clinical diagnosis of NS. In the cohort, 11/87 (13%) had PH. There were significant differences in NS-associated genetic mutations; variants in RAF1 (p = 0.013) and KRAS (p = 0.015) were more common among PH patients. Hypertrophic cardiomyopathy (p = 0.009) was more common in PH patients, while valvar, subvalvar, or supravalvar PS (p = 0.011) was less common. PH patients had more aortic-valve interventions (p = 0.025) and fewer valvar right-sided heart interventions (p = 0.037). Transplant-free survival was lower in the PH group than the non-PH group (p = 0.004). There are novel features to cardiovascular outcomes for genetically defined subsets of patients with NS, including significant differences in the NS-associated causative genes, associated hypertrophic cardiomyopathy, structural heart disease, interventions, and transplant-free survival.
Severe Aortic Regurgitation with New Congestive Heart Failure Following Fetal Aortic Valvuloplasty - A Case Report.
Hochpoechler J, Tulzer G, Tulzer A
Pediatr Cardiol · 2026 Mar · PMID 41862724 · Publisher ↗
Fetal aortic valvuloplasty (FAV) is performed in fetuses with critical aortic stenosis (CAS) to prevent progression to hypoplastic left heart syndrome (HLHS). While successful FAV often results in new-onset aortic regurg...
Fetal aortic valvuloplasty (FAV) is performed in fetuses with critical aortic stenosis (CAS) to prevent progression to hypoplastic left heart syndrome (HLHS). While successful FAV often results in new-onset aortic regurgitation (AR), this typically improves during gestation. We present a rare case of persistent severe AR following FAV, complicated by fetal congestive heart failure. A 28-year-old woman was referred at 23 + 0 weeks of gestation due to CAS of the fetus. Fetal echocardiography showed CAS with a peak velocity of 2.79 m/s, significant LV dysfunction and grade 2–3 endocardial fibroelastosis. No signs of heart failure were seen. The decision to perform FAV was made and the aortic valve was dilated to 115% of the valve diameter. After successful intervention, fetal echocardiography on the next day showed improved antegrade aortic valve flow with severe AR and signs of new RV dysfunction. Three days after intervention, development of ascites was observed. Due to further increase of ascites and poor cardiac function, the already established transplacental digitalization was increased. In the following weeks, hemodynamic stabilization could be observed with right ventricular function gradually improving and ascites resolving completely by 37 weeks of gestation. After elective C-section, the decision to perform early Ross-Konno surgery on the second day of life was made due to severe diastolic runoff. This case highlights the drastic hemodynamic changes associated with new significant AR after FAV and shows that successful in-utero treatment of congestive heart failure in this condition is possible.
Correction: Comprehensive Functional Echocardiographic Assessment of Transposition of the Great Arteries: From Fetus to Newborn.
Walter C, Soveral I, Bartrons J … +5 more , Escobar-Diaz MC, Carretero JM, Quirado L, Gómez O, Sánchez-de-Toledo J
Pediatr Cardiol · 2026 Jun · PMID 41843015 · Publisher ↗
Abstract loading — click title to view on PubMed.
Modification of Nicks Technique for Aortic Annular Enlargement in Children.
Masaki N, Kumae M, Sai S
Pediatr Cardiol · 2026 Mar · PMID 41843014 · Publisher ↗
We present a case of a 9-year-old boy with severe aortic insufficiency and left ventricular dilatation. The patient underwent aortic valve replacement with annular enlargement using a modified Nicks technique. This appro...
We present a case of a 9-year-old boy with severe aortic insufficiency and left ventricular dilatation. The patient underwent aortic valve replacement with annular enlargement using a modified Nicks technique. This approach successfully enlarged the aortic annulus, enabling the implantation of a larger prosthesis. The procedure was performed without any complications, offering a potential surgical strategy to prevent postoperative patient-prosthetic mismatch in pediatric patients with aortic valve disease.
Diastolic Function Unchanged After Lifestyle Intervention Despite Improved VO in Pediatric Heart Transplant Patients.
Brennan A, Mardy C, Punn R … +1 more , Tierney S
Pediatr Cardiol · 2026 Mar · PMID 41843013 · Publisher ↗
Pediatric heart transplant recipients commonly exhibit diastolic dysfunction, which may contribute to reduced exercise capacity. In our Healthy Hearts study, a supervised exercise and nutrition intervention improved exer...
Pediatric heart transplant recipients commonly exhibit diastolic dysfunction, which may contribute to reduced exercise capacity. In our Healthy Hearts study, a supervised exercise and nutrition intervention improved exercise performance and endothelial function. We hypothesized that this intervention would also improve resting echocardiographic markers of diastolic function in this cohort. Fourteen stable heart transplant patients aged 8 to 19 years, at least one year post-transplant, participated in a live-video-supervised exercise and nutrition intervention from 2014 to 2018. Participants completed a 12-16-week intervention, followed by a maintenance phase of similar duration with mostly self-directed sessions. Echocardiograms taken near baseline, post-intervention, and post-maintenance visits were reviewed retrospectively. Diastolic dysfunction at baseline was defined by any of the following: E/A ratio > 2, mitral deceleration time < 160 ms, septal or lateral E/e' > 14, or tricuspid regurgitant jet velocity > 2.8 m/s. Atrial strain was measured using TOMTEC software. No significant improvements from baseline to post-intervention were observed in conventional diastolic function parameters or atrial strain, including patients with baseline diastolic dysfunction (n = 9). Among those with baseline diastolic dysfunction, there was a strong inverse correlation between the change in E/A ratio and the change in peak VO from baseline to post-intervention (r = - 0.94). Despite improvements in exercise capacity and endothelial function following a nutrition and exercise intervention in pediatric heart transplant patients, echocardiographic markers of diastolic function remained unchanged. These findings suggest that peripheral rather than cardiac adaptations may underlie the observed functional improvements.
Performance of Kawasaki Disease Risk Scores in a 30-Year Regional Spanish Cohort.
Viadero MT, Caldeiro MJ, Garde J … +5 more , Suárez NF, Gijón I, Llorca J, Cabero MJ, González-Lamuño D
Pediatr Cardiol · 2026 Mar · PMID 41843012 · Publisher ↗
Several risk scores have been developed to predict intravenous immunoglobulin (IVIG) resistance and coronary artery involvement in Kawasaki disease (KD), but their performance outside Asian populations remains uncertain....
Several risk scores have been developed to predict intravenous immunoglobulin (IVIG) resistance and coronary artery involvement in Kawasaki disease (KD), but their performance outside Asian populations remains uncertain. To evaluate the diagnostic performance of major KD risk scores in predicting IVIG resistance and coronary artery involvement in a regional cohort from Northern Spain. Retrospective observational study including 81 KD patients diagnosed at a tertiary pediatric center (1994–2024). We applied the following IVIG-resistance scores: Kobayashi, Egami, Sano, Kawanet, Kawanet-echo, Kawa-Race, and the Spanish Pediatric Association (AEP) consensus. For coronary involvement prediction, the Son score and the Kawa-Race coronary score were used. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were calculated, restricting IVIG-resistance analyses to patients who received IVIG as initial therapy. An exploratory stratified analysis according to therapeutic era (pre-2017 vs. ≥ 2017), reflecting the progressive introduction of adjunctive corticosteroids, was performed. IVIG resistance occurred in 7/81 (8.6%) and coronary artery involvement (dilatation or aneurysm) in 23/81 (28.4%). Most IVIG-resistance scores showed low sensitivity but high negative predictive value, while the AEP criteria achieved maximal sensitivity at the expense of very low specificity. Coronary involvement frequently occurred in IVIG-responsive patients (20/23, 87.0%). For coronary prediction, the Son score at the ≥ 2-point threshold showed the most balanced performance (sensitivity 34.8%, specificity 93.1%), whereas the Kawa-Race coronary score demonstrated high specificity (94.8%) but very low sensitivity (13.0%). Stratified analysis by therapeutic era showed persistently low positive predictive values and heterogeneous sensitivity across IVIG-resistance scores, with no clear improvement in discriminative performance after the introduction of adjunctive corticosteroid therapy. KD risk scores showed limited sensitivity for identifying children at risk of coronary artery involvement in this Western cohort and this limited performance remained consistent across therapeutic eras, including after the progressive introduction of adjunctive corticosteroid therapy. The marked discordance between IVIG resistance and coronary outcomes suggests that refractoriness-based prediction alone may be insufficient to guide early treatment intensification. A sequential strategy integrating early clinical assessment with coronary-focused prediction tools may support individualized management. Prospective multicenter validation is warranted.
Neo-Aortic Valve Insufficiency and Root Dilatation During and After the Staged Palliation for Hypoplastic Left Heart Syndrome and Variants.
Matsubara M, Telescu I, Schaeffer T … +9 more , Lemmen T, Röhlig C, Palm J, Heinisch PP, Piber N, Hager A, Ewert P, Hörer J, Ono M
Pediatr Cardiol · 2026 Mar · PMID 41831014 · Publisher ↗
In hypoplastic left heart syndrome (HLHS) and variants, the pulmonary valve functions as the neo-aortic valve after the Norwood procedure. Neo-aortic valve insufficiency (Neo-AI) represents an emerging complication with...
In hypoplastic left heart syndrome (HLHS) and variants, the pulmonary valve functions as the neo-aortic valve after the Norwood procedure. Neo-aortic valve insufficiency (Neo-AI) represents an emerging complication with implications for long-term survival. This study investigated the clinical impact and patterns of Neo-AI development following staged palliation. Neonates undergoing the Norwood procedure (2001–2023) were retrospectively reviewed. Neo-AI was evaluated using serial echocardiograms, with patients classified into two groups: early onset (during the Norwood hospitalization) and late onset (after the Norwood discharge). Neo-aortic root dimensions were measured at four time points during staged palliation, with Z-scores calculated for patient growth. Among 378 patients, significant Neo-AI developed in 30 (7.9%) during median follow-up of 4.3 years. Early onset neo-AI occurred in 13 patients (43.3%) and late onset neo-AI in 17 (56.7%). Cox regression revealed 7.9-fold increased mortality risk in early onset patients (hazard ratios 7.87, p = 0.009), with 5-year survival of 38.5% versus 87.8% in late onset patients. All early onset deaths occurred within the first year. Late onset neo-AI patients demonstrated progressive sinus of Valsalva dilatation (annual progression 0.33 Z-score units). At Fontan completion, late onset neo-AI patients had larger Z-scores than early onset patients [5.14 vs. 2.18, p = 0.006], while surviving early onset patients demonstrated Neo-AI improvement after bidirectional cavopulmonary shunt with subsequent stable root dimensions. Early onset neo-AI associates with acute hemodynamic compromise and higher perioperative mortality. Late onset neo-AI demonstrates progressive root dilatation with superior survival, raising concerns for future valve intervention.
Prenatal Diagnosis of Obstructive Accessory Mitral Valve Tissue.
Zou P, Xu X, Huang J … +1 more , Sun S
Pediatr Cardiol · 2026 Mar · PMID 41831013 · Publisher ↗
Accessory mitral valve tissue (AMVT) is a rare congenital cardiac anomaly. The majority of affected patients are under 18 years of age, and AMVT may lead to left ventricular outflow tract (LVOT) obstruction. We present a...
Accessory mitral valve tissue (AMVT) is a rare congenital cardiac anomaly. The majority of affected patients are under 18 years of age, and AMVT may lead to left ventricular outflow tract (LVOT) obstruction. We present a case of a 33-year-old pregnant woman (gravida 2, para 2) who underwent detailed fetal echocardiography at 28 weeks of gestation, which demonstrated obstructive AMVT. At 4 months of age, the infant underwent surgical resection owing to an increased pressure gradient across the LVOT and ventricular septal thickening at the LVOT. This case highlights the diagnostic value of fetal echocardiography in identifying AMVT and the importance of regular postnatal follow-up.
Technical Feasibility of Virtual Reality-Enhanced Three-Dimensional Rotational Angiography: A Retrospective Analysis of a Novel Imaging Approach for Interventional Procedures in the Pediatric Cardiac Catheterization Laboratory.
Szeliga J, Cabaj-Włodarczyk K, Góreczny S
Pediatr Cardiol · 2026 Mar · PMID 41831012 · Publisher ↗
Three-dimensional rotational angiography (3DRA) is useful in congenital interventional cardiology but remains limited by two-dimensional screen visualization, which may restrict comprehensive analysis of complex cardiova...
Three-dimensional rotational angiography (3DRA) is useful in congenital interventional cardiology but remains limited by two-dimensional screen visualization, which may restrict comprehensive analysis of complex cardiovascular anatomy. Virtual reality (VR) enables immersive visualization of volumetric data. However, the technical feasibility of VR models derived from intraprocedural 3DRA remains insufficiently explored. To evaluate the technical feasibility and processing time of VR models generated from 3DRA in pediatric cardiac catheterizations, focusing on anatomical assessment, procedural simulation and fluoroscopic projection planning. This retrospective single-centre study included 62 3DRA guided procedures performed in 2024. Virtual and conventional work station based (C-3D) models were generated from anonymized 3DRA-datasets. Models were evaluated using a structured framework across four predefined categories: assessment of target-site anatomy, visualization beyond the target region, procedural simulation capability, and theoretical fluoroscopic projection planning. All assessments were performed by a multidisciplinary clinical team. VR models were successfully generated in all cases. Median VR processing times were 3.0 min for model generation, 5.0 min for refinement, 4.0 min for procedural simulation, and 7.0 min for extended anatomical analysis, defined as immersive evaluation of anatomical structures beyond the primary contrast-opacified target region. Visualization of target anatomy was feasible in 98%, beyond-target region in 84%, procedural simulation in 94%, and projection planning in all cases. Semi-quantitative comparative analysis demonstrated statistically significant deviation from the neutral reference value for target-site anatomy (p = 0.0318) and beyond-target visualization (p = 0.0495). VR-based assessment of 3DRA datasets is technically feasible and enables immersive anatomical evaluation in a heterogeneous pediatric population with congenital heart disease, providing a technical basis for future prospective studies of VR-enhanced analysis of intraprocedural 3DRA data.
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