Pediatr Cardiol [JOURNAL]
Sun
200 papers
Pulmonary Artery Diameters in Children, Teenagers and Young Adults Derived from Quiescent Interval Slice Selective (QISS) Magnetic Resonance Angiography.
Zalas-Piotrowiak D, Rozewicz Juraszek M, Gabbert DD … +3 more , Krupickova S, Caliebe A, Voges I
Pediatr Cardiol · 2026 Jun · PMID 42340447 · Publisher ↗
The radial Quiescent Interval Slice Selective (QISS) technique, which is used in cardiovascular magnetic resonance (CMR), is a reliable, contrast-free technique for obtaining high-quality vascular images. It is distingui...
The radial Quiescent Interval Slice Selective (QISS) technique, which is used in cardiovascular magnetic resonance (CMR), is a reliable, contrast-free technique for obtaining high-quality vascular images. It is distinguished by its safety, insensitivity to motion and arrhythmias, and its overall simplicity and adaptability. However, previously published studies assessing pulmonary artery (PA) dimensions in children have not yet incorporated this technique. CMR scans of 139 patients (95 males), aged 6 weeks - 20 years, with no evidence of PA disorders were analyzed. Main PA (MPA), right PA (RPA) and left PA (LPA) diameters and cross-sectional areas (CSA) were measured at three locations, respectively, using the radial QISS technique. CMR was most performed for aortic pathology (n = 80), followed by other congenital heart diseases (n = 15), cardiomyopathies (n = 14) and others (n = 30). Consistent decreases in vessel calibre from proximal to distal MPA, RPA and LPA segments were demonstrated. Males showed slightly larger PA diameters and CSA than females at all measured locations. Univariate regression analysis demonstrated highest associations with body height for most PA parameters. This study employed the radial QISS method to present data for PA diameters and CSA in children and young adults. These values may help to detect pulmonary vascular abnormalities and inform clinical decisions regarding intervention and management.
Efficacy of Right Ventricle-to-Pulmonary Artery Conduit as a Pulmonary Blood Source at Unifocalization of Major Aortopulmonary Collateral Arteries.
Yuasa E, Hoashi T, Kojima T … +5 more , Hirano A, Hosoda R, Fuchigami Y, Iijima Y, Suzuki T
Pediatr Cardiol · 2026 Jun · PMID 42329380 · Publisher ↗
Multimodality treatment with catheter-based intervention is still commonly used for pulmonary atresia with ventricular septal defect (PA/VSD) or hemi-truncus with major aortopulmonary artery collateral arteries (MAPCAs)...
Multimodality treatment with catheter-based intervention is still commonly used for pulmonary atresia with ventricular septal defect (PA/VSD) or hemi-truncus with major aortopulmonary artery collateral arteries (MAPCAs) requiring unifocalization. A total of 12 patients with PA/VSD with MAPCAs (n = 10) or hemi-truncus (n = 2) who had undergone unifocalization since 1994 were enrolled. The development of the central pulmonary artery (cPA) was absent in 2 patients, diminutive (< 2 mm) in 3, sizable (> 2 mm) in 5, and unilateral (hemi-truncus) in 2. Treatment strategy was determined according to the morphology of the central PA. VSD closure was not performed at the same time of unifocalization. The source of pulmonary blood flow from completion of unifocalization to VSD closure was either systemic to pulmonary artery shunt (SPS) or right ventricle-to-pulmonary artery (RV-PA) conduit. A total of 39 catheterizations were performed. Median follow-up was 13.2 years [interquartile range: 2.3-16.5 years]. All patients achieved VSD closure. Cumulative survival rate was 83.3% at 10 years. Median RV to aortic pressure (AoP) ratio during VSD closure was 0.57 [0.51-0.75]. After a patient with partial anomalous pulmonary venous connection and pulmonary hypertension was eliminated RV/AoP was lower in RV-PA conduit (n = 5) than in SPS cases (n = 6) (0.49 vs. 0.76, p = 0.014). RV-PA conduit as a source of pulmonary blood flow facilitates blood flow control and effective catheter-based intervention to reconstructed PA after unifocalization then contributed maintaining low RV pressure at VSD closure.
CardioMEMS Utilization in Pediatric Heart Failure: A Single Center Experience.
Benvenuto V, Rockefeller T, Barnes A … +4 more , Birnbaum B, Garner K, Juhl R, Sutcliffe D
Pediatr Cardiol · 2026 Jun · PMID 42329379 · Publisher ↗
CardioMEMS is an innovative device that offers the ability to monitor pulmonary artery pressures remotely. This provides a less invasive way to monitor hemodynamic changes and allow for earlier intervention with the aim...
CardioMEMS is an innovative device that offers the ability to monitor pulmonary artery pressures remotely. This provides a less invasive way to monitor hemodynamic changes and allow for earlier intervention with the aim of reducing heart failure complications and need for cardiac catheterization. However, the use of CardioMEMS in pediatric heart failure is limited. We performed a retrospective review of all patients presenting for CardioMEMS placement in our center. Safety outcomes were based on cases of device embolization and tissue injury. Effectiveness outcomes included ability to reliably obtain pressure readings remotely. Overall, 20 pediatric patients presented to the catheterization lab. 18 patients had successful implantation with a CardioMEMS device in our center, with median age of 14.3 years (14-18.5) and median weight at implant of 57.6 kg (40.3-63). The smallest implanted patient was 28 kg. In those implanted there was no clinical evidence of device embolization, thrombosis, nor pulmonary artery injury during, or after, implant. Overall, there were minimal issues obtaining remote readings however, one patient with Fontan circulation was unable to reliably obtain readings post-placement. There is a significant decrease in reading frequency further out from implant. In the largest description to date of CardioMEMS usage in pediatric patients, we demonstrate that the CardioMEMS device can be a safe and effective tool in the management of pediatric heart failure with more data needed to further optimize the use of CardioMEMS in pediatric patients.
Clinical Utility of Acute Maternal Hyperoxygenation Testing for Fetal D-Transposition of the Great Arteries.
Madan N, Hancock H, Donofrio MT … +5 more , Warta A, Cloutier D, Brattrud K, Landes Z, Kiaffas M
Pediatr Cardiol · 2026 Jun · PMID 42322354 · Publisher ↗
Acute maternal hyperoxygenation (MH) testing may be useful to predict need for balloon atrial septostomy (BAS) in fetal d-transposition of the great arteries/intact ventricular septum (d-TGA/IVS). We present our d-TGA/IV...
Acute maternal hyperoxygenation (MH) testing may be useful to predict need for balloon atrial septostomy (BAS) in fetal d-transposition of the great arteries/intact ventricular septum (d-TGA/IVS). We present our d-TGA/IVS MH experience. MH was performed in late third trimester with O2 at 8 L/min via a non-rebreather mask for 10 min followed by evaluation of septum primum position, patent foramen ovale (PFO) dimension, and pulmonary vasoreactivity (PV). Mean gestational age of 18 d-TGA/IVS fetuses at MH and birth were 35.9 and 39 weeks respectively. Nine newborns underwent BAS. During MH, in BAS group, septum primum phasically centralized but stayed leftwards of the septum secundum in 8/9 fetuses. Atrial septum was assessed in 8/9 fetuses in the no BAS group. In all 8, septum primum was bidirectional phasically crossing rightwards of the septum secundum. Mean MH sagittal PFO dimension between BAS and no BAS groups were 2.5 ± 0.6 and 4.7 ± 0.9 mm respectively (p < 0.001). PV was noted in 15/16 fetuses with available data. Interobserver reliability for septum primum position showed 100% agreement. d-TGA/IVS fetuses commonly demonstrate PV with MH. Smaller PFO dimension and septum primum not crossing rightwards of the septum secundum despite PV predicted need for BAS. Larger prospective studies are needed to validate these findings.
The FUEL FALD Study: Effects of Udenafil on Liver Stiffness and Fibrosis after Fontan.
Schumacher KR, Dillman J, Goldberg DJ … +21 more , Cartoski M, DiMaria M, Frischhertz B, Ginde S, Goldstein BH, Kasi N, Lee CK, Leung DH, Mao CY, McCrindle BW, Menon S, O'Connor M, Opotowsky AR, Palermo J, Paridon SM, Penny D, Rathod R, Wagner JB, Yetman A, Zak V, Russell M
Pediatr Cardiol · 2026 Jun · PMID 42322353 · Publisher ↗
Fontan-associated liver disease (FALD) is a fibrotic liver disease in individuals with Fontan physiology. This study sought to determine the efficacy of treatment with udenafil, a phosphodiesterase type 5 inhibitor, for...
Fontan-associated liver disease (FALD) is a fibrotic liver disease in individuals with Fontan physiology. This study sought to determine the efficacy of treatment with udenafil, a phosphodiesterase type 5 inhibitor, for reducing liver congestion leading to a decreased liver stiffness and reduction in serologic biomarkers of liver fibrosis. In patients enrolling in the FUEL trial open label extension, liver stiffness measurements were obtained by ultrasound shear wave elastography (SWE) and magnetic resonance elastography (MRE); serum markers of liver fibrosis were measured by the Enhanced Liver Fibrosis (ELF) score. Change in liver stiffness and ELF score after 12 months of udenafil treatment were analyzed. In 88 subjects, 36 were previously treated with udenafil and 52 were udenafil-naïve. The cohort's baseline liver stiffness was elevated by SWE, mean 2.0 ± 0.4 m/sec (adult upper limit normal 1.5 m/sec), and MRE, mean 5.3 ± 1.2 kPa (adult upper limit normal 2.8 kPa). After 12 months of udenafil treatment, there was no change in SWE (post-treatment mean 1.9 ± 0.3 m/sec, p = 0.28) nor change in MRE (post-treatment mean 5.2 ± 0.97 kPa, p = 0.68). The cohort's baseline ELF score was elevated at 10.4 ± 1.0 according to values correlating with liver fibrosis in other diseases. ELF score decreased at 12 months to 9.8 ± 0.8 (p < 0.0001). Liver stiffness did not change following 12 months of udenafil treatment, but ELF scores significantly decreased. Further study is needed to understand the relationship of ELF score to age, but these results suggest the possibility that udenafil may alter the overall Fontan fibrotic milieu.
Percutaneous Recanalization of Chronic Total Inferior Vena Cava Occlusion During ASD Closure.
Dowaikh A, Almohizy O, Alsahari A … +1 more , Momenah T
Pediatr Cardiol · 2026 Jun · PMID 42322352 · Publisher ↗
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Bifurcating Patent Ductus Arteriosus: A Rare Anomaly Associated with a Congenital Portosystemic Shunt.
Taha AZ, Abou Zahr R, Al-Ata J … +2 more , El Mahdi Y, Mohammad Nijres B
Pediatr Cardiol · 2026 Jun · PMID 42322351 · Publisher ↗
Malformation of the ductus arteriosus (DA) is uncommon in otherwise healthy newborns. We report an extremely rare aneurysmal DA with bifurcation, in which the superior branch connects at the typical location on the roof...
Malformation of the ductus arteriosus (DA) is uncommon in otherwise healthy newborns. We report an extremely rare aneurysmal DA with bifurcation, in which the superior branch connects at the typical location on the roof of the main pulmonary artery, while the inferior branch connects to the left aspect of the left pulmonary artery origin. This unusual DA configuration is associated with an intrahepatic congenital portosystemic shunt.
Combined Analysis by Peak and Submaximal Exercise Parameters Delineates Underlying Mechanisms of Poor Exercise Performance in Fontan Patients.
Tsuda T, Kernizan D, Hossain MJ
Pediatr Cardiol · 2026 Jun · PMID 42313130 · Publisher ↗
Fontan patients have reduced exercise performance primarily due to the absence of a sub-pulmonary ventricle mitigating ventricular filling during exercise, but other factors may be involved. Comprehensive cardiopulmonary...
Fontan patients have reduced exercise performance primarily due to the absence of a sub-pulmonary ventricle mitigating ventricular filling during exercise, but other factors may be involved. Comprehensive cardiopulmonary exercise testing (CPET) analysis may delineate the underlying mechanisms. We retrospectively reviewed CPET parameters of Fontan and age-matched control adolescents. Both peak and submaximal parameters were analyzed. Fifty-one Fontan patients (15.4 ± 3.5 years old, male 21) and 65 age-matched controls (14.7 ± 2.0 years old, male 32) were studied. Peak values of heart rate (HR), oxygen consumption (VO2)/kg, and work rate (WR)/kg were significantly lower in Fontan than control patients in both sexes. Peak oxygen pulse (pOP/kg) and submaximal slope of [VO2/kg]/HR (Δ[VO2/kg]/ΔHR), surrogates of stroke volume, were significantly reduced whereas ΔHR/Δ[WR/kg], HR-dependency, was significantly increased in Fontan patients, indicating limited stroke volume reserve during exercise resulting in higher HR increase during exercise despite lower peak HR. When dividing Fontan patients into upper and lower halves at the average pVO2/kg, the upper half showed significantly higher peak WR/kg, ventilatory anaerobic threshold (VAT)/kg, oxygen uptake efficiency slope (OUES)/kg, and ΔVO2/ΔWR (aerobic work efficiency), suggesting the difference in muscle conditioning and breathing efficiency. There were some sex differences in CPET parameters that predict exercise performance among Fontan patients. Our proposed CPET assessment methods can address the underlying exercise physiology of Fontan patients. Enhancing muscle conditioning and ventilatory efficiency by routine exercise may help improve functional reserve of Fontan patients, which can be monitored by these CPET parameters.
Characteristics and Outcomes of Patients with Regression of Coronary Artery Aneurysms Caused by Kawasaki Disease.
Tsuda E, Kotoku A, Ito Y
Pediatr Cardiol · 2026 Jun · PMID 42313129 · Publisher ↗
Most medium to small-sized coronary artery aneurysms (CAAs) caused by Kawasaki disease (KD) can regress. To clarify the outcomes of patients with regressed-CAAs (r-CAAs). We reviewed characteristics and outcomes in 139 p...
Most medium to small-sized coronary artery aneurysms (CAAs) caused by Kawasaki disease (KD) can regress. To clarify the outcomes of patients with regressed-CAAs (r-CAAs). We reviewed characteristics and outcomes in 139 patients (male, 101; female, 38) with r-CAAs between 1978 and 2022 using medical records, including coronary angiograms and coronary computed tomography angiograms (CCTAs). The prevalence of localized stenosis (LS) and outcomes were calculated using the Kaplan-Meier method. Furthermore, the cut-off points of the maximum CAA diameter in the initial angiogram for coronary artery calcification (CAC) in late CCTAs were analyzed. The 25-year incidence of LS was 9.8% (95% CI, 3.6-23.8), and the 25-year cardiac event-free survival rate was 95.3% (95% CI 72.9-99.3). All LS cases were associated with CAC. Cut-off values of the initial maximum CAA diameter for CAC in branches and at bifurcations were 4.9 mm (n = 129, AUC 0.859, p < 0.0001) and 5.7 mm (n = 43, AUC 0.951, p = 0.0013), respectively. The corresponding Z scores were 6.75 (AUC 0.846, p < 0.0001) and 6.65 (AUC 0.921, p = 0.0014), respectively. The cut-off value of the interval time from the onset of KD to CAC detection was 10.0 years (AUC 0.673, p = 0.0053) in branches. LS with CAC in patients with r-CAAs can occur more than 10 years after the onset of KD. CAC after CAA regression is likely to appear at a maximum diameter of more than 5.0 mm (z score 6.7) in young adults; however, it may appear with aging in smaller r-CAAs.
Radiation Reduction Through the Use of a Novel Ultra-Low Dose Fluoroscopy Imaging Protocol in Congenital Cardiac Catheterization.
Shibbani K, Justino H, El-Said H … +1 more , Gordon BM
Pediatr Cardiol · 2026 Jun · PMID 42313128 · Publisher ↗
Increased radiation exposure during congenital heart disease procedures may have a detrimental effect on patients, especially in the pediatric population. Advances in imaging systems and adherence to the ALARA (As Low As...
Increased radiation exposure during congenital heart disease procedures may have a detrimental effect on patients, especially in the pediatric population. Advances in imaging systems and adherence to the ALARA (As Low As Reasonably Achievable) principle are paramount in minimizing radiation exposure. This is a single center, retrospective study designed to understand the degree of radiation reduction achievable with a novel ultra-low dose fluoroscopy protocol (ULDFP). Patients were included in the study if they underwent any one of the following procedures between 12/1/2021 and 12/31/2023: atrial septal defect (ASD) device closure, coarctation balloon or stent angioplasty, patent ductus arteriosus (PDA) closure, pulmonary valvuloplasty, and aortic valvuloplasty. Our radiation doses were compared to published benchmarks and current registry data. Phantom studies were done to evaluate the radiation reduction achieved by using the novel ULDFP as compared to our standard protocol. We report significant reduction in radiation exposure for pediatric patients undergoing interventional cardiac catheterization procedures achieved predominantly by near-complete elimination of cine angiography and use of ULDFP. Values reported in this study represent a dose reduction of greater than 90% compared to current benchmark doses by Cevallos [1] et al. and Lamers [2] et al., and greater than 85% compared to current CRISP registry data. Use of an ULDFP and the near elimination of cine angiography drastically reduces radiation dose in pediatric patients undergoing cardiac catheterization procedures even when compared to current registry data. Reducing radiation exposure in this vulnerable cohort may potentially play a significant role in lowering morbidity associated with cardiac catheterization.
Long-term CMR Findings in Pediatric COVID-19 Related Myocarditis.
Vasquez Choy AL, Adebo DA, Patel MD … +3 more , Uppu SC, Kharouf RM, Xiong L
Pediatr Cardiol · 2026 Jun · PMID 42313127 · Publisher ↗
Cardiac magnetic resonance (CMR) is a key modality for diagnosing and monitoring myocarditis, including cases related to COVID-19 infection and Multisystem Inflammatory Syndrome in Children (MIS-C). While acute findings...
Cardiac magnetic resonance (CMR) is a key modality for diagnosing and monitoring myocarditis, including cases related to COVID-19 infection and Multisystem Inflammatory Syndrome in Children (MIS-C). While acute findings are often favorable, the persistence of abnormalities over time remains uncertain. This retrospective study included 21 pediatric patients with suspected COVID-19-related myocarditis between March 2020 and July 2023. All underwent CMR as part of clinical evaluation. Imaging findings were analyzed at early (< 30 days), mid (30-364 days), and long-term (≥ 365 days) intervals. Myocarditis was defined using the 2009 Lake Louise criteria. Of the 21 patients (mean age 13.1 years, 57% male), 8 had follow-up CMRs (median time to follow-up 347 days). Etiologies included MIS-C (57%), confirmed COVID-19 (19%), and suspected COVID-19 (24%). A total of 30 CMR studies were analyzed. Initial CMR met 2009 Lake Louise criteria in 8 patients (38%). Across temporally categorized studies, LGE declined from 46% of early studies to 25% of mid-term studies and was absent on long-term studies; pericardial effusions similarly declined from 46% to 25% to 0%. EGE remained frequent across timepoints (77%, 83%, and 80%, respectively). Among patients with serial CMR, no patient had edema on follow-up, LGE persisted in 1 of 8 (13%), pericardial effusion in 1 of 8 (13%), and only 1 of 8 (13%) continued to meet Lake Louise criteria. Biventricular systolic function remained normal on follow-up imaging. Initial LGE demonstrated marked lateral wall predominance (Cochran's Q = 13.38, p = 0.004). Median clinical follow-up was 20 months (IQR 11-38), with no documented arrhythmias, heart failure symptoms, cardiac-related rehospitalizations, or mortality. In pediatric myocarditis associated with COVID-19, most CMR abnormalities improve over time, with resolution of edema and substantial reduction in LGE and pericardial effusion. Persistent EGE remains common, although its clinical significance is uncertain. Medium-term clinical outcomes were favorable. Larger prospective studies are needed to define the long-term significance of persistent imaging abnormalities.
Association of LVOT Gradient and LA Strain with Cardiac Events in Pediatric HCM.
Gearhart A, Epstein SF, Gauvreau K … +6 more , Ferraro A, Mangano CM, Roth I, Harrild D, Colan SD, Chen MH
Pediatr Cardiol · 2026 Jun · PMID 42313126 · Publisher ↗
In adults with hypertrophic cardiomyopathy (HCM), left ventricular outflow tract (LVOT) obstruction on exercise stress echocardiography (ESE) and reduced left atrial (LA) strain increase heart failure and cardiac outcome...
In adults with hypertrophic cardiomyopathy (HCM), left ventricular outflow tract (LVOT) obstruction on exercise stress echocardiography (ESE) and reduced left atrial (LA) strain increase heart failure and cardiac outcome risk. However, limited pediatric data exist. We evaluated the association of exercise-induced LVOT obstruction and LA strain with cardiac events in young HCM patients. Patients who underwent ESE (n = 114, 32%F, median = 17 yrs) from 2014-2022 at a single center were stratified by LVOT gradients: Group 1 (n = 44, rest/exercise gradients < 30mmHg); Group 2 (n = 41, rest < 30mmHg; exercise ≥ 30mmHg); Group 3 (n = 29, rest/exercise ≥ 30mmHg). LA reservoir (LASr), conduit (LAScd), and contractile strain were analyzed with TOMTEC AutoStrain LA. Composite cardiac event included: heart failure, non-sustained/sustained ventricular tachycardia, cardiac syncope, heart transplantation, and HCM-related death. At follow-up (median = 2.0 years), there was a greater hazard of the composite event in Group 3 vs. 2 (hazard-ratio [HR] = 4.97, p = 0.013). Severe events were rare and largely observed in Group 3. Increased HR was seen in Group 2 vs. 1 but the difference was not significant. Adjusting for LVOT gradient (Group 3), lower LASr or LAScd or increased LA stiffness (E/e' to LASr ratio) were associated with composite event (HR LASr = 1.57; LAScd = 1.70; LA stiffness = 1.31; all p < 0.01). Concordance indices were similar for these three strain/stiffness models (0.81-0.82) and higher than in models including LVOT group with traditional diastolic indices (LA volume index or E/e'; c-index = 0.76). In one of the largest pediatric HCM cohorts undergoing ESE, reduced LA strain demonstrated greater incremental value to LVOT gradient than traditional diastolic indices when evaluating association with cardiac events.
Utility of the Frontal-Plane QRS-T Angle in a Real-World Screening Population, for Identification of Hypertrophic Cardiomyopathy.
Wayne KS, Liu H, Canales KK … +1 more , Cortez D
Pediatr Cardiol · 2026 Jun · PMID 42313125 · Publisher ↗
The frontal QRS-T angle, derived from a standard 12-lead electrocardiogram (ECG), quantifies the relationship between ventricular depolarization and repolarization. Although a widened angle has been associated with myoca...
The frontal QRS-T angle, derived from a standard 12-lead electrocardiogram (ECG), quantifies the relationship between ventricular depolarization and repolarization. Although a widened angle has been associated with myocardial disarray and arrhythmic risk, its diagnostic utility for detecting hypertrophic cardiomyopathy (HCM) in children, adolescents, and young adults has not been well established. We analyzed 6,128 participants aged ≤ 30 years from the Heartfelt community cardiovascular screening program. HCM was defined as septal or posterior wall thickness ≥ 2.5 SD above the mean for body-surface area in pediatric participants or ≥ 15 mm in adults. In a secondary analysis, the ≥ 15 mm threshold was applied irrespective of age. Electrocardiographic variables were evaluated using logistic regression and receiver operating characteristic (ROC) analyses. Among the 6,128 screened participants, 162 met criteria for HCM. Compared with unaffected individuals, participants with HCM were younger and had shorter PR intervals, QRS durations, and QTc values (all p < 0.001). The frontal QRS-T angle did not differ significantly between groups (23° [12-34] vs 24° [14-36]; p = 0.175) and demonstrated poor discriminatory performance (AUC 0.47; 95% CI 0.43-0.51). Findings were similar in the secondary analysis applying the ≥ 15 mm threshold across all ages (AUC 0.43; p = 0.67). In this large cohort of young community-screened participants, the frontal QRS-T angle did not distinguish individuals with hypertrophic cardiomyopathy. These findings suggest that adult-derived electrocardiographic thresholds may not be directly applicable to pediatric or athletic populations and highlight the need for age-specific ECG screening criteria.
Design and Implementation of My Health, My Heart: A Pilot Healthcare Transition Program for Adolescents with Congenital Heart Disease.
Park A, Szymanski L, Wevers M … +5 more , Mathews A, Saraf S, Phillippi R, Feindt C, John AS
Pediatr Cardiol · 2026 Jun · PMID 42307674 · Publisher ↗
Effective healthcare transition for congenital heart disease (CHD) patients to adult-centered care remains a significant challenge. Virtual, family-centered transition programs incorporating individualized goal setting m...
Effective healthcare transition for congenital heart disease (CHD) patients to adult-centered care remains a significant challenge. Virtual, family-centered transition programs incorporating individualized goal setting may improve readiness. My Health, My Heart is a 12-month virtual, family centric transition program for patients (12-22 years) with CHD. It includes a social work assessment, individualized goal setting, and sessions focused on medical knowledge, care coordination, and mental health. Validated assessments (transition readiness, health knowledge, PedsQL 4.0, PROMIS anxiety and depression, and parental PSS) were delivered at baseline and completion. Primary outcome was feasibility (enrollment, retention, and barriers); secondary outcomes included changes in transition readiness, medical knowledge, and quality of life. A total of 68 participants (median age 16[13.75-18], 38% F] enrolled, with 41 (60%, median age 16[13-18], 39% F) patients completing both goals. Attrition was highest (37%) from completion of initial surveys to the first goal. Participants reported improvements in confidence managing heart health (median score 6.5[4.25-8] to 8[6-9]) and transition readiness (median score 5[4-8] to 7[5-9]). Quality-of-life scores increased significantly (PedsQL total: 79.4 ± 15.3 → 84.4 ± 15.7, p = 0.006). Among completers, 80% of participants and 90% of caregivers reported being very or extremely satisfied. Qualitative feedback identified ongoing needs in mental health support, practical life skills, and peer connection. The My Health, My Heart telemedicine transition program is feasible and suggests early improvements in quality of life and transition readiness, underscoring the importance of individualized support and caregiver engagement.
Home is Where the Heart is: Disparities Identified with a Digital Program for Congenital Heart Surgery Follow-Up.
Bacaj C, Fryberger L, Gauvreau K … +3 more , Liu H, Roy N, Nathan M
Pediatr Cardiol · 2026 Jun · PMID 42307673 · Publisher ↗
Consistent longitudinal follow-up of congenital heart surgery (CHS) patients is often difficult to achieve and varies based on cardiac complexity and families' socioeconomic status. We assessed CHS patient/caregiver resp...
Consistent longitudinal follow-up of congenital heart surgery (CHS) patients is often difficult to achieve and varies based on cardiac complexity and families' socioeconomic status. We assessed CHS patient/caregiver responses to our questionnaire-based follow-up program, Discharge Communications (DisCo), to address disparities in follow-up. Single-center retrospective review of consecutive patients discharged after CHS between 10/2016 and 5/2024. Outcomes included response rate and responses to questions at 30-days and 1-year post-discharge. Child Opportunity Index (COI) was categorized as very low, low, moderate, high, and very high using home address. Covariates were patient/procedural characteristics. Associations between response rates and covariates were evaluated. DisCo received responses from 5438 (88%) of 6211 30-day questionnaires and 3767 (70%) of 5372 1-year questionnaires. Upon analysis, respondents (versus non-respondents) were less likely to have low or very low COI and more likely to have very high COI (p < 0.001, both). Other significant predictors included age, procedural complexity, race, and ethnicity. Patients with lower COI levels (versus very high) were more likely to report rehospitalization (p = 0.002 at 30-day, p = 0.011 at 1-year) and surgical reintervention (p = 0.012 at 1-year). Patients with very low or low COI, Black or Other race, and Hispanic ethnicity were more likely to respond by phone call versus other outreach (p < 0.001, all). DisCo is a useful digital tool for staying in touch with families, particularly with populations vulnerable to loss of follow-up. While child opportunity was associated with adverse outcomes, optimizing DisCo outreach may be an effective strategy to identify disparities in CHS follow-up.
The Prevalence of Adverse Childhood Experiences in the Congenital Heart Disease Population in Los Angeles County.
Mehta K, Draycott C, Parsons JR … +4 more , Thompson N, Detterich J, Williams R, Thyne SM
Pediatr Cardiol · 2026 Jun · PMID 42287431 · Publisher ↗
Adverse Childhood Experiences (ACEs) correlate with increased risk of cardiovascular disease in adulthood and children with congenital heart disease (CHD) are also at increased risk of negative cardiovascular outcomes as...
Adverse Childhood Experiences (ACEs) correlate with increased risk of cardiovascular disease in adulthood and children with congenital heart disease (CHD) are also at increased risk of negative cardiovascular outcomes as adults. This study sought to identify the prevalence of ACEs in the pediatric CHD population in Los Angeles County and to identify any differences in healthcare utilization between pediatric patients with and without CHD. Data on children ages 0-26 with at least one ACE score in the study period were collected. Pediatric patients with CHD were identified from this cohort and propensity matching across gender, race and ethnicity, and age was performed using the patients with CHD as the case population. Statistical analysis was performed to evaluate the prevalence of ACEs in the CHD population and controls and to evaluate healthcare utilization differences. 249 pediatric patients with CHD were identified of the 45,000 patients who receive primary care within a safety net hospital system in LA County. When comparing ACE scores between CHD patients and controls, patients with CHD were less likely to have any exposure to ACEs (p = 0.0093) and less likely to have 1-3 ACEs (p = 0.028) than their matched cohorts. Pediatric patients with CHD had significantly higher rates of healthcare utilization as compared to their propensity-matched peers. The prevalence of ACEs in a large CHD population is lower than a group of propensity-matched patients without CHD, and children with CHD had significantly more interactions with the healthcare system. This may provide some early insight into protective factors against ACEs in a high-risk population of pediatric patients.
Impact of Irrigated Contact-Force Sensing on Fluoroscopy Exposure and Efficiency in Pediatric AVNRT Ablation.
Akif Atlan M, Oksuz S, Yıldız K … +8 more , Cirkinoglu GG, Dedecengiz H, Bacak T, Alimoglu B, Gerceker E, Elmalı F, Narin N, Karadeniz C
Pediatr Cardiol · 2026 Jun · PMID 42257767 · Publisher ↗
Atrioventricular nodal reentrant tachycardia (AVNRT) is one of the most common forms of supraventricular tachycardia in children and adolescents. Radiofrequency (RF) catheter ablation is an established and effective trea...
Atrioventricular nodal reentrant tachycardia (AVNRT) is one of the most common forms of supraventricular tachycardia in children and adolescents. Radiofrequency (RF) catheter ablation is an established and effective treatment; however, the close anatomical relationship between the slow pathway and the compact atrioventricular (AV) node poses a risk of conduction system injury. Irrigated contact-force (CF) sensing catheters provide real-time feedback on catheter-tissue interaction and may enhance lesion delivery and procedural efficiency. Despite increasing use in adult populations, data regarding their use in pediatric AVNRT remain limited. To evaluate the safety, efficacy, and procedural characteristics of CF-guided RF ablation versus conventional non-contact-force (non-CF) catheters in pediatric patients undergoing slow-pathway modification for AVNRT. This retrospective study included consecutive pediatric patients (< 18 years) who underwent AVNRT ablation between January 2021 and December 2024. Patients were treated using either an irrigated CF-sensing catheter (n = 130) or a non-CF catheter (n = 48). Continuous variables were analyzed according to their distribution, and appropriate statistical tests were applied. Time-to-recurrence was assessed using Kaplan-Meier survival analysis with log-rank testing. The CF group demonstrated significantly shorter procedure time (median 58 vs. 90 min; p < 0.001), less frequent fluoroscopy use (1.5% vs. 14.6%; p = 0.002), and shorter RF duration (73 vs. 89.5 s; p = 0.024) compared with the non-CF group. Acute procedural success was achieved in all patients in the CF group and in 89.6% of patients in the non-CF group (p = 0.001). No major complications were observed in either group. Recurrence rates were numerically higher in the CF group (6.1% vs. 2.1%; p = 0.448). Kaplan-Meier analysis demonstrated a statistically significant difference in recurrence-free survival (log-rank p = 0.015); however, this finding should be interpreted cautiously due to differences in follow-up duration and the limited number of events. Irrigated CF-guided RF ablation appears to be a safe and effective option for the treatment of pediatric AVNRT. CF-guided ablation was associated with improved procedural efficiency and high acute success. Differences in recurrence patterns should be interpreted cautiously in light of potential confounding factors, including follow-up duration and temporal learning effects.
Late Gestation Fetal Growth in Infants Undergoing Stage 1 Palliation for Single Ventricle Heart Disease.
Schneider K, Marcuccio E, Cnota JF … +4 more , Heydarian H, Gao Z, Khoury PR, Hill GD
Pediatr Cardiol · 2026 Jun · PMID 42257766 · Publisher ↗
Improved understanding of late gestation fetal growth patterns and postnatal outcomes in fetuses with single ventricle heart disease may inform decisions about elective delivery timing. This was a retrospective cohort an...
Improved understanding of late gestation fetal growth patterns and postnatal outcomes in fetuses with single ventricle heart disease may inform decisions about elective delivery timing. This was a retrospective cohort analysis of patients in the National Pediatric Cardiology Quality Improvement Collaborative database from 2016 to 2019 anticipated to need stage 1 palliation (S1P) and had a gestational age (GA) of ≥ 36 weeks. Birthweight (BW) and BW z-scores were compared across GA. Survival to stage 2 palliation (S2P), post-S1P length of stay, and duration of mechanical ventilation were compared by GA. In 1290 infants, the mean BW increased with increasing GA (p < 0.001). Mean BW z-scores were below zero in late gestation (p < 0.001), with declining growth velocity 38 to > 40 weeks GA. Of 1216 patients with mechanical ventilation data, those at later GA had shorter durations of ventilation (p < 0.001). Among 1083 survivors to discharge or S2P if not discharged, infants born at later GA had shorter S1P hospitalizations (p = 0.015) and were more likely to undergo S2P (p = 0.003). Infants with single ventricle heart disease requiring S1P have decreased late gestation growth velocity, but growth continues. Infants born after longer gestation have shorter duration of ventilation and greater likelihood of undergoing S2P.
Adenosine Dose and Cardioversion in Pediatric Supraventricular Tachycardia: A Systematic Review and Meta-Analysis.
Dos Santos L, Shehan TM, Lang C … +4 more , Algarin RS, de Oliveira Ribeiro IB, de Mesquita PCMD, de Carvalho GD
Pediatr Cardiol · 2026 Jun · PMID 42246961 · Publisher ↗
Although adenosine is recommended as first-line therapy for pediatric supraventricular tachycardia (SVT), the optimal weight-based dose for reliable first-pass cardioversion remains uncertain. To evaluate the association...
Although adenosine is recommended as first-line therapy for pediatric supraventricular tachycardia (SVT), the optimal weight-based dose for reliable first-pass cardioversion remains uncertain. To evaluate the association between weight-based adenosine dosing and cardioversion probability in children with SVT. PubMed, Embase, Scopus, and the Cochrane Library were searched from inception through July 2025. Of 3,195 records identified, 9 observational studies including 480 patients and 1,369 intravenous adenosine bolus administrations met inclusion criteria. Two reviewers independently screened studies and extracted bolus-level cardio version data. Risk of bias was assessed with ROBINS-I and certainty of evidence with GRADE. Logit-transformed cardioversion proportions were pooled using a three-level random-effects model to account for clustering of multiple bolus administrations within studies. Successful cardioversion to sinus rhythm following an individual intravenous adenosine bolus. Conversion probability increased across prespecified weight-based dose categories: 25% (95% CI, 14%-40%) at doses < 100 µg/kg, 38% (95% CI, 31%-46%) at 100 µg/kg, 47% (95% CI, 39%-55%) at 150-225 µg/kg, and 59% (95% CI, 48%-69%) at 250-400 µg/kg. In the three-level model, overall conversion probability was 41% (95% CI, 35% to 47%). Increasing dose was associated with higher odds of cardioversion (β = 0.0054 per µg/kg; OR per 100 µg/kg, 1.72 [95% CI, 1.43 to 2.07]; P < .001), with residual heterogeneity primarily attributable to between-study variability. Higher weight-based adenosine doses were consistently associated with greater conversion probability across observational cohorts. These findings suggest that commonly used initial doses may underperform in many children and justify prospective trials evaluating mechanism-specific initial dosing strategies.
Infliximab as an Effective Second-Line Treatment for IVIG-Resistant Kawasaki Disease Accompanied by Severe Dyslipidemia.
Sunaga Y, Hasebe Y, Yoshizawa M … +16 more , Kono Y, Koizumi K, Katsumata N, Toda T, Hoshiai M, Kobayashi K, Hokibara S, Oshiro H, Sawanobori E, Sano T, Ohta M, Tsuruta M, Nakamura M, Sato K, Sato H, Inukai T
Pediatr Cardiol · 2026 Jun · PMID 42240641 · Publisher ↗
For high-dose intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD), individualized selection of second-line therapy according to clinical subgroups could be useful to prevent progression of coronary artery a...
For high-dose intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD), individualized selection of second-line therapy according to clinical subgroups could be useful to prevent progression of coronary artery abnormalities. We retrospectively analyzed IVIG-resistant KD patients aged ≥ 1 year treated with additional IVIG (n = 70) or infliximab (IFX) (n = 54) by applying clustering approaches. Consistent with previous studies, fever resolved significantly faster in the IFX group (median 72 h) than in the second IVIG group (96 h; p = 0.02). However, the incidence of residual coronary artery abnormalities (Z-score ≥ 2.5) at 30 days, 6 months, and 1 year did not differ significantly between the two treatment groups. In the first clustering approach, which was based on ten baseline laboratory variables, the two identified subgroups were not clearly associated with differential treatment responses. In a secondary clustering analysis using four treatment-associated factors (serum sodium, C-reactive protein, total cholesterol, and HDL cholesterol), the three identified stable subgroups were associated with differential treatment responses. Among the three clusters, Cluster 1 showed a favorable response to second IVIG (resistance rate, 7/36 [19.4%]) but a relatively poor response to IFX (12/28 [42.9%]). In contrast, Clusters 2 and 3, characterized by dyslipidemia with decreased total cholesterol and HDL cholesterol levels, were resistant to second IVIG (7/17 [41.2%] and 8/17 [47.1%], respectively) but relatively sensitive to IFX (0/7 [0%] and 6/19 [31.6%], respectively). These findings suggest that IFX may be an effective second-line therapy for IVIG-resistant KD patients with severe dyslipidemia, whereas additional IVIG may remain beneficial for those without dyslipidemia.
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