Pediatric Congenital Heart Disease Patients Research Articles

Associations Between Clinical Factors and Postoperative Thrombosis in Pediatric Cardiac Surgery Patients: A Single-Center Retrospective Study.

Postoperative thrombosis is a significant complication in pediatric cardiac surgery patients, contributing to morbidity and mortality. Identifying clinical factors associated with thrombosis can improve patient outcomes by guiding early detection and intervention. This study aimed to assess factors associated with postoperative thrombosis or thromboembolism in pediatric patients under 12 months old who underwent surgery for congenital heart disease (CHD). Design, Setting, and Participants: This retrospective cohort study analyzed electronic medical records from pediatric patients admitted to the Pediatric Cardiovascular Intensive Care Unit (PCICU) at the German Paediatric Heart Center, Bonn, between March 1, 2020, and March 1, 2021. A total of 197 children under 12 months old who underwent cardiac surgery were included in the analysis. Thrombosis was diagnosed postoperatively using imaging modalities such as ultrasound, echocardiography, and computed tomography. The primary outcome was the incidence of thrombosis and its association with clinical factors such as age, central venous catheter (CVC) duration, CRP levels, and D-dimer levels. Among 197 patients, the incidence of thrombosis was 8.63%, predominantly venous (70.6%). Initial associations were observed between thrombosis and younger age, lower body weight, higher hematocrit, cyanosis, longer central venous catheter (CVC) use, and elevated C-reactive protein (CRP) and d-dimer levels. Receiver operating characteristic analysis indicated a higher risk in patients with d-dimer levels above 5.47 mg/L. The stepwise multiregression analysis identified longer CVC duration in situ (β = 0.553; p < 0.001), higher CRP levels (β = 0.217; p = 0.022), and younger age at admission (β = -0.254; p = 0.006) as significant predictors of thrombosis. Decision tree analysis identified CVC use longer than 12.5 days and CRP levels above 118.01 mg/L as the most critical risk factors. Postoperative thrombosis is a notable risk in pediatric CHD patients, particularly in neonates. Prolonged CVC use and elevated CRP levels are critical risk factors. Routine monitoring of D-dimer and CRP levels, along with timely sonographic screening, can aid early thrombosis detection and intervention. Further research is warranted to optimize thrombosis prevention strategies in this population.

Caught in the Act: A Detailed Analysis of Cardiac Event Monitoring in a Cohort of Pediatric and ACHD Patients.

Event monitors are being increasingly used in pediatric and adult congenital heart disease (ACHD) patients for arrhythmia evaluation. Data on their diagnostic yield are limited. To evaluate the diagnostic yield of event monitors, patient characteristics associated with critical events, and clinical response to events. We retrospectively assessed event monitors prescribed to patients at our institution's Heart Center from 2017 to 2020. Thirty-day event monitor tracings were reviewed by an electrophysiologist (EP) to identify critical events defined as supraventricular tachycardia (SVT, re-entrant, atrial tachycardia, atrial flutter, and atrial fibrillation), ventricular tachycardia (VT), atrioventricular block, and pauses greater than 3 s. Patient characteristics and treatment data were collected. Characteristics associated with events were assessed using multivariable logistic regression. Trends in monitor prescription over time, diagnostic yield, and clinical response to events were analyzed. 204/2330 (8.8%) event monitors had EP-confirmed critical events. Critical events included SVT (51.5%), VT (38.5%), atrioventricular block (4%), and pauses (6%). 129/198 (65%) patients with critical events underwent treatment. Event monitoring usage increased by 52% between 2017 and 2020 (p<0.0001). Complex CHD (OR 2.1, 95% CI 1.3-3.4, p=0.004), cardiomyopathy (OR 2.9, 95% CI 1.5-4.8, p<0.001), and EP-ordered monitors (OR 1.6, 95% CI 1.2-2.1, p=0.001) were more highly associated with critical events. Event monitor use is common, and critical events were captured in 8.8% of patients. The majority of patients with critical events underwent treatment. Factors associated with critical events include EPs as ordering providers, complex CHD, and cardiomyopathy.

Implementing Patient Protection Radiation Dose Alerts for Pediatric Cardiac Catheterization Examinations

Advancements in cardiac catheterization have improved survival for pediatric congenital heart disease patients, but the associated ionizing radiation risks necessitate ethical consideration. This study presents an empirical model to establish reference air kinetic energy released per unit mass (KERMA) and air KERMA area product, also referred to as dose area product, alert levels based on pediatric patient size during diagnostic or interventional cardiac catheterization. Recognizing the significant size variations among pediatric patients, the model provides a universal application for institutions to set quality assurance programs to prevent overexposure. Developed from 3131 unique procedures, the model uses linear regression of logarithmic reference air KERMA and dose area product against the patient's lateral thickness of the thorax for various procedural categories, setting alert levels at the top 95% and 99% of patient data. This allows institutions to tailor dose alert levels to their specific pediatric populations.

Combined Exercise and Mindset Training During a Pediatric Cardiac Fitness and Rehabilitation Program Benefits Youth with a Range of Congenital Heart Disease.

Pediatric cardiac fitness and rehabilitation programs vary widely in structure and content. The Cardiac Fitness Program (CFP) is built on traditional training pillars of aerobic, strength, and flexibility, and adds a fourth, training a positive mindset. This study assesses whether the systematic and comprehensive framework of the CFP results in broad benefits for a range of patients with congenital heart disease (CHD). Data from participants between 01/2017 and 12/2022 were analyzed. Pre- and post-CFP cardiopulmonary exercise test parameters, strength and flexibility metrics, and mindset survey results were compared overall, and by sex, age, diagnosis, and hemodynamic level. Of 62 participants (median age 15.5years, range 8 to 23, 50% female), 3% had simple, 37% complex, 24% single ventricle CHD, and 35% arrhythmia, cardiomyopathy, or transplant. Significant improvements were noted in aerobic fitness (mean 9 ± 15% increase in % predicted peak oxygen consumption, p < 0.001). Strength metrics significantly improved (each p < 0.001), as did flexibility (p < 0.001). Patient-reported positive mindset scores did not improve significantly (mean increase 1.8 ± 5.1, p = 0.10, n = 25), whereas parents reported significant improvements (5.9 ± 10.4, p = 0.02, n = 20). Improvements were not significantly different by sex, age, diagnosis, or hemodynamic level. Comprehensive training across four pillars of fitness yields significant improvements in aerobic fitness, strength, flexibility, and parent-reported mindset scores for pediatric CHD patients, regardless of patient characteristics, diagnosis type, or severity of hemodynamic limitation. Further study is warranted into optimal standardization of training and whether a comprehensive approach amplifies individual pillars to create more than the sum of its parts.

Quantitative assessment of preoperative brain development in pediatric congenital heart disease patients by synthetic MRI

ObjectivesThis study investigated the quantitative assessment and application of Synthetic MRI (SyMRI) for preoperative brain development in children with congenital heart disease (CHD).MethodsForty-three CHD patients aged 2–24 months were prospectively included in the observation group, and 43 healthy infants were included in the control group. The SyMRI scans were processed by postprocessing software to obtain T1, T2, and PD maps. The values of T1, T2, and PD in different brain regions were compared with the scores of the five ability areas of the Gesell Development Scale by Pearson correlation analysis.ResultsIn the observation group, the T1 values of the posterior limb of the internal capsule (PLIC), Optic radiation (PTR), cerebral peduncle, centrum semiovale, occipital white matter, temporal white matter, and dentate nucleus were greater than those in the control group. In the observation group, the T2 values of the PLIC, PTR, frontal white matter, occipital white matter, temporal white matter, and dentate nucleus were greater than those in the control group. Pearson correlation analysis revealed that the observation group had significantly lower Development Scale scores. In the observation group, the T2 value of the splenium of the corpus callosum was significantly positively correlated with the personal social behavior score. The AUCs for diagnosing preoperative brain developmental abnormalities in children with CHD using T1 values of the temporal white matter and dentate nucleus were both greater than 0.60.ConclusionsQuantitative assessment using SyMRI can aid in the early detection of preoperative brain development abnormalities in children with CHD.Critical relevance statementT1 and T2 relaxation values from SyMRI can be considered as a quantitative imaging marker to detect abnormalities, allowing for early clinical evaluation and timely intervention, thereby reducing neurodevelopmental disorders in these children.Key PointsT1 and T2 relaxation values by SyMRI are related to myelin development.Evaluated development quotient markers were lower in the observation compared to the control group.SyMRI can act as a reference indicator for brain development in CHD children.Graphical

Platelet-serotonin dynamics: elucidating their role in pulmonary arterial hypertension

Background and Objectives:&lt;br /&gt; Pulmonary arterial hypertension (PAH) is a significant complication in pediatric patients with congenital heart disease (CHD). The role of platelets and serotonin in the pathogenesis of PAH has been increasingly recognized. This study aims to investigate the correlation between platelet count, serotonin levels, and PAH in children with CHD, and to understand the impact of surgical intervention on these parameters.&lt;br /&gt; &lt;br /&gt; Material and Methods:&lt;br /&gt; This study included 26 children with CHD and PAH (Group I) and an 11-child control group without PAH. Pre- and post-operative platelet counts, mean platelet volume (MPV), platelet distribution width (PDW), plateletcrit (PCT), and serotonin levels in plasma and platelets were measured. Group I underwent surgical correction for CHD, and the control group received no such intervention. Data were analyzed to determine the relationships between these hematological and biochemical markers and PAH.&lt;br /&gt; &lt;br /&gt; Results:&lt;br /&gt; Group I showed higher pre-operative platelet counts and serotonin levels compared to the control group. Post-surgical data indicated a significant decrease in platelet serotonin levels, aligning more closely with the control group. The study also observed lower plasma serotonin levels in the control group, suggesting altered serotonin metabolism in PAH patients.&lt;br /&gt; &lt;br /&gt; Conclusion:&lt;br /&gt; The study suggests a strong association between elevated platelet counts, increased serotonin levels, and the presence of PAH in children with CHD. Surgical correction of CHD appears to normalize these parameters, indicating a potential pathophysiological link. These findings emphasize the need for further research to understand the underlying mechanisms and to explore targeted therapeutic strategies for PAH in pediatric CHD patients.

Pilot Assessment of Platelet Thrombus Area and Contractile Force in Pediatric Congenital Heart Surgery Patients Using a Microfluidic Device

ObjectivesEvaluate maximum platelet contractile force and thrombus area before and after cardiopulmonary bypass (CPB) in pediatric patients having congenital heart disease (CHD) surgery using a microfluidic device. DesignProspective cohort study. SettingAcademic medical center. ParticipantsTwenty pediatric CHD patients ≤ 8 years of age with expected CPB time >30 minutes. InterventionsNone Measurements and Main ResultsBlood was collected at baseline and post-CPB. Maximum platelet contractile force and thrombus area were evaluated in vitro using a microfluidic device (ATLAS PST). Post-CPB samples were supplemented with recombinant von Willebrand Factor (rVWF) to explore impact on contractile force and thrombus area. At baseline, maximum thrombus area was 0.06 [0.05, 0.07] and maximum force was 123.3 nN [68.4, 299.5]. Linear mixed effects regression models showed that maximum thrombus area was larger post-CPB and post-CPB + rVWF compared to pre-CPB (Est=0.04, p=0.002 and Est = 0.09, p < .001, respectively). Maximum thrombus area was also larger post-CPB + rVWF compared to post-CPB (Est=0.04, p=0.001). Force was higher post-CPB + rVWF compared to pre-CPB (Est=173.32, p=0.044). ConclusionsIn pediatric CHD patients, microfluidic testing demonstrated that platelet thrombus area increased slightly after CPB, while platelet contractile force did not change. In vitro addition of rVWF further increased thrombus area, suggesting augmentation of primary hemostasis. Microfluidic assessment of platelet contractile force and thrombus area in pediatric CHD patients appears feasible and can demonstrate changes after CPB. Further studies are needed to determine its accuracy and clinical utility, as well as normal values for pediatric patients.

Predictive value of NT-proBNP and hs-TnT for outcomes after pediatric congenital cardiac surgery.

The available evidence regarding the predictive value of troponins and natriuretic peptides for early postoperative outcomes in pediatrics is limited, controversial, and based on small sample sizes. We aimed to investigate the association of N-terminal pro B-type natriuretic peptide (NT-proBNP) and high-sensitivity troponin T (hs-TnT) with the in-hospital adverse outcomes after congenital cardiac surgeries. A secondary analysis based on a prospective study of pediatric congenital heart disease (CHD) patients was conducted to investigate the association of NT-proBNP and hs-TnT tested within 6 hours postoperatively with in-hospital adverse events. A multivariate logistic regression analysis with a minimum P value approach was used to identify the optimal thresholds of NT-proBNP and hs-TnT for risk stratification. NT-proBNP and hs-TnT are positively correlated with cardiopulmonary bypass time, mechanical ventilation duration, and pediatric intensive care unit stay. The predictive performance of NT-proBNP is excellent for adverse events in both patients younger than 1 year [area under the curve (AUC): 0.771, 0.693-0.850] and those older than 1 year (AUC: 0.839, 0.757-0.922). However, hs-TnT exhibited a satisfactory predictive value solely in patients aged over 1 year. (AUC: 0.784, 0.717-0.852). NT-proBNP levels of 2000 to 10000ng/L [Odds Ratio (OR): 3.79, 1.47-9.76) and exceeding 10000ng/L (OR: 12.21, 3.66-40.80) were associated with a higher risk of postoperative adverse events in patients younger than 1 year. Patients older than 1 year, with NT-proBNP higher than 500ng/L (OR: 15.09, 6.05-37.66) or hs-TnT greater than 1200ng/L (OR: 5.50, 1.47-20.59), had a higher incidence of postoperative adverse events. NT-proBNP and hs-TnT tested within postoperative 6 hours demonstrated significant predictive value for postoperative adverse events in CHD patients older than 1 year. However, among CHD patients younger than 1 year, only NT-proBNP exhibited commendable predictive performance for postoperative adverse events.

Cumulative radiation dose from medical imaging in paediatric congenital heart disease patients with epicardial cardiac implantable electronic devices.

To determine whether paediatric congenital heart disease (CHD) patients with epicardial cardiac implantable electronic devices (CIEDs) receive high cumulative effective doses (CEDs) of ionizing radiation from medical imaging tests. We compared 28 paediatric CHD patients with epicardial CIEDs (cases) against 40 patients with no CIED matched by age at operation, sex, surgical era, and CHD diagnosis (controls). We performed a retrospective review of radiation exposure from medical imaging exams between 2006 and 2022. Radiation dose from computed tomography (CT) and X-ray radiography was calculated using the National Cancer Institute Radiation Dosimetry Tool. We performed univariate analysis to compare the CED between the two groups. In the case subgroup, we convened experts' review to adjudicate the prevalence of CT exams that should have been performed with magnetic resonance imaging (MRI) in the absence of a CIED. Children (median age 2.5 years at implant) with CIEDs received significantly higher median CED compared with matched controls (6.90 vs. 1.72 mSv, P = 0.0018). In cases, expert adjudication showed that 80% of the CT exams would have been performed with MRI in the absence of a CIED. This resulted, on average, a five-fold increase in the effective dose (ED) from post-lead implant CTs. Paediatric CHD patients with CIED received four times higher CED than matched controls. Improved access to medical imaging tests without ionizing radiation, such as MRI, could potentially reduce the ED in CIED patients by up to five times.

Case Management Implications for Pediatric Patients With Congenital Heart Disease in China: A Randomized Controlled Trial.

Objectives. Case management, which is defined as a fully collaborative process that includes evaluation, planning, execution, coordination and supervision, has been widely used in the field of chronic diseases. However, the clinical effect of case management in pediatric patients with congenital heart disease (CHD) is unclear. This study was to explore the effects of case management model in pediatric patients with CHD. Methods. A total of 110 pediatric CHD patients referred to our center from January 2018 to January 2020 were enrolled for analysis. Patients were randomly assigned to a case management (experimental) group or a conventional nursing (control) group. Patient satisfaction, quality of life, and clinical outcomes were compared between the 2 groups. Results. Compared with that in the control group, patient satisfaction rate was significantly greater in the experimental group. Furthermore, the experimental group showed more significant improvement in quality of life than the control group did (73.8 ± 12.3 vs 66.5 ± 14.2, P < .001). In addition, the readmission rate in the experimental group was significantly lower than that in the control group (5% vs 20%, P = .022). Conclusions. Case management mode can be effectively applied in pediatric patients with CHD, which can improve patient satisfaction rate, health-related quality of life and lower the readmission rate.

Current State of Cardiac Implantable Electronic Device Remote Monitoring in Pediatrics and Congenital Heart Disease: A PACES-Sponsored Quality Improvement Initiative.

Cardiac implantable electronic device (CIED) remote transmissions are an integral part of longitudinal follow-up in pediatric and adult congenital heart disease (ACHD) patients. To evaluate baseline CIED remote monitoring(RM) data among pediatric and ACHD centers prior to implementation ofa Pediatric and Congenital Electrophysiology Society (PACES)-sponsored quality improvement (QI) project. This is a cross-sectional study of baseline CIED RM. Centers self-reported baseline data: individual center RM compliance was defined as high if there was > 80% achievement and low if < 50%. A total of 22 pediatric centers in the USA and Australia submitted baseline data. Non-physicians were responsible for management of the RM program in most centers: registered nurse (36%), advanced practice provider (27%), combination (23%), and third party (9%). Fifteen centers (68%) reported that > 80% of their CIED patients are enrolled in RM and only two centers reported < 50% participation. 36% reported high compliance of device transmission within 14 days of implant and 77% of centers reported high compliance of CIED patients enrolled in RM. The number of centers achieving high compliance differed by device type: 36% for pacemakers, 50% for ICDs, and 55% for Implantable Cardiac Monitors(ICM). All centers reported at least 50% adherence to recommended follow-up for PM and ICD, with 23% low compliance rate for ICMs. Based on this cross-sectional survey of pediatric and ACHD centers, compliance with CIED RM is sub-optimal. The PACES-sponsored QI initiative will provide resources and support to participating centers and repeat data will be evaluated after PDSA cycles.

Abstract 16051: Heart Transplantation With a Positive Crossmatch in Pediatric Recipients is Associated With Increased Mortality in the Current Era: An Analysis of the United Network for Organ Sharing Database

Introduction: Congenital heart disease (CHD) patients undergoing heart transplantation (HT) often have anti-HLA antibodies (sensitization), increasing the likelihood of a positive crossmatch and in turn an increased risk of mortality post-HT. The burden of sensitization in pediatric HT recipients and outcomes in such patients in the current era are unknown. Methods: The United Network for Organ Sharing (UNOS) database was queried for patients &lt;18 y/o with CHD undergoing HT between 4/1/2015 and 12/31/2019. Panel reactive antibody (PRA) at HT was collected; patients were grouped into 3 categories: nonsensitized (PRA &lt;10%), moderately sensitized (PRA 10-79%), and highly sensitized (PRA ≥80%). Data regarding crossmatch at HT were collected. One-year post-HT mortality based on sensitization category and crossmatch result were evaluated using multivariable Cox regression. Results: During the study period, 881 CHD patients with PRA data underwent HT; 69% nonsensitized, 22% moderately sensitized, and 9% highly sensitized. Positive crossmatch by flow cytometry or cytotoxicity was more commonly seen in highly sensitized patients (Table 1). Highly sensitized patients had 28% 1-year mortality. After adjustment, both 1-year mortality and graft loss were higher in highly sensitized patients than nonsensitized patients (HR 4, CI 2.4-6.7, P&lt;0.001 and HR 6.8, CI 3.6-12.8, P&lt;0.001, respectively); positive crossmatch by flow cytometry or cytotoxicity was also associated with increased risk of mortality and graft loss (HR 2.1, CI 1.3-3.4, P = 0.002 and HR 3.1, CI 1.7-5.6, P&lt;0.001, respectively). Conclusions: Highly sensitized pediatric CHD patients have 1-year post-HT mortality of nearly 30%, a 4-fold increase compared to nonsensitized patients; positive crossmatch also increased the risk of mortality and graft loss. Due to these poor outcomes, aggressive desensitization as well as alternatives to transplant should be considered in these high-risk patients.

Bioactive adrenomedullin (bio-ADM) is associated with endothelial dysfunction in infants and children with complex congenital heart disease undergoing open-heart surgery on cardiopulmonary bypass.

Children with congenital heart disease (CHD) undergoing cardiac surgery on cardiopulmonary bypass (CPB) are at risk for systemic inflammation leading to endothelial dysfunction associated with increased morbidity. Bioactive adrenomedullin (bio-ADM) is a peptide regulating vascular tone and endothelial permeability. Theaim of this study was to evaluate the dynamics of plasma bio-ADM in this patient cohort and its role in capillary leak. Plasma samples from 73 pediatric CHD patients were collected for bio-ADM measurement at five different timepoints (TP) in the pre-, intra-, and post-operative period. The primary endpoint was a net increase in bio-ADM levelsafter surgery on CPB. Secondary endpoints included association of bio-ADM levels with clinical signs for endothelial dysfunction. Bio-ADM levels increased after surgery on CPB from pre-operative median of 12 pg/mL (IQR [interquartile range] 12.0-14.8 pg/mL) to a maximum post-operative median of 48.8 pg/mL (IQR 34.5-69.6 pg/mL, p<0.001). Bio-ADM concentrations correlated positively with post-operative volume balance, (r=0.341; p=0.005), increased demand for vasoactive medication (duration: r=0.415; p<0.001; quantity: TP3: r=0.415, p<0.001; TP4: r=0.414, p<0.001), and hydrocortisone treatment for vasoplegia (bio-ADM median [IQR]:129.1 [55.4-139.2] pg/mL vs. 37.9 [25.2-64.6] pg/mL; p=0.034). Patients who required pleural effusion drainage revealed higher bio-ADM levelscompared to those who did not (median [IQR]: 66.4 [55.4-90.9] pg/mL vs. 40.2 [28.2-57.0] pg/mL; p<0.001). Bio-ADM is elevated in children after cardiac surgery and higher levels correlate with clinical signs of capillary leakage. The peptide should be considered as biomarker for endothelial dysfunction and as potential therapeutic target in this indication.

First experience with real-time magnetic resonance imaging-based investigation of respiratory influence on cardiac function in pediatric congenital heart disease patients with chronic right ventricular volume overload

BackgroundCongenital heart disease (CHD) is often associated with chronic right ventricular (RV) volume overload. Real-time magnetic resonance imaging (MRI) enables the analysis of cardiac function during free breathing.ObjectiveTo evaluate the influence of respiration in pediatric patients with CHD and chronic RV volume overload.Methods and materialsRV volume overload patients (n=6) and controls (n=6) were recruited for cardiac real-time MRI at 1.5 tesla during free breathing. Breathing curves from regions of interest reflecting the position of the diaphragm served for binning images in four different tidal volume classes, each in inspiration and expiration. Tidal volumes were estimated from these curves by data previously obtained by magnetic resonance-compatible spirometry. Ventricular volumes indexed to body surface area and Frank-Starling relationships referenced to the typical tidal volume indexed to body height (TTVi) were compared.ResultsIndexed RV end-diastolic volume (RV-EDVi) and indexed RV stroke volume (RV-SVi) increased during inspiration (RV-EDVi/TTVi: RV load: + 16 ± 4%; controls: + 22 ± 13%; RV-SVi/TTVi: RV load: + 21 ± 6%; controls: + 35 ± 17%; non-significant for comparison). The increase in RV ejection fraction during inspiration was significantly lower in RV load patients (RV load: + 1.1 ± 2.2%; controls: + 6.1 ± 1.5%; P=0.01). The Frank-Starling relationship of the RV provided a significantly reduced slope estimate in RV load patients (inspiration: RV load: 0.75 ± 0.11; controls: 0.92 ± 0.02; P=0.02).ConclusionIn pediatric patients with CHD and chronic RV volume overload, cardiac real-time MRI during free breathing in combination with respiratory-based binning indicates an impaired Frank-Starling relationship of the RV.Graphical

The Effects of Cardiac Implantable Electronic Device Personal Protective Equipment Use in Patients with Pediatric and Adult Congenital Heart Disease.

Pediatric and adult congenital heart disease (ACHD) patients encounter physical and emotional barriers. Cardiac implantable electronic devices (CIEDs), including pacemakers and implantable cardiac defibrillators (ICD) often compound these issues. Patient fear associated with damaging the CIED system can lead to avoidance of physical activity and reduced quality of life (QOL). CIED personal protective equipment (PPE) is a potential treatment for decreasing this fear. We sought to determine the effects of CIED PPE use in the pediatric and ACHD population. Patients 5 years or older with a CIED at a single pediatric and ACHD heart rhythm center received a CIED protector and customized athletic shirt. QOL was assessed using the PedsQL TM 4.0 SF15 prior to and after 6-12months. Of the 77 patients enrolled, 26 completed repeat assessment. The mean age at enrollment was 15.6years (range 5-36) with a mean device age of 2.2years (< 1-10years). Pacemakers were present in 77% and 23% had ICDs. The PPE was used in 92% with no PPE malfunction. Fear associated with physical activity was reduced, z = -4, p < 0.001, with a large effect size (r = 0.55). There was a trend toward increased physical activity. Total QOL scores improved, z = -2.771, P < 0.05, with a medium effect size (r = 0.4). This first study of CIED PPE in children shows that providing CIED PPE to pediatric and ACHD patients may decrease their fear of damaging their device system, increasing physical activity levels, and improving quality of life.

Open Field Exercise Testing in Pediatric Congenital Heart Disease Patients: A Subsumption of Cardiovascular Parameters

Heart failure is a common phenomenon in congenital heart disease patients. Cardiopulmonary exercise testing is used for a reliable assessment of heart failure but is still challenging, especially for young children. Implementing mobile cardiopulmonary exercise testing (CPET) can close that diagnostic gap. While average values for healthy children have already been published, this study aims to describe typical ranges of cardiovascular performance parameters of young children with congenital heart disease performing an 8-min running cardiopulmonary exercise test. Children aged 4–8 years with common congenital heart defects after corrective surgery (Tetralogy of Fallot; transposition of the great arteries and univentricular hearts after palliation) were included. The outdoor running protocol consisted of slow walking, slow jogging, fast jogging, and maximum speed running. Each exercise was performed for 2 min, except the last, in which children were instructed to keep up maximal speed as long as possible. A total of 78 children (45 male/33 female, mean age 6,24) with congenital heart disease participated in the study, of which 97% completed the CPET successfully. A detailed description of participating patients, including data on cardiac function and subjective fitness levels, is given to help physicians use this method to classify their patients. This study presents a typical range for cardiovascular performance parameters in a population of 4–8-year-old children with congenital heart disease tested in a newly developed outdoor running protocol for CPET.

Calciferol Status and Clinical Outcomes in Children with Congenital Heart Disease after Heart Surgery

Highlights: Calciferol has cardioprotective properties, and a severe deficiency of calciferol is associated with septic shock, a longer duration of mechanical ventilation and treatment in the intensive care unit, and mortality. The role of calciferol in critical conditions has been described in several research reports, but relatively little is known about its function in pediatric cardiac surgery. Abstract Calciferol is a micronutrient and a secosteroid hormone that plays a role in maintaining the health of bones and soft tissues in the body, such as the myocardium, as well as the immune system. Hypovitaminosis D has been reported to be associated with poor clinical outcomes and child mortality in pediatric intensive care units. Children with congenital heart disease (CHD) are vulnerable to critical conditions and require early intervention due to heart failure. This study aimed to investigate the influence of calciferol status on the clinical outcomes of pediatric CHD patients who underwent heart surgery. A systematic literature review was conducted using the electronic databases from PubMed, Elsevier, and Cochrane. This study included observational and randomized control studies that assessed the calciferol status of pediatric CHD patients undergoing cardiac surgery. From a total of 168 studies, 8 studies were selected for review. The preoperative and postoperative calciferol status as well as clinical outcomes following pediatric cardiac surgery were reviewed. According to the findings, most pediatric CHD patients suffered from calciferol deficiency prior to corrective heart surgery, which further decreased postoperatively and was associated with clinical outcomes in the intensive care unit (ICU). Preoperative calciferol supplementation has been reported to enhance serum calciferol levels and is associated with good clinical outcomes in pediatric patients undergoing cardiac surgery.

Liver Fibrosis Markers Represent Central Venous Pressure in Post-pubertal Patients With Congenital Heart Disease.

Background Central venous pressure (CVP) is one of the most important hemodynamic parameters in patients with congenital heart disease (CHD). In adults, it is well-known that liver fibrosis markers reflect CVP, but this is notwell-understood in children. We investigated the liver fibrosis markers in pediatric CHD patients and their ability to predict CVP. Methods We studied 160 patients who underwent cardiac catheterization in our hospital between January 2017 and December 2020. The levels of the fibrotic markers, including type IV collagen 7s, procollagen type III peptide, and hyaluronic acid, were measured. Results Procollagen type III peptide was markedly elevated in infants younger than one year of age. From one to 15 years of age, it was slightly lower than in the infant group, with a peak at around 10 years of age. In the age group of 16 years and older, most of its values were generally high. Type IV collagen 7s and hyaluronic acid levels were high in infants, with no significant differences at later ages. Procollagen type III peptide and hyaluronic acid showed no significant correlation with CVP in any of the age groups, whereas type IV collagen 7s significantly correlated with CVP in the age group above one yearold. Conclusions We found that elevated liver fibrosis markers, particularly type IV collagen 7s, correlated with central venous pressure in CHD patients older than one year. Measurement of liver fibrosis markers may allow the early detection of changes in CVP and liver function in patients with CHD.

The implication of chromosomal abnormalities in the surgical outcomes of Chinese pediatric patients with congenital heart disease.

Copy number variations (CNVs) have been shown to be overrepresented in children with congenital heart disease (CHD). Genetic evaluation of CHD is currently underperformed in China. We sought to determine the occurrence of CNVs in CNV regions with disease-causing potential among a large cohort of Chinese pediatric CHD patients and investigate whether these CNVs could be the important critical modifiers of surgical intervention. CNVs screenings were performed in 1,762 Chinese children who underwent at least one cardiac surgery. CNV status at over 200 CNV locus with disease-causing potential was analyzed with a high-throughput ligation-dependent probe amplification (HLPA) assay. We found 378 out of 1,762 samples (21.45%) to have at least one CNV and 2.38% of them were carrying multiple CNVs. The detection rates of ppCNVs (pathogenic and likely pathogenic CNVs) were 9.19% (162/1,762), significantly higher than that of the healthy Han Chinese individuals from The Database of Genomic Variants archive (9.19% vs. 3.63%; P = 0.0012). CHD cases with ppCNVs had a significantly higher proportion of complex surgeries compared to CHD patients with no ppCNVs (62.35% vs. 37.63%, P < 0.001). Duration of cardiopulmonary bypass and aortic cross clamp procedures were significantly longer in CHD cases with ppCNVs (all P < 0.05), while no group differences were identified for complications of surgery and one-month mortality after surgery. The detection rate of ppCNVs in the atrioventricular septal defect (AVSD) subgroup was significantly higher than that in other subgroups (23.10% vs. 9.70%, P = 0.002). CNV burden is an important contributor to Chinese children with CHD. Our study demonstrated the robustness and diagnostic efficiency of HLPA method in the genetic screening of CNVs in CHD patients.

Lympho-hematopoietic malignancies risk after exposure to low dose ionizing radiation during cardiac catheterization in childhood.

Pediatric patients with congenital heart disease (CHD) often undergo low dose ionizing radiation (LDIR) from cardiac catheterization (CC) for the diagnosis and/or treatment of their disease. Although radiation doses from a single CC are usually low, less is known about the long-term radiation associated cancer risks. We aimed to assess the risk of lympho-hematopoietic malignancies in pediatric CHD patients diagnosed or treated with CC. A French cohort of 17,104 children free of cancer who had undergone a first CC from 01/01/2000 to 31/12/2013, before the age of 16 was set up. The follow-up started at the date of the first recorded CC until the exit date, i.e., the date of death, the date of first cancer diagnosis, the date of the 18th birthday, or the 31/12/2015, whichever occurred first. Poisson regression was used to estimate the LDIR associated cancer risk. The median follow-up was 5.9 years, with 110,335 person-years. There were 22,227 CC procedures, yielding an individual active bone marrow (ABM) mean cumulative dose of 3.0 milligray (mGy). Thirty-eight incident lympho-hematopoietic malignancies were observed. When adjusting for attained age, gender and predisposing factors to cancer status, no increased risk was observed for lympho-hematopoietic malignancies RR/mGy = 1.00 (95% CI: 0.88; 1.10). In summary, the risk of lympho-hematopoietic malignancies and lymphoma was not associated to LDIR in pediatric patients with CHD who undergo CC. Further epidemiological studies with greater statistical power are needed to improve the assessment of the dose-risk relationship.