Pediatric Congenital Heart Disease Patients Research Articles

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

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.

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.

PO-03-147 EVALUATION OF A PEDIATRIC ELECTROPHYSIOLOGY CASE COMPLEXITY PREDICTION MODEL TO OPTIMIZE PROCEDURAL SCHEDULING

While operational efficiency has been evaluated in adult interventional and surgical specialties, limited data exist regarding the prediction of case complexity in the pediatric and congenital electrophysiology (EP) laboratory. To identify pre-procedural variables that predict prolonged case durations among pediatric and adult congenital patients presenting for diagnostic EP study and catheter ablation. A retrospective cross-sectional study was performed of all EP studies performed at Boston Children’s Hospital between 1/1/2015-1/1/2020 including pediatric and adult congenital heart disease patients of all ages. The primary outcome was case duration ≥180 minutes. Univariate and multivariate analyses were performed for the primary outcome using logistic regression, with a sub-analysis repeated for pediatric patients <20 years of age. During the 5-year period, 1025 patients underwent EP study and 292 (28.5%) had a case duration ≥180 minutes. Factors associated with the primary outcome on multivariate analysis were: age ≥20 years at EP study (OR 1.95, 95% CI 1.24-3.07, p 0.004), arrhythmia diagnosis of premature ventricular contractions/ventricular tachycardia (PVC/VT, OR 3.70, 95% CI 2.10-6.55, p <0.001), Fontan (OR 3.74, 95% CI 1.69-8.30, p=0.001) or Mustard/Senning physiology (OR 4.13, 95% CI 1.48-11.60, p=0.007), and the presence of non-cardiac comorbidities (OR 2.47, 95% CI 1.29-4.73, p 0.006). Restricting the analysis to pediatric patients <20 years old, factors associated with the primary outcome on multivariate analysis were an arrhythmia diagnosis of PVC/VT (OR 3.04, 95% CI 1.64-5.63, p <0.001), Fontan physiology (OR 3.13, 95% CI 1.10-8.92, p 0.033), non-cardiac comorbidities (OR 4.32, 95% CI 2.07-9.01, p <0.001), and prior ablation (OR 1.58, 95% CI 1.03-2.41, p 0.035). Intra-atrial reentrant tachycardia was not associated with prolonged case duration in either multivariate model. A weighted risk score using factors identified on multivariate analyses correctly classified procedures with prolonged case duration with reasonable accuracy (c-statistic 0.70). Age-dependent anatomic, electrophysiologic, and comorbid factors are associated with prolonged case duration in the pediatric and congenital EP laboratory. Applying a simple risk score using pre-procedural variables can discriminate cases with and without prolonged duration, and may aid in efficient procedural scheduling practices.

Use of Rivaroxaban in Children with Congenital Heart Disease: A Single-Center Case Series.

Rivaroxaban is a direct oral anticoagulant approved for therapeutic and prophylactic anticoagulation in both adults and children. Studies on rivaroxaban use in pediatric patients with congenital heart disease (CHD) are limited. Currently, warfarin (oral) and enoxaparin (injection) are the primary options for pediatric outpatient anticoagulation. Rivaroxaban may be a less burdensome alternative, but its use has not been well described in the pediatric CHD population. We describe our single-center experience. From May 2020-July 2022, we identified all pediatric CHD patients started on rivaroxaban. Dosing was based on recommendations reported in the EINSTEIN-Jr (Male et al. in Lancet Haematol 7:e18-e27, 2020) and UNIVERSE study (McCrindle et al. in J Am Heart Assoc 10:e021765, 2021) protocols. Qualitative outcomes on safety and efficacy are reported. There were 27 patients studied with an age range of 4months-15years at time of medication initiation. Single ventricle heart disease was present in 70% (19/27) of patients. Of the 27 patients initiated on rivaroxaban, 15 (56%) were started for VTE prophylaxis and 12 (44%) were started for VTE treatment. No patients started on rivaroxaban for prophylaxis developed a VTE. There was resolution or lack of propagation in 10 of the 12 patients started for treatment. There were two clinically relevant bleeding episodes. Our single-center case series describes the experience with rivaroxaban for VTE prophylaxis and treatment in pediatric congenital heart disease. Larger studies are required to further investigate safety, efficacy, and use indications.

CURRENT TRENDS OF CARDIAC SEPTAL DEFECTS IN CHILDREN IN EASTERN INDIA: AN OBSERVATIONAL STUDY

Congenital Heart Disease (CHD) is the most common among all congenital anomalies. CHDs are majorly classied into either cyanotic or acyanotic variety and both may have septal defects. Isolated cardiac septal defects mainly constitute the acyanotic CHDs namely Ventricular Septal Defect (VSD), Atrial Septal Defect (ASD) and Endocardial Cushion Defects (ECD). With recent advances and ease of availability of diagnostic aids and specialists, CHDs have become a common disease entity faced by Paediatric practitioners today. Acyanotic cardiac septal defects are relatively easier to manage and treat as compared to the cyanotic and complex CHDs. Still data detailing the epidemiology of these defects in India are scarce. This study was aimed to typify ndings from pediatric CHD patients with septal defects at a medical college hospital in northern part of West Bengal, catering to a previously understudied demographic. A sequence of CHD cases, picked contiguously from the pediatric indoor and outdoor patients over a period, were surveyed. Data was recorded under multiple headings including demographics, clinical ndings, investigations and outcomes

Comparison of lipid profile values in pediatric patients with cyanotic and acyanotic congenital heart disease

Background Incidence of congenital heart disease (CHD) is about 0.8% of every child born. This heart defect is associated with dyslipidemia in children. Lipid profiles examination in patients with CHD can be used to determine risk factors for atherosclerosis.&#x0D; Objective To examine differences in lipid profiles in children with cyanotic and acyanotic CHD.&#x0D; Methods This was a cross-sectional study on 60 pediatric CHD patients at Haji Adam Malik Hospital, Medan, North Sumatera, from December 2020 to March 2021. Subjects were included by consecutive sampling. Data of patient’s age, gender, weight, height, complete blood count, blood glucose, and lipid profiles were recorded. Unpaired T-test analysis and Mann-Whitney test were then performed to analyze variables in cyanotic and acyanotic CHD patients.&#x0D; Results Of a total of 60 CHD children, 26 subjects had a diagnosis of cyanotic CHD and 34 subjects had a diagnosis of acyanotic CHD. The most common cause of cyanotic CHD was tetralogy of Fallot (76.9%), while the most common cause of acyanotic CHD were ventricular septal defect and patent ductus arteriosus (32.4% each). Analysis of lipid profiles on both groups revealed that low density lipoprotein (LDL) was significantly lower in the cyanotic group than in the acyanotic group (P&lt;0.05). However, other lipid profile values, were not significantly different between groups. In addition, there was no significant difference in incidence of dyslipidemia between cyanotic and acyanotic CHD.&#x0D; Conclusion Low density lipoprotein is significantly lower in the cyanotic CHD group than in the acyanotic CHD group. But there are no significant differences in the other lipid profiles measurement and incidence of dyslipidemia between groups.

Habilitation of Executive Functions in Pediatric Congenital Heart Disease Patients through LEGO®-Based Therapy: A Quasi-Experimental Study.

Congenital heart disease is defined as an abnormality in the cardiocirculatory structure or function. Various studies have shown that patients with this condition may present cognitive deficits. To compensate for this, several therapeutic strategies have been developed, among them, the LEGO® Education sets, which use the pedagogic enginery to modify cognitive function by didactic material based on mechanics and robotics principles. Accordingly, the goal of this study was to evaluate the effect of cognitive habilitation by using LEGO®-based therapy in pediatric congenital heart disease patients. This was a quasi-experimental study; eligible patients were identified, and their general data were obtained. In the treatment group, an initial evaluation with the neuropsychological BANFE-2 test was applied; then, once a week, the interventions were performed, with a final test at the end of the interventions. In the control group, after the initial evaluation, a second appointment was scheduled for the final evaluation. Our results show that &gt;50% of children presented cognitive impairment; nevertheless, there was an overall improvement in treatment patients, showing a significant increase in BANFE scores in areas related to executive functions. LEGO®-based therapy may be useful to improve cognitive abilities; however, future research should be performed to strengthen the data.

Anaesthesia administered as S(+)-ketamine for cardiac intervention in children with common congenital heart disease.

Safety and efficacy were assessed of different S(+)-ketamine doses combined with propofol administered as anesthesia to common pediatric congenital heart disease (CHD) patients undergoing cardiac interventional surgery to provide reference data as guidance for use in clinical settings. Sixty CHD children admitted to Beijing Anzhen Hospital, Capital Medical University from December 2020 to December 2021 who underwent elective cardiac intervention were assigned to 3 groups (H, L, M, 20 patients/group) using a random number table-based method. Patients received 1% propofol (2 mg/kg) and intravenous injections of S(+)-ketamine (Group L, 0.4 mg/kg; Group M, 0.5 mg/kg; Group H, 0.6 mg/kg) followed by intravenous pumping of 1% propofol (4-6 mg/kg/h). Heart rate (HR), mean arterial pressure, and pulse oxygen saturation were recorded preoperatively (T0), at the time of anesthesia maintenance (T1), at the time of arteriovenous puncture (T2), and when they awakened (T3). Additionally, propofol dose and incidence rates of intraoperative body movement, postoperative agitation, and postoperative nausea/vomiting were recorded. For the 3 groups, Group H awakening time was significantly longer than that of Group L (P = .039). Notably, intergroup intraoperative propofol times differed significantly (P = .009). Meanwhile, T0 to T3 intragroup HR values differences were significant (P = .017; P = .001; P = .005, respectively). Group L HR was significantly elevated at T2 relative to T0 (P = .003), Group M HR was significantly elevated at T1 and T2 relative to T0 (P = .019; P = .003, respectively), and Group H HR values were significantly elevated at T1 and T2 relative to T0 (P = .012; P = .005, respectively). At all 4 time points no statistically significant intergroup differences in mean arterial pressure values were observed (P = .587). T1 to T3 pulse oxygen saturation values for all 3 groups were significantly greater than corresponding T0 values. Although intergroup intraoperative body movement incidence differed significantly (P = .044), intergroup differences in awakening time agitation and postoperative nausea/vomiting incidence rates were insignificant (P = .732, P = .887, respectively). Use of 0.6 mg/kg S(+)-ketamine with propofol was most effective as anesthesia for common pediatric CHD patients undergoing cardiac interventional surgery.