Congenital Heart Disease Patients Research Articles

Absorbable metal stents for vascular use in pediatric cardiology: progress and outlook.

The past five years have yielded impressive advancements in fully absorbable metal stent technology. The desired ultimate ability for such devices to treat a vascular stenosis without long-term device-related complications or impeding future treatment continues to evoke excitement in clinicians and engineers alike. Nowhere is the need for fully absorbable metal stents greater than in patients experiencing vascular anomalies associated with congenital heart disease (CHD). Perhaps not surprisingly, commercially available absorbable metal stents have been implanted in pediatric cardiology patients with conditions ranging from pulmonary artery and vein stenosis to coarctation of the aorta and conduit/shunt reconstructions. Despite frequent short term procedural success, device performance has missed the mark with the commercially available devices not achieving degradation benchmarks for given applications. In this review we first provide a general overview detailing the theory of absorbable metal stents, and then review recent clinical use in CHD patients since the release of current-generation absorbable metal stents around 2019. We also discuss the challenges and our center's experience associated with the use of absorbable metal stents in this pediatric population. Lastly, we present potential directions for future engineering endeavors to mitigate existing challenges.

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.

Heart failure in patients with congenital heart disease after a cancer diagnosis.

Individuals with congenital heart disease (CHD) are at an increased risk for cancer. As cancer survival rates improve, the prevalence of late side effects, such as heart failure (HF), is becoming more evident. This study aims to evaluate the risk of developing HF following a cancer diagnosis in patients with CHD, compared with those without CHD and with CHD patients who do not have cancer. CHD patients (n=69799) and randomly selected non-CHD controls (n=650406), born in Sweden between 1952 and 2017, were identified from the Swedish National Health Registers and Total Population Register (excluding those with syndromes and transplant recipients). CHD patients who developed cancer (n=1309) were propensity score-matched with non-CHD patients who developed cancer (n=9425), resulting in a cohort of 1232 CHD patients with cancer and 2602 non-CHD controls with cancer (after exclusion of individuals with HF prior to cancer diagnosis). In a separate analysis, CHD patients with cancer were propensity score-matched with CHD patients without cancer (n=68490). A total of 1233 CHD patients with cancer and 2257 CHD patients without cancer were included in the study. Among CHD patients with cancer, 73 (5.9%) developed HF during a mean follow-up time of 8.5±8.7. Comparatively, in the propensity-matched control population, 29 (1.1%) non-CHD cancer patients (mean follow-up time of 7.3±7.5) and 101 (4.5%) CHD patients without cancer (mean follow-up time of 9.9±9.2) developed HF. CHD patients exhibited a significantly higher risk of HF post-cancer diagnosis compared with the non-CHD control group [hazard ratio (HR) 4.39, 95% confidence interval (CI) 2.83-6.81], after adjusting for age at cancer diagnosis and comorbidities. In the analysis between CHD patients with cancer and those without cancer, the results indicated a significantly higher risk of developing HF in CHD patients with cancer (HR 1.53, 95% CI 1.13-2.07). CHD patients face a more than four-fold increased risk of developing HF after a cancer diagnosis compared with cancer patients without CHD. Among CHD patients, the risk of HF is only modestly higher for those with cancer than for those without cancer. This suggests that the increased HF risk in CHD patients with cancer, relative to non-CHD cancer patients, may be more attributable to CHD itself than to cancer treatment-related side effects.

High-Quality Hospital Status on Health Care Costs for Pediatric Congenital Heart Disease Care for U.S. Military Beneficiaries.

Congenital heart disease (CHD) is the most common and resource demanding birth defect managed in the United States, with approximately 40,000 children undergoing CHD surgery year. Researchers have compared high-volume to low-volume hospitals and found significant hospital-level variation in major complications, health resource utilization, and health care costs after CHD surgery. Using nationwide representative claim data from the United States Military Health System from 2016 to 2020, TRICARE beneficiaries diagnosed with CHD were tabulated based on ICD-10 codes (International Classification of Diseases, 10th revision). We examined the relationships between total costs and total hospitalizations costs post 1-year CHD diagnosis and presence or absence of High-Quality Hospital (HQH) designation. We applied both the naive generalized linear model (GLM) to control for the observed patient and hospital characteristics and the 2-stage least squares (2SLS) model to account for the unobserved confounding factors. This study was approved by University of Maryland, College Park Institutional Review Board (IRB) (Approval Number: 1576246-2). A relationship between HQH designation and total CHD related costs was not seen across 2SLS model specifications (marginal effect; -$41,579; 95% CI, -$83,429 to $271). For patients diagnosed with a moderate-complex or single ventricle CHD, the association of HQH status was a statistically significant reduction in total costs (marginal effect; -$84,395; 95% CI, -$140,560 to -$28,229) and hospitalization costs (marginal effect; -$73,958; 95% CI, -$121,878 to -$26,039). It is very imperative for clinicians and patient support advocates to urge policymakers to deliberate the establishment of a quality designation authority for CHD management. These efforts will not only help to identify and standardize quality care metrics but to improve long-term health, effectiveness, and equity in the management of CHD. Furthermore, these efforts can be used to navigate patients to proven HQH, thereby improving care and reducing associated treatment costs for CHD patients.

Lung Ultrasound Evaluation of Pulmonary Abnormalities in Children with Congenital Heart Diseases

Abstract Background and Aims Congenital heart disease (CHD) can cause several abnormalities of the lungs, such as pulmonary overflow (PO), pulmonary hypertension and chest infection like pneumonia. We aimed to assess pulmonary abnormalities using lung ultrasound (LUS) in children with congenital heart diseases. Patients and Methods This is a cross-sectional study, which included (33) CHD patients aged between 0.5 to 3.5 years who were admitted to the Cardiology Unit,Ain Shams University, Children’s Hospital. The patients were subjected to detecting any consolidation and measuring B vertical lines in all lung zones. LUS score was recorded at baseline and after course of management. Results There was statistically reduction in the after management group compared to the before management group according to LUS score with p-value (p &amp;lt; 0.001). There was a difference between types of management according to LUS score and its severity in the before management group, with p-value (p &amp;lt; 0.001) whereas, surgical type of management had the highest mean value of the LUS score compared to other types of management among our studied patients before management with a highly statistically significant difference, with p-value (p &amp;lt; 0.001). Conclusion Lung ultrasound is an ideal tool for the diagnosis and follow-up of pulmonary complications such as lung congestion, pulmonary edema and pneumonia. Also, there was improvement in pulmonary congestion after management among CHD children.

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

Peripartum anesthesia management and outcomes of patients with congenital heart disease: a single-center retrospective analysis (2009–2023)

BackgroundAdvances in medicine have enabled more patients with congenital heart disease (CHD) to become pregnant. However, these patients face significant challenges during the peripartum period. Current peripartum anesthesia guidelines for CHD patients mainly rely on case reports and small series. MethodsIn this retrospective study at a high-volume tertiary care center, we analyzed peripartum anesthetic approaches, postpartum hemorrhage (PPH) incidence, and maternal outcomes in CHD patients stratified by the modified World Health Organization (mWHO) classification. ResultsAmong 85 473 deliveries between 2009 and 2023, 409 occurred in 282 patients with CHD. Cesarean deliveries were significantly more frequent in mWHO class III, p=0.005. Labor epidural analgesia was the most common analgesic modality for vaginal deliveries (epidural rate was 71.1% with no differences between mWHO classes). Anesthesia management for cesarean deliveries varied significantly by class p<0.001. While spinal anesthesia was predominant in classes I and II, combined spinal-epidural anesthesia was more common in class III. PPH incidence was 6.4%, with no significant difference across classes, and no association was found between mWHO class severity and PPH risk (OR 0.97; 95% CI; 0.93 to 1.02, p=0.2). Higher mWHO classes correlated with significantly higher intensive care unit (ICU) admission rates, longer hospital stays, and one-year cardiac hospitalizations. ConclusionIn this retrospective study on the peripartum anesthetic management and outcomes of CHD patients stratified by mWHO class, cases with greater mWHO class were more likely to deliver preterm, by cesarean delivery, with a combined spinal-epidural anesthetic and an arterial line placement for that cesarean delivery. They overall had a longer hospital stay and were more likely to be admitted to the ICU. However, the overall risk of PPH did not increase with mWHO class severity.

Profile Of Congenital Heart Disease (CHD) Patients In Cirebon, Indonesia: Maternal Passive Smoking Risk

Background: Congenital heart disease (CHD) is the most common congenital disorder and a leading cause of infant mortality. Maternal pregnancy history is considered a potential risk factor for CHD. This cross-sectional study aimed to investigate the patient and maternal profile of CHD cases in Cirebon, Indonesia. Methods: The study was conducted at Hasna Medika Cardiovascular Hospital from November 2019 to March 2020, and data were collected through questionnaires and medical records. The study included 28 CHD patients, of whom 57.1% were female, and most had acyanotic CHD (82.1%). Results: None of the mothers had a history of active smoking, but all were exposed to passive smoking. Additionally, 17.9% of the samples had a history of fever during the first trimester, 17.9% took medication during pregnancy, and all consumed vitamins and supplements. Hormonal contraceptive failure accounted for 17.9% of CHD cases, while 7.1% of mothers had gestational hypertension. None of the mothers had gestational diabetes mellitus. Conclusions: The study suggests that passive smoking may be closely related to CHD, but further research is needed to confirm this association. Therefore, it is crucial for pregnant women to avoid exposure to cigarette smoke. Health promotion programs that educate the public about the risks of smoking during pregnancy and infancy are highly encouraged.

Surgical Outcomes of Congenital Heart Disease in the Eastern Region of Libya

Congenital heart disease (CHD) refers to a group of structural abnormalities in the heart that are present at birth. However, due to advancements in medical and surgical care, more children with CHD in developed countries are now surviving into adulthood. This study aims to assess the outcomes of surgically repaired congenital heart disease in the eastern part of Libya. Data was obtained from the medical records of 374 patients, of which 54.2% were male and 45.7% were female, who underwent surgical correction and palliative cardiac surgery between February 2015 and February 2019. The majority of patients were from Benghazi and Al-Jabal Al-Akhdar, and the most common diagnoses were ventricular septal defects (VSD) (25.4%) and complex congenital heart disease (CCHD) (21.9%). The death rate in atrioventricular canal (AVC) defects was 7.4%, CCHD had a mortality rate of 4.8%, and the total mortality rate was 2.9%. In the eastern part of Libya, pediatric cardiac surgery and cardiac surgical intensive care for children with congenital heart disease (CHD) still heavily rely on foreign cardiac surgery missions. There is a need for a comprehensive treatment plan and the development of local capabilities to manage CHD patients independently.

Update on Transcatheter Interventions in Adults with Congenital Heart Disease.

The field of adult congenital interventions is unique in the world of cardiac catheterization, combining the structural concepts commonly employed in pediatric heart disease and applying them to adult patients, who are more amenable to device intervention as they no longer experience somatic growth. Rapid advances in the field have been made to match the growing population of adult patients with congenital heart disease, which currently surpasses the number of pediatric patients born with congenital heart disease. Many congenital defects, which once required surgical intervention or reoperation, can now be addressed via the transcatheter approach, minimizing the morbidity and mortality often encountered within adult congenital surgeries. In this paper, we aim to provide a focused review of the more common procedures that are utilized for the treatment of adult congenital heart disease patients in the catheterization laboratory, as examples of current practices in the United States, as well as emerging concepts and devices awaiting approval in the future.

Risk of Cardiac Reoperation: An Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database

BackgroundThe increasing number of congenital heart disease patients undergoing reoperative cardiac surgery presents critical and growing challenges. Our objective was to evaluate the association between the number of prior cardiopulmonary bypass operations and operative mortality and morbidity in a national cohort. MethodsThe Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) was reviewed for index cardiac operations on cardiopulmonary bypass during 2016 to 2021. Infants and patients with functionally univentricular physiology were excluded. Multivariable logistic regression adjusted for covariates in the STS-CHSD Mortality Risk Model, the STS-European Association for Cardio-Thoracic Surgery (STAT) Mortality Category, and institutional volume. ResultsOf 50,625 eligible operations, 22,100 (44%) were performed on patients with ≥1 prior cardiopulmonary bypass operations. Most common diagnoses were tetralogy of Fallot (4340 of 22,100 [19.6%]), pulmonary atresia/ventricular septal defect (1334 of 22,100 [6.0%]), and aortic stenosis (966 of 22,100 [4.4%]). Operative mortality correlated with number of prior cardiopulmonary bypass operations: 157 of 28,525 (0.6%) for 0, 127 of 13,488 (0.9%) for 1, 81 of 5,664 (1.4%) for 2, 61 of 2039 (3.0%) for 3, 35 of 623 (5.6%) for 4, 10 of 207 (4.8%) for 5, and 5 of 79 (6.3%) for ≥6 operations (P < .001). On multivariable analysis, patients with ≥3 prior cardiopulmonary bypass operations had higher risk of operative mortality (odds ratio, 2.31; P < .001) and major morbidity (odds ratio, 1.60; P < .001). Annual institutional volume and age were not associated with either outcome. ConclusionsThree or more prior cardiopulmonary bypass operations was an independent risk factor for operative mortality/morbidity, even after controlling for risk factors and institutional volume. Future research is needed to identify modifiable factors to optimize outcomes, particularly for those with ≥3 prior cardiopulmonary bypass operations.

Comparative proteomics reveals that fatty acid metabolism is involved in myocardial adaptation to chronic hypoxic injury.

Congenital heart disease (CHD) is the most serious form of heart disease, and chronic hypoxia is the basic physiological process underlying CHD. Some patients with CHD do not undergo surgery, and thus, they remain susceptible to chronic hypoxia, suggesting that some protective mechanism might exist in CHD patients. However, the mechanism underlying myocardial adaptation to chronic hypoxia remains unclear. Proteomics was used to identify the differentially expressed proteins in cardiomyocytes cultured under hypoxia for different durations. Western blotting assays were used to verify protein expression. A Real-Time Cell Analyzer (RTCA) was used to analyze cell growth. In this study, 3881 proteins were identified by proteomics. Subsequent bioinformatics analysis revealed that proteins were enriched in regulating oxidoreductase activity. Functional similarity cluster analyses showed that chronic hypoxia resulted in proteins enrichment in the mitochondrial metabolic pathway. Further KEGG analyses found that the proteins involved in fatty acid metabolism, the TCA cycle and oxidative phosphorylation were markedly upregulated. Moreover, knockdown of CPT1A or ECI1, which is critical for fatty acid degradation, suppressed the growth of cardiomyocytes under chronic hypoxia. The results of our study revealed that chronic hypoxia activates fatty acid metabolism to maintain the growth of cardiomyocytes.

Cardiopulmonary Exercise Test and Daily Physical Activity in Pediatric Congenital Heart Disease: an Exploratory Analysis

Sedentary lifestyle is reported to be associated with diminished exercise capacity resulting in increased cardiovascular risk in adults with congenital heart disease (CHD). This cross-sectional study examined the association between objectively measured physical activity (PA) and exercise capacity in children and adolescents with CHD. Therefore, 107 patients (13.0 ± 2.7 years, 41 girls) with various CHD performed a cardiopulmonary exercise test (CPET) to quantify their peakV'O2. Moderate-to-vigorous physical activity (MVPA) and daily step count were assessed with “Garmin vivofit jr.®” for 7 consecutive days. For association between PA and (sub-) maximal exercise capacity Spearman's correlation was performed. CHD patients showed almost normal values compared to reference (79.5 ± 17.2% [31.6-138.1] %peakV'O2 predicted) with roughly normal ventilatory anaerobic thresholds (50.6±14.0% [20.3-97.9] %VATV'O2). Step counts are below recommendations (9,304 ± 3,792 steps/day [1,701-20,976]), whereas MVPA data are above recommendations for children with ≥ 60min/day (83.6 ± 34.6 min/day [10.1-190.9]). Spearman's Rho showed significant positive correlations to VATV'O2 (r=0.353, p<0.001), and %VATV'O2 (r=0.307, p=0.001) with similar results regarding MVPA (VATV'O2: r=0.300, p=0.002, and %VATV'O2: r=0.270, p=0.005). Concluding, submaximal exercise capacity and PA correlate positively making both assessments relevant in a clinical setting - PA in the context of cardiovascular prevention and peakV'O2 as the strongest predictor for morbidity and mortality.

Epidemiology of Congenital Heart Disease in Kazakhstan: Data from the Unified National Electronic Healthcare System 2014-2021

The aim of this study was to investigate the epidemiology of congenital heart disease (CHD) in Kazakhstan, using the data from the Unified National Electronic Healthcare System (UNEHS) for the period of 2014-2021. This retrospective cohort study included all patients diagnosed with CHD in Kazakhstan and registered in the UNEHS between January 2014 and December 2021. CHDs were defined based on ICD-10 codes Q20-Q26. Incidence, prevalence, and all-cause mortality rates were calculated per 100,000 population. Survival analysis was performed using Cox proportional hazards regression modeling and Kaplan-Meier method. The cohort consisted of 68,371 CHD patients, of whom 61,285 (89.6%) had a single CHD type, 40,767 (59.6%) were diagnosed before the age of 1 year, and 5,225 (7.6%) died over the study period. Incidence of CHD decreased from 64.6 to 47.3 cases per 100,000 population in males, and from 68.7 to 42.5 cases in females between 2014 and 2020. All-cause mortality rates per 100,000 population increased from 3.3 to 4.7 cases among males, and from 2.7 to 3.7 among females between 2014 and 2020. Survival analysis showed that in patients diagnosed with CHD before 1 year of age, risk of death was significantly associated with male sex (hazard ratio [HR] 1.17), multiple CHD types (HR 1.70), and no performed surgery (HR 0.57). In patients diagnosed with CHD after 1 year of age, risk factors were male sex (HR 1.65), multiple CHD types (HR 1.55), and no performed surgery (HR 1.82).

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.

Experiences and Attitudes Toward Telemedicine in an Adult Congenital Heart Disease Clinic: Lessons Learned from the COVID-19 Pandemic.

The coronavirus disease 2019 (COVID-19) pandemic has driven a broader adoption of telemedicine (TM). We aim to describe adult congenital heart disease (ACHD) patient experiences with TM and explore factors associated with positive attitude toward future TM visits. This is a cross-sectional, single-center study in an outpatient ACHD clinic from February to June, 2022. Between-group comparisons were made using Wilcoxon-Rank Sum, Chi-Square, or Fisher-Exact tests. Univariate logistic regression was performed for variables that could correlate with a "positive" attitude toward future TM visits. Significance was determined using an alpha level of 0.05. Of 262 patients (median age 33years, 55% female, 81% White), 115 (44%) had a prior TM visit and 110 (96%) reported a positive experience. There were 64 (24%) with a positive attitude toward future TM visits. Concerns include lack of cardiac testing and limited quality of visit. Patients with visits every 3-6months (Odds Ratio [OR] 2.44; p < 0.01) and prior TM visit (OR 1.89; p = 0.03) had higher odds of a positive attitude toward future TM, whereas males had lower odds (OR 0.53; p = 0.04). Age, annual income, disease complexity, distance from clinic, and employment status were not associated. There is high rate of satisfaction with TM among ACHD patients but only one-quarter indicated interest in using TM in the future. Factors associated with interest in TM visits are identified, and together with patient feedback, can be used to understand potential role of TM for the ACHD population in the post-pandemic era.

Association between congenital heart disease and cancer: A meta-analysis of 30 million patients.

10568 Background: Birth defects have been associated with increased cancer risk; however, the association between congenital heart disease (CHD) and cancer risk has not been well established with limited and conflicting literature till date. Children afflicted with CHD possess multiple risk factors for oncogenesis, such as chromosomal and genetic abnormalities, ionising radiation exposure during cardiac imaging and interventions, chronic hypoxia and debility. We sought to evaluate the relationship between CHD and cancer risk and its various subtypes. Methods: We performed a systematic literature search on PubMed, EMBASE, and Scopus for relevant articles from inception until February 4th, 2023. Odds ratios (OR) and 95% confidence intervals (CI) were pooled using a random-effects model, and a p-value of &lt;0.05 was considered statistically significant. Results: A total of 5 studies with 30,437,431 patients (CHD group=213,229, and non-CHD group = 30,224,202) were involved in the analysis. Mean age of patients with CHD and non CHD was 48.4 and 51.3 years. The pooled analysis revealed that patients with CHD had a higher risk of skin cancer (OR, 1.16(95%CI: 1.10-1.24), p&lt;0.00001), and thyroid cancer (OR, 1.47(95%CI: 1.20-1.80), p&lt;0.001) compared with non-CHD patients. However, leukemia (OR, 0.74(95%CI: 0.22-2.48), p=0.62), gastrointestinal cancer (OR, 1.50(95%CI: 0.79-2.87), p=0.22), central nervous systems tumor (OR, 1.18(95%CI: 0.74-1.88), p=0.48), respiratory system cancer (OR, 0.94(95%CI: 0.67-1.30), p=0.69), breast cancer (OR, 95%CI: 0.96-1.48), p=0.87, genitourinary system cancer (OR, 1.06(95%CI: 0.73-1.54), p=0.76), and musculoskeletal cancer (OR, 1.40(95%CI: 0.92-2.41), p=0.12) was comparable between CHD and non-CHD groups of patients. Conclusions: Our findings indicate that CHD patients are at higher risk of thyroid and skin cancer compared with non-CHD patients; however, no such association with other cancers was observed. The implications of these results extend to the delineation of subsequent research, and the results underscore the necessity for further exploration into the underlying pathophysiological mechanisms. Potential implications include the establishment of enhanced surveillance and screening protocols to protect this vulnerable patient group. [Table: see text]

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.

Using artificial intelligence and deep learning to optimise the selection of adult congenital heart disease patients in S-ICD screening

IntroductionThe risk of complications associated with transvenous ICDs make the subcutaneous implantable cardiac defibrillator (S-ICD) a valuable alternative in patients with adult congenital heart disease (ACHD). However, higher S-ICD ineligibility and higher inappropriate shock rates-mostly caused by T wave oversensing (TWO)- are observed in this population. We report a novel application of deep learning methods to screen patients for S-ICD eligibility over a longer period than conventional screening. MethodsAdult patients with ACHD and a control group of normal subjects were fitted with a 24-h Holters to record their S-ICD vectors. Their T:R ratio was analysed utilising phase space reconstruction matrices and a deep learning-based model to provide an in-depth description of the T: R variation plot for each vector. T: R variation was compared statistically using t-test. Results13 patients (age 37.4 ± 7.89 years, 61.5 % male, 6 ACHD and 7 control subjects) were enrolled. A significant difference was observed in the mean and median T: R values between the two groups (p < 0.001). There was also a significant difference in the standard deviation of T: R between both groups (p = 0.04). ConclusionsT:R ratio, a main determinant for S-ICD eligibility, is significantly higher with more tendency to fluctuate in ACHD patients when compared to a population with normal hearts. We hypothesise that our novel model could be used to select S-ICD eligible patients by better characterisation of T:R ratio, reducing the risk of TWO and inappropriate shocks in the ACHD patient cohort.

Safety and feasibility of left bundle branch pacing for pediatric and congenital heart disease patients

Abstract Background Conduction system pacing (CSP) has evolved and validated in adults. Due to technical challenges, pediatric and congenital heart disease (CHD) patients were excluded from original trials in this field. We report the safety and feasibility results of our initial experience with pediatric and CHD patients undergoing LBBAP (Left bundle branch area pacing). Purpose To analyze the indications, intraoperative outcomes, electrocardiographic and clinical data from the initial follow-up of pediatric and CHD patients who underwent LBBAP. Methods Indications, intraoperative technical issues, electronic parameters of the implant and early complications of consecutive pediatric and CAD patients submitted to stimulation of the conduction system were evaluated. Results This study was performed between February 2022 and November 2023 and recruited 11 consecutive patients with complete AV block (age: 15-43 years; 7 women) who underwent LBBAP. Four patients had congenital heart disease repaired surgically and two patients with congenitally corrected transposition of the great arteries. Upgrading from VVIR to LBBAP underwent in four patients. Right ventricle (RV) leads were extracted in three patients before CSP. DDD pacemakers with Lumneless 3830 pacing leads were implanted in all patients. The pre-implant QRS duration was 146 (104- 200) ms and 120 (110-130) ms after the procedure. The mean left ventricle activation time was 72 (50-90) ms. The R-wave amplitude was 10.00 (6-29) mV, with a stimulation threshold of 0.8 (0.4-1) V x 0.4 ms and impedance of 676 (534-780) ohms. Transition from non-selective to either myocardial or selective capture and fixation beats were respectively demonstrated in five and three patients. The procedure duration was 125 (70-130) min, and the fluoroscopy time was 15.2 (4-23.6) min. LBBAP substantially shortened the QRS duration and improved LV dyssynchrony echocardiographic parameters in four patients with prior epicardial or endocardial pacing. The LBBAP was successful in all patients with no early complications occured. At mean three months follow-up, QRS duration showed no significant changes compared with baseline. Conclusion Our initial experience on cardiac stimulation of the His-Purkinje conduction system through the stimulation of the left bundle branch is a safe and feasible technique in pediatric and CHD patients. The widespread use of this pacing technique is very useful and cost-effective to prevent heart failure in this vulnerable population.