Congenital Heart Disease Patients Research Articles

Transcatheter Pulmonary Flow Restrictors: Current Trends and Future Perspectives.

Transcatheter Pulmonary Flow Restrictors (TPFRs) represent a significant advancement in managing pulmonary blood flow for congenital heart disease patients. However, there is a paucity of comprehensive studies addressing the diversity of these devices and identifying their critical features. This review aims to consolidate the existing knowledge on TPFRs, pinpoint crucial design and development aspects, identify gaps in current practices, and spotlight directions for future research and advancement. An exhaustive search was conducted across multiple databases, using specific search terms related to transcatheter and percutaneous pulmonary artery banding. Between 2005 and 2024, 82 patients were reported to have received TPFR implants, including fenestrated atrial septal defect occluders, diabolo-shaped stents, and MVP™ Micro Vascular Plug with polytetrafluoroethylene (PTFE) membranes partially removed. Microvascular plugs were the most commonly used and the most successful devices. However, the primary complications and challenges associated with MVPs included pulmonary overflow, unprotected flow to the right upper lobe, difficulty in creating an appropriately sized fenestration, the need for device replacement due to incorrect sizing, distal migration into the right pulmonary artery, left pulmonary artery stenosis, partial device collapse, thrombosis, jailing of the right upper lobe, potential injury to the pulmonary arterial wall, as well as device fracture and infection. TPFRs can be categorized based on the duration they are designed to remain within the pulmonary artery. Strategies should be devised to enable the device's easy removal without harming the pulmonary arterial wall while also preventing embolization. The ideal device should minimize migration, embolization, thrombosis, inflammation, and endothelialization risks. It should also prevent peri-device flow and adapt to the growth of the pulmonary artery, ensuring long-term efficacy and safety. The long-term outcomes and the potential for employing biodegradable and smart biomaterials remain areas for further investigation. Successful development of these devices requires a collaborative effort among biomaterial engineers, device developers, and interventional cardiologists.

Extended Donor Criteria Outcomes in Adult Congenital Heart Disease Patients Undergoing Heart Transplantation in the United States.

Background: There are very few published outcomes for the use of extended criteria donor (ECD) organs in adult congenital heart disease (ACHD) patients undergoing cardiac transplantation. We investigated outcomes with the use of ECD organs in this population. We hypothesized that ECD organs may be safely used in this population in patients who are younger and carry less vascular and renal disease than the general cardiac transplant population. Methods: United Network of Organ Sharing data from all ACHD heart transplant recipients between October 18, 2018, and September 30, 2023, was analyzed. Patients were grouped by ECD transplantation. The primary outcome of interest was one-year survival. Secondary outcomes included length of stay, posttransplant stroke, retransplantation, kidney transplantation, dialysis, pacemaker implantation, and rejection. Results: Extended criteria donor organs were utilized in 39.1% (200/512) of ACHD heart transplantation cases. There was marked regional variation in the use of ECD organs. Extended criteria donor criteria in this population were solely met by ischemic time ≥4 h in 89.5% (179/200) of cases. Transplantation with ECD donors was associated with lower survival (one-year survival 82.76% vs 90.62%). A multivariate Cox regression analysis suggested a hazard ratio of 1.96 with the use of these organs. Conclusion: Compared with organs accepted under traditional donor criteria, ECD organs appear to compromise short- and long-term survival in ACHD transplant candidates.

Risk Factors for Chronic Kidney Disease in Adult Patients with Congenital Heart Disease and Its Relationship with Cardiovascular Mortality.

Background: Patients with congenital heart disease (CHD) show risk factors for chronic kidney disease (CKD) and it is well known that CKD has a large negative impact on survival. Methods: Observational and prospective cohort study. Adult CHD patients and controls were matched for age and sex. Results: A total of 657 CHD adult patients (cases) and 1954 controls were studied. Median age in CHD patients was 30 (17-62) years and 373 (57%) were male. The prevalence of CKD (Glomerular filtration rate (GFR) < 60 mL/min/1.73 m2) was 0.2% and 4.5% in the control and CHD groups, respectively. Binary logistic regression analysis determined as risk factors for CKD in CHD patients: age [1.54 (1.04-1.28), p = 0.009], dyslipidemia [19.8 (1.35-301.1), p = 0.031], low iron concentration [0.96 (0.96-0.93), p = 0.048], cyanosis [25.7 (1.60-411.8), p = 0.022], and Down syndrome [46.8 (8.09-2710), p = 0.003]. During a follow-up time of 6.8 (1.2-10.5) years, cardiovascular mortality occurred in 31 patients with CHD showing, through the Kaplan-Meier test, a worse outcome among patients with CKD (p < 0.05) as was also seen in the univariate Cox regression survival analysis. However, after adjusting for other variables, this significance was lost, with age remaining as the unique independent prognostic factor. Conclusions: The prevalence of CKD was much higher in patients with CHD than in the control group; age, cyanosis, and Down syndrome were the predictors of a higher risk of CKD among CHD patients. Although CKD was associated with worse survival in CHD patients, only age was identified as an independent prognostic factor for cardiovascular mortality.

CARDIAC REHABILITATION PROGRAM AS AN EFFORT TO IMPROVE THE QUALITY OF LIFE OF PATIENTS WITH CONgenital HEART DISEASE

ABSTRACT Introduction: Congenital Heart Disease (CHD) is the most common congenital disorder found throughout the world, including Indonesia. Currently, the main treatment for CHD patients is open cardiothoracic surgery. However, its implementation in Indonesia still has some obstacles due to several things which cause the life expectancy of CHD patients in Indonesia is estimated to be not as good as the life expectancy of CHD patients in developed countries. Apart from being related to life expectancy, CHD patients will also experience a decrease in their quality of life, especially those who are not treated properly. The cardiac rehabilitation program is one of the programs to overcome this. Discussion: Cardiac rehabilitation program is able to improve physical and psychosocial quality and ensure that CHD patients' cognitive and socioemotional development is maintained. Cardiac rehabilitation includes physical exercise, psychosocial management, education, risk factor modification, and nutritional management. Several studies have shown that cardiac rehabilitation has many benefits and is safe for CHD patients. In its implementation, cardiac rehabilitation faces several challenges, especially in center-based cardiac rehabilitation (CBCR), so it is recommended to increase home-based cardiac rehabilitation (HBCR) while ensuring that information regarding HBCR is delivered completely and well received by the patients. Conclusion: Based on this literature review, the authors suggest an increase in the implementation of cardiac rehabilitation for CHD patients throughout Indonesia, especially HBCR which can be carried out independently or in the community to improve the patient’s quality of life in the long term. Keywords: congenital, cardiac, rehabilitation, quality of life

Clinical characteristics and long-term outcomes of Venoarterial extracorporeal membrane oxygenation in children with congenital heart disease.

To analyze the clinical outcomes of extracorporeal membrane oxygenation (ECMO) support in children with congenital heart disease (CHD) after surgery and explore the risk factors associated with mortality during long-term follow-up for 3-5 years. We conducted a retrospective observational study at Shanghai Children Medical Center (SCMC) from 2017 to 2021 and reviewed the clinical results and laboratory findings of 188 CHD patients who received ECMO support during this period. The 5-year overall survival rate was 56.38% (106/188) among CHD patients who received ECMO support. Kaplan-Meier curve showed residual anatomical malformation (RAM) (p < 0.0001), gastrointestinal bleeding (p = 0.019), single ventricular (SV) (p = 0.028), and pre-ECMO lactate level >10 mmol/L (p < 0.0001) were significantly associated with higher mortality in follow-up. Cox analysis identified RAM (p = 0.039) and pre-ECMO lactate level >10 mmol/L (p < 0.001) as independent risk factors for overall survival. Conversely, a minimum platelet count ≥50 × 109/L (p < 0.001) was found to be a protective factor. Moreover, a competing risk model showed that a CPR time ≥60 min (p < 0.001) was identified as a risk factor for death in patients who failed to be discharged from the hospital. Our study showed characteristics of long-term follow-up patients and revealed several risk factors associated with mortality in children with CHD who received ECMO support. These findings can provide valuable insights for clinical decision-making and contribute to improving patient outcomes.

Factors contributing to severe scoliosis after open chest surgery for congenital heart disease: a case-control analysis.

Previous reports have identified factors associated with open chest surgery for congenital heart disease (CHD) and scoliosis. However, these reports included conditions such as Down syndrome and Marfan syndrome, which involve both cardiac disease and scoliosis. The relationships between these factors and open chest surgery remain unclear. This study aimed to identify factors contributing to severe scoliosis in CHD patients who have undergone open chest surgery. Seventy-four post-CHD surgery patients with severe scoliosis (Scoliosis group) and 30 post-CHD surgery patients without scoliosis (NS group), excluding those with any syndrome or intellectual disability, were retrospectively analyzed. Patient background characteristics and radiographic parameters were compared between the NS and Scoliosis groups. Furthermore, the patients in the Scoliosis group were classified into three categories, namely, mild scoliosis, moderate scoliosis, and severe scoliosis, and the results were compared among the four groups. Eighteen patients in the NS group and 63 in the Scoliosis group met the inclusion criteria. Compared with the NS group, the Scoliosis group included significantly more girls and patients who had younger ages at first CHD surgery and multiple open chest surgeries. Severe scoliosis progression was observed in patients who underwent multiple surgeries for severe CHD with cardiomegaly. Progression to severe scoliosis was noted in patients with younger ages at first CHD surgery and those who underwent multiple surgeries for severe CHD. Assessing spinal deformities should be a key aspect of postoperative care for CHD, particularly in patients with severe CHD who are undergoing multiple chest surgeries. III.

Abstract 4138620: Assessment of the Accuracy of the Perioperative Mortality Risk in Adults with Congenital Heart Disease (PEACH) Score

INTRODUCTION: Previous mortality risk scores for adult cardiac surgery patients did not include adult congenital heart disease (ACHD) patients. Development of ACHD specific mortality risk scores include the procedure-specific ACHS score and recently derived PEACH score. While validated in Europe, further evaluation of the PEACH score in additional settings is necessary. RESEARCH QUESTION: We assessed the efficacy of the PEACH score to predict mortality in ACHD patients undergoing congenital cardiac surgery at our institution. We compared the PEACH score to the ACHS and STS scores and assessed for additional risk factors which may further augment the PEACH score. Methods: A retrospective cohort study was conducted of patients who underwent congenital cardiac surgery at the University of Colorado Hospital and Children’s Hospital Colorado between 2010-2022. Patients were identified by the institutional STS congenital cardiac surgery database and were eligible if 18 years or older and underwent congenital cardiac surgery. Components of the PEACH score and additional postulated risk factors were evaluated by univariate analysis. A receiver operating characteristic (ROC) curve was used to compare the PEACH score to existing risk models. Logistic regression analysis determined additional risk factors to augment the PEACH score. Results: A total of 516 patients were included with a median age of 30 (IQR 23-42) and 51% were male. There were nine deaths overall. The PEACH score was predictive of mortality (OR: 2.09, p&lt;0.001) with a score of 0: 0 deaths/240 patients (0%); 1-2: 6 deaths/229 patients (2.6%); 3+: 3 deaths/41 patients (7.3%). Of additional risk factors tested, platelets less than 184,000 per mcL (OR: 8.36, p=0.01) was significantly associated with mortality. Comparison of the models revealed PEACH score AUC 0.832 (95% CI: 0.740,0.924), ACHS score AUC 0.869 (95% CI: 0.806, 0.932), and STAT AUC 0.769 (95% CI: 0.616, 0.922, Figure 1). The addition of platelet level improved the predictive ability of the PEACH score (AUC 0.881). Conclusion: In this single-center analysis, the PEACH score provided strong predictive ability for peri-operative mortality. The addition of platelet levels may further augment this score.

Outcomes of Congenital Heart Diseases in Duhok, Kurdistan Region of Iraq

Background: Congenital heart diseases (CHD) are the most common birth defects worldwide, significantly impacting morbidity and mortality, especially in low- and middle-income countries where early diagnosis and access to surgical care are limited. In Iraq, congenital heart surgeries are relatively new, and few studies have investigated CHD outcomes in this context. Aim: This study aimed to evaluate the prevalence, distribution, and surgical outcomes of congenital heart diseases among patients treated at the Azadi Heart Center in Duhok, Kurdistan Region of Iraq. Patients and Methods: A retrospective, cross-sectional study was conducted involving 111 patients aged 1-69 years with CHD who underwent surgery at the Azadi Heart Center between March 2019 and March 2024. Data were collected on demographic characteristics, clinical profiles, surgical details, and postoperative complications, including mortality rates. Statistical analysis was performed using Microsoft Excel, with data presented as means, standard deviations, frequencies, and percentages. Results: The average age of patients in the study was 13 years (±16), with congenital heart disease (CHD) being most common among children, who made up 80.18% of cases. Male patients accounted for 54.95%. The most frequent CHD types observed were Tetralogy of Fallot (25%), isolated Ventricular Septal Defect (20%), and Atrial Septal Defect (10%). The surgeries were highly successful, with a 95.5% success rate. However, some patients experienced postoperative complications, such as pneumonia (11.71%), arrhythmias (10.96%), and surgical site infections (5.41%). The overall mortality rate was 10.81%, with infections being a leading cause of death after surgery. Conclusions: CHD is prevalent among young patients in Duhok, with favorable surgical outcomes and acceptable complication rates. These findings highlight the need for early detection, enhanced surgical resources, and continued surveillance to improve outcomes for CHD patients in Iraq.

A novel non-contrast agent-enhanced 3D whole-heart magnetic resonance sequence for congenital heart disease patients: the REACT Study.

The three-dimensional balanced-steady-state-free-precession (3D bSSFP) whole-heart (WH) technique has long been used to depict cardiac morphology in congenital heart disease (CHD) but is prone to banding artifacts. The Relaxation Enhanced Angiography without Contrast and Triggering (REACT) sequence is an alternative method that is resistant to off-resonance effects. To evaluate cardiac structures and great vessels in CHD patients using 3D WH REACT sequence and compare it to 3D WH bSSFP sequence. This study was approved by the Institutional Review Board. Thirty CHD patients were prospectively enrolled. Contrast-to-noise ratio (CNR), image quality, and cross-sectional area (CSA) were analyzed. Categorical data were compared with a Wilcoxon signed-rank test and normally distributed variables with a t-test. Thirty patients (16 females) participated in this study (median age 17, range 5months to 52years). REACT showed higher CNR in all pulmonary veins (all P<0.05), while 3D bSSFP had higher CNR in the right ventricle (P<0.001) and right pulmonary artery, (P=0.04). Image quality favored 3D bSSFP in the right atrium and ventricle (both P<0.001), main pulmonary artery (P=0.02), and coronary arteries (left: P<0.001, right: P=0.01). REACT outperformed 3D bSSFP for the pulmonary veins (all P<0.05) from image quality perspective. CSA measurements were not significantly different between REACT and 3D bSSFP (all P≥0.05). The REACT method is associated with improved image quality and CNR for pulmonary veins, with CSA measurements concordant with 3D bSSFP in CHD patients, while bSSFP shows better performance for imaging cardiac chambers and coronary arteries.

Utility of Serum Matrix Metalloproteinase-7 as a Biomarker in Cholestatic Infants with Congenital Heart Disease.

Matrix metalloproteinase 7 (MMP-7) is a novel biomarker for diagnosis of biliary atresia (BA), the most common cholestatic liver disease in infancy. There is a pressing need to determine the utility of MMP-7 levels in infants with congenital heart disease (CHD) to avoid unnecessary invasive diagnostic procedures in this high-risk population. We investigated the utility of MMP-7 in discriminating BA from non-BA cholestasis in infants with CHD and whether MMP-7 elevation was present in infants requiring treatment for clinically significant PH. This is a single-center cross-sectional study including infants < 180days of age with cholestasis and serum MMP-7 levels collected from 2019 to 2023. Demographic data and descriptive statistics were summarized with medians with interquartile ranges and frequencies with percentages. Median MMP-7 levels were assessed via Wilcoxon rank-sum test. A total of 149 patients were included. Patients with CHD had significantly elevated MMP-7 levels relative to the non-CHD cohort (50 vs. 34ng/mL, p = 0.009). Sub-analysis comparing infants with and without PH revealed significantly elevated median MMP-7 levels in those with clinically significant PH (125 vs. 39ng/mL, p = 0.010). CHD patients with PH had greater median MMP-7 compared to CHD patients without PH (154 vs 43ng/mL, p = 0.028). Serum MMP-7 levels in infants with congenital heart disease with cholestasis (CHD-C) were significantly elevated compared to those with cholestasis alone. MMP-7 may help identify non-BA cholestatic infants who have concurrent clinically significant pulmonary hypertension. Larger, prospective studies are needed to validate this finding and establish CHD-specific MMP-7 cut-offs.

Interventional Management of a Rare Case of Complex Congenital Heart Disease in an Adult Patient: A Case Report

Congenital heart disease (CHD), which accounts for about one-third of all congenital defects and affects approximately 1% of all live births worldwide, has maintained a stable incidence rate while experiencing a decrease in mortality rates since 1990. Despite advancements in fetal cardiac ultrasound examinations and routine pulse oximetry screening of newborns, a significant proportion of CHD patients may still be undiagnosed until adulthood. This oversight can lead to considerable morbidity and mortality due to physical limitations and reduced quality of life. In this case report, we describe a 29-year-old woman who was initially misdiagnosed as having an inoperable case of CHD and was referred to our center with symptoms of palpitations and exertional dyspnea. During her evaluation, she was diagnosed with severe pulmonary hypertension associated with patent ductus arteriosus (PDA), pre-ductal aortic coarctation, ventricular septal defect (VSD), and bicuspid aortic valve, all of which had been overlooked since infancy. Following initial medical treatment for pulmonary hypertension, a simultaneous transcatheter approach was employed, during which the PDA was closed using an occluder device, and the coarctation was repaired concurrently with a self-expanding stent. Eight months later, her VSD was successfully closed using a muscular VSD occluder device through an interventional technique. This case underscores the importance of adult CHD fellowship training, which enables cardiologists specializing in this field to accurately diagnose and treat complex cases of CHD that may have been missed during infancy and early childhood. This timely intervention ultimately led to the complete relief of her symptoms and prevented progression to Eisenmenger syndrome.

Lead Longevity in Pediatric and Congenital Heart Disease Patients: The Impact of Patient Somatic Growth.

Pacemakers and implantable cardioverter-defibrillators in pediatric and congenital heart disease (CHD) patients may be required for decades. In this population, there are sparse data on long-term lead functionality. The aims of this study were to assess pacemaker and defibrillator lead survival in pediatric and CHD patients beyond 10 years after implantation and to identify patient- and lead-related factors associated with earlier lead failure. This was a retrospective study reviewing all patients with a pacemaker or defibrillator who received care at a single large children's hospital during a 30-year timespan. The log-rank test and Cox proportional hazards model were used to identify risk factors associated with earlier lead failure. Data were collected from 952 leads in 396 patients. Overall lead survival was 87% at 10 years, 78% at 15 years, and 69% at 20 years. Male sex, younger patient age, greater somatic growth, left ventricular lead location, and epicardial implantation approach were associated with higher likelihood of lead failure (log-rank test P<0.05). On multivariate analysis, the most significant predictor of lead failure was patient somatic growth≥5cm/year (HR 3.33; 95%CI: 1.78-6.25). The presence of CHD, lead insulation, and lead manufacturer had no impact on lead longevity. Greater patient somatic growth is an important predictor of lead failure. Greater somatic growth may account for the observation in this study (and prior studies) that leads in male patients, younger patients, and implanted via epicardial approach were more likely to fail.

Preoperative nutritional status and serum insulin-like growth factor of children with cyanotic and acyanotic congenital heart disease

BackgroundMalnutrition is common among children with congenital heart disease (CHD). We compared the anthropometric indices, serum insulin-like growth factor (IGF), and IGF acid-labile subunit (IGFALS) of children with cyanotic and acyanotic CHD before corrective surgery. MethodsThis 82-patient case-control study included 1- to 24-month-old CHD patients referred for corrective surgery. (41 with tetralogy of Fallot [TOF] and 41 with ventricular septal defect [VSD] or atrial septal defect [ASD]). Anthropometric indices represented as Z-scores were used to determine nutritional status. Serum IGF-1 and IGFALS levels were measured. ResultsThe median [quartile] age of the acyanotic group was 8 [7,11] months which was lower than the cyanotic group (11 [8,14.5] months). The prevalence of underweight (weight for age Z [WAZ] < −2), wasting (weight for length Z [WLZ] < −2), and thinness (body mass index Z [BMIZ] < −2) was significantly higher in children with acyanotic than cyanotic children with. WAZ, WLZ, and BMIZ were significantly lower in acyanotic children than cyanotic children with CHD (-2.5±1.2 vs -1.0±1.2, p<0.001 for WAZ, -2.5± 1.5 vs -0.8±1.4, p<0.001 for WLZ, and -2.5±1.5 vs -0.8±1.4, p<0.001 for BMIZ), but length for age Z was not different between the two groups (-1.2±1.0 vs -0.8±1.1, p=0.31). A comparison of preoperative serum albumin, IGF-1, and IGFALS showed no differences. ConclusionsIn CHD children without corrective surgery, moderate to severe underweight and wasting were more common in acyanotic CHD (VSD and ASD) than in cyanotic CHD (TOF), but the higher prevalence of malnutrition was not associated with lower IGF-1 and IGFALS levels.

Physical Activity Levels in Children with Esophageal Atresia and Congenital Heart Disease: A Comparative Multicenter Study.

Esophageal atresia (EA) is associated with impaired motor development, cardiopulmonary function, and physical activity (PA). Despite missing scientific evidence, this fact is often attributed to associated congenital heart disease (CHD). The aim of this study was to investigate PA in EA patients without CHD compared with CHD patients and healthy controls. In this multicenter study, EA patients aged 6 through 17 years were included. Moderate-to-vigorous PA (MVPA, minutes per week) was assessed using the standardized and validated questionnaire Motorik-Modul Physical Activity Questionnaire. EA patients were randomly matched 1:4 for gender and age with patients with CHD (n = 1,262) and healthy controls (n = 6,233). Patients born with both EA and CHD were excluded. Means and 95% confidence intervals (95% CIs) were calculated. To identify associated factors, Spearman's correlation was performed. Overall, 69 EA patients were matched with 276 CHD patients and 276 controls (57% male, 43% female, mean age 10,3 years, 95% CI: 9.5-11.1). Mean MVPA was reduced in EA (492 minutes, 95% CI: 387-598) and CHD patients (463 minutes, 95% CI: 416-511) compared with controls (613 minutes, 95% CI: 548-679). In subgroup analysis, MVPA was reduced further in females and older age groups with EA and CHD. For EA patients, there was no statistical association between Gross type, current symptoms, surgical approaches, and MVPA. Isolated EA and CHD were associated with reduced PA, especially in females and teenagers. To avoid additional morbidity associated with sedentary behavior, PA should be promoted during follow-up.

A meta-analysis of the effects of sacubitril-valsartan on surrogate markers of systemic right ventricular dysfunction in congenital heart disease

Abstract Introduction Dysfunction of systemic right ventricle (sRV) in adult congenital heart disease (ACHD) patients is expectable and often associated with heart failure and other dismal cardiovascular events. Despite limited evidence, several neurohormonal modulators approved for heart failure with reduced ejection fraction (HFrEF) have been used off-label in patients with RV dysfunction1. Sacubitril-valsartan (ARNI) effects on sRV remains poorly studied, with current evidence being limited to small cohorts. Purpose Review and summarize current evidence about the effects of ARNI in ACHD patients with sRV. Methods We performed a systematic review on MEDLINE, EMBASE, COCHRANE, and SCOPUS for articles that included "systemic right ventricle" and "sacubitril valsartan" until January 2024. Two investigators screened the literature and extracted data. Treatment effects were measured by Cohen's d effect-size (EZ) pre and post-ARNI, with a 95% confidence interval (CI), using a restricted maximum likelihood model. Results From 197 publications screened, five studies fulfilled our criteria, comprising a total of 135 patients. Follow-up after ARNI therapy ranged from 12 to 27 months. No randomized clinical trials were found, and only two studies had a prospective design. Dextro-transposition of the great arteries (D-TGA) after atrial switch operation was the most common type of congenital heart disease with sRV (n=95; 70%). Meta-analysis demonstrates that ARNI reduced NT-proBNP levels during follow-up (EZ= -0.34, CI -0.59 to 0.08; p=0.01; I2=4.0%). Regarding RV function, ARNI improved RV fractional area change (RVFAC) (EZ= 0.75, CI 0.01 to 1.50; p= 0.048; I2=85.0%). A trend for RV global longitudinal strain improvement was also observed with ARNI (EZ= 1.74, CI -0.05 to 3.54; p=0.06; I2=95.9%) but not for tricuspid annular plane systolic excursion (EZ= -0.07, CI -0.33 to 0.20; p=0.62; I2=0.0%). RV diastolic diameter did not change significantly with ARNI (EZ= 0.07, CI -0.43 to 0.56; p=0.79; I2=68.2%). A trend for improvement on the 6-minute walk test was observed (EZ= 1.89, CI -0.13 to 3.91; p=0.07; I2=96.3%), but no effect on exercise oxygen consumption (VO2) (EZ= -0.09, CI -1.32 to 1.15; p=0.89; I2=91.4%). During follow-up ARNI did not change significantly systolic blood pressure (EZ= -0.32, CI -0.74 to 0.11; p=0.15; I2=58.4%) or serum creatinine (EZ= 0.23, CI -0.04 to 0.49; p=0.09; I2=0.0%). Conclusions Despite heterogeneity among these studies, this meta-analysis demonstrates that ARNI might improve NT-proBNP levels and RV function. ARNI seems safe and well tolerated, with no significant effect on blood pressure and renal function. More well-powered studies are needed to confirm the beneficial effects of ARNI in ACHD with sRV.

E-selectin and asymmetric dimethylarginine plasma levels in adult cyanotic congenital heart disease patients: relation to biochemical parameters, vascular function, and clinical status.

Abstract Background and Aim Patients with cyanosis secondary to congenital heart disease (CHD) are characterized by erythrocytosis and increased blood viscosity, which contributes to endothelial dysfunction, increased arterial stiffness, and impaired vascular function, which may affect the final clinical presentation. Asymmetric dimethylarginine (ADMA) and e-selectin (e-sel) are valuable biomarkers for prognosis of endocardial dysfunction and vascular damage. Their concentration levels in blood serum have the potential to be a rapid and easily accessible tool that reflects the severity of the disease. We aimed to assess e-sel and ADMA levels, their relationship with clinical status, as well as endothelial and vascular function. Methods A cross-sectional study including 36 adult CHD cyanotic patients [(17 males)(42.3 +/- 16.3 years)] with arterial blood oxygen saturation less than 92% and 20 healthy controls [(10 males)( 38.2 +/- 8.5 years)] was performed. All patients underwent clinical examination, blood testing, and cardiopulmonary tests. Endothelial function was assessed using intima-media thickness and flow-mediated dilatation (FMD). Vascular function using applanation tonometry methods was completed while considering aortic systolic pressure, aortic pulse pressure (AoPP), augmentation pressure (AP), augmentation index (AI), pulse pressure amplification (PPampl), and pulse wave velocity (PWV). Results Concentrations of e-sel and ADMA were significantly higher in patients with CHD than in controls (56.3 ± 13.6 ng/mL vs. 15.5 +/- 8.6 ng/mL, p&amp;lt;0.001) and (1.48 ± 0.48 ng/mL vs. 0.46 ± 0.1 ng/mL, p&amp;lt;0.001) respectively. E-sel levels correlated with gender, blood oxygen saturation, red blood cell, hemoglobin concentration, hematocrit, augmentation pressure, forced expiratory one second volume, forced vital capacity, and oxygen uptake. ADMA levels were found to correlate with age only. Conclusions E-sel level, unlike ADMA concentration, reflects the degree of severity of erythrocytosis and hypoxia and, thus, the physical status of patients with cyanotic CHD. E-selectin predicts increased augmentation pressure.Figure 1.A) ROC analysis for e-selectin

Connecting transcriptomics with computational modeling to reveal developmental adaptations in pediatric human atrial tissue.

Nearly 1% of babies are born with congenital heart disease-many of whom will require heart surgery within the first few years of life. A detailed understanding of cardiac maturation can help to expand our knowledge on cardiac diseases that develop during gestation, identify age-appropriate drug therapies, and inform clinical care decisions related to surgical repair and postoperative management. Yet, to date, our knowledge of the temporal changes that cardiomyocytes undergo during postnatal development is limited. In this study, we collected right atrial tissue samples from pediatric patients (n = 117) undergoing heart surgery. Patients were stratified into five age groups. We measured age-dependent adaptations in cardiac gene expression and used computational modeling to simulate action potential and calcium transients. Enrichment of differentially expressed genes revealed age-dependent changes in several key biological processes (e.g., cell cycle, structural organization), cardiac ion channels, and calcium handling genes. Gene-associated changes in ionic currents exhibited age-dependent trends, with changes in calcium handling (INCX) and repolarization (IK1) most strongly associated with an age-dependent decrease in the action potential plateau potential and increase in triangulation, respectively. We observed a shift in repolarization reserve, with lower IKr expression in younger patients, a finding potentially tied to an increased amplitude of IKs that could be triggered by elevated sympathetic activation in pediatric patients. Collectively, this study provides valuable insights into age-dependent changes in human cardiac gene expression and electrophysiology, shedding light on molecular mechanisms underlying cardiac maturation and function throughout development.NEW & NOTEWORTHY To date, our knowledge of the temporal changes that cardiomyocytes undergo during postnatal development is limited. In this study, we demonstrate age-dependent adaptations in the gene expression profile of >100 atrial tissue samples collected from congenital heart disease patients. We coupled transcriptomics datasets with computational modeling to simulate action potentials and calcium transients for different pediatric age groups.

Management and outcomes of acute appendicitis in children with congenital heart disease.

Congenital heart disease (CHD) care has evolved during the past decades. Advances in care have contributed to improved survival among CHD patients. Children with CHD are requiring interventions for non-CHD related medical issues that occur in the general pediatric population. A retrospective review of the Pediatric Health Information System (PHIS) database from January 1, 2004, to July 31, 2023. Discharges of patients with an admitting/principal diagnosis of appendicitis were evaluated and categorized as CHD or non-CHD. A total of 319,228 patients were identified with 1,25,858(39.4%) female, 1,38,966(43.5%) white, and median age of 11[IQR:8-14] years. 708(0.2%) had CHD with 85(12%) of them having a diagnosis consistent with single-ventricle CHD (SV-CHD). In univariate analysis, CHD patients were more likely to undergo conservative treatment (n = 172(24.2%)vs n = 59,358(18.6%)) and less likely to undergo laparoscopic appendectomy (n = 483(68.2%) vs n = 2,35,324(73.8%))(p < 0.001) compared to non-CHD. After adjustment, CHD patients had increased odds of undergoing open appendectomy compared to non-CHD. CHD patients were more likely to have an ICU admission (OR:8.36(95%CI 6.35-10.00),p < 0.001) and had a 77.6%(95%CI 40.89-123.93) increase in length of stay (LOS) (p < 0.001). CHD patients are more likely to have an open appendectomy than non-CHD patients. These findings suggest a distinctive pattern in the care of CHD patients compared to non-CHD. Overall, CHD patients had a more intense level of care with longer LOS and increased ICU admissions. Further work is needed to evaluate drivers of management decisions, the role of conservative treatment with antibiotics alone in the CHD population, and the potential impacts and safety of a laparoscopic approach.

Hematological Profile of Congenital Heart Disease Patients undergoing Surgical Correction: A Case–control Observational Study from North India

Hematological Profile of Congenital Heart Disease Patients undergoing Surgical Correction: A Case–control Observational Study from North India

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.