Congenital Heart Disease Patients Research Articles

Chronotropic Response to Exercise is Decreased in Patients With Congenital Heart Disease Compared to Cardiomyopathy Following Pediatric Heart Transplantation.

Two common indications for pediatric heart transplantation are congenital heart disease and cardiomyopathy. Prior studies suggest differences in chronotropy on cardiopulmonary exercise testing outcomes depending on indication for heart transplantation. We aimed to determine whether the number of pretransplant sternotomies is associated with differences in heart rate response during exercise testing. A retrospective analysis of our institutional pediatric heart transplant data between 2004 and 2022 was performed. Patients were categorized by indication for transplantation into a cardiomyopathy (CM) group if they had a congenital or acquired cardiomyopathy or a congenital heart disease (CHD) group including all other forms of congenital cardiac anatomic abnormalities. CHD patients (n = 40) differed from CM patients (n = 53) by mean number of sternotomies prior to transplant (2.4 ± 1.8 vs. 0.5 ± 0.9, p < 0.001). There were no significant differences in echocardiographic function or catheterization hemodynamics. In cardiopulmonary exercise testing performance, the congenital heart disease group had a significantly higher resting heart rate (91.8 ± 11.2 vs. 86.4 ± 10.2 bpm, p = 0.019), lower percent predicted age-predicted maximal heart rate achieved (78.3 ± 8.5% vs. 83.2 ± 11.4%, p = 0.032), and lower heart rate reserve (68.6 ± 19.8 vs. 84.4 ± 24.0 bpm, p = 0.001) despite a similar age and average time from transplantation. Regression analysis confirmed number of pretransplant sternotomies as a main predictor of heart rate metrics. There is greater chronotropic incompetence in patients who underwent transplantation due to congenital heart disease compared to cardiomyopathy. The groups differ significantly by number of sternotomies, potentially supporting the hypothesis that prior surgical disruption of cardiac innervation may cause decreased chronotropic response to exercise following transplantation.

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

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.

A 3D Statistical Shape Model of the Right Ventricular Outflow Tract in Pulmonary Valve Replacement Patients Post-Surgical Repair.

Assessment of the right ventricular outflow tract and pulmonary arteries (RVOT) for percutaneous pulmonary valve implantation (PPVI) uses discrete measurements (diameters and lengths) from medical images. This multi-centre study identified the 3D RVOT shape features prevalent in patients late after surgical repair of congenital heart disease (CHD). A 3D RVOT statistical shape model (SSM) was computed from 81 retrospectively selected CHD patients (14.7 ± 6.8 years) who required pulmonary valve replacement late after surgical repair. A principal component analysis identified prevalent shape features (modes) within the population which were compared with standard geometric measurements (diameter, length and surface area) and between sub-groups of diagnosis, RVOT type and dysfunction. Shape mode 1 and 2 represented RVOT size and curvature and tapering and length, respectively. Shape modes 3-5 related to branch pulmonary artery calibre, conical vs. bulbous RVOTs and RVOT curvature, respectively. Tetralogy of Fallot, transannular patch type and regurgitant RVOTs were larger and straighter while conduit and stenotic types were longer and more cylindrical than other subgroups. This SSM analysed the main 3D shape features present in a population of RVOTs, exploiting the wide 3D anatomical information provided by routine imaging. This morphological information may have implications for PPVI patient selection and device design.

Inter-atrial Septum Stenting in Congenital Heart Disease Patient: A Case Series in Indonesia.

Inter-atrial septum (IAS) stenting in duct-dependent congenital heart disease patient has shown to be an effective way to maintain inter-atrial blood flow, however it is still considered a high risk procedure and inter-atrial septum stenting remains a low-frequency procedure. A single-center observational cohort study was carried out at the National Cardiovascular Center Harapan Kita (NCCHK) between April 2019 and April 2023. This study included duct-dependent congenital heart disease patients. The extracted data were baseline characteristics, clinical findings, complications, and outcomes of the patients. Eleven patients with duct-dependent physiology were intervened with inter-atrial septum stenting. The patients were 4 females and 7 males with the median age of implantation being 150 days (range 11-703 days) and the median weight being 3.9 (range 2.8-9) kg, with 2 patients weighing less than 3 kg. The average stent diameter was 8.50 (2.03) mm with an average length of 24.45 (7.94) mm. Non-restrictive atrial flow was successfully achieved in 90.90% of the procedures, corresponding to 10 patients. Inter-atrial septum stenting in duct-dependent congenital heart disease patients produces reliable patency with a very good intra-procedural success rate.

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

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

The impact of COVID-19 vaccination on patients with congenital heart disease in England: a case-control study

BackgroundStudies predating widespread COVID-19 vaccination identified patients with congenital heart disease (CHD) as a group at increased risk of severe outcomes from COVID-19. Here we evaluate the impact of vaccination...

Genetic investigation and diagnosis in adults with congenital heart disease with or without structural or neurodevelopmental comorbidity: a retrospective chart review.

Genetic evaluation is indicated for individuals with congenital heart disease (CHD), especially if extracardiac anomalies are also present. Timely recognition of genetic diagnoses can facilitate medical management and as well as provide assessment of reproductive risk. At least 20% of the pediatric population with CHD has a syndrome or genetic diagnosis. Further, at least 30% have extracardiac congenital malformations and/or neurodevelopmental differences (NDD), and this is known to increase the likelihood of a genetic/syndromic diagnosis. However, little is known regarding whether these statistics also apply to the current population of adults living with CHD, many of whom were born prior to currently available genetic testing. The primary aim of this study was to determine the prevalence of documented genetic and syndromic diagnoses in a cohort of adults with CHD followed by a dedicated adult CHD (ACHD) clinic. The secondary aims were to describe genetic testing and genetic referral patterns in this population and identify the presence of extracardiac comorbidities which are known to be indicative of an underlying genetic diagnosis in the pediatric CHD population. To answer these questions, we performed a retrospective chart review on a sample of adults with CHD (excluding those with isolated bicuspid aortic valve) seen at Cincinnati Children's Hospital in the ACHD clinic between 2010-2021. Among 233 adult CHD patients, 36 (14%) had a documented genetic or syndromic diagnosis but only 29 (13.7%) had received genetic testing, while 27 (11.6%) had received genetic referrals. Furthermore, of 170 patients without any documented genetics related care (defined as genetic testing, genetic referrals, or genetic diagnosis), 35 (20%) had at least one congenital and/or neurodevelopmental comorbidity. Factors associated with individuals having received genetics related care included younger age (<40), male sex, and presence of extracardiac comorbidities. Our results indicate important gaps in genetics-related care for adults living with CHD. The subset of our cohort with congenital and/or neurodevelopmental comorbidities who received no genetic-related care, represent a population of adults with CHD who may have unrecognized genetic diagnoses.

Self-gated free-running 5D whole-heart MRI using blind source separation for automated cardiac motion extraction.

To compare two blind source separation (BSS) techniques to principal component analysis and the electrocardiogram for the identification of cardiac triggers in self-gated free-running 5D whole-heart MRI. To ascertain the precision and robustness of the techniques, they were compared in three different noise and contrast regimes. The repeated superior-inferior (SI) projections of a 3D radial trajectory were used to extract the physiological signals in three cardiac MRI cohorts: (1) 9 healthy volunteers without contrast agent injection at 1.5T, (2) 30 ferumoxytol-injected congenital heart disease patients at 1.5T, and (3) 12 gadobutrol-injected patients with suspected coronary artery disease at 3T. Self-gated cardiac triggers were extracted with the three algorithms (principal component analysis [PCA], second-order blind identification [SOBI], and independent component analysis [ICA]) and the difference with the electrocardiogram triggers was calculated. PCA and SOBI triggers were retained for image reconstruction. The image sharpness was ascertained on whole-heart 5D images obtained with PCA and SOBI and compared among the three cohorts. SOBI resulted in smaller trigger differences in Cohorts 1 and 3 compared to PCA (p < 0.01) and in all cohorts compared to ICA (p < 0.04). In Cohorts 1 and 3, the sharpness increased significantly in the reconstructed images when using SOBI instead of PCA (p < 0.03), but not in Cohort 2 (p = 0.4). We have shown that SOBI results in more precisely extracted self-gated triggers than PCA and ICA. The validation across three diverse cohorts demonstrates the robustness of the method against acquisition variability.

Electrocardiographic correlates of ventricular arrhythmias in repaired congenital heart disease

BackgroundSudden cardiac death (SCD) is a major cause of mortality in congenital heart disease (CHD). Ventricular arrhythmias (VAs) are responsible for 80% of SCD. However, the clinical characteristics of patients with CHD and VAs are not fully understood. ObjectiveTo evaluate clinical and electrocardiographic characteristics of patients with CHD based on the presence or absence of VAs. MethodsWe identified 28 CHD patients with VAs (males, n = 15; 35.9 ± 16.5 years). Two patients with channelopathies were excluded from the analysis. ResultsThe most common diagnoses in patients with VAs were a transposition of the great arteries (n = 8, 30.8%), followed by tetralogy of Fallot (n = 7, 26.9%). Compared to patients without VA (n=43), those with VAs had a longer QRS (160.6 ± 52.9ms versus 133.4 ± 29.3ms; P = 0.004), a higher prevalence of fragmented QRS (65.4% versus 37.2%; P = 0.022) and more epsilon wave (23.1% versus 2.3%; P = 0.005). Multivariable analysis showed that QRS>180ms (odd ratio [OR], 22.23; 95% confidence interval [CI] 2.40–206.53) and epsilon wave (OR 14.33; 95% CI, 1.47–139.58) were significantly associated with VAs. During a median follow-up duration of 5.4 years, five patients (19.2%) received appropriate ICD therapy, and three patients (11.5%) died of heart failure. ConclusionThe assessment of QRS duration and the presence of fQRS and epsilon waves in patients with CHD could improve risk prediction for the development of VAs. These ECG features could assist in clinical decision-making and might consequently reduce mortality.

Outcomes in adult congenital heart disease patients with Down syndrome undergoing cardiac surgery.

BACKGROUNDAs the life expectancy of patients with Down syndrome (DS) improves, the number of older DS patients requiring cardiac surgery for congenital heart disease will increase. Perioperative risk factors and outcomes in these patients are unknown. METHODSIn a multicenter retrospective study, teenage and adult DS patients undergoing cardiac surgery between 2008-2018 were matched by age and surgical procedure with non-DS patients. Demographic, medical, and surgical characteristics were compared. Outcome measures were length of stay (LOS), duration of mechanical ventilation, need for non-invasive positive pressure ventilation (NIPPV) and reintubation, additional cardiac interventions, postoperative infections, and early postoperative mortality. Risk factors for extended hospital LOS (>10 days) were explored using multivariable logistic regression. RESULTSWe compared 121 DS patients to 121 non-DS patients. DS patients had a longer median LOS (7 vs. 5 days, p<0.001), longer duration of mechanical ventilation (12.5 vs. 6.7 hours, p<0.001), greater need for NIPPV or reintubation (26% vs. 4%, p<0.001), and higher likelihood of postoperative infections (10% vs. 2%, p=0.035). There was no early mortality. Pre-operative risk factors for extended LOS for DS patients included pulmonary medication use (OR 4.0, p=0.046), history of immunodeficiency (OR 10.4, p=0.004), or ≥moderate tricuspid regurgitation (OR 12.7, p<0.001). CONCLUSIONSTeenage and adult DS patients undergoing congenital cardiac surgery had a longer hospital LOS and more postoperative respiratory and infection complications compared to non-DS patients without increased mortality. Cardiac surgery can be performed safely in older DS patients. Management of pulmonary disease, immunodeficiency, and tricuspid regurgitation may mitigate risk.

A new strategy for monitoring of direct oral anticoagulants in patients with cyanotic and complex congenital heart disease

BackgroundPatients with congenital heart disease (CHD) often require an oral anticoagulation. Vitamin K antagonists (VKA) are the standard treatment, however, an increased hematocrit in patients with secondary erythrocytosis due to cyanosis complicates the correct measurement of the international normalized ratio. Direct oral anticoagulants (DOAC) could be an alternative, but data on their efficacy and safety in complex and cyanotic CHD patients are scarce. This study proposes a new strategy of DOAC monitoring in these patients using D-dimers and DOAC trough levels. MethodsThis is a retrospective study including cyanotic and complex CHD patients requiring oral anticoagulation. Clinical, cardiac imaging and laboratory data were collected before and after start of DOAC. The new monitoring strategy consists of determination of D-dimers and DOAC trough levels at 1–4 weeks, 1–6 months, 6–12 months, >1 year after start of DOAC. ResultsEleven patients were included. For 10 patients D-dimers and DOAC trough levels were in target range. In one patient, D-dimers increased continuously after start of DOAC despite dose escalation, suggesting insufficient DOAC efficacy and finally requiring a switch to VKA. D-dimers subsequently decreased under VKA to the therapeutic range. In three patients, one thromboembolic and two minor bleeding complications occurred. No major complications were observed. ConclusionsWe propose a new strategy of monitoring of oral anticoagulation with DOAC and report its implementation in clinical routine. Highlighting the importance of pharmacokinetic and -dynamic monitoring, this strategy could improve safety and efficacy of DOAC in cyanotic and complex CHD which, however, requires a prospective validation.

Olfactory Bulb Volume and Asymmetry as Predictors of Executive Dysfunction in Adolescents with Congenital Heart Disease.

Common sequelae for patients with congenital heart disease (CHD) are neurodevelopmental disabilities including executive function, attention, and socio-emotional deficits. Although these are common diagnoses for patients with CHD, limited research has investigated the mechanistic underpinnings of these findings. Our previous research examined the association between abnormal respiratory ciliary motion and brain abnormalities in infants with CHD. Results suggested that abnormal ciliary motion correlated to a spectrum of subtle dysplasia, notably within the olfactory bulb (OB)1. Our current study investigates whether OB anomalies predict neurodevelopmental outcomes for pediatric patients with CHD. We hypothesize that adolescents with CHD who exhibit aberrant morphological measurements in the OB are more likely to suffer from executive functional deficits. A prospective, observational study of 54 CHD and 75 healthy subjects, ages 6-25 years old, was completed under the supervision of a senior pediatric neuroradiologist. T2 3D Space and T2 Blade 2MM MRI images were manually segmented to extract volumetric bilateral regions of the OB and cerebrospinal fluid (CSF) using ITK-SNAP. Imaging metrics were correlated to OB asymmetry, CSF to OB ratio, total CSF volume, total OB volume, and independent left and right CSF and OB volumes. Linear regression was used to evaluate MRI morphologic measurements with co-variates: CHD status, sex, MRI age, and segmenter. Executive function was determined by the Behavioral Rating Inventory of Executive Function (BRIEF) Parent Report and Delis-Kaplan Executive Function System (D-KEFS) for subjects ages 6-16. Cognition and olfactory function were measured with the NIH Toolbox Cognitive Battery and Odor Identification Test, respectively. No statistically significant results were reported between cohorts for asymmetry of OB, CSF to OB ratio, total CSF volume, total OB volume, nor between independent left and right CSF and OB volumes. Increased OB volume was associated with worse outcomes on the BRIEF Parent Report (p≤0.03). Asymmetry of OB predicted poorer executive functioning as reported by parents on the BRIEF (p≤0.05). Overall, the CHD cohort demonstrated worse scores on the BRIEF Parent Report compared to controls. Across groups, no significant association was reported for olfaction function measured by the NIH Toolbox Odor Identification Test on a limited subset of participants. As survival rates for CHD improve, there is an increased risk of long-term neurodevelopmental impairments. Our findings identify adolescents who are at risk for executive dysfunction, particularly those showing increased OB volume and/or asymmetry of the OB. This is particularly concerning for the CHD population with atypical OB morphology, who also exhibit significantly poorer outcomes on the BRIEF Parent Report and face a higher overall risk. Increased OB volume and OB asymmetry are olfactory-based biomarkers that may help identify at-risk CHD patients earlier, enabling more timely intervention and support.

Factors Associated with Depressive Syndrome in Adult Congenital Heart Disease Patients at RSUP Haji Adam Malik Medan

Besides being one of the causes of death in the world, adult congenital heart disease sufferers seem to be forgotten and neglected from what is called depression and anxiety. Not much has been described about depressive psychopathology in adult congenital heart disease patients. The purpose of this study was to analyze the factors associated with depressive syndrome in congenital heart disease patients. This cross-sectional study was conducted by 97 adult congenital heart disease patients recruited sequentially, consisting of 8 independent variables and 1 dependent variable. Depression scores were assessed using the BDI-II measuring instrument. Most sexes were female, with as many as 59 subjects (60.2 %). The most marital status was 66 subjects (67.3 %). The most occupations were those who worked as many as 70 subjects (71.4 %), the most previous history of surgery/intervention were those who did not have a history of prior surgery/intervention as many as 56 subjects (57.1 %), the most comorbid diseases were those who did not have a history of comorbid diseases as many as 86 subjects (87.8 %), age with a median value of 33 (18 - 45), the median value of education for adult CHD patients was 15 (12 - 18), the median NYHA grade value of adult CHD patients is 2 (1 - 4). The characteristics of patients who are highly educated, NYHA classmates, and who have jobs should be a concern because they will be more likely to experience depressive syndrome. HIGHLIGHTS Relationship between NYHA class and depression syndrome: Patients with higher NYHA grades are prone to depression. This indicates that the more severe the heart condition, the greater the level of depression. The role of education in depression: The higher the education of heart patients, the more likely they are to develop depression. Effect of work on depression: Patients without work are more prone to depression than patients who work, demonstrating the importance of job stability for inner well-being. Use of BDI-II tool: Depression measurement is carried out using BDI-II, a screening tool that effectively measures depression. GRAPHICAL ABSTRACT

Resuscitation arterial waveform quantification and outcomes in pediatric bidirectional Glenn and Fontan patients.

Resuscitation with chest compressions and positive pressure ventilation in Bidirectional Glenn (BDG) or Fontan physiology may compromise passive venous return and accentuate neurologic injury. We hypothesized that arterial pressure and survival would be better in BDG than Fontan patients. Secondary analyses of the Pediatric Intensive Care Quality of CPR and Improving Outcomes from Pediatric Cardiac Arrest databases. P-values were considered significant if < 0.05. In total, 64 patients had either BDG (42/64, 66%) or Fontan (22/64, 34%) anatomy. Return of spontaneous circulation was achieved in 76% of BDG patients versus 59% of Fontan patients and survival with favorable neurologic outcome in 22/42 (52%) BDG versus 6/22 (27%) Fontan patients, p = 0.067. Twelve of 24 (50%) BDG and 2/7 (29%) Fontan patients who survived to discharge suffered new morbidity as defined by worsening Functional Status Score. More BDG patients achieved adequate DBP (≥25 mmHg for neonates and infants; ≥ 30 mmHg for children) than Fontan patients (21/23 (91%) vs. 5/11 (46%), p = 0.007). Only 27% of Fontan patients survived to hospital discharge with favorable neurologic outcome after CPR, likely driven by inadequate diastolic blood pressure during resuscitation. One half of the BDG patients who survived to hospital discharge had new neurologic morbidity. Cardiac arrest in patients with congenital heart disease (CHD) may present challenges to resuscitation based on the unique cardiovascular physiology resulting from surgical palliation. Recent resuscitation guidelines for CHD patients highlight the lack of data surrounding these special patient populations.1 Univentricular heart disease is palliated by a series of cardiac surgeries that stepwise result in passive pulmonary perfusion from the systemic venous system directly to the pulmonary vascular bed. The bidirectional Glenn (BDG) palliation directly anastomoses the superior vena cava (SVC) to the pulmonary arterial system and leaves normal inferior vena cava (IVC) venous return to the heart.2 The Fontan palliation baffles IVC flow directly to the pulmonary vascular bed which relieves cyanosis due to right to left shunting, but requires systemic ventricular preload to be directly dependent upon pulmonary vascular resistance and intrathoracic pressures.3 IMPACT STATEMENT: Hemodynamic waveforms from 2 large prospective observational studies now allow for exploration of physiology during cardiopulmonary resuscitation for unique anatomy associated with single ventricle congenital heart disease. Fewer patients with Fontan physiology (46%) achieved an adequate diastolic blood pressure (defined as ≥ 25 mmHg for neonates and infants and ≥ 30 mmHg for children) than bidirectional Glenn patients during cardiopulmonary resuscitation (91%, p = 0.007). Only 27% of Fontan patients survived to hospital discharge with favorable neurologic outcome after cardiopulmonary resuscitation. Of the bidirectional Glenn patients who survived, 50% developed a new morbidity as quantified by the Functional Status Score.

ADVANCES IN DIAGNOSIS AND TREATMENT OF CONGENITAL HEART DISEASE: A COMPREHENSIVE REVIEW

The purpose of this article is to provide a comprehensive review of recent advances in the diagnosis and treatment of congenital heart disease (CHD). CHD, which affects about 1% of live births worldwide, is still a major problem in pediatric cardiology. The most prevalent congenital cardiac defects—Ventricular Septal Defect (VSD), Tetralogy of Fallot (TOF), Coronary Artery Fistula, Coarctation of the Aorta (CoA), Aortic and Pulmonary Stenosis, Valve Insufficiency, Double Outlet Right Ventricle (DORV), Ebstein Anomaly, Transposition of the Great Arteries (TGA), Anomalous Venous Return, and Hypoplastic Left Heart Syndrome (HLHS) are all thoroughly examined in this review. The review examines current diagnostic and treatment strategies, highlighting the benefits, drawbacks, sensitivity, and specificity of several imaging modalities, including as CT, MRI, and echocardiography. It also discusses the historical evolution of treatment outcomes, emphasizing the tremendous advances in patient care and surgical procedures that have significantly mproved survival rates and quality of life for CHD patients.

Risk of cardiovascular events after influenza infection-related hospitalizations in adults with congenital heart disease: A nationwide population based study

BackgroundCardiovascular complications due to viral infection pose a significant risk in vulnerable patients such as those with congenital heart disease (CHD). Limited data exists regarding the incidence of influenza and its impact on cardiovascular outcomes among this specific patient population. MethodsA retrospective cohort study was designed using the Canadian Congenital Heart Disease (CanCHD) database - a pan-Canadian database of CHD patients with up to 35 years of follow-up. CHD patients aged 40 to 65 years with influenza virus-associated hospitalizations between 2010 and 2017 were identified and 1:1 matched with CHD patients with limb fracture hospitalizations on age and calendar time. Our primary endpoint was cardiovascular complications: heart failure, acute myocardial infarction, atrial arrhythmia, ventricular arrhythmia, heart block, myocarditis, and pericarditis. ResultsOf the 303 patients identified with incident influenza virus-associated hospitalizations, 255 were matched to 255 patients with limb fracture hospitalizations. Patients with influenza virus-related hospitalizations showed significantly higher cumulative probability of cardiovascular complications at one year (0.16 vs. 0.03) and five years (0.33 vs. 0.15) compared to patients hospitalized with bone fracture. Time-dependent hazard function modeling demonstrated a significantly higher risk of cardiovascular complications within nine months post-discharge for influenza-related hospitalizations. This association was confirmed by Cox regression model (average hazard ratio throughout follow-up: 2.48; 95% CI: 1.59 – 3.84). ConclusionsThis pan-Canadian cohort study of adults with CHD demonstrated an association between influenza virus-related hospitalization and risk of cardiovascular complications during the nine months post discharge. This data is essential in planning surveillance strategies to mitigate adverse outcomes and provides insights into interpreting complication rates of other emerging pathogens, such as COVID-19.