Congenital Heart Disease Patients Research Articles

Counting on random number generation: Uncovering mild executive dysfunction in congenital heart disease

Congenital heart disease (CHD) is associated with various neurocognitive deficits, particularly targeting executive functions (EFs), of which random number generation (RNG) is one indicator. RNG has, however, never been investigated in CHD. We administered the Mental Dice Task (MDT) to 67 young adults with CHD and 55 healthy controls. This 1-minute-task requires the generation of numbers 1 to 6 in a random sequence. RNG performance was correlated with a global EF score. Participants underwent MRI to examine structural-volumetric correlates of RNG. Compared to controls, CHD patients showed increased backward counting, reflecting deficient inhibition of automatized behavior. They also lacked a small-number bias (higher frequency of small relative to large numbers). RNG performance was associated with global EF scores in both groups. In CHD patients, MRI revealed an inverse association of counting bias with most of the volumetric measurements and the amount of small numbers was positively associated with corpus callosum volume, suggesting callosal involvement in the “pseudoneglect in number space”.In conclusion, we found an impaired RNG performance in CHD patients, which is associated with brain volumetric measures. RNG, reportedly resistant to learning effects, may be an ideal task for the longitudinal assessment of EFs in patients with CHD.

Altered white matter connectivity in children with congenital heart disease with single ventricle physiology

Children born with congenital heart disease (CHD) have seen a dramatic decrease in mortality thanks to surgical innovations. However, there are numerous risk factors associated with CHD that can disrupt neurodevelopment. Recent studies have found that psychological deficits and structural brain abnormalities persist into adulthood. The goal of the current study was to investigate white matter connectivity in early school-age children (6–11 years), born with complex cyanotic CHD (single ventricle physiology), who have undergone Fontan palliation, compared to a group of heart-healthy, typically developing controls (TPC). Additionally, we investigated associations between white matter tract connectivity and measures on a comprehensive neuropsychological battery within each group. Our results suggest CHD patients exhibit widespread decreases in white matter connectivity, and the extent of these decreases is related to performance in several cognitive domains. Analysis of network topology showed that hub distribution was more extensive and bilateral in the TPC group. Our results are consistent with previous studies suggesting perinatal ischemia leads to white matter lesions and delayed maturation.

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

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

Moderate and Severe Congenital Heart Diseases Adversely Affect the Growth of Children in Italy: A Retrospective Monocentric Study.

Children with congenital heart disease (CHD) are at increased risk for undernutrition. The aim of our study was to describe the growth parameters of Italian children with CHD compared to healthy children. We performed a cross-sectional study collecting the anthropometric data of pediatric patients with CHD and healthy controls. WHO and Italian z-scores for weight for age (WZ), length/height for age (HZ), weight for height (WHZ) and body mass index (BMIZ) were collected. A total of 657 patients (566 with CHD and 91 healthy controls) were enrolled: 255 had mild CHD, 223 had moderate CHD and 88 had severe CHD. Compared to CHD patients, healthy children were younger (age: 7.5 ± 5.4 vs. 5.6 ± 4.3 years, p = 0.0009), taller/longer (HZ: 0.14 ± 1.41 vs. 0.62 ± 1.20, p < 0.002) and heavier (WZ: -0,07 ± 1.32 vs. 0.31 ± 1.13, p = 0.009) with no significant differences in BMIZ (-0,14 ± 1.24 vs. -0.07 ± 1.13, p = 0.64) and WHZ (0.05 ± 1.47 vs. 0.43 ± 1.07, p = 0.1187). Moderate and severe CHD patients presented lower z-scores at any age, with a more remarkable difference in children younger than 2 years (WZ) and older than 5 years (HZ, WZ and BMIZ). Stunting and underweight were significantly more present in children affected by CHD (p < 0.01). In conclusion, CHD negatively affects the growth of children based on the severity of the disease, even in a high-income country, resulting in a significant percentage of undernutrition in this population.

Right ventricular cardiac resynchronization therapy in patients with right ventricular conduction delay and heart failure

Right ventricular cardiac resynchronization therapy in patients with right ventricular conduction delay and heart failure

Overview of Risk Factors for Toddlers with Congenital Heart Disease Suffering from Pneumonia at Dr. M. Djamil General Hospital, Padang, Indonesia

Background: Congenital heart disease (CHD) includes structural and non-structural abnormalities that arise from birth. Disorders of the circulatory system can also affect the respiratory system, causing symptoms and disturbances that similar to primary respiratory disease. This study aimed to describe the risk factors for toddlers with congenital heart disease who suffer from pneumonia at Dr. M. Djamil General Hospital, Padang, Indonesia.&#x0D; Methods: Descriptive observational study using secondary data. A total of 58 research subjects participated in the study. The research subjects met the inclusion criteria in the form of patients who had been diagnosed with congenital heart disease, aged 29 days – 60 months, and were treated at Dr. M. Djamil General Hospital, Padang, Indonesia. Data analysis was performed using SPSS software univariately.&#x0D; Results: The majority of patients with various age levels have a cured outcome. The majority of subjects with medium and high parental education had a greater recovery percentage than subjects with low parental education. Subjects with medium and high socioeconomic status had a greater percentage of recovery outcomes than subjects with low socioeconomic status. The majority of subjects with normal nutritional status and normal birth weight had a higher percentage of recovered outcomes than those who died. The history of DPT immunization and the type of congenital heart disease did not show a large percentage difference between patients who recovered and died. History of measles immunization and leukocytosis showed a greater percentage of patients with cured outcomes than death outcomes.&#x0D; Conclusion: CHD patients with pneumonia are under 1 year old, male, come from families with low-medium socioeconomic status, low-medium educational level, and malnutrition status have worse patient outcomes.

Prophylaxis protects infants with congenital heart disease from severe forms of RSV infection: an Italian observational retrospective study

BackgroundIn children with congenital heart disease (CHD) respiratory syncytial virus (RSV) infection may have a severe course, with increased risk of morbidity and mortality, requiring hospital admission and intensive care. The aim of the present study was to evaluate the effect of prophylaxis with palivizumab in preventing RSV-associated hospitalization in infants with CHD.MethodsWe carried out an observational, retrospective study in a paediatric cardiology division at a secondary-care centre in Italy, extracting from the database children with CHD who, from November 2004 to March 2022, matched the criteria for palivizumab prophylaxis, to evaluate the hospitalization rate in CHD patients with and without palivizumab prophylaxis and their RSV-related hospitalization characteristics compared with a group of children without CHD and no other underlying clinical conditions (control group, CG), hospitalized for RSV infection.ResultsOne hundred twenty-eight children with CHD were enrolled in the study, mainly (71.9%) with increased pulmonary flow, and received palivizumab prophylaxis. Twenty-seven received hospital care for bronchiolitis. Almost all CHD patients hospitalized for bronchiolitis (26 out of 27) received partial prophylaxis (≤ 3 doses). CHD patients with bronchiolitis stay longer in the hospital than control (14.4 ± 21.7 days vs 6.2 ± 2.3 days) some of which require intensive care (n = 4).ConclusionsOur study provides evidence of the efficacy of palivizumab in protecting patients with hemodynamically significant CHD under the age of 2 years from RSV disease and its life-threatening complications. Reducing hospitalisation rate, morbidity, and mortality in this category of patients, passive immune prophylaxis with palivizumab may impact healthcare resource availability and utilisation.

SCUBA Diving in Adult Congenital Heart Disease.

Conventionally, scuba diving has been discouraged for adult patients with congenital heart disease (ACHD). This restrictive sports advice is based on expert opinion in the absence of high-quality diving-specific studies. However, as survival and quality of life in congenital heart disease (CHD) patients have dramatically improved in the last decades, a critical appraisal whether such restrictive sports advice is still applicable is warranted. In this review, the cardiovascular effects of diving are described and a framework for the work-up for ACHD patients wishing to engage in scuba diving is provided. In addition, diving recommendations for specific CHD diagnostic groups are proposed.

Neurodevelopmental Assessment in Children With Congenital Heart Disease by Applying the Denver Developmental Screening Test 2: A Prospective Cross-Sectional Study.

Background Congenital heart conditions often cause developmental delays and impact neurodevelopment throughout one's lifetime. Hence, it is crucial to analyze the impact that heart defects have on the developing brain of a child. The present cross-sectional study was undertaken given the paucity of studies on the developmental status in children with congenital heart diseases (CHDs) in central India, where we tried to evaluate and compare the prevalence of neurodevelopmental delay in individuals with different congenital cardiac disorders. The objectives of our study were, firstly, to utilize the Denver Developmental Screening Test 2 (DDST-2) to evaluate the neurodevelopmental conditions in children with CHD; secondly, to compare the neurodevelopmental state of children with acyanoticCHD (ACHD) and cyanotic CHD (CCHD); andthirdly, to ascertain the prevalence of developmental delay in children with CHD. Methodology The study population comprised children aged six months to six years with two-dimensional (2D) echocardiography confirmation of CHD; those who were critically ill, had genetic syndromes, and were not willing to participate in the study were excluded.The neurodevelopmental assessment was conducted using the DDST-2. The screening looked at each patient's progress in four areas: personal-social, fine motor-adaptive, language, and gross motor. Based on these observations, results were obtained and interpreted. Result Out of 82 children with CHD, the prevalence rate of developmental delay according to the DDST-2 was found to be maximum in the gross motor domain and the least affected in the social domain, which was similar to the analysis of developmental delay by developmental quotient (DQ).The comparative analysis of developmental delay in ACHD and CCHD according to the DDST-2 showed a significant P value only in the gross motor domain. Conclusion The DDST-2 is a straightforward screening tool for determining how well-developed infants with CHD are. The gross motor domain is the most frequently damaged in ACHD and CCHD, followed by the fine motor domain, and the social domain is the least affected. Cyanotic CHD patients are more susceptible to developmental delay than children with ACHD.

Sifrim-Hitz-Weiss/CHD4-related syndrome: A new case report.

Sifrim-Hitz-Weiss/CHD4-related syndrome: A new case report.

Factors Associated with Mortality in Children with Congenital Heart Disease and Cerebral Abscess

BACKGROUND: In children, a cerebral abscess is a rare yet life-threatening condition. Children account for about 25% of all cerebral abscess cases in the general population. One of the most common predisposing factors for developing a cerebral abscess with increased mortality risk is congenital heart disease (CHD). AIM: This study aims to determine the prevalence of death and factors associated with death in children with CHD and cerebral abscesses. METHODS: This study is a cross-sectional design which collects retrospective data. Secondary data of CHD patients with cerebral abscesses were retrieved from patients’ medical records. RESULTS: From January 1, 2012, to December 31, 2021, a total of 107 children with a history of CHD were diagnosed with cerebral abscesses during this study. Thus, mortality rates in cases with cerebral abscess were 38.3% in children with a history of CHD. Patients with a focal infection in the ear, nose, and throat (ENT) area have a 3.6 times higher risk of dying than those with a focal infection elsewhere (odds ratio [OR] = 3.6 [95% confidence interval [CI], 1.57–8.32, p = 0.004). Patients who did not have neurosurgery had a 2.5- fold increased risk of death than those who did (OR = 2.5, 95% CI, 1.11–5.96, p &lt; 0.05). Nutritional status, anemia status, and culture results, which were anticipated to be linked with death in CHD children with cerebral abscess, were not statistically significant. CONCLUSION: We conclude that this study provides an overview of the prevalence of mortality and variables related to death in incidents of cerebral abscess in children with CHD in Indonesia.

Environmental changes surrounding congenital heart disease.

As the outcomes of patients with congenital heart disease (CHD) improve, the number of patients is accumulating and the proportion of adult CHD patients is gradually increasing. Accordingly, the proportion of typical adult diseases such as coronary arterial disease, diabetes, dyslipidemia, and hypertension among patients with CHD is increasing, as is the medical burden due to residual cardiac problems, heart failure, and arrhythmias. Due to increasing genetic knowledge, efforts are being made to provide active medical support through risk stratification. Surgical techniques and prostheses are also developing, the instruments used for transcatheter intervention are diversifying, and the range of therapeutic options is expanding. In addition, owing to the development of electronic devices, heart rhythm and blood pressure monitoring instruments have been miniaturized and diversified. In this process, a vast amount of information is collected; therefore, the current systematic review examined their clinical usefulness. A team approach consisting of experts from various fields is essential for the treatment of patients with CHD and advanced heart failure.

Neonatal Risk Factors for Growth Retardation in Infants With Congenital Heart Disease.

While the association of congenital heart disease (CHD) and growth retardation (GR) is known, data remain limited. This study investigated the incidence of GR and its neonatal risk factors in patients with CHD using nationwide population-based claims data. The study population was extracted from Korean National Health Insurance Service claims data from January 2002 to December 2020. We included patients diagnosed with CHD under one year of age. GR was defined as an idiopathic growth hormone deficiency or short stature on the claims data. We investigated the neonatal risk factors for GR. The number of patients diagnosed with CHD within the first year of birth was 133,739. Of these, 2,921 newborns were diagnosed with GR. The cumulative incidence of GR was 4.8% at 19 years of age for individuals diagnosed with CHD at infancy. In the multivariable analysis, the significant risk factors for GR were preterm birth, small for gestational age, low birth weight, respiratory distress, bronchopulmonary dysplasia, bacterial sepsis, necrotizing enterocolitis, feeding problems and cardiac procedure. Several neonatal conditions were significant risk factors for GR in CHD patients, and appropriate monitoring and treatment programs are required in CHD neonates with these factors. Considering this study is limited to claims data, further studies are warranted, including genetic and environmental factors affecting GR in CHD patients.

Quality of Life in Congenital Heart Disease Patients According to Their Anatomical and Physiological Classification

Background: Living well is as important as living longer. The objective of this study is to assess quality of life (QoL) in congenital heart disease (CHD) according to current AHA/ACC anatomical and physiological classifi- ... | Find, read and cite all the research you need on Tech Science Press

Triclosan in paired-maternal and cord blood, and their relationships with congenital heart disease of baby.

Prenatal triclosan (TCS) exposure has been reported to be associated with various birth outcomes and thyroid function, while the study of TCS exposure for congenital heart disease (CHD) patients is limited. In the present study, paired mother-fetus blood samples from CHD and healthy participants were collected to measure TCS exposure levels, and then check their relationship. Coupled with the concentrations of thyroid function biomarkers [free thyroxine (FT4), free triiodothyronine (FT3), thyroid-stimulating hormone (TSH), and thyroid antibodies (TgAb)] in maternal blood, we aimed to investigate whether the hormone-disrupting properties of TCS will affect its association with CHD. Our results indicated that the maternal TCS concentrations in the CHD group (median 0.31 ng/mL) were significantly lower than those in the control group (0.48 ng/mL, Mann Whitney U test, p = 0.01). Higher interquartile of TCS levels in maternal blood was associated with decrease odds of CHD (adjusted OR = 0.61, 95%CI: 0.41-0.91, p = 0.02). Maternal blood TCS higher than the cut-off value (25th quantile, 0.17 ng/mL) was significantly negatively associated with CHD risk (adjusted OR = 0.24, 95%CI: 0.09-0.62, p < 0.01). Besides, none of the thyroid biomarkers were significantly associated with maternal TCS exposure. However, maternal FT4 concentrations were positively correlated with TCS transplacental transfer rate and cord blood TCS levels (general linear regression, both p < 0.01). The results of molecular docking and dynamics simulation suggested that these correlations might be related to the transthyretin, a thyroid hormone-binding protein involved in the placental thyroid hormone transport system. Overall, our findings indicated that at normal exposure levels, the increase of maternal blood TCS concentration may have an inverse association with CHD, which merits further investigation.

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

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

Catheter-based Interventions to Reduce or Modify Surgical Risk in High-Risk Adult Congenital Heart Disease Patients.

The field of adult congenital heart disease has changed greatly over the past sixty years. As patients are now surviving longer into adulthood due to various improvements in surgical technique and medical technology, the demographic of patients with congenital heart disease (CHD) has changed, such that there are now more adults with CHD than there are children with CHD. This older and more medically complex population needs more interventions to treat residual defects or sequelae of their initial surgeries, and many of these patients are now deemed high risk for surgery. When the surgical risk becomes too great, either due to patient complexity, surgical complexity, or both, then transcatheter procedures may have a role in either mitigating or avoiding the risk altogether.

Impact of 2016 UNOS pediatric heart allocation policy changes on VAD utilization, waitlist, and post-transplant survival outcomes in children with CHD versus Non-CHD.

We analyzed the impact of the revised pediatric heart allocation policy on types of ventricular assist device (VAD) utilization, and waitlist (WL) and post-heart transplant (HT) survival outcomes in congenital heart disease (CHD) versus non-CHD patients before (Era-1) and after (Era-2) pediatric heart allocation policy implementation. We retrospectively reviewed the UNOS database from December 16, 2011, through March 31, 2021, for patients<18 years old and listed for primary HT. We compared the differences observed between Era-1 and Era-2. 5551 patients were listed for HT, of whom 2447(44%) were in Era-1 and 3104(56%) were in Era-2. CHD patients were listed as status 1A unchanged, but the number of patients listed as status 1B decreased in Era-2, whereas the number of non-CHD patients listed as status 1A decreased, but status 1B increased. In Era-2 compared to Era-1, both temporary (1% to 4%, p<.001) and durable VAD (13.6% to 17.8%, p<.001) utilization increased, and the transplantation rate per 100-patient years increased in both groups. The median WL period for CHD patients increased marginally from 70 to 71 days (p=.06), whereas for non-CHD patients it decreased from 61 to 54 days (p<.001). Adjusted 90-day WL survival increased from 84% to 88%, p=.016 in CHD, but there was no significant change in non-CHD patients (p=.57). There was no significant difference in 1-year post-HT survival in CHD and non-CHD patients between Era-1 and Era-2. In summary, after the revised heart allocation policy implementation, temporary and durable VAD support increased, HT rate increased, waitlist duration marginally increased in the CHD cohort and decreased in the non-CHD cohort, and 90-day WL survival probability improved in children with CHD without significant change in 1-year post-HT outcomes. Future studies are needed to identify changes to the policy that may further improve the listing criteria to improve WL duration and post-HT survival.

Remote Care Adoption in Underserved Congenital Heart Disease Patients During the COVID-19 Era.

The COVID-19 pandemic restricted in-person appointments and prompted an increase in remote healthcare delivery. Our goal was to assess access to remote care for complex pediatric cardiology patients. We performed a retrospective chart review of Texas Children's Hospital (TCH) pediatric cardiology outpatient appointments from March 2020 to December 2020 for established congenital heart disease (CHD) patients 1 to 17 yo. Primary outcome variables were remote care use of telemedicine and patient portal activation. Primary predictor variables were age, sex, insurance, race/ethnicity, language, and location. Descriptive statistics were used to analyze patient demographics. Multivariate logistic regression determined associations with remote care use (p < 0.05). We identified 5,410 established patients with clinic appointments during the identified timeframe. Adopters of telemedicine included 13% of patients (n = 691). Of the prior non patient portal users, 4.5% activated their accounts. On multivariate analysis, older age (10-17 yo) was associated with increased telemedicine (OR 2.04, 95%CI 1.71, 2.43) and patient portal use (OR 1.70, 95%CI 1.33, 2.17). Public insurance (OR 1.66, 95%CI 1.25, 2.20) and Spanish speaking were associated with increased patient portal adoption. Race/ethnicity was not significantly associated with telemedicine use or patient portal adoption. Telehealth adoption among older children may be indicative of their ability to aid in the use of these technologies. Higher participation in patient portal activation among publicly insured and Spanish speaking patients is encouraging and demonstrates ability to navigate some degree of remote patient care. Adoption of remote patient care may assist in reducing access to care disparities.

Effect of clinical decision support systems on emergency medicine physicians' decision-making: A pilot scenario-based simulation study.

Children with congenital heart disease (CHD) are predisposed to rapid deterioration in the face of common childhood illnesses. When they present to their local emergency departments (ED) with acute illness, rapid and accurate diagnosis and treatment is crucial to recovery and survival. Previous studies have shown that ED physicians are uncomfortable caring for patients with CHD and there is a lack of actionable guidance to aid in their decision making. To support ED physicians' key decision components (sensemaking, anticipation, and managing complexity) when managing CHD patients, a Clinical Decision Support System (CDSS) was previously designed. This pilot study evaluates the effect of this CDSS on ED physicians' decision making compared to usual care without clinical decision support. In a pilot scenario-based simulation study with repeated measures, ED physicians managed mock CHD patients with and without the CDSS. We compared ED physicians' CHD-specific and general decision-making processes (e.g., recognizing sepsis, starting antibiotics, and managing symptoms) with and without the use of CDSS. The frequency of participants' utterances related to each key decision components of sensemaking, anticipation, and managing complexity were coded and statistically analyzed for significance. Across all decision-making components, the CDSS significantly increased ED physicians' frequency of "CHD specific utterances" (Mean = 5.43, 95%CI: 3.7-7.2) compared to the without CDSS condition (Mean = 2.05, 95%CI: 0.3-3.8) whereas there was no significant difference in frequencies of "general utterances" when using CDSS (Mean = 4.62, 95%CI: 3.1-6.1) compared to without CDSS (Mean = 5.14 95%CI: 4.4-5.9). A CDSS that integrates key decision-making components (sensemaking, anticipation, and managing complexity) can trigger and enrich communication between clinicians and enhance the clinical management of CHD patients. For patients with complex and subspecialized diseases such as CHD, a well-designed CDSS can become part of a multifaceted solution that includes knowledge translation, broader communication around interpretation of information, and access to additional expertise to support CHD specific decision-making.