Spectrum of Heart Diseases in Children in a National Cardiac Referral Center Tanzania, Eastern Africa: A Six-Year Overview.

Background: While communicable diseases have long been the primary focus of healthcare in Africa, the rising impact of paediatric and congenital heart disease (CHD) cannot be overlooked. This research aimed to estimate the frequency and pattern of heart diseases in children who underwent their first echocardiography at a national cardiac referral hospital in Tanzania. Methods: A retrospective observational study was conducted on children aged 0 to 18 years referred for first-time cardiological evaluation from January 2017 to December 2022. Retrieval of social and echocardiogram data and descriptive analysis were performed. Results: There were 6,058 children with complete reports. Of these, 52.8% (3,198) had heart disease, of whom 2,559 (80%) had CHD, while (340/639; 53.2%) with acquired heart disease (AHD) had rheumatic heart disease (RHD). Children with CHD had a median age 1.0 years (IQR: 0.3-3.5) and were predominantly 51.2% male. Children with RHD had a median age 9.7 years (IQR: 3.2-13.8) with equal gender distribution. Shunt lesions were common in 1,487 (58.1%), mainly VSD 19.3%, PDA 19.1%, ASD 15.1%, and atrioventricular septal defect (AVSD) 4.6%. Pulmonary valve stenosis was in 97 (3.8%). Around 35% (718) had cyanotic CHD, with TOF being most common (13.3%), followed by double outlet right ventricle (DORV) (3.6%). Compared to global average truncus arteriosus was higher in 69 (2.3%) children. In contrast, TGA and hypoplastic left heart syndrome (HLHS) were lower than the estimated global average seen in 2.3% and 0.5% of the cases, respectively. Atresia of the right-side valves was more common (174 vs. 24), and approximately 40% of the patients referred for first-time echocardiographic evaluation required hospitalization. Conclusion: Congenital heart disease is the primary cause of heart disease in children presenting at a national referral hospital, surpassing RHD. With its distinct distribution pattern, acyanotic lesions are more frequent than cyanotic heart diseases. The observed late referral tendencies suggest improving the referral system, enhancing CHD awareness among healthcare professionals, and instituting nationwide screening programs.

Spatial Distribution and Birth Prevalence of Congenital Heart Disease in Iran: A Systematic Review and Hierarchical Bayesian Meta-analysis.

BACKGROUND: This study aimed to comprehensively analyze the overall congenital heart disease (CHD) prevalence in live births and children in Iran, along with evaluating the spatial distribution of CHD birth prevalence across various geographical regions within the country. METHODS: A Bayesian hierarchical meta-analysis (PROSPERO 2022: CRD42022331281) was performed to determine the pooled prevalence. A systematic search was conducted using Web of Science, ScienceDirect, PubMed, Iranian Research Institute for Information Science and Technology (IranDoc), Scientific Information Database (SID), and Magiran until October 4, 2023. Cross-sectional and cohort studies in both English and Persian languages, focusing on the age range of 0-10 years, were considered for the study population. The study quality was evaluated using the Agency for Healthcare Research and Quality (AHRQ) Risk of Bias tool. Heterogeneity was assessed by I2 and τ2 statistics, and publication bias by Egger's and Begg's tests. RESULTS: The meta-analysis included 62 studies, revealing an overall CHD prevalence of 2.5 per 1000 births. Over time, CHD birth prevalence in Iran has consistently increased. Spatial distribution analysis, including spatial autocorrelation and local spatial autocorrelation, indicated no spatial clustering (P=.46) or aggregation (P=.65) among Iran's provinces. Geographic disparities were significant (P=.000), with the northern and eastern regions showing the highest and lowest CHD prevalence, respectively. CONCLUSION: The overall CHD prevalence in Iran is lower than global rates, but it continues to rise. Furthermore, there are variations in birth prevalence among different regions of Iran. Environmental, genetic, socioeconomic, and diagnostic accessibility differences are possibly involved in regional variation. The limitations like heterogeneity among studies, the potential inaccuracy of reports due to limited use of accurate diagnostic methods in some studies, and the absence of population-based models to investigate prevalence, underscore the urgent need for standardized diagnostic approaches, and the utilization of population-wide birth defect registries to accurately assess CHD prevalence in Iran.

Measuring misclassification and sample bias in passive surveillance systems: Improving prevalence estimates of critical congenital heart defects in state-based passive surveillance systems.

OBJECTIVES: We assessed reporting misclassification for 12 critical congenital heart defects (CCHDs) identified through administrative diagnosis codes within a passive surveillance system. We measured the effect of misclassification on prevalence estimation. Lastly, we investigated a sample-based review strategy to estimate surveillance misclassification resulting from administrative diagnosis codes for case detection. METHODS: We received 419 reports of CCHDs between 2007 and 2018; 414 were clinically reviewed. We calculated confirmation probabilities to assess misclassification and adjust prevalence estimates. Random samples of reported cases were taken at proportions between 20% and 90% for each condition to assess sample bias. Sampling was repeated 1000 times to measure sample-estimate variability. RESULTS: Misclassification ranged from a low of 19% (n = 4/21) to a high of 84% (n = 21/25). Unconfirmed prevalence rates ranged between one and six cases per 10,000 live births, with some conditions significantly higher than national estimates. However, confirmed rates were either lower or comparable to national estimates. CONCLUSION: Passive birth defect surveillance programs that rely on administrative diagnosis codes for case identification of CCHDs are subject to misclassification that bias prevalence estimates. We showed that a sample-based review could improve the prevalence estimates of 12 cardiovascular conditions relative to their unconfirmed prevalence rates.

Prenatal exposure to air pollutants and the risk of congenital heart disease: a Korean national health insurance database-based study.

Air pollution and heavy metal exposure are emerging public health concerns. Prenatal exposure to air pollutants and heavy metals has been implicated in the development of congenital heart disease (CHD). However, the relationship between exposure to airborne heavy metals and CHD has not yet been investigated. Therefore, in this large population-based study, we investigated the association between air pollutants, including airborne heavy metals, and the risk of CHD using national health insurance claims data from South Korea. Data regarding 1,129,442 newborns and their mothers were matched with air pollutant levels during the first 8 weeks of gestation. In the five-air pollutant model, we found significant positive correlations between prenatal exposure to sulfur dioxide (SO2; odds ratio [OR] 6.843, 95% confidence interval [CI] 5.746-8.149) and cadmium (Cd; OR 1.513, 95% CI 1.187-1.930) and the risk of ventricular septal defects in newborns. This study highlights the association between prenatal exposure to air pollutants, including airborne heavy metals, and an elevated CHD risk. Further research is essential to validate and expand these findings, with the ultimate goal of enhancing public health outcomes.

Intervention needs assessment in children with heart diseases presenting to Tibebe-Ghion Specialized Teaching Hospital, Bahir Dar, North West Ethiopia - Tip of the iceberg of the intervention gap in Low-and-middle-income Countries (LMICs).

BACKGROUND: Pediatric heart disease is becoming a major contributor to childhood mortality. Almost half of congenital heart defects require intervention, either surgical or trans-catheter. Rheumatic heart disease also remains a global health problem in Low-and-middle-income-countries (LMICs). Intervention timing depends on the natural course and hemodynamic significance of the lesion with an emphasis on earlier intervention to prevent the damage of volume or pressure load and hypoxia. OBJECTIVE: describe the current unmet intervention need of children with heart diseases presenting to Bahir Dar University Tibebe-Ghion Specialized Teaching Hospital. METHODS: This is a descriptive cross-sectional study involving three-hundred-seventy-six children (376) who had echocardiography-confirmed cardiac diseases over fifteen months from August 01, 2022, to October 30, 2023. Variables were analysed using IBM SPSS version 27 software. RESULTS: Of the 376 children with heart diseases, 54% were boys. The mean (SD) age was 53 (58) months, the median (IQR) being 26 (5-96). Congenital heart defects (CHDs) account for 68% of pediatric heart diseases. 77% of CHDs were acyanotic. Isolated Ventricular septal defect (21%) is the most common acyanotic CHD followed by patent ductus arteriosus (13.2%). Tetralogy of Fallot is the most common cyanotic CHD (41%). Rheumatic heart disease is the most common acquired heart disease (76.9%). 89.4% (336) of children were eligible for intervention need assessment. 35.4% (119) of intervention-eligible children had delayed presentation. 79.8% (268) of intervention-eligible children need intervention at the current presentation. 61%(163) of children who need intervention had delayed intervention timing. 242/268 (90.3%) of children with intervention needs were candidates for surgery. Only 0.7% of children had intervention. CONCLUSION: Our study illustrates the unmet intervention need for pediatric heart diseases our health facilities faced to achieve the 2030 Sustained-development-goal (SDG) target.

Risk-prediction nomogram for congenital heart disease in offspring of Chinese pregnant women.

BACKGROUND: The identification and assessment of environmental risks are crucial for the primary prevention of congenital heart disease (CHD). We were aimed to establish a nomogram model for CHD in the offspring of pregnant women and validate it using a large CHD database in Northwest China. METHODS: A survey was conducted among 29,204 women with infants born between 2010 and 2013 in Shaanxi province, Northwest China. Participants were randomly assigned to the training set and to the validation set at a ratio of 7:3. The importance of predictive variables was assessed using random forest. A multivariate logistic regression model was used to construct the nomogram for the prediction of CHD. RESULTS: Multivariate analyses revealed that the gravidity, preterm birth history, family history of birth defects, infection, taking medicine, tobacco exposure, pesticide exposure and singleton/twin pregnancy were significant predictive risk factors for CHD in the offspring of pregnant women. The area under the receiver operating characteristic curve for the prediction model was 0.716 (95% CI: 0.671, 0.760) in the training set and 0.714 (95% CI: 0.630, 0.798) in the validation set, indicating moderate discrimination. The prediction model exhibited good calibration (Hosmer-Lemeshow χ2 = 1.529, P = 0.910). CONCLUSIONS: We developed and validated a predictive nomogram for CHD in offspring of Chinese pregnant women, facilitating the early prenatal assessment of the risk of CHD and aiding in health education.

Factors associated with the indication of neonatal echocardiography in the investigation of congenital heart diseases.

OBJECTIVE: To analyze the factors associated with the indication of echocardiography for the investigation of congenital heart disease among newborns. METHOD: Retrospective sectional study through the collection of 848 medical records of patients admitted to maternity hospitals in Rio de Janeiro-Brazil, respecting the time frame from September to December 2022. RESULTS: The average age of mothers was 26.5±6.3 years; 52.7% were classified as brown. The average age of the newborns was 3.5±5.6 days. Maternal variables: gestational age (OR=6.93, CI:3.76-12.80), number of gestational risk factors (1.90: 1.47-2.45) and number of medications (1. 97: 1.40-2.77); and neonatal variables: age (1.07: 1.03-1.02), prematurity (10.55: 5.29-21.03) and number of risk factors (2.62: 2.03-3 .38) were significantly associated with the indication for echocardiography (p<0.001). CONCLUSION: It is concluded that the different maternal and neonatal variables, gestational age, number of gestational risk factors, number of medications, age, prematurity and number of risk factors, respectively, showed a significant association for the indication of echocardiography. Therefore, the identification of these factors will enable the investigation of congenital heart disease at an opportune time among newborns.

Preeclampsia and neonatal outcomes in pregnancies with maternal congenital heart disease: A nationwide cohort study from Norway.

INTRODUCTION: The prevalence of congenital heart disease (CHD) among women of reproductive age is rising. We aimed to investigate the risk of preeclampsia and adverse neonatal outcomes in pregnancies of mothers with CHD compared to pregnancies of mothers without heart disease. MATERIAL AND METHODS: In a nationwide cohort of pregnancies in Norway 1994-2014, we retrieved information on maternal heart disease, the course of pregnancy, and neonatal outcomes from national registries. Comparing pregnancies with maternal CHD to pregnancies without maternal heart disease, we used Cox regression to estimate the adjusted hazard ratio (aHR) for preeclampsia and log-binomial regression to estimate the adjusted risk ratio (aRR) for adverse neonatal outcomes. The estimates were adjusted for maternal age and year of childbirth and presented with 95% confidence intervals (CIs). RESULTS: Among 1 218 452 pregnancies, 2425 had mild maternal CHD, and 603 had moderate/severe CHD. Compared to pregnancies without maternal heart disease, the risk of preeclampsia was increased in pregnancies with mild and moderate/severe maternal CHD (aHR1.37, 95% CI 1.14-1.65 and aHR 1.62, 95% CI 1.13-2.32). The risk of preterm birth was increased in pregnancies with mild maternal CHD (aRR 1.33, 95% CI 1.15-1.54) and further increased with moderate/severe CHD (aRR 2.49, 95% CI 2.03-3.07). Maternal CHD was associated with elevated risks of both spontaneous and iatrogenic preterm birth. The risk of infants small-for-gestational-age was slightly increased with mild maternal CHD (aRR 1.12, 95% CI 1.00-1.26) and increased with moderate/severe CHD (aRR 1.63, 95% CI 1.36-1.95). The prevalence of stillbirth was 3.9 per 1000 pregnancies without maternal heart disease, 5.6 per 1000 with mild maternal CHD, and 6.8 per 1000 with moderate/severe maternal CHD. Still, there were too few cases to report a significant difference. There were no maternal deaths in women with CHD. CONCLUSIONS: Moderate/severe maternal CHD in pregnancy was associated with increased risks of preeclampsia, preterm birth, and infants small-for-gestational-age. Mild maternal CHD was associated with less increased risks. For women with moderate/severe CHD, their risk of preeclampsia and adverse neonatal outcomes should be evaluated together with their cardiac risk in pregnancy, and follow-up in pregnancy should be ascertained.

Maternal Oxidative Balance Score during Pregnancy and Congenital Heart Defects.

The relationship between maternal oxidative balance score (OBS) in pregnancy, representing overall oxidative balance status by integrating dietary and lifestyle factors, and congenital heart defects (CHD) remains unclear; therefore, this study attempted to explore their associations among the Chinese population. We conducted a case-control study including 474 cases and 948 controls in Northwest China. Pregnant women were interviewed to report diets and lifestyles in pregnancy by structured questionnaires. Logistic regression models were used to estimate the adjusted ORs (95%CIs). Maternal OBS ranged from 6 to 34 among cases, and 5 to 37 among controls. Comparing the highest with the lowest tertile group, the adjusted OR for CHD was 0.31 (0.19-0.50). The CHD risk was reduced by 7% (OR = 0.93, 95%CI = 0.90-0.95) in association with per 1 higher score of OBS during pregnancy. The inverse relationship between maternal OBS and CHD risk appeared to be more pronounced among participants in urban areas (OR = 0.89, 95%CI = 0.86-0.93). Maternal OBS during pregnancy showed good predictive values for fetal CHD, with the areas under the receiver operating characteristic curve 0.78 (0.76-0.81). These findings highlighted the importance of reducing oxidative stress through antioxidant-rich diets and healthy lifestyles among pregnant women to prevent fetal CHD.

Distribution and Characteristics of Congenital Cardiac Surgery Centers within Bangladesh.

Congenital heart disease is a leading cause of non-communicable childhood death. This is especially true in nations with limited resources where shortages of skilled workforce, healthcare facilities, and essential equipment limit the ability to provide care. This retrospective study was designed to determine the volume and distribution of surgical care being provided to patients with congenital heart disease in Bangladesh, as well as to characterize the facilities providing such care. Pre-existing survey data of hospitals performing congenital heart surgery in the year 2022 in Bangladesh was obtained. Additional information was gathered on these facilities, including hospital location and type. The distribution of care by geographic location, type of facility, and volume of cases was reported. Overall, a total of 2333 surgeries were performed in 2022 at 28 facilities. The majority of hospitals were performing <50 cases per year, while a small number (5) provided greater than 50.0% of all surgeries. In addition, while the majority of hospitals were private in nature, the majority of surgeries occurred at not-for-profit hospitals. There was a large geographic skew of surgeries and hospitals being located within the city of Dhaka (79.0% of centers and 94.0% of surgeries). The data suggests that, though there has been great progress in increasing the number of surgeries performed in Bangladesh, the vast majority of patients still do not have access to care. In addition, nearly all care is being provided in Dhaka, which presents challenges for patients who come from across the nation seeking care. Finally, there is a great need for further research to fully understand the challenges faced and find workable solutions.

Metal stannum exposure increases the risk of congenital heart defects occurence in offspring: A case-control study in Lanzhou.

BACKGROUND: The purpose of this study was to analyse the association between stannum exposure during pregnancy and congenital heart diseases in offspring. METHODS: Based on a prospective birth cohort study conducted in Gansu Maternal and Child Health Hospital from 2010 to 2012, 14,359 pregnant women were followed up using a nested case-control study method. 97 pregnant women whose offspring were diagnosed with CHDs were used as the case group, and 194 pregnant women whose offspring did not suffer from congenital heart diseases were used as the control group in a ratio of 1:2 according to their age and place of birth. Inductively coupled plasma mass spectrometry was used to determine elemental stannum in blood samples from pregnant women hospitalized for delivery and in fetal cord blood samples. Multifactorial logistic regression analysis was used to assess the association between stannum and offspring CHDs. RESULTS: There was a moderate positive correlation between the concentration of stannum in pregnant women's blood and that in umbilical cord blood. A higher concentrations of maternal blood stannum level was associated with a greater risk of CHDs (aOR 3.409, 95%CI 1.785-6.826), isolated CHDs (aOR 4.044, 95%CI 1.803-9.070), multiple CHDs (aOR 2.625, 95%CI 1.137-6.061), patent ductus arteriosus (aOR 2.882, 95%CI 1.443-5.756), atrial septal defects (aOR 3.067, 95%CI 1.406-6.690), ventricular septal defects (aOR 7.414, 95%CI 1.414-38.874). There was a correlation between the maternal and cord blood sample suggesting stannum crosses the placenta.

Iron deficiency in patients with a Fontan circulation and its impact on exercise capacity.

BACKGROUND: Iron deficiency (ID) has been reported in patients with congenital heart disease. There is, however, a scarcity of data on its prevalence in patients with a Fontan circulation. The aim of this study is to investigate the prevalence of ID in Fontan patients and to investigate the association between ID and exercise capacity in this population. METHODS AND RESULTS: Blood count and haematological parameters were determined in plasma of 61 Fontan patients (51% female, mean age 29±9 years). ID was defined as transferrin saturation (TSAT) ≤19.8%. The prevalence of ID was 36% (22/61 patients). Especially among women, the diagnosis of ID was highly prevalent (52%) despite normal haemoglobin levels (153.7±18.4 g/L). Mean ferritin levels were 98±80 µg/L and mean TSAT levels were 22%±12%. Cardiopulmonary exercise testing was performed in 46 patients (75%). Patients with ID had a lower peak oxygen uptake (V̇O2peak) (1397±477 vs 1692±530 mL/min; p=0.039), although this relationship was confounded by sex. The presence of ID increased the likelihood of not achieving a respiratory exchange ratio (RER) ≥1.1 by 5-fold (p=0.035). CONCLUSION: ID is highly prevalent among patients with a Fontan circulation. V̇O2peak is lower in patients with ID. Fontan patients with ID are less likely to achieve an RER≥1.1 during cardiopulmonary exercise testing.

Perinatal deaths attributable to congenital heart defects in Hunan Province, China, 2016-2020.

OBJECTIVE: To explore the association between demographic characteristics and perinatal deaths attributable to congenital heart defects (CHDs). METHODS: Data were obtained from the Birth Defects Surveillance System of Hunan Province, China, 2016-2020. The surveillance population included fetuses and infants from 28 weeks of gestation to 7 days after birth whose mothers delivered in the surveillance hospitals. Surveillance data included demographic characteristics such as sex, residence, maternal age, and other key information, and were used to calculate the prevalence of CHDs and perinatal mortality rates (PMR) with 95% confidence intervals (CI). Multivariable logistic regression analysis (method: Forward, Wald, α = 0.05) and adjusted odds ratios (ORs) were used to identify factors associated with perinatal deaths attributable to CHDs. RESULTS: This study included 847755 fetuses, and 4161 CHDs were identified, with a prevalence of 0.49% (95%CI: 0.48-0.51). A total of 976 perinatal deaths attributable to CHDs were identified, including 16 (1.64%) early neonatal deaths and 960 (98.36%) stillbirths, with a PMR of 23.46% (95%CI: 21.98-24.93). In stepwise logistic regression analysis, perinatal deaths attributable to CHDs were more common in rural areas than urban areas (OR = 2.21, 95%CI: 1.76-2.78), more common in maternal age <20 years (OR = 2.40, 95%CI: 1.05-5.47), 20-24 years (OR = 2.13, 95%CI: 1.46-3.11) than maternal age of 25-29 years, more common in 2 (OR = 1.60, 95%CI: 1.18-2.18) or 3 (OR = 1.43, 95%CI: 1.01-2.02) or 4 (OR = 1.84, 95%CI: 1.21-2.78) or > = 5 (OR = 2.02, 95%CI: 1.28-3.18) previous pregnancies than the first pregnancy, and more common in CHDs diagnosed in > = 37 gestional weeks (OR = 77.37, 95%CI: 41.37-144.67) or 33-36 gestional weeks (OR = 305.63, 95%CI: 172.61-541.15) or < = 32 gestional weeks (OR = 395.69, 95%CI: 233.23-671.33) than diagnosed in postnatal period (within 7 days), and less common in multiple births than singletons (OR = 0.48, 95%CI: 0.28-0.80). CONCLUSIONS: Perinatal deaths were common in CHDs in Hunan in 2016-2020. Several demographic characteristics were associated with perinatal deaths attributable to CHDs, which may be summarized mainly as economic and medical conditions, severity of CHDs, and parental attitudes toward CHDs.

Environmental Signals.

Environmental factors have long been known to play a role in the pathogenesis of congenital heart disease (CHD), but this has not been a major focus of research in the modern era. Studies of human exposures and animal models demonstrate that demographics (age, race, socioeconomic status), diseases (e.g., diabetes, hypertension, obesity, stress, infection, high altitude), recreational and therapeutic drug use, and chemical exposures are associated with an increased risk for CHD. Unfortunately, although studies suggest that exposures to these factors may cause CHD, in most cases, the data are not strong, are inconclusive, or are contradictory. Although most studies concentrate on the effects of maternal exposure, paternal exposure to some agents can also modify this risk. From a mechanistic standpoint, recent delineation of signaling and genetic controls of cardiac development has revealed molecular pathways that may explain the effects of environmental signals on cardiac morphogenesis and may provide further tools to study the effects of environmental stimuli on cardiac development. For example, environmental factors likely regulate cellular signaling pathways, transcriptional and epigenetic regulation, proliferation, and physiologic processes that can control the development of the heart and other organs. However, understanding of the epidemiology and risk of these exposures and the mechanistic basis for any effects on cardiac development remains incomplete. Further studies defining the relationship between environmental exposures and human CHD and the mechanisms involved should reveal strategies to prevent, diagnose, and treat CHD induced by environmental signals.

Survival and Risk Factors for Mortality in Infants With Congenital Heart Disease in South Korea.

BACKGROUND/AIM: The survival of patients with congenital heart disease (CHD) has dramatically improved over recent decades. However, a disparity exists depending on the country and medical system. This study aimed to analyze the survival of infants with CHD until the age of 18 years using large-scale population data in South Korea and investigate the effect of neonatal conditions at birth. PATIENTS AND METHODS: We retrospectively extracted the Korean National Health Insurance Service claims data from January 2002 to December 2020. We included patients diagnosed with CHD who were less than one year of age. The follow-up duration was until their death or until they were censored before the age of 18 years. The CHD lesions were classified hierarchically (conotruncal, severe non-conotruncal, coarctation of the aorta, ventricular septal defect, atrial septal defect, and others). Several neonatal conditions were adopted as risk factors. RESULTS: Overall, 127,958 infants had been diagnosed with CHD and 2,275 died before the age of 18 years. The survival rate of infants with CHD during childhood was 97.9%. The highest childhood mortality rate was associated with non-conotruncal defects (19.7%), followed by conotruncal defects (10.2%). The significant risk factors for childhood mortality were complex CHD, pulmonary hypertension, birth asphyxia, small for gestational age, respiratory distress, pulmonary hemorrhage, bronchopulmonary dysplasia, and convulsions. CONCLUSION: The survival of infants with CHD has been favorable in South Korea. Several neonatal conditions are risk factors for childhood mortality. Individualized risk assessment and optimal treatment strategies may help improve their survival rate.

Addressing Disparities in Pediatric Congenital Heart Disease: A Call for Equitable Health Care.

While significant progress has been made in reducing disparities within the US health care system, notable gaps remain. This article explores existing disparities within pediatric congenital heart disease care. Congenital heart disease, the most common birth defect and a leading cause of infant death, has garnered substantial attention, revealing certain disparities within the US health care system. Factors such as race, ethnicity, insurance coverage, socioeconomic status, and geographic location are all commonalities that significantly affect health disparities in pediatric congenital heart disease. This comprehensive review sheds light on disparities from diverse perspectives in pediatric care, demonstrates the inequities and inequalities leading to these disparities, presents effective solutions, and issues a call to action for providers, institutions, and the health care system. Recognizing and addressing these disparities is imperative for ensuring equitable care and enhancing the long-term well-being of children affected by congenital heart disease. Implementing robust, evidence-based frameworks that promote responsible and safe interventions is fundamental to enduring change.

Impact of maternal body mass index (BMI) and the challenges of fetal echocardiography.

OBJECTIVES: Fetal echocardiography is the gold standard modality to detect suspected congenital heart disease (CHD). Accurate diagnosis and subsequent prognosis is even more challenging in the presence of a raised maternal body mass index (BMI). This retrospective study aimed to gain insight into the prevalence of obesity within the cohort of patients referred for fetal echocardiography. STUDY DESIGN/METHODS: Retrospective analysis of all pregnant patients referred to the Scottish National Fetal Cardiology Service between 2015 and 2021 due to a suspected fetal cardiac abnormality and examining the associated trends in maternal BMI and the Scottish Index of Multiple Deprivation (SIMD). RESULTS: BMI data were available for 962 (96.3%) of the 998 patients referred during the study period. Median BMI during the study period was 31. BMI range in the seven-year period was 16-63. There was no association between BMI group and year (P = 0.889). A median of 58% of patients referred were classified as overweight (BMI > 25 kg/m2), and only 37% were reported to have a BMI within normal limits. Referral BMI was relatively consistent in the seven years with no dramatic increase in the obese categories. Mean BMI in SIMD 5 (lowest level of deprivation), was significantly lower (P = 0.001), than in SIMD 1 (highest deprivation). CONCLUSIONS: People of child bearing age should be aware the potential limitations that a raised BMI may have upon diagnostic/screening accuracy impacting subsequent ability to provide accurate fetal cardiac diagnoses and prognostic fetal cardiac imaging.

Educational outcomes among children with congenital heart disease compared to peers: a Scotland-wide record-linkage study of 715,850 schoolchildren.

BACKGROUND: Nine in every thousand children born in the United Kingdom have congenital heart disease, and 250,000 adults are living with the condition. This study aims to investigate the associations between congenital heart disease and educational outcomes among school-aged children in Scotland. METHODS: Routine health and education databases were linked to produce a cohort of all singleton children born in Scotland and attending a local authority run primary, secondary, or special school in Scotland at some point between 2009 and 2013. Children with congenital heart disease within this cohort were compared with children unaffected by congenital conditions. Outcomes investigated were special educational need (SEN), absenteeism, exclusion, academic attainment, and unemployment. All analyses were adjusted for sociodemographic and maternity confounders. Absenteeism was investigated as a mediating factor in the associations with attainment and unemployment. RESULTS: Of the 715,850 children, 6,295 (0.9%) had congenital heart disease and 4,412 (6.1%) had isolated congenital heart disease. Congenital heart disease and isolated congenital heart disease were both significantly associated with subsequent special educational need (OR 3.45, 95% CI 3.26-3.65, p < 0.001 and OR 1.98, 95% CI 1.84-2.13, p < 0.001 respectively), absenteeism (IRR 1.13, 95% CI 1.10-1.16, p < 0.001 and IRR 1.10, 95% CI 1.06-1.13, p < 0.001 respectively), and low academic attainment (OR 1.69, 95% CI 1.39-2.07, p < 0.001 and OR 1.35, 95% CI 1.07-1.69, p = 0.011 respectively). Neither congenital heart disease nor isolated congenital heart disease were associated with school exclusion. Only congenital heart disease (OR 1.21, 95% CI 1.03-1.42, p = 0.022) but not isolated congenital heart disease was associated with unemployment. When days absent were included in the analyses investigating attainment and unemployment, the conclusions were not altered. CONCLUSION: Children with congenital heart disease have greater special educational need, lower school attendance, attain lower examination grades and have greater unemployment compared to peers. In addition to healthcare support, affected children need educational support to avoid additional impact on their long-term wellbeing.

Association of congenital heart defects (CHD) with factors related to maternal health and pregnancy in newborns in Puerto Rico.

Background: Given the pervasive issues of obesity and diabetes both in Puerto Rico and the broader United States, there is a compelling need to investigate the intricate interplay among BMI, pregestational, and gestational maternal diabetes, and their potential impact on the occurrence of congenital heart defects (CHD) during neonatal development. Methods: Using the comprehensive System of Vigilance and Surveillance of Congenital Defects in Puerto Rico, we conducted a focused analysis on neonates diagnosed with CHD between 2016 and 2020. Our assessment encompassed a range of variables, including maternal age, gestational age, BMI, pregestational diabetes, gestational diabetes, hypertension, history of abortion, and presence of preeclampsia. Results: A cohort of 673 patients was included in our study. The average maternal age was 26 years, within a range of 22 to 32 years. The mean gestational age measured 39 weeks, with a median span of 38 to 39 weeks. Of the 673 patients, 274 (41%) mothers gave birth to neonates diagnosed with CHD. Within this group, 22 cases were linked to pre-gestational diabetes, while 202 were not; 20 instances were associated with gestational diabetes, compared to 200 without; and 148 cases exhibited an overweight or obese BMI, whereas 126 displayed a normal BMI. Conclusion: We identified a statistically significant correlation between pre-gestational diabetes mellitus and the occurrence of CHD. However, our analysis did not show a statistically significant association between maternal BMI and the likelihood of CHD. These results may aid in developing effective strategies to prevent and manage CHD in neonates.

Evaluation and Management of Chronic Heart Failure in Children and Adolescents With Congenital Heart Disease: A Scientific Statement From the American Heart Association.

With continued medical and surgical advancements, most children and adolescents with congenital heart disease are expected to survive to adulthood. Chronic heart failure is increasingly being recognized as a major contributor to ongoing morbidity and mortality in this population as it ages, and treatment strategies to prevent and treat heart failure in the pediatric population are needed. In addition to primary myocardial dysfunction, anatomical and pathophysiological abnormalities specific to various congenital heart disease lesions contribute to the development of heart failure and affect potential strategies commonly used to treat adult patients with heart failure. This scientific statement highlights the significant knowledge gaps in understanding the epidemiology, pathophysiology, staging, and outcomes of chronic heart failure in children and adolescents with congenital heart disease not amenable to catheter-based or surgical interventions. Efforts to harmonize the definitions, staging, follow-up, and approach to heart failure in children with congenital heart disease are critical to enable the conduct of rigorous scientific studies to advance our understanding of the actual burden of heart failure in this population and to allow the development of evidence-based heart failure therapies that can improve outcomes for this high-risk cohort.