Incidence Of Congenital Heart Disease Research Articles

Neonatal outcomes in preterm infants with severe congenital heart disease: a national cohort analysis.

Prematurity and congenital heart disease (CHD) are the leading causes of neonatal mortality and morbidity. Limited data are available about the outcomes of premature infants with severe CHD. We queried The National Inpatient Database using ICD-10 codes for premature patients (<37 weeks) with severe CHD from 2016 to 2020. Severe CHDs were grouped into three categories: A. left-sided lesions with impaired systemic output, B. Cyanotic CHD, and C. Shunt lesions with pulmonary overcirculation. Patients with isolated atrial or ventricular septal defects and patent ductus arteriosus were excluded. We also excluded patients with chromosomal abnormalities and major congenital anomalies. Patients' demographics, clinical characteristics, and outcomes were evaluated by comparing premature infants with vs. without CHD adjusting for gestational age (GA), birth weight, and gender. A total of 27710 (1.5%) out of 1,798,245 premature infants had severe CHD. This included 27%, 58%, and 15% in groups A, B, and C respectively. The incidence of severe CHD was highest between 25 and 28 weeks of gestation and decreased significantly with increasing GA up to 36 weeks (p < 0.001). Premature infants with severe CHD had a significantly higher incidence of neonatal morbidities including necrotizing enterocolitis (NEC) [OR = 4.88 (4.51-5.27)], interventricular hemorrhage [OR = 6.22 (5.57-6.95)], periventricular leukomalacia [OR = 3.21 (2.84-3.64)] and bronchopulmonary dysplasia [OR = 8.26 (7.50-10.06) compared to preterm infants of similar GA without CHD. Shunt lesions had the highest incidence of NEC (8.5%) compared to 5.3% in cyanotic CHD and 3.7% in left-sided lesions (p < 0.001). Mortality was significantly higher in premature infants with CHD compared to control [11.6% vs. 2.5%, p < 0.001]. Shunt lesions had significantly higher mortality (11.0%) compared to those with left-sided lesions (8.3%) and cyanotic CHD (6.4%), p < 0.001. Premature infants with severe CHD are at high risk of neonatal morbidity and mortality. Morbidity remains increased across all GA groups and in all CHD categories. This significant risk of adverse outcomes is important to acknowledge when managing this patient population and when counseling their families. Future research is needed to examine the impact of specific rather than categorized congenital heart defects on neonatal outcomes.

Clinical implications of dextrocardia based on four visceroatrial situs studies

BackgroundPrevious studies on congenital heart diseases (CHD) associated with dextrocardia were based on selective patient databases and did not reflect the full spectrum of dextrocardia in the general population. Additionally, these studies had complex classification and presentation. Nor did these studies elaborate on the distribution of the associated CHD's complexity, the various segmental connections, and associated CHD among the four visceroatrial situs. MethodsWe retrospectively reviewed the medical records of 211 children with primary dextrocardia. We used a segmental approach to diagnose CHD. We then analyzed and compared the distribution of the above-mentioned issues among the four visceroatrial situs. ResultsDextrocardia occurred most commonly with situs inversus (52.6%), followed by situs solitus (28.4%), asplenia (17.1%), and polysplenia (1.9%). Although some patients had a structurally normal heart (22.7%) or they were associated with simple CHD (17.5%), most patients had complex CHD (59.7%) consisting of a single ventricle (34.6%) or conotruncal anomaly (25.1%) (double-outlet right ventricle [7.6%], corrected transposition of the great arteries [6.2%], complete transposition of the great arteries [5.7%], tetralogy of Fallot [4.7%], etc.). Situs inversus or polysplenia had a higher prevalence of a structurally normal heart or associated with simple CHD, two patent atrioventricular (AV) valves connections, and biventricular AV connections. Situs solitus or asplenia had a higher prevalence of associated complex CHD, common AV valve connection, univentricular AV connection, pulmonary outflow tract obstruction, and anomalous pulmonary venous drainage. ConclusionOur study finds that situs inversus is the most common visceroatrial situs in dextrocardia. Although some patients had a structurally normal heart or were associated with simple CHD, most patients have associated complex CHD consisting of a single ventricle or conotruncal anomaly. Dextrocardia is associated with a higher incidence of complex CHD in situs solitus and asplenia groups than in situs inversus and polysplenia groups.

Impact of Congenital Heart Disease on the Outcomes of Very Low Birth Weight Infants.

To investigate the association of congenital heart disease (CHD) with morbidity and mortality of very low birth weight (VLBW) infants. This matched case-control study included VLBW infants born at a single institution between 2001 and 2015. The primary outcome was mortality. Secondary outcomes included necrotizing enterocolitis, bronchopulmonary dysplasia (BPD), sepsis, retinopathy of prematurity, and intraventricular hemorrhage. These outcomes were assessed by comparing VLBW-CHDs with control VLBW infants matched by gestational age within a week, birth weight within 500 g, sex, and birth date within a year using conditional logistic regression. Multivariable logistic regression analyzed differences in outcomes in the VLBW-CHD group between two birth periods (2001-2008 and 2009-2015) to account for changes in practice. In a cohort of 44 CHD infants matched with 88 controls, the mortality rate was 27% in infants with CHD and 1% in controls (p < 0.0001). The VLBW-CHDs had increased BPD; (odds ratio [OR]: 7.70, 95% confidence interval [CI]: 1.96-30.29) and sepsis (OR: 10.59, 95% CI: 2.99-37.57) compared with the control VLBWs. When adjusted for preoperative ventilator use, the VLBW-CHDs still had significantly higher odds of BPD (OR: 6.97, 95% CI: 1.73-28.04). VLBW-CHDs also had significantly higher odds of both presumed and culture-positive sepsis as well as late-onset sepsis than their matched controls. There were no significant differences in outcomes between the two birth periods. VLBW-CHDs showed higher odds of BPD, sepsis, and mortality than VLBW infants without CHD. Future research should focus on the increased mortality and specific complications encountered by VLBW infants with CHD and implement targeted strategies to address these risks. · Incidence of CHD is higher in preterm infants than in term infants but the incidence of their morbidities is not well described.. · VLBW infants with CHD have higher odds of mortality, bronchopulmonary dysplasia, and sepsis.. · Future research is needed to implement targeted preventive responses..

Sex-specific difference in phenotype of Kabuki syndrome type 2 patients: a matched case-control study.

Kabuki syndrome (KS) is a monogenic disorder leading to special facial features, mental retardation, and multiple system malformations. Lysine demethylase 6A, (KDM6A, MIM*300128) is the pathogenic gene of Kabuki syndrome type 2 (KS2, MIM#300867), which accounts for only 5%-8% of KS. Previous studies suggested that female patients with KS2 may have a milder phenotype. We summarized the phenotype and genotype of KS2 patients who were diagnosed in Shanghai Children's Medical Center since July 2017 and conducted a 1:3 matched case-control study according to age and sex to investigate sex-specific differences between patients with and without KS2. There were 12 KS2 cases in this study, and 8 of them matched with 24 controls. The intelligence quotient (IQ) score of the case group was significantly lower than that of the control group (P < 0.001). In addition, both the incidence of intellectual disability (ID) (IQ < 70) and moderate-to-severe ID (IQ < 55) were significantly higher in the case group than those in the control group. No sex-specific difference was found in the incidence of ID or moderate-to-severe ID between the female cases and female controls, whereas there was a significant difference between male cases and male controls. Furthermore, the rate of moderate-to-severe ID and congenital heart disease (CHD) was significantly higher in the male group than that in the female group. Our results showed that a sex-specific difference was exhibited in the clinical phenotypes of KS2 patients. The incidence of CHD was higher in male patients, and mental retardation was significantly impaired. However, the female patients' phenotype was mild.

Maternal Gestational Diabetes Mellitus and Congenital Heart Disease in Offspring: A Meta-Analysis.

Maternal diabetes has been related to an increased risk of congenital heart disease (CHD) in offspring. However, inconsistent results were retrieved for studies evaluating the association between gestational diabetes mellitus (GDM) and CHD in offspring. We therefore performed a systematic review and meta-analysis for comprehensive investigation. Observational studies were identified by searching PubMed, Embase, and Web of Science according to the aim of the meta-analysis. A randomized-effects model was used to pool the data by incorporating the influence of potential heterogeneity. Twenty-three observational studies, involving 46953078 mother-child pairs, were available for the meta-analysis. Among them, 2131800 mothers were diagnosed as GDM and 214379 newborns had CHD. Overall, maternal GDM was associated with a higher incidence of CHD in offspring [odds ratio (OR): 1.32, 95% confidence interval (CI): 1.21 to 1.45, p<0.001; I2=62%]. Sensitivity analysis limited to studies with adjustment of maternal age and other potential confounding factors showed similar results (OR: 1.40, 95% CI: 1.30 to 1.51, p<0.001; I2=47%). Subgroup analysis suggested that the association between maternal GDM and CHD in offspring was not significantly affected by methods for diagnosis of GDM, methods for confirmation of CHD, or study quality scores (p for subgroup difference all>0.05). Subsequent analysis according to types of CHD showed that maternal GDM was associated with higher risks of atrial septal defect, ventricular septal defect, and Tetralogy of Fallot. Maternal GDM may be associated with a higher risk of CHD in offspring.

Echocardiographic Findings of Children with Down Syndrome

Objective: Down syndrome is the most common well-known chromosomal disorder. The incidence of congenital heart disease (CHD) in these patients is high and it is the most important cause of mortality. In this study, we reviewed the comorbid CHDs and their characteristics in patients with the diagnosis of Down syndrome. We compared our results with the results of studies conducted in different regions of our country in different years. Materials and Methods: Between January 2020 and December 2022, data from a total of 385 patients (175 females, 210 males) with Down syndrome were evaluated. The age of the mothers at the time of delivery and the availability of prenatal diagnosis information in accessible patients were determined. Results: Of 385 patients with Down syndrome, 38.2% (n=147) had CHD. Among patients with CHD, 57 patients had atrioventricular septal defect (AVSD) (38.7%), 35 patients had perimembranous ventricular septal defect (23.8%), 24 patients had secundum atrial septal defect (16.3%), 8 patients had patent ductus arteriosus (5.4%), and 10 patients without AVSD had CHD with multiple left-to-right shunts (6.8%). The most common cyanotic CHD was tetralogy of Fallot (n=7, 4.7%). Complete AVSD was present in 63.1% of AVSD patients. CHD was present in 36.2% of boys and 40.6% of girls. Among all female patients, 17.1% had AVSD, while this rate was 12.8% among all male patients (p&lt;0.05). The mean maternal age at delivery was 32.59±6.9 (17-48) years. It was found that children with Down syndrome whose mothers had a maternal age of 33.5 years and above had a lower rate of CHD. Of the 170 mothers for whom prenatal diagnosis information was available, 58.2% (n=99) had a prenatal diagnosis. Conclusion: Prenatal diagnosis opportunities have increased and become widespread over the years. However, no significant change was observed in the rates of CHD, which is most frequently responsible for mortality in children with Down syndrome. It is appropriate for every expectant mother with a prenatal diagnosis to be referred for fetal echocardiography or postnatal transthoracic echocardiography.

Congenital Heart Disease In Preterm Infants

Background: Congenital heart defects and preterm birth are two important causes of neonatal and infant mortality. However, the relationship between them has not yet been fully clarified. Objective: To find the association of congenital heart disease with premature delivery. Patients and Methods: The study is an observational cross sectional study, done in Al-Batool teaching hospital for maternity and children from July 2022 to January 2023. A self-prepared questionnaire was used to gather data. Gestational age was calculated mainly on postnatal gestational assessment by Dubowitz/Ballard method by a pediatrician. Echocardiography was done by Echocardiography specialist doctor to identify the presence and types of congenital heart defects. SPSS version 25 was used for statistical analysis. Results: Total number of births (term and preterm) was 1616, congenital heart diseases were detected in 60(3.71%) of them, most of them were isolated heart defect. In preterm babies, the congenital heart diseases were detected in 8% (p value was &lt;0.001), they were of different types as followings: patent ductus arteriosus (n=10,26%), atrial septal defect (n=8,21%), ventricular septal defect (n=7,18%), transposition of the great arteries (n=4,10%), tetralogy of Fallot (n=2,5%), and complex of congenital heart disease (n=7,18%), while in full term babies, the rate of congenital heart diseases was only 1.9%. Conclusion: The incidence of congenital heart disease was higher in preterm than full term delivered babies, mostly in the group of transposition of the great arteries, ventricular septal defect, complex heart defects, followed by atrial septal defect. Keywords: Preterm, delivery, congenital heart disease.

Congenital Heart Disease In Preterm Infants

Background: Congenital heart defects and preterm birth are two important causes of neonatal and infant mortality. However, the relationship between them has not yet been fully clarified.&#x0D; Objective: To find the association of congenital heart disease with premature delivery.&#x0D; Patients and Methods: The study is an observational cross sectional study, done in Al-Batool teaching hospital for maternity and children from July 2022 to January 2023. A self-prepared questionnaire was used to gather data. Gestational age was calculated mainly on postnatal gestational assessment by Dubowitz/Ballard method by a pediatrician. Echocardiography was done by Echocardiography specialist doctor to identify the presence and types of congenital heart defects. SPSS version 25 was used for statistical analysis.&#x0D; Results: Total number of births (term and preterm) was 1616, congenital heart diseases were detected in 60(3.71%) of them, most of them were isolated heart defect. In preterm babies, the congenital heart diseases were detected in 8% (p value was &lt;0.001), they were of different types as followings: patent ductus arteriosus (n=10,26%), atrial septal defect (n=8,21%), ventricular septal defect (n=7,18%), transposition of the great arteries (n=4,10%), tetralogy of Fallot (n=2,5%), and complex of congenital heart disease (n=7,18%), while in full term babies, the rate of congenital heart diseases was only 1.9%.&#x0D; Conclusion: The incidence of congenital heart disease was higher in preterm than full term delivered babies, mostly in the group of transposition of the great arteries, ventricular septal defect, complex heart defects, followed by atrial septal defect.

Incidence of congenital heart disease in fetuses diagnosed with single isolated umbilical artery. Systematic review and meta-analysis.

To estimate the proportion of heart disease in fetuses with a prenatal diagnosis of a single isolated umbilical artery. We performed a search strategy in MEDLINE (OVID), EMBASE, and the Cochrane Central Register of Controlled Trials (CENTRAL) from inception to the present. We assessed the risk of bias and performed a meta-analysis. We completed the subgroup analysis according to the region. We found 1384 studies by the search strategy. After carefully reviewing the full-text, 15 studies were included. A total of 2008 fetuses with a single isolated umbilical artery were included, and 177 had cardiac malformations. There was an overall incidence of 9% 95%CI (0.05-0.14) I2 90%. The incidence by country of origin was between 5% and 19%. The most common heart disease reported was a ventricular septal defect. Seven studies were found describing 25 cases. We described other malformations, such as tetralogy of Fallot, coarctation of the aorta, and hypoplastic left ventricle, among others. The incidence of congenital heart disease in fetuses with a single isolated umbilical artery was high. In addition, half of these correspond to significant heart disease. Based on the above, we suggest that fetuses with a single isolated umbilical artery should have a complete anatomic evaluation emphasizing cardiac evaluation.

Congenital Heart Diseases: Risk Factors and Ultrasound Diagnostic Potential at the First Screening

Introduction: Due to the high incidence of congenital heart diseases (CHD) it is important to identify risk factors for abnormal organogenesis. There is a crucial medical need to understand cardiogenesis and possible markers of abnormalities in fetuses.Objective: To determine risk factors for CHD in pregnant women and their partners and assess CHD markers on the first ultrasound screening.Materials and methods: We analyzed 221 medical records of pregnant women with prenatally diagnosed fetal CHD and divided the patients into 2 groups: group I (n = 177) with CHD compatible with postnatal development and group II (n = 44) with CHD incompatible with postanatal development. We evaluated physical findings and somatic and reproductive history in both groups. We retrospectively analyzed 2272 ultrasound scans and first screening markers: nuchal translucency (NT) thickness and ductus venosus pulsatility index (DVPI).Results: We identified possible risk factors for fetal CHD: body mass index, P = .000; age of menarche, P = .037; chronic tonsillitis, Р = .046; cervical erosion, Р = .045; vulvovaginal candidiasis, Р = .005; vaginitis, Р = .027; cervicitis, Р = .027; chronic hepatitis B and C viruses, Р = .005; respiratory tract infection with fever during the current pregnancy, Р = .012. We built 500 networks with the highest performance for a predictive model and found that the model’s predictors did not fully represent the risk factors for CHD. CHD with increased NT thickness (≥2.5 mm) was detected in 2.24% of cases. NT thickness and DVPI were 3.37 ± 0.92 mm and 1.098 ± 0.158, respectively. According to our correlation analysis, increased DVPI and NT thickness were associated with CHD (R = 0.510).Conclusions: It is not possible to identify risk factors for CHD in fetuses by medical history, reproductive history and physical status of pregnant women, and unhealthy habits of their sexual partners. Increased NT thickness and DVPI moderately correlate with CHD incidence.

TRENDS IN THE INCIDENCE AND CHARACTERISTICS OF CONGENITAL HEART DISEASE IN LOPBURI PROVINCE, CENTRAL THAILAND, 2017-2021

Background: Congenital heart disease (CHD) is the newborn’s most common congenital anomaly. Nevertheless, limited evidence is available of CHD incidence in Thailand. Therefore, we aimed to determine the trends in CHD incidence from 2017 to 2021.&#x0D; Methods: A descriptive epidemiology study was conducted using data from 2017-2021. We collected data from the medical records of pediatric patients with CHD at the Pediatric Cardiology Clinic at Ananda Mahidol Hospital, central Thailand. The pediatric cardiologist reviewed medical records to obtain participants’ characteristics and medical data, including CHD diagnosis, sex, child order and maternal risk factors during pregnancy.&#x0D; Results: In total, 27,882 live births were recorded between 2017 and 2021 in Lopburi Province, Thailand. The study included 584 pediatric patients with CHD born between 2017 and 2021. Of these, 312 (53.4%) were males, and 89 (15.2%) presented cyanotic CHD. Sex-adjusted CHD incidence was 22.4 per 1000 live-births (95% CI: 17.6-34.5) in 2017; then rose to 25.7 per 1000 live-births (95% CI 21.7- 30.2) in 2019 and dropped to 15.4 per 1000 live-births (95% CI 12.1-19.3) in 2021 (p for trend = 0.317). Two hundred and fourteen patients (36.6%) presented a ventricular septal defect. In 2017, the proportion of patients receiving a diagnosis of CHD before one year of age was 57.7% and continuously rose to 100% in 2021 (p-for trend &lt;0.001). The proportion of alcohol consumption during pregnancy was consistently high, ranging from 61.6 to 74.8% (p for trend = 0.189). In addition, contraindicated drug use during the first trimester of pregnancy was also constantly high (73.1%) among study participants over five years (p for trend = 0.235).&#x0D; Conclusion: CHD incidence in Lopburi Province has been persistently high from 2017 to 2021. The average age at diagnosis with CHD in this study population continuously declined over five years. Characteristics of CHD risk factors during pregnancy constituted a constantly high proportion among these study participants over one half decade.

Sociodemographic profile associated with congenital heart disease among infants

PurposeCongenital heart disease affects thousands of newborns each year in the United States. Previous United States-based research has explored how sociodemographic factors may impact health outcomes in infants with congenital heart disease; however, their impact on the incidence of congenital heart disease is unclear. We explored the sociodemographic profile related to congenital heart disease to help address health disparities that arise from race and social determinants of health. Defining the sociodemographic factors associated with congenital heart disease will encourage implementation of potential preventative measures. Design and methodsWe conducted a secondary analysis of longitudinally collected data comparing 39 infants with congenital heart disease and 30 healthy controls. We used a questionnaire to collect sociodemographic data. Pearson's chi-square test/Fisher's exact tests analyzed the associations among different sociodemographic factors between infants with congenital heart disease and healthy controls. ResultsWe found a statistically significant difference in maternal education between our 2 groups of infants (p = 0.004). ConclusionMaternal education was associated with congenital heart disease. Future studies are needed to further characterize sociodemographic factors that may predict and impact the incidence of congenital heart disease and to determine possible interventions that may help decrease health disparities regarding the incidence of congenital heart disease. Practice implicationsUnderstanding the associations between maternal sociodemographic factors and infant congenital heart disease would allow clinicians to identify mothers at higher risk of having an infant with congenital heart disease.

Lower Socioeconomic Status is Associated with an Increased Incidence and Spectrum of Major Congenital Heart Disease and Associated Extracardiac Pathology.

Several studies have suggested an inverse relationship between lower socioeconomic status (SES) and the incidence of congenital heart disease (CHD) among live births. We sought to examine this relationship further in a Canada-wide population study, exploring CHD subtypes, trends, and associated noncardiac abnormalities. Infants born in Canada (less Quebec) from 2008 to 2018 with CHD requiring intervention in the first year were identified using ICD-10 codes through the Canadian Institute for Health Information Discharge Abstract Database. Births of CHD patients were stratified by SES (census-based income quintiles) and compared against national birth proportions using X2 tests. Proportions with extracardiac defects (ED) and nonlethal genetic syndromes (GS) were also explored. From 2008 to 2018, 7711 infants born with CHD were included. The proportions of major CHD distributed across SES quintiles were 27.1%, 20.1%, 19.2%, 18.6%, and 15.0% from lowest to highest, with significant differences relative to national birth proportions (22.0%, 20.0%, 20.6%, 20.7%, and 16.7% from lowest (1) to highest (5)) (p < 0.0001). No temporal trends in the CHD proportions across SES categories were observed over the study period. The distribution across SES quintiles was different only for specific CHD subtypes (double-outlet right ventricle (n = 485, p = 0.03), hypoplastic left heart syndrome (n = 547, p = 0.006), heterotaxy (n = 224, p = 0.03), tetralogy of Fallot (n = 1007, p = 0.008), truncus arteriosus (n = 126, p < 0.0001), and ventricular septal defect (n = 1916, p < 0.0001)), with highest proportions observed in the lowest quintile. The proportion of the total population with ED but not GS was highest in lower SES quintiles (< 0.0001) commensurate with increased proportion of CHD. Our study suggests a negative association between SES and certain CHD lesions and ED.

Incidence and types of congenital heart disease at a referral hospital in Jordan: retrospective study from a tertiary center.

Congenital heart disease (CHD) is the most common birth defect and accounts for significant global morbidity and mortality. Relatively little is known about the epidemiology of CHD in Jordan or the manner in which CHD is identified. A retrospective medical record review was conducted for all neonates who had an abnormal echocardiogram performed at a tertiary referral hospital. All included neonates had echocardiography performed by the same pediatric cardiologist at the discretion of the treatment team. Descriptive statistics were used to describe CHD incidence, types of CHD identified, and mechanism of identification. The incidence of congenital heart disease was 17.8 per 1,000 live births. This rose to 24.6 per 1,000 if patent ductus arteriosus in preterm infants was included. The most common identified abnormalities were PDA, atrial septal defects, persistent pulmonary hypertension, septal hypertrophy, and ventricular septal defects. Most children were evaluated either for a murmur heard on exam or as a part of screening due to other comorbidities or risk factors. Less than 1% of children had a prenatal diagnosis. There was a higher rate of persistent pulmonary hypertension during the COVID-19 pandemic than before (p < 0.001). There is a high incidence of CHD in Jordan. Increased prenatal and perinatal screening for CHD may allow for earlier detection.

Comprehensive program development for congenital heart disease in the United Arab Emirates and in Singapore

Background/Objective. Establishment and development of tertiary-care centres for congenital heart disease (CHD) face multiple challenges as they need to be accessible, affordable/sustainable and provide the best possible outcomes. We report our experience of foundation a new tertiary-care CHD centre in the United Arab Emirates (UAE) and program development in Singapore.&#x0D; Methods. Prevalence and incidence of CHD in the context of local sociocultural environment was reviewed. Having established program elements of team, premises and workflow, quality assurance system was established. Outcome analysis, continuous audit modified internal therapeutic policies in accordance to international databases.&#x0D; Results. Consanguinity, segregated pattern of the population, high birth rate and low prenatal screening rate increased the incidence/prevalence and complexity of CHD in the UAE. Higher complexity/redo presentation and presence of associated anomalies were prevalent in Singapore at a lower birth rate. Increasing patient-load, strict professional working environment, strong governmental financial mandate for care nominated our centre into a market-leading position in the UAE. Singapore has a highly-developed, regulated, still competitive healthcare infrastructure that represents challenges for program development. Additional surgical activity from underserviced regions could provide the adequate CHD patient-pool that is critical to achieve team-expertise and ensure the best possible patient and program outcomes.&#x0D; Conclusion. CHD has significant public health aspects, however, new and/or developing centres face different challenges depending on the sociocultural and economic environment they function in. External demographic factors, case-load, complexity, referral patterns, health-care financing structure are difficult to adjust; internal factors: program structure, quality assurance, professional working framework are subjects for improvement.

Associated congenital heart disease with Hirschsprung's disease: a retrospective cohort study on 2,174 children.

To examine the incidence and phenotypes of congenital heart disease (CHD) in a large cohort of patients with Hirschsprung's disease (HSCR). Retrospective data review of children with HSCR between 2003 and 2020 was conducted at the Provincial Key Laboratory for Structural Birth Defects in Guangzhou, Guangdong, China. HSCR was confirmed by pathological diagnosis. CHD was defined as a gross structural abnormality of the heart or intrathoracic great vessels that is of functional significance. A total of 2,174 HSCR patients (84.7% males) were studied and 306 of them underwent echocardiography. Overall, 27 children (1.2%) had associated CHD. Among them, CHDs mostly presented as atrial and ventricular septal defects (n = 5 and 12 respectively) and patent ductus arteriosus (n = 4). Three patients (1.4‰) presented as a severe CHD including complete atrioventricular canal, congenitally corrected transposition of the great arteries and double-outlet of right ventricle. Among 14 patients carrying a chromosomal abnormality, CHD was detected in 4 infants (28.6%), all being mild forms of septal defects. Some new and severe types of CHD were found in patients with HSCR. Patients with syndromic features had higher incidence of CHD.

The effects of catheter choice on accessory pathway mapping efficiency

Abstract Funding Acknowledgements: Type of funding sources: None. Recent studies have assessed the use of a novel high density (HD) mapping catheter in pediatric patients, finding that the catheter is safe and effective in this population. However, there is a lack of understanding on use patterns of HD mapping technologies in this population and how these patterns affect intraprocedural workflows. The objective of this analysis was to assess the role of mapping catheter choice on the efficiency of accessory pathway localization and ablation in patients with structurally normal hearts treated in a pediatric EP setting. Acute data from 61 accessory pathway ablation cases in patients treated in a pediatric EP setting were prospectively collected from 14 hospitals in 6 countries in Europe and the US. Choice of mapping tools and strategy was left to physician discretion. Procedure data such as map collection duration and statistics were compared across three mapping catheter choices: Ablation Catheter (AC) (n=42), Linear Catheter (LC) (n=9), and HD Grid Catheter (HDC) (n=10). Of the 61 procedures, 16 cases were patients aged 0-12 years (26%), 34 aged 13-17 years (56%), and 5 aged 18+ years (18%). Distribution of catheter use was similar across age groups with HDC use of 13%, 15%, and 27%, respectively. Pathway location did not correlate with catheter choice. There was 0% incidence of congenital heart disease. Mean mapping times in the AC, LC, and HDC groups were 20.6±15.8, 18.8±12.9, and 14.2±13.8 minutes, respectively; differences in mapping times were not statistically significant but trended lower with higher density mapping. The mean number of mapping points used in each group was 44±46, 101±62, and 1521±908 points, respectively (p&amp;lt;.01). Ablation was attempted in 57 (93%) of cases with 100% acute success eliminating accessory pathway conduction. Ablation time was analyzed in patients receiving RF ablation (93% of treated; n=53); mean RF time was 526s in the AC group, 369s in the LC group, and 367s in the HDC group; differences were not statistically significant (p&amp;gt;.05). All groups had similar ablation target technique distributions including timing map and signal morphology. Mean procedure time was 2:11 (h:mm) in the AC group, 1:56 in the LC group, and 1:44 in the HDC group; differences were not statistically significant. Analysis of this cohort of pathways in a pediatric setting shows multipolar catheters may provide more mapping information in shorter times. RF ablation and procedure times also trend shorter in procedures with HD Grid used for mapping, regardless of anatomic location of pathway. Further analysis in a larger cohort may be warranted to validate these findings and explore use of open window mapping described elsewhere.

Effect of Arsenic Contamination in Drinking Water During Pregnancy with the Incidence of Congenital Heart Disease: A Meta-Analysis

Background: Water is a basic human need. The need for clean water continues to increase along with the increasing human population. Drinking water providers are required to provide harmless water. Several studies have discussed the effect of arsenic contamination in drinking water on the incidence of congenital heart disease. Objective: To evaluate the impact of arsenic contamination in drinking water during pregnancy on the incidence of congenital heart disease. Method: Search for published scientific articles using the PRISMA (Preferred Reporting, Items for Systematic Reviews and Meta-Analysis) method conducted on PubMed using the following keywords: “arsenic”, “drinking water” “contamination”, “congenital heart desease”, “congenital heart defect” as the keyword, also combined by using “or” and “and”. Further meta-analysis using Jamovi version 2.2.5 software were performed based on the included published scientific articles. Result: Based on 3 studies included about effect of arsenic contamination in drinking water during pregnancy with the incidence of congenital heart disease, the analytical results showed a statistically significant positive relationship (z=1.42, p&lt;0.001;95% CI[-0.038;0.240]) but in the low category (r=0.101). Conclusion: Arsenic contamination in drinking water during pregnancy has a low effect on the incidence of congenital heart disease.&#x0D; Keyword: drinking water, arsenic, congenital heart disease

Outcome of Management of Tetralogy of Fallot in a Cardiac Center of a Tertiary Care Hospital in Bangladesh

Background: Congenital heart diseases (CHD) represent one of the major groups of birth defects and make up approximately 1% of human malformations. Incidence of congenital heart disease is 8-10/1000 live births. The incidence is higher in premature infants. In Bangladesh incidence of congenital heart diseases was found up to 25/1000 live birth in a one year study conducted in CMH Dhaka. Congenital cyanotic heart disease accounts for less than 25% of total congenital cardiac defects. In TOF the lungs are under-perfused as blood shunts from right to left bypassing the lungs. TOF constitutes 5-7% of congenital heart disease. It is the commonest congenital cyanotic heart disease in Bangladesh. Objective: The aim of this study was to assess outcome of management of Tetralogy of Fallot in a cardiac center of a tertiary care hospital in Bangladesh. Methodology: It was a retrospective observational study and was conducted in the department of Pediatric Cardiology of Combined Military Hospital (CMH), Dhaka, Bangladesh from January 2015 to December 2016. We included 50 patients with TOF taking treatment from our department. All the patients were divided into three groups according to treatment type. Medical management group, palliative surgery group and total intra cardiac repair group and their outcome were assessed. Results: In our study we found 6 (12%) patients were in medical management group, 8 (16%) were in palliative surgery group and 36 (72%) were in total intra cardiac repair group. Among them 33 (66%) were male and 17 (34%) were female. Majority patients 30 (60%) were in less than 3 years of age. In medical management group all the patients had oxygen saturation &lt;80%, 4 (66.68%) patients reduced frequency of hyper-cyanotic spell, 1 (16.67%) patient had cerebral abscess. In palliative surgery group 6 (75%) patients had &gt;90% oxygen saturation, 2 (25%) had &lt;90% oxygen saturation, tet spell reduced in all the cases. After total intra cardiac repair no patient had .....

Incidence of congenital heart disease with consanguinity: 10 years of experience in a single-center

Incidence of congenital heart disease with consanguinity: 10 years of experience in a single-center