Prevalence Of Congenital Heart Disease Research Articles

Clinical Profile and Natural History of Children with Ventricular Septal Defects in Tripoli, Libya from 1996 through 2009

Introduction and Objectives: Ventricular septal defect (VSD) is the most common congenital heart diseases, it accounts 40%. This study carried out to describe the epidemiological characters and clinical presentation of patients with VSD in Western and Southern Libya, and to evaluate spontaneous versus surgical closure in different types.  Patients and Methods It is hospital case series study conducted by reviewing the medical records of 1092 patients followed at cardiology outpatient department (OPD) of Tripoli Children Hospital from 1996 through 2009, the data abstracted from medical records and filed in a worked sheet, Excel and SPSS software used to analyze the data.  Results We identified 1092 patients. The results revealed that 30.9% diagnosed during the first month, and 49.7% from1 to 12 months. From 1092 patients; 50.8% have peri- membranous VSD, 18.8% muscular, and 13% having an apical type. 97.2% of males have peri-membranous VSD where muscular and apical seen more in female, p value = 0.001. Cardiac murmur is the common presenting sign 61.3%, 9.3% have feature of Down syndrome. Positive Family history of CHD in 9.9% with significant relationship between family history and type of VSD (peri- membranous type), P value = 0.001. Small VSDs accounts 66.9%, large VSDs 16.1%, followed by medium size VSD 11.8% of cases. Patients with large VSDs more prone for hospital admission as 101 (57.4%) from 176 patients were admitted to hospital, followed by moderate size (45%), P value = 0.001. 70.3% of patients with large VSD need either medical or surgical treatment, P value = 0.001. Spontaneous closure seen in 17%, 0.6% died, while 41.9% still on follow up. Muscular VSD shows the highest tendency for spontaneous closer as 72% closed by the age of 2 years.  Conclusion We conclude that peri- membranous VSD is the most common type. Two thirds of patients had been diagnosed in the first year of life. Big defects and some of moderate VSDs need either medical or surgical treatment. Spontaneous closer is usually seen in the first 2 years of life.  Recommendations We highly recommend for further multi-center study all over the country to get reliable data on the prevalence of congenital heart diseases and the incidence of ventricular septal defect in our population.

A “twelve-section ultrasonic screening and diagnosis method” and management system for screening and treating neonatal congenital heart disease at the grassroots level in Tang County, Hebei Province, China

BackgroundTo explore a method for screening and diagnosing neonatal congenital heart disease (CHD) applicable to grassroots level, evaluate the prevalence of CHD, and establish a hierarchical management system for CHD screening and treatment at the grassroots level.MethodsA total of 24,253 newborns born in Tang County between January 2016 and December 2020 were consecutively enrolled and screened by trained primary physicians via the “twelve-section ultrasonic screening and diagnosis method” (referred to as the “twelve-section method”). Specialized staff from the CHD Screening and Diagnosis Center of Hebei Children’s Hospital regularly visited the local area for definite diagnosis of CHD in newborns who screened positive. Newborns with CHD were managed according to the hierarchical management system.ResultsThe centre confirmed that, except for 2 newborns with patent ductus arteriosus missed in the diagnosis of ventricular septal defect combined with severe pulmonary hypertension, newborns with other isolated or concomitant simple CHDs were identified at the grassroots level. The sensitivity, specificity and diagnostic coincidence rate of the twelve-section method for screening complex CHD were 92%, 99.6% and 84%, respectively. A total of 301 children with CHD were identified. The overall CHD prevalence was 12.4‰. According to the hierarchical management system, 113 patients with simple CHD recovered spontaneously during local follow-up, 48 patients continued local follow-up, 106 patients were referred to the centre for surgery (including 17 patients with severe CHD and 89 patients with progressive CHD), 1 patient died without surgery, and 8 patients were lost to follow-up. Eighteen patients with complex CHD were directly referred to the centre for surgery, 3 patients died without surgery, and 4 patients were lost to follow-up. Most patients who received early intervention achieved satisfactory results. The mortality rate of CHD was approximately 28.86 per 100,000 children.ConclusionsThe “twelve-section method” is suitable for screening neonatal CHD at the grassroots level. The establishment of a hierarchical management system for CHD screening and treatment is conducive to the scientific management of CHD, which has important clinical and social significance for early detection, early intervention, reduction in mortality and improvement of the prognosis of complex and severe CHDs.

Congenital Heart Disease in a Tertiary Care NICU; Pattern, Risk Factors and Outcome

Background: Identification of congenital heart disease (CHD) is increasing, may be due to advancement of diagnostic technology and availability of trained man power. Several studies have been conducted in Bangladesh to identify the prevalence and pattern of CHD but paucity of data regarding etiology and outcome. Objective: The aim of this study is to identify the frequency and risk factors of so that preventive measures can be taken. Methods: This retrospective case control study was conducted in the neonatal intensive care unit (NICU), BSMMU, Dhaka from January 2021 to June 2022. All admitted neonates in NICU with the diagnosis of CHD were considered as cases. Twice number of newborn having gestational age and birth weight matched neonates diagnosed other than CHD in NICU were considered as control. Data were extracted from the Medical records of patient files and computer electronic data records of “Newborn Birth Defect (NBBD) surveillance in Bangladesh.” The level of significance was set at 5% (P <0.05). Result: Total 914 patient were admitted during the study period among them 119 patient were CHD (frequency rate 13%). Mean birth weight and gestational age of CHD group were 2376±783.85g & 35.80±3.26 weeks respectively. About 85.7% had acyanotic CHD, among them most prevalent were atrial septal defect (42%) then patent ductus arteriosus (11.8%). Cyanotic CHD were 17 (14.3%). Maternal DM significantly increase the risk of developing CHD, Odd’s ratio 1.971 (CI: 1.182 - 3.285). Death were more in CHD group (34.5%) than control group (20.4%), p values = 0.005. Conclusion: Frequency (13%) of congenital heart disease was high. Maternal diabetes mellitus increases the risk of developing CHD of their baby. Death were more among babies with CHD. BANGLADESH J CHILD HEALTH 2022; VOL 46 (3) : 122-129

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

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

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.

Time trends and birth rates in women with congenital heart disease; a nationwide cohort study from Norway 1994–2014

BackgroundMore women with congenital heart disease (CHD) reach reproductive age, but little is known of their success in having children. We investigated time trends of CHD in women of reproductive age and maternal CHD in childbirth and compared birth rates in women with CHD to birth rates in women without heart disease. Methods and resultsIn a national cohort, we combined information from five registries in Norway 1994–2014. Among 1,644,650 women aged 15–45 years, 5672 had CHD. Among 1,183,851 childbirths, 3504 were registered with maternal CHD. The prevalences of mild and moderate/severe CHD in women increased by an average of 3–4% per year 1994–2014, as did the prevalences of mild and moderate/severe maternal CHD in childbirth. Compared to women without heart disease, the likelihood of having children was similar for women with mild CHD (rate ratio 1.03, 95% confidence interval 0.97–1.09) but lower for women with moderate/severe CHD (rate ratio 0.75, 95% confidence interval 0.68–0.84). The mean number of childbirths was similar in women with mild CHD and women without heart disease (1.81 vs 1.80, p = 0.722) but lower in women with moderate/severe CHD (1.42, p < 0.001). ConclusionIn a national cohort over two decades of women of reproductive age, the prevalence of maternal CHD in childbirth reflected the increasing prevalence of CHD in the population. Birth rates were similar for women with mild CHD and women without heart disease, whereas women with moderate/severe CHD were less likely to have children and had a lower mean number of childbirths.

Congenital cardiac anomalies in non-syndromic cleft lip and cleft palate patients: A systematic review and meta-analysis.

The aim was to establish a specific and definite connection between non-syndromic orofacial cleft patients and associated congenital heart disease (CHD). Following PRISMA guidelines, selective databases were searched for data collection. Studies showing a definite association of CHD with orofacial cleft were included, and studies non-specific of the association of orofacial cleft with CHD were excluded. Data extraction criteria were study design, frequency of CHD in overall non-syndromic orofacial cleft and in specific cleft type, and most prevalent congenital cardiac anomaly. DerSimonian Laird random effects model was used to estimate the pooled proportion of CHD, along with corresponding 95% confidence intervals (CIs) for each measure. Publication bias was assessed using Fail-Safe N analysis and the Rosenthel approach. Of a total of 182 articles searched, only 30 studies were assessed. The overall pooled estimate of the proportion of CHD in total cleft lips/palates was 16% (95% CI: 13-19). The odds of developing CHD in cleft palates was 4.08 times more as compared to cleft lips with 95% CIs of 3.86-4.33, and 1.65 more as compared to cleft lips and palates both with 95% CI of 1.52-1.68. We affirm the upsurging prevalence of CHD in non-syndromic cleft children and vehemently propose that it is of utmost importance to inculcate it in practice and policy-making to screen all non-syndromic orofacial cleft children for congenital cardiac anomaly. This study was registered on PROSPERO (ID no. CRD42023391597) on February 24, 2023.

Epidemiological Survey of Congenital Heart Disease Among Children Aged from 2 to 18 in Suo County, Nagqu, Tibet.

Lin, Tian, Huaping Jia, Yunming Li, Yongxing Xu, Bei Zhao, Dong Zheng, Hongfeng Yan, Meihui Zhao, Yanlei Li, Liping Xia, Fengxia Zhou, Cuiping Liu, Ke Ma, Ma Mi, and Jianwen Gu. Epidemiological survey of congenital heart disease among children aged from 2 to 18 in Suo County, Nagqu, Tibet. High Alt Med Biol. 00:000-000, 2024. Background: Studies have reported the prevalence of congenital heart disease (CHD) in parts of Tibet, but relative epidemiological surveys are rare. We aimed to explore the prevalence of CHD in children and its relationship with family history in Suo County, Nagqu, Tibet, an altitude of 3,980 meters. Methods: We recruited 4,002 children aged 2-18 years. Subjects underwent a family history investigation, cardiac auscultation, and clinical manifestation examination and then received echocardiographic screening. Results: The prevalence of CHD among children in Suo County was 0.97% (39 cases), much higher than the prevalence at sea level. The most common subtype was atrial septal defect, accounting for 53.9% of CHD, followed by patent ductus arteriosus (33.3%) and ventricular septal defect (12.8%). We also found that children whose mothers had previously borne children with CHD had a higher risk of CHD than those without (p = 0.002); other factors related to CHD during pregnancy, such as smoking, drinking, drug use, and viral infection, showed no statistical differences between children with and without CHD. Conclusions: The prevalence of CHD in children in Suo County is much higher than at low altitude, consisting mostly of simple forms with left-to-right shunt, with rare complex CHD. These results support implementing diagnostic and treatment plans to prevent CHD in Suo County.

Clinical Features and Cardiac Anomalies of Children with Down Syndrome. A Literature Report

Background: Clinical diagnosis of Down syndrome is based on the characteristic features and associated malformations. Nonetheless, there is significant individual diversity in the clinical presentation. Not every physical characteristic may be present, particularly in infants. At the same, congenital heart abnormalities (CHD) remain a major predictor of death in children with Down syndrome (DS) despite improvements in surgical therapy for these conditions. The effects of DS vary from person to person, with some people having a significant impact while others are well and capable of living unassisted as adults. So, this study is done to understand the pattern of clinical features and cardiac anomalies in various research reports.Aim: This scoping review aims to describe the frequency and distribution of clinical features and cardiac anomalies in children with Down syndrome and to consider the clinical implications of this knowledge.Methods: Medline, CINHAL, and PubMed databases were searched electronically to identify pertinent articles from 2000 to 2023. Children with Down syndrome and cardiac comorbidities aged 18 years or younger met the inclusion criteria. Articles that were not peer-reviewed or written in English were disqualified at the title or abstract level.Results: Literature revealed that the common physical and dysmorphic features found in individuals with Down syndrome include flat facial profile, epicanthal folds, upward slanting eyes, hypotonia, small ears, short neck, protruding tongue, small hands and feet, brushfield spots, sandal gap, and short stature. It's important to note that while these physical features are commonly associated with Down syndrome, not all individuals will exhibit every characteristic, and the severity can vary greatly among individuals.A high prevalence of CHD was reported in DS children from a group where consanguinity was relatively frequent. The prevalence of congenital heart disease in children with Down syndrome is the highest reported, especially when the researchers have used diagnostic ultrasound. VSD and AVSD, followed by persistent ductus arteriosus, and tetralogy of Fallot are the most common CHD in DS children. Recent research suggests that though the incidence of CHD in DS children has remained stable over time, there may be trends in some forms of CHD, with a rise in isolated, less severe kinds and a reduction in complicated, more severe ones. Individuals with Down syndrome can lead fulfilling lives with appropriate support and resources.Conclusion: All neonates with a new diagnosis or suspicion of DS must undergo comprehensive screening, which includes clinical examination, ECG, and echocardiography in the second trimester combined with fetal echocardiography when the fetal ultrasonography raises the likelihood of an abnormality. Literature proves that regardless of the existence of DS, early CHD repair is advised for newborns susceptible to biventricular surgery. For the most part, DS is not linked to an increased perioperative risk for CHD.Understanding DS heterogeneity will help professionals provide better prenatal counseling, assist parents in establishing focused early interventions to improve daily activities and the quality of life for their children, and assist policy-makers in providing and allocating resources for disability services. A sustaining commitment to scientific and clinical research studies is necessary to enhance the quality of life and survival for DS patients from infancy into adulthood.

Congenital heart disease: clinical characterization in newborns from a referral hospital, Manaus, Amazonas, Brazil

In Brazil, the impact of congenital heart disease (CHD) remains obscured by limited data, especially in the northern region. For this reason, our study seeks to uncover the prevalence and clinical variations of CHD in Manaus, Amazonas. This study followed a cross-sectional model performed in 110 newborns with CHD diagnosed between March 2022 to April 2023. Complex CHD was present in about 80% of the newborns, which increased the risk of requiring multiple surgical interventions. We found and characterized 19 CHD phenotypes, with a higher prevalence of complex CHD cases. We documented 12 fatalities, mostly attributed to septic and cardiogenic shock related to complex CHD with high levels of urea, creatinine, AST, ALT, CRP, GGT, NT-proBNP and acidosis (p&lt;0.001). Our findings suggest a close relationship between CHD and a systemic impact with chronic/acute kidney failure and hepatopathies. Inadequate data from the information system on live births (SINASC) results in underreporting and increased CHD mortality rates, emphasizing the urgency for new strategies to enhance case identification and improve early diagnosis of critical CHD with important systemic repercussions.

The detailed profile of congenital heart diseases in 254 children with Down syndrome in Saudi Arabia

BackgroundDown syndrome is the most common chromosomal abnormality in humans. It is associated with several congenital anomalies, including a spectrum of congenital heart diseases. Understanding the true prevalence and distribution of congenital heart diseases is essential for health resource planning, outcomes, and family counseling.This study aimed to assess the prevalence and distribution of congenital heart disease in children with Down syndrome. It is a retrospective cohort review that included all patients treated at King Abdulaziz University Hospital. Frequencies were estimated using the SPSS software and comparisons were made using Student’s t test.ResultsThe ages of the 254 subjects ranged from less than 1 year to 53 years. Of these, 44.5% were female and 40.6% were Saudi nationals. Congenital heart disease was present in 66.5% with a significant difference between Saudi nationals 44.6%) and non-Saudi nationals 71.5% (P = 0.01). The atrioventricular septal defect was the most common pathology, representing 33.1% of all congenital heart diseases followed by perimembranous ventricular septal defect 18.9%, and right ventricular pathology 10.2%.ConclusionThe prevalence of congenital heart diseases in Saudi children with Down Syndrome is similar to that reported worldwide. Septal defects and right-sided pathologies are the dominant forms of congenital heart diseases, with atrioventricular septal defect and perimembranous ventricular septal defect representing the most common pathologies.

Arrhythmias including atrial fibrillation and congenital heart disease in Kleefstra syndrome: a possible epigenetic link.

Kleefstra syndrome (KS), often diagnosed in early childhood, is a rare genetic disorder due to haploinsufficiency of EHMT1 and is characterized by neuromuscular and intellectual developmental abnormalities. Although congenital heart disease (CHD) is common, the prevalence of arrhythmias and CHD subtypes in KS is unknown. Inspired by a novel case series of KS patients with atrial tachyarrhythmias in the USA, we evaluate the two largest known KS registries for arrhythmias and CHD: Radboudumc (50 patients) based on health record review at Radboud University Medical Center in the Netherlands and GenIDA (163 patients) based on worldwide surveys of patient families. Three KS patients (aged 17-25 years) presented with atrial tachyarrhythmias without manifest CHD. In the international KS registries, the median [interquartile range (IQR)] age was considerably younger: GenIDA/Radboudumc at 10/13.5 (12/13) years, respectively. Both registries had a 40% prevalence of cardiovascular abnormalities, the majority being CHD, including septal defects, vascular malformations, and valvular disease. Interestingly, 4 (8%) patients in the Radboudumc registry reported arrhythmias without CHD, including one atrial fibrillation (AF), two with supraventricular tachycardias, and one with non-sustained ventricular tachycardia. The GenIDA registry reported one patient with AF and another with chronic ectopic atrial tachycardia (AT). In total, atrial tachyarrhythmias were noted in six young KS patients (6/213 or 3%) with at least four (three AF and one AT) without structural heart disease. In addition to a high prevalence of CHD, evolving data reveal early-onset atrial tachyarrhythmias in young KS patients, including AF, even in the absence of structural heart disease.

Investigating the Risk Factors of Congenital Heart Diseases in Newborns Admitted to Amir al-Momenin Hospital in Semnan from 2020-2021

Introduction: Congenital heart defects refer to conditions that affect the structure and function of a newborn’s heart. The aim of this study was to investigate the prevalence and risk factors of congenital heart diseases in hospitalized infants in the neonatal intensive care unit of Amiralmomenin Hospital in Semnan. Methods: In this study, 300 medical records of hospitalized infants who underwent echocardiography in the neonatal intensive care unit of Amiralmomenin Hospital in Semnan during 2020-2021 were reviewed. The overall prevalence of congenital heart diseases and their types (ASD/VSD/PDA/TOF) were determined, and the association of study variables (infant gender, gestational age, maternal age, infant weight, parental family history, medication use during pregnancy, and history of heart disease and gestational diabetes) with congenital heart disease was evaluated. Results: The results showed that out of the total 300 hospitalized infants who underwent echocardiography, 132 infants (44%) were diagnosed with congenital heart disease. PDA and VSD were the most common types of congenital heart diseases with a prevalence of 8.56% and 5.23%, respectively. Conclusion: According to the results, there was a significant association between congenital heart disease and diabetes, hypertension, as well as medication use during pregnancy and family history of heart disease , infant gender, gestational age, and weight did not show a significant association. However, there was a significant association between the type of congenital heart disease and gestational age and infant weight, with an increase in gestational age and infant weight leading to a decrease in the prevalence of congenital heart disease

Infant congenital heart disease prevalence and mortality in French Guiana: a population-based study

Few studies have assessed the prevalence and mortality of simple or complex congenital heart diseases (CHD) in newborns. In Latin America and Caribbean (LAC), CHD epidemiology seems highly variable, with few population-based assessments and different methodologies between studies. To date, the situation in French Guiana, a French overseas territory located in South America between Brazil and Suriname, has never been described. We analysed CHD prevalence, characteristics and related infant mortality in French Guiana, with a population-based registry analysis of all fetal and live birth CHD cases in infants under 1 year (January 2012-December 2016). Overall, 33,796 births (32,975 live births) were registered, with 231 CHD (56 fetuses), including 215 live births. Most frequent CHD categories were anomalies of the ventricular outflow tract and extra-pericardial trunks, and ventricular septal defects. 18.6% (43/231) chromosomal or genetic anomalies, and 6.5% (15/231) terminations of pregnancy were observed. Total CHD prevalence was 68.4 [95% CI: 67.9-68.8] per 10,000, while live birth prevalence was 65.2 [95% CI: 64.7-65.7] per 10,000. Total infant mortality was 9.4/10,000 live births [95% CI 9.1-9.7], with highest rates for functionally univentricular hearts (FUH). A distinct profile for CHD is highlighted in French Guiana with elevated mortality linked to FUH. A potential determinant of the recognized excess mortality risk might be the presence of chromosomal or genetic anomalies in about a fifth of all CHD. This helps us to better understand CHD burden in this part of South America and provides future keys towards reducing CHD-related infant mortality. The authors received no financial support for the present research, authorship, and/or publication of this article.

Anorectal malformations (ARM) and VACTERL association and severity of congenital heart diseases (CHD): Experience of 396 consecutive patients in a tertiary center

ObjectiveCongenital heart diseases (CHD) are the most frequently associated anomalies with anorectal malformations (ARM). Nevertheless, many specific aspects of CHD in ARM patients have yet to be studied. The aims of this study were to evaluate the prevalence and distribution of CHD in neonates-infants with ARM, and to explore whether the severity of ARM, and the presence of VACTERL association, had an impact on CHD rate, severity, and timing at first cardiac surgery. Study designAll consecutive newborn-infants with ARM managed in our tertiary center (January-1999; December-2021) were collected from a prospective database and retrospectively analyzed. Prevalence and distribution of CHD in ARM patients were assessed. Patients were divided into groups depending on ARM severity and presence of VACTERL association. Pairwise comparison for CHD prevalence, severity, and timing at first cardiac surgery was performed between groups. ResultsOf 396 ARM patients identified, those with severe ARM showed a higher number of overall CHD compared to patients with non-severe ARM (36.7 % vs. 25.2 %, p = 0.032). VACTERL + patients had a significantly higher prevalence of CHD (73.4 % vs. 16.4 %; p <0.001) and major CHD (51.7 % vs. 26.9 %; p = 0.008) when compared with VACTERL-patients. Furthermore, VACTERL + patients underwent first cardiac surgery at a significantly younger age than VACTERL-patients (5.2 ± 15.2 months vs. 11.9 ± 6.3, p = 0.039). ConclusionsPatients with severe ARM had a higher number of CHD compared to patients with non-severe ARM. VATERL + patients had significantly more CHD and more severe CHD than VACTERL-patients. Early screening for CHD is strongly recommended in all newborns diagnosed with ARM before surgery. Level of evidenceIII retrospective comparative study.

Liver transplantation for pediatric patients with congenital heart disease: A single-center study in mainland China.

Liver transplantation (LT) is a serious cardiovascular stressor for patients with end-stage liver disease (ESLD). Data on the effects of cardiovascular diseases on pediatric LT is limited. No study on LT for pediatric patients with ESLD combined with congenital heart disease (CHD) has been reported from mainland China. A total of 1005 patients were included in this study. The Kaplan-Meier method with log-rank testing was used to evaluate survival outcomes between groups. Univariable and multivariable Cox regression models were used to determine the risk factors for patient and graft survival. The most common indication for LT was biliary atresia (BA 90.3%). The prevalence of CHD was 3.8% (38). 42 CHD were found in 38 patients. The incidence of death and graft loss was more common in the CHD group than in the no-CHD group (13.2% vs. 5.0%, p = .045 and 15.8% vs. 6.2%, p = .019, respectively). The 5-year patient survival and graft survival in the CHD group versus the no-CHD group was 86.8% versus 94.7% (log-rank p = .022) and 84.2% versus 93.5% (log-rank p = .015), respectively. No significant differences were observed in re-transplantation, hepatic artery thrombosis (HAT), and portal vein thrombosis (PVT). After adjusting for age, BMI, etiology of LT, and other confounding factors, we can still find that the presence of CHD was associated with patient and graft survival after LT. The presence of CHD was associated with higher mortality and lower graft survival after LT. If possible, the cardiac defects should be addressed prior to LT.

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.

CHARGE syndrome and congenital heart diseases: systematic review of literature.

CHARGE syndrome (CS) is a rare genetic disease that affects many areas of the body. The aim of the present systematic review was to evaluate the prevalence and types of congenital heart diseases (CHDs) in CS and their impact on clinical outcome. A systematic review from 1981 to September 2022 was conducted. Clinical studies that reported the association between CS and CHDs were identified, including a case report of a rare congenital anomaly of the aortic arch (AA) with persistent fifth aortic arch (PFAA). Demographic, clinical and outcome data were extracted and analyzed. Sixty-eight studies (44 case reports and 24 case series; n=943 CS patients) were included. The prevalence of CHDs was 76.6%, patent ductus arteriosus (PDA) 26%, ventricular (VSD) 21%, atrial septal defects (ASD) 18%, tetralogy of Fallot 11%, aortic abnormalities 24%. PFAA has not been previously reported in CS. Cardiac surgery was performed in more than half of CS patients (150/242, 62%). In-hospital mortality rate was about 9.5% (n=86/900) in case series studies and 12% (n=5/43) in case reports, including cardiovascular (CV) and non-CV causes. CHDs and feeding disorders associated with CS may have a substantial impact on prognosis. CHDs were usually associated with CS and represent important causes of morbidity and mortality. PFAA, although rare, may also be present. The prognosis is highly dependent on the presence of cardiac and non-cardiac developmental abnormalities. Further studies are needed to better identify the main causes of the long-term outcome of CS patients.

The spectrum of congenital heart defects in neonates of diabetic mothers

Introduction: Insulin-dependent diabetes mellitus (IDDM) in a mother is considered a risk factor for congenital malformation, including congenital heart diseases. Many studies signify a five to six times increased frequency of congenital heart diseases in neonates born from insulin-dependent diabetic mothers. This study aimed to identify the spectrum of congenital heart diseases (CHDs) in neonates born from insulin-dependent diabetes mellitus (IDDM) mothers. Methods: Between April 2019 and March 2021, a descriptive cross-sectional study involved 120 consecutive neonates aged 0-28 days of mothers with insulin-dependent diabetes mellitus (IDDM) at Maiwand Teaching Hospital, Kabul University of Medical Sciences. Neonates born to non-diabetic mothers were excluded. IDDM refers to mothers using Insulin upon admission to control blood glucose. CHD encompasses various heart defects affecting function. Family and maternal history were noted, focusing on health issues and delivery details. Neonatal mothers' ages ranged from 23 to 45 (mean 33), with diabetes onset spanning 2.5 to 25 years. Pediatric cardiologists conducted physical and echocardiographic exams. All CHD forms were documented using 2D echocardiography and Doppler studies (Sono Ace X-6 Machine). Result: 120 consecutive neonates born to mothers with insulin-dependent diabetes mellitus (IDDMs) were investigated during the two-year study period. Of them, 18 (15%) had congenital heart abnormalities. The prevalence of solitary and multiple CHD was 66.6 percent (12 patients) and 33.3 percent (6 patients) in the 18 infants born from IDDMs. The most prevalent isolated defects were PDA (22.2%) and Ventricular septal defect (16.6%). The most common multiple CHDs (16.6 percent) were PDA and ASD. PDA + ASD (16.6%) and VSD + PDA were the most frequent correlations with CHD (11.1 percent). Conclusion: Maternal insulin-dependent diabetes mellitus is an important risk factor for congenital heart disease. Careful assessment and early diagnosis of CHD in this high-risk group are very important in a pediatric population. Expansion of prenatal screening programs for CHDs in mothers suffering from insulin-dependent diabetes mellitus is necessary. Keywords: congenital heart diseases; diabetic mothers; insulin dependent; neonates

Accuracy of the NADAS Criteria in Helping Establish the Diagnosis in Children With Suspected to Have Congenital Heart Disease

Background : Prevalence of congenital heart disease is approximately 8/1000 live births and majority of them are diagnosed during evaluation of a murmur detected either during a routine follow-up or while assessment during an intercurrent illness. NADAS criteria can be used as screening test with hight a sensitivity and specificity regarding ability to differentiate pathologic from innocent murmurs.&#x0D; Aim : The aim of the study was to assess the accuracy of the NADAS criteria in helping to diagnose disease in children suspected of having congenital heart disease.&#x0D; Methods : This was a diagnostic study with a cross-sectional approach and Inclusion criteria were children aged 1 month - 12 years from history and physical examination found a history of recurrent ARI, poor nutritional status, failure to thrive, found cardiac murmurs, without/any cyanosis who attended the outpatient cardiology pediatric at Haji Adam Malik General Hospital between Januari - july 2021. Data Analysis was created using the program IBM SPSS Statistics version 25&#x0D; Results : 70 children were screened. 60 children were detected to have CHD. 63,3% of the study population in the age group &lt;5 year had a heart disease. All CHD patients had poor nutritional status, abnormal ECG and 88.33% had a history of recurrent ARI, 80% had abnormal chest x-ray, and 55% had abnormal S2. A grade 3 or higher systolic murmur was found in 78.33% of patients had CHD.&#x0D; Conclusion : NADAS criteria can be used as a screening test for helping to diagnose disease in children suspected of having CHD with accuracy 87.14%, sensitivity of 88.33%, specificity was 80% when score at 3 (1 major + 2 minor) and AUC value is 0.842.