Types Of Congenital Heart Disease Research Articles

Association between type of congenital heart disease with child growth and development status: A cross-sectional study in Medan, Indonesia.

Congenital heart disease (CHD) is a congenital disorder primarily affecting newborns and children. Children with CHD have a greater risk of experiencing growth delays or disorders compared to healthy children. CHD also affects various aspects of a child's development. The aim of this study was to determine the association of CHD types (cyanotic and acyanotic) with the growth and development status of children. A cross-sectional study was conducted among CHD patients at a national reference hospital in Sumatra, H. Adam Malik General Hospital in Medan, Indonesia. The children's growth status was assessed using the WHO growth chart, and the developmental condition was evaluated through the Denver Developmental Screening Test-II. Chi-squared test and Fisher's exact test were used to assess the association between the type of CHD with growth and development status in children. Using a consecutive sampling method, a total of 53 individuals were included in this study. Almost half of CHD patients (48.1%) were within the age group of 0-2 years and more than half (61.1%) were girls. Acyanotic CHD (74.1%) was more prevalent than cyanotic CHD (25.9%), with ventricular septum defect (VSD) as the most common diagnosis. A total of 37% of children with CHD suffered from malnutrition, whereas the remaining 62.9% had good nutrition. The Denver Developmental Screening Test-II indicated that 81.4% of the children were normal, whereas 18.5% had developmental disorders. Our data suggested a significant association between cyanotic CHD and poor growth status in children based on weight-for-age, weight-for-length, and body mass index (BMI)-for-age. However, there was no association between the type of CHD and developmental status in children. This study highlights that the type of CHD is significantly associated with the growth status of children, but not with their developmental status.

Transcriptome studies of congenital heart diseases: identifying current gaps and therapeutic frontiers.

Congenital heart disease (CHD) are genetically complex and comprise a wide range of structural defects that often predispose to - early heart failure, a common cause of neonatal morbidity and mortality. Transcriptome studies of CHD in human pediatric patients indicated a broad spectrum of diverse molecular signatures across various types of CHD. In order to advance research on congenital heart diseases (CHDs), we conducted a detailed review of transcriptome studies on this topic. Our analysis identified gaps in the literature, with a particular focus on the cardiac transcriptome signatures found in various biological specimens across different types of CHDs. In addition to translational studies involving human subjects, we also examined transcriptomic analyses of CHDs in a range of model systems, including iPSCs and animal models. We concluded that RNA-seq technology has revolutionized medical research and many of the discoveries from CHD transcriptome studies draw attention to biological pathways that concurrently open the door to a better understanding of cardiac development and related therapeutic avenue. While some crucial impediments to perfectly studying CHDs in this context remain obtaining pediatric cardiac tissue samples, phenotypic variation, and the lack of anatomical/spatial context with model systems. Combining model systems, RNA-seq technology, and integrating algorithms for analyzing transcriptomic data at both single-cell and high throughput spatial resolution is expected to continue uncovering unique biological pathways that are perturbed in CHDs, thus facilitating the development of novel therapy for congenital heart disease.

Longitudinal development of central SBP in children with congenital heart disease.

Central SBP (cSBP) was shown to be increased already in children with congenital heart disease (CHD). However, its development over time has not yet been investigated. The aim of this study was to evaluate the natural course of cSBP over time from longitudinal assessment in children with CHD. In this longitudinal study, 306 children and adolescents (11.3 ± 2.9 years, 34% girls) with various CHD were prospectively examined from July 2014 to May 2022. Over a mean follow-up length of 30.1 ± 18.9 months, 467 follow-up assessments have been conducted. cSBP was noninvasively assessed by oscillometric measurement via Mobil-O-Graph. A linear mixed effect model was performed to examine the course of cSBP. cSBP increased significantly over time by 1.22 mmHg per year of age (P < 0.001). The longitudinal increase in cSBP over time remained significant when including sex (b = 0.68, P < 0.001), BMI (b = 1.12, P < 0.001), hypertensive medication (b = 1.13, P < 0.001), disease severity (b = 1.04, P < 0.001), and CHD type (b = 3.74, P = 0.03) in the model. Patients with transposition of the great arteries (TGA) after arterial switch had a significantly higher cSBP increase over time (b = 1.78, P < 0.001). The longitudinal cSBP increase was significantly higher in obese CHD children (b = 2.52, P = 0.005) and in boys (b = 0.85, P < 0.001). This study shows a longitudinal increase in cSBP in children with CHD. Whether observed trajectories of cSBP are normal or abnormal needs to be investigated in further studies. Monitoring of the vascular function with a special focus on patients with TGA and obese CHD children seems indicated.

The Value of Fetal Echocardiography for Early Detection of Congenital Heart Defects: A Report of our Experience in a Great Referral Heart Center in Iran

Background: Prenatal diagnosis of cardiac defects in fetus is of paramount importance to enable a better monitoring of heart defects and to allow planning a proper management. We reported our experience on fetal heart echocardiography in a great referral heart center in Iran with the approach to assessment of the diagnostic value of this tool for detecting Congenital Heart Diseases (CHDs). Methods: This retrospective cohort study included 234 pregnant women attended at a great referral heart center in Tehran, Iran between 2012 and 2016 for heart abnormalities screening. All women were evaluated by trans-abdominal fetal echocardiography from 12 weeks of gestation to term and after birth. Results: Fetal echocardiography showed different types of CHDs in 5.9%. CHDs detected in 9.9% of neonates by echocardiography after birth. The most common heart abnormalities detected was VSD in 4.3%. Considering echocardiography findings after birth as the reference, fetal echocardiography had a sensitivity of 92.8%, a specificity of 66.6%, a positive predictive value of 59.8%, a negative predictive value of 86.6%, and an accuracy of 84.1% for detection of heart defects. Conclusion: Fetal heart echocardiography is a sensitive tool for detection of CHDs, but its specificity is moderate.

Association of genetic and sulcal traits with executive function in congenital heart disease.

Persons with congenital heart disease (CHD) are at increased risk of neurodevelopmental disabilities, including impairments to executive function. Sulcal pattern features correlate with executive function in adolescents with single-ventricle heart disease and tetralogy of Fallot. However, the interaction of sulcal pattern features with genetic and participant factors in predicting executive dysfunction is unknown. We studied sulcal pattern features, participant factors, and genetic risk for executive function impairment in a cohort with multiple CHD types using stepwise linear regression and machine learning. Genetic factors, including predicted damaging de novo or rare inherited variants in neurodevelopmental disabilitiesrisk genes, apolipoprotein E genotype, and principal components of sulcal pattern features were associated with executive function measures after adjusting for age at testing, sex, mother's education, and biventricular versus single-ventricle CHD in a linear regression model. Using regression trees and bootstrap validation, younger participant age and larger alterations in sulcal pattern features were consistently identified as important predictors of decreased cognitive flexibility with left hemisphere graph topology often selected as the most important predictor. Inclusion of both sulcal pattern and genetic factors improved model fit compared to either alone. We conclude that sulcal measures remain important predictors of cognitive flexibility, and the model predicting executive outcomes is improved by inclusion of potential genetic sources of neurodevelopmental risk. If confirmed, measures of sulcal patterning may serve as early imaging biomarkers to identify those at heightened risk for future neurodevelopmental disabilities.

Trends in mortality risk of patients with congenital heart disease during the COVID-19 pandemic

Cardiovascular conditions are considered risk factors for poor outcomes associated with COVID-19. However, the effect of the COVID-19 pandemic on the mortality of patients with congenital heart disease (CHD) is unclear. Our study aims to examine the trends in mortality risk of CHD patients during the COVID-19 pandemic. This is a retrospective cohort study from the Pediatric Cardiac Care Consortium, a US-based registry of interventions for CHD. We included patients having US residence and direct identifiers; death events were captured by matching with the National Death Index. The observation window (2017-2022) was divided into pre-COVID-19 and COVID-19 era defined around the national onset of COVID-19 disease in 2020. Stratified Cox model was used to assess all-cause mortality between the pre- and the COVID-19 era. Among 45,130 patients with CHD (median age in 2017: 23.3 years, IQR: 19.0-28.4), 503 deaths occurred during the pandemic with 44 deaths (8.7%) attributed to COVID-19 (COVID-19 mortality rate of 0.09%). The overall risk of death for patients with all types of CHD during the pandemic was significantly higher compared to the pre-COVID-19 era (aHR 1.28, 95%CI: 1.08-1.53), with a differential trend towards increased risk in patients with two-ventricle (aHR 1.44, 95% CI: 1.19-1.76) vs unchanged risk for those with single ventricle CHD (aHR=0.83, 95% CI: 0.57-1.21). Adjusted subgroup analysis revealed a higher risk of death during the pandemic for CHD patients with male and chromosomal abnormalities. The excess deaths during the pandemic were attributed to COVID-19 itself rather than CHD or cardiovascular conditions. In this large CHD cohort study, there was a higher risk of death among CHD patients with male and chromosomal abnormalities. A differential trend towards higher risk for those with two vs. unchanged risk for single ventricle CHD was presented. The excess mortality was attributed to the COVID-19 itself and not to conditions potentially related to deferral of care. These results justify targeted protective measures towards the CHD population and may provide guidance for public health and medical care response in future epidemics.

Identification of abnormal ventricular asymmetry in 2nd trimester fetal ultrasound using artificial intelligence

Abstract Introduction Prenatal ultrasound screening for congenital heart disease (CHD) suffers from poor detection yield (10%-56%)[1, 2], with a strong impact on neonatal morbidity and mortality. The need for expertise in fetal cardiology and the high volume of screening cases limit the current detection rates. Computer-assisted prenatal screening could support clinicians in improving diagnostic yield, including for abnormal ventricular asymmetry, which is often hard to discriminate from physiological asymmetry. Here we aim at evaluating a deep neural network (DNN) for detection of abnormal ventricular asymmetry, which can be linked to multiple types of CHD, including coarctation of the aorta, pulmonary or aortic valve stenosis, ventricular hypoplasia or univentricular heart. Purpose To determine if a novel DNN can identify cases of abnormal ventricular asymmetry in 2nd trimester fetal ultrasound videos. Methods We used as an endpoint the presence of any abnormality causing a significantly abnormal asymmetry of the ventricles as seen in the fetal ultrasound. Consecutive patients who had an echocardiography performed at one center were included retrospectively starting from 2022, January 1st. Inclusion criteria were single pregnancy between 18 and 25 weeks of gestation. To have a high number of positive patients, patients were included separately if they were expected to be positive (n=35) or negative (n=75) to the endpoint, based on their medical record. Each case was reviewed by one of two physicians expert in fetal echocardiography interpretation to confirm the presence or absence of the endpoint. For each patient, a single video showing an interpretable four chamber view was selected. Patients with no such video were excluded. The DNN takes as input fetal ultrasound videos, and predicts the absence or presence of the endpoint for each video. If its confidence is low, the DNN predicts an "inconclusive" output instead. The DNN has a ResNet-50 architecture[3] adapted to videos and was trained on data from patients not included in the evaluation. The detection performance of the DNN and the proportion of conclusive outputs were assessed. Results Overall, 37 positive and 66 negative ultrasound videos were included. The DNN achieved an AUC of 75.5%, a sensitivity of 71.4% (54.9-83.7, 95%CI) and a specificity of 64.4% (51.7-75.4, 95%CI) in identifying cases of abnormal ventricular asymmetry, after excluding inconclusive diagnosis. The DNN predicted a conclusive diagnosis in 91.3% of cases. Conclusion A DNN could accurately identify cases of abnormal ventricular asymmetry in fetal heart ultrasound videos. Importantly, this evaluation was performed in a population of patients who had an echocardiogram, and which therefore contains more abnormal cases compared to patients undergoing standard screening. This work lays the ground for diagnostic support to enable a more efficient, accurate, and scalable analysis of fetal heart ultrasounds.

Automated segmentation of 3D cine cardiovascular magnetic resonance imaging.

As the life expectancy of children with congenital heart disease (CHD) is rapidly increasing and the adult population with CHD is growing, there is an unmet need to improve clinical workflow and efficiency of analysis. Cardiovascular magnetic resonance (CMR) is a noninvasive imaging modality for monitoring patients with CHD. CMR exam is based on multiple breath-hold 2-dimensional (2D) cine acquisitions that should be precisely prescribed and is expert and institution dependent. Moreover, 2D cine images have relatively thick slices, which does not allow for isotropic delineation of ventricular structures. Thus, development of an isotropic 3D cine acquisition and automatic segmentation method is worthwhile to make CMR workflow straightforward and efficient, as the present work aims to establish. Ninety-nine patients with many types of CHD were imaged using a non-angulated 3D cine CMR sequence covering the whole-heart and great vessels. Automatic supervised and semi-supervised deep-learning-based methods were developed for whole-heart segmentation of 3D cine images to separately delineate the cardiac structures, including both atria, both ventricles, aorta, pulmonary arteries, and superior and inferior vena cavae. The segmentation results derived from the two methods were compared with the manual segmentation in terms of Dice score, a degree of overlap agreement, and atrial and ventricular volume measurements. The semi-supervised method resulted in a better overlap agreement with the manual segmentation than the supervised method for all 8 structures (Dice score 83.23 ± 16.76% vs. 77.98 ± 19.64%; P-value ≤0.001). The mean difference error in atrial and ventricular volumetric measurements between manual segmentation and semi-supervised method was lower (bias ≤ 5.2 ml) than the supervised method (bias ≤ 10.1 ml). The proposed semi-supervised method is capable of cardiac segmentation and chamber volume quantification in a CHD population with wide anatomical variability. It accurately delineates the heart chambers and great vessels and can be used to accurately calculate ventricular and atrial volumes throughout the cardiac cycle. Such a segmentation method can reduce inter- and intra- observer variability and make CMR exams more standardized and efficient.

Clinical utility of repeat fetal echocardiography in congenital heart disease.

To investigate the utility of repeat fetal echocardiography (FE) following a diagnosis of structural congenital heart disease (CHD) on the initial FE. We evaluated how often changes in management and counseling occurred based on subsequent FE findings and sought to determine which types of CHD were more likely to have changes in management and/or counseling based on repeat FE. This was a retrospective review of all patients who presented to our center between January 2012 and January 2019 and who had more than one FE performed for structural CHD. We reviewed consultation notes to determine whether management or counseling had changed based on FE findings at follow-up visits. Management variables included a change in location or mode of delivery, plan for atrial septostomy, initiation of prostaglandin infusion, umbilical line placement and planned admission location (nursery vs neonatal intensive care unit). We defined a counseling change as any of the above changes in management as well as any meaningful refinements in the cardiac diagnosis that led to a change in the overall prognosis or future management. Initial diagnoses were grouped into anatomically/hemodynamically relevant subgroups. Fisher's exact test was used to assess the relationship between the initial diagnosis and changes in management. Post-hoc pairwise comparisons were performed using Dunnett's test. Between January 2012 and January 2019, 267 patients underwent 534 follow-up FE assessments performed for structural CHD. Management change based on repeat FE occurred in 41/267 (15.4%) cases. A change in management was associated with the diagnosis made at the initial visit (P < 0.001). The proportion of cases with a management change was highest among those with an initial diagnosis of pulmonary valve abnormality/non-critical pulmonary stenosis (4/11 (36.4%)), followed by balanced atrioventricular canal (AVC) defect (5/17 (29.4%)) and left ventricular outflow tract obstruction/aortic valve abnormality or coarctation/interrupted aortic arch (19/68 (27.9%)). No management change occurred in fetuses diagnosed with isolated ventricular septal defect (VSD), truncus arteriosus, pulmonary vein anomaly or isolated arch sidedness/branching abnormality. Compared to those with a VSD, management was significantly more likely to be changed in fetuses with a balanced AVC defect (P = 0.025) and left heart lesions (P = 0.002). Right heart lesions showed a trend towards an increased incidence of management change (P = 0.05). A counseling change based on repeat FE occurred in 108/267 (40.4%) cases. The proportion of cases with a counseling change was highest among those with an initial diagnosis of pulmonary valve abnormality/non-critical pulmonary stenosis (8/11 (72.7%)) and hypoplastic left heart syndrome/critical aortic stenosis (5/9 (55.6%)). The clinical utility of follow-up FE is associated with the type of CHD diagnosed. Follow-up FE led to changes in management in several types of CHD, most commonly in cases with an initial diagnosis of right and left outflow obstructive lesions and balanced AVC defect. When developing programmatic protocols for the frequency of FE assessments, the type of CHD should be a major determinant, but additional studies are required to reach a consensus on how often serial FE should be performed for each type of CHD. © 2023 International Society of Ultrasound in Obstetrics and Gynecology.

Semi-Automatic Measurement of Fetal Cardiac Axis in Fetuses with Congenital Heart Disease (CHD) with Fetal Intelligent Navigation Echocardiography (FINE).

Congenital heart disease (CHD) is one of the most common organ-specific birth defects and a major cause of infant morbidity and mortality. Despite ultrasound screening guidelines, the detection rate of CHD is limited. Fetal intelligent navigation echocardiography (FINE) has been introduced to extract reference planes and cardiac axis from cardiac spatiotemporal image correlation (STIC) volume datasets. This study analyses the cardiac axis in fetuses affected by CHD/thoracic masses (n = 545) compared to healthy fetuses (n = 1543) generated by FINE. After marking seven anatomical structures, the FINE software generated semi-automatically nine echocardiography standard planes and calculated the cardiac axis. Our study reveals that depending on the type of CHD, the cardiac axis varies. In approximately 86% (471 of 542 volumes) of our pathological cases, an abnormal cardiac axis (normal median = 40-45°) was detectable. Significant differences between the fetal axis of the normal heart versus CHD were detected in HLHS, pulmonary atresia, TOF (p-value < 0.0001), RAA, situs ambiguus (p-value = 0.0001-0.001) and absent pulmonary valve syndrome, DORV, thoracic masses (p-value = 0.001-0.01). This analysis confirms that in fetuses with CHD, the cardiac axis can significantly deviate from the normal range. FINE appears to be a valuable tool to identify cardiac defects.

We Have to Really Decide: The Childbearing Decisions of Women With Congenital Heart Disease.

Medical management and surgical improvement techniques permit persons with congenital heart conditions to live longer. Adults with congenital heart disease (CHD) have more childbearing options than previously available to them. However, there is an increased childbearing risk associated with certain types of CHD. Minimal investigation has been given to the childbearing decision-making experiences and adaptation of women with CHD. The aim of this study was to gain insight into the childbearing decision-making and adaptation experiences of women with CHD. Using a narrative inquiry approach, 17 adult women with CHD of any severity, of childbearing age, who had, within the last 5 years, made a decision regarding childbearing, were interviewed. In this study, we applied the key components of the Roy Adaptation Model to understand childbearing decision-making experiences and their adaptation. Data were analyzed using thematic analysis. Data analysis revealed 5 stages of childbearing decision making: (1) prologue: stimulus to consider childbearing; (2) exploring childbearing options; (3) considering childbearing options; (4) choosing to bear or not to bear a child; and (5) epilogue: adapting to the childbearing decision. Adaptation occurred in the areas of self-concept (ie, emotional adaptation), role function (ie, relational adaptation), and interdependence (ie, interactional adaptation). Childbearing decision making is a complex personal decision that is carefully and deliberately made. Women with CHD long for children and seek childbearing information from various resources and may experience grief regarding the inability to bear children. A greater understanding of childbearing decision making can be useful in addressing women's childbearing emotions and assist with adaptation to childbearing needs.

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.

Is maternal pre-pregnancy Body Mass Index associated with type of Congenital Heart Disease in offspring?

HIGHLIGHTS The most common CHD was atrial septal defect for acyanotic CHD and Tetralogy of Fallot for cyanotic CHD. There was no association between maternal pre-pregnancy BMI and type of CHD in offspring. ABSTRACT Objectives: This study aimed to determine the association between maternal pre-pregnancy BMI and type of congenital heart disease (CHD) in offspring. Materials and Methods: This retrospective cross-sectional study involved all mothers of children with CHD who visited Pediatric Outpatient Unit at Dr. Soetomo General Academic Hospital, Surabaya, Indonesia, from January to December 2019. The maternal data were obtained from the KIA's (Maternal and Child Health) book or through anamnesis by telephone, while the offspring’s data were collected from medical records. The data were analyzed using the Chi-Square test. Significance was determined at a 5% level (p &lt;0.05). Results: We studied 117 mothers of children with CHD. The most frequent maternal pre-pregnancy body mass index (BMI) was normal (BMI 17-23 kg/m2) accounting for 56.4% of the study population. The most common CHD was atrial septal defect (33.3%) among acyanotic patients and Tetralogy of Fallot (8.5%) among cyanotic patients. The Chi-Square test showed p=0.958 for the association between maternal pre-pregnancy BMI and type of CHD in offspring. Conclusion: There was no association between maternal pre-pregnancy BMI and type of CHD in offspring.

An 18-year evolution of congenital heart disease in China: An echocardiographic database-based study

ObjectiveData on the evolution of congenital heart disease (CHD) in China remain scarce. Based on a Chinese echocardiography database, we analyzed the observed rate (OR) and spectrum changes of CHD over the past 18 years with a focus on the congenital aortic valve malformation (CAVM) and Adult CHD (ACHD). Methods and resultsThe transthoracic echocardiographic data of 682,565 records from 2003 to 2020 were retrospectively reviewed at Fujian Medical University Union Hospital, China. A total of 37,200 CHD cases were recruited in this study. Over the three periods (from 2003 to 2008, 2009–2014, to 2015–2020), the OR of Total CHD decreased (106.72, 90.64, and 67.43 per 1000 cases, respectively); the proportion of Simple CHD to Total CHD increased (80.96%, 83.41%, and 87.97%, respectively), with a decrease in the proportion of Complex CHD (18.11%, 15.51%, and 10.42%, respectively) (p < 0.05 for all). The proportion of ACHD increased in most types of CHD [Total CHD: 25.79%, 27.84%, and 31.43%; CAVM: 69.02%, 73.42%, and 78.16%; CAVM with aortic stenosis (AS): 67.42%, 70.73%, and 79.25%; respectively, p < 0.05 for all], with a much higher proportion in both CAVM and CAVM with AS than in the other CHD types. The proportion of CHD patients receiving intervention increased over the designated periods. ConclusionsThis study depicts the longitudinal changes of CHD in the Chinese population with a single-center echocardiographic data, revealing an increased proportion of Simple CHD, ACHD (including CAVM and CAVM with AS), and a decreased OR of Total CHD and proportion of Complex CHD.

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.

From Illness to Resilience: Mediating Factors of Quality of Life in Patients with Congenital Heart Disease.

Congenital heart disease (CHD) is a leading cause of childhood morbidity, with an estimated prevalence of 0.8-1%. However, advances in diagnosis and treatment now allow 90% of childhood CHD patients to survive to adulthood, leading to increased interest in their quality of life (QoL). In this study, we examine the impact of clinical and psychosocial variables, including the number of surgical interventions (NSI), age at surgery, school achievement, and social support, as mediating factors of QoL in CHD patients. The study included 233 CHD patients (132 males) with an average age of 15.2 2.07 years, including 80 with cyanotic CHD and 153 with acyanotic CHD. The severity of illness ranged from mild to severe, with 30 patients having a severe illness, 119 having a moderate illness, and 84 having a mild illness. One-hundred-sixty-three patients underwent surgery. Clinical data on diagnosis, the severity of CHD, the type of CHD, and surgical interventions were collected from patient records, and a semi-structured interview was conducted to explore the relationship between CHD diagnosis and various aspects of life. QoL was assessed using the Abbreviated World Health Organization Quality of Life questionnaire (WHOQOL-Bref) questionnaire. Ten mediation models were analyzed, each with three hypotheses (paths). In all models the first hypothesis was supported. Analyses of the second and third hypotheses revealed three feasible models of mediation through the effect of NSI on QoL in CHD patients. Our findings indicate that patients with more severe and cyanotic CHD generally require more surgical interventions, which may increase the risk of negative outcomes and affect patients' perception of QoL. These results have important implications for healthcare providers and psychologists who work with childhood CHD patients.

Outcomes of out-of-hospital cardiac arrest in adult congenital heart disease: a Danish nationwide study.

The risk, characteristics, and outcome of out-of-hospital cardiac arrest (OHCA) in patients with congenital heart disease (CHD) remain scarcely investigated. An epidemiological registry-based study was conducted. Using time-dependent Cox regression models fitted with a nested case-control design, hazard ratios (HRs) with 95% confidence intervals of OHCA of presumed cardiac cause (2001-19) associated with simple, moderate, and severe CHD were calculated. Moreover, using multiple logistic regression, we investigated the association between pre-hospital OHCA characteristics and 30-day survival and compared 30-day survival in OHCA patients with and without CHD. Overall, 43 967 cases (105 with simple, 144 with moderate, and 53 with severe CHD) and 219 772 controls (median age 72 years, 68.2% male) were identified. Any type of CHD was found to be associated with higher rates of OHCA compared with the background population [simple CHD: HR 1.37 (1.08-1.70); moderate CHD: HR 1.64 (1.36-1.99); and severe CHD: HR 4.36 (3.01-6.30)]. Pre-hospital cardiopulmonary resuscitation and defibrillation were both associated with improved 30-day survival in patients with CHD, regardless of CHD severity. Among patients with OHCA, simple, moderate, and severe CHD had a similar likelihood of 30-day survival compared with no CHD [odds ratio 0.95 (0.53-1.69), 0.70 (0.43-1.14), and 0.68 (0.33-1.57), respectively]. A higher risk of OHCA was found throughout the spectrum of CHD. Patients with and without CHD showed the same 30-day survival, which relies on the pre-hospital chain of survival, namely cardiopulmonary resuscitation and defibrillation.

Pregnancy loss in major fetal congenital heart disease: incidence, risk factors and timing.

Fetuses with congenital heart disease (CHD) are at increased risk of pregnancy loss compared with the general population. We aimed to assess the incidence, timing and risk factors of pregnancy loss in cases with major fetal CHD, overall and according to cardiac diagnosis. This was a retrospective, population-level cohort study of fetuses and infants diagnosed with major CHD between 1997 and 2018 identified by the Utah Birth Defect Network (UBDN), excluding cases with termination of pregnancy and minor cardiovascular diagnoses (e.g. isolated aortic/pulmonary pathology and isolated septal defects). The incidence and timing of pregnancy loss were recorded, overall and according to CHD diagnosis, with further stratification based on presence of isolated CHD vs additional fetal diagnosis (genetic diagnosis and/or extracardiac malformation). Adjusted risk of pregnancy loss was calculated and risk factors were assessed using multivariable models for the overall cohort and prenatal diagnosis subgroup. Of 9351 UBDN cases with a cardiovascular code, 3251 cases with major CHD were identified, resulting in a study cohort of 3120 following exclusion of cases with pregnancy termination (n = 131). There were 2956 (94.7%) live births and 164 (5.3%) cases of pregnancy loss, which occurred at a median gestational age of 27.3 weeks. Of study cases, 1848 (59.2%) had isolated CHD and 1272 (40.8%) had an additional fetal diagnosis, including 736 (57.9%) with a genetic diagnosis and 536 (42.1%) with an extracardiac malformation. The observed incidence of pregnancy loss was highest in the presence of mitral stenosis (< 13.5%), hypoplastic left heart syndrome (HLHS) (10.7%), double-outlet right ventricle with normally related great vessels or not otherwise specified (10.5%) and Ebstein's anomaly (9.9%). The adjusted risk of pregnancy loss was 5.3% (95% CI, 3.7-7.6%) in the overall CHD population and 1.4% (95% CI, 0.9-2.3%) in cases with isolated CHD (adjusted risk ratio, 9.0 (95% CI, 6.0-13.0) and 2.0 (95% CI, 1.0-6.0), respectively, based on the general population risk of 0.6%). On multivariable analysis, variables associated with pregnancy loss in the overall CHD population included female fetal sex (adjusted odds ratio (aOR), 1.6 (95% CI, 1.1-2.3)), Hispanic ethnicity (aOR, 1.6 (95% CI, 1.0-2.5)), hydrops (aOR, 6.7 (95% CI, 4.3-10.5)) and additional fetal diagnosis (aOR, 6.3 (95% CI, 4.1-10)). On multivariable analysis of the prenatal diagnosis subgroup, years of maternal education (aOR, 1.2 (95% CI, 1.0-1.4)), presence of an additional fetal diagnosis (aOR, 2.7 (95% CI, 1.4-5.6)), atrioventricular valve regurgitation ≥ moderate (aOR, 3.6 (95% CI, 1.3-8.8)) and ventricular dysfunction (aOR, 3.8 (95% CI, 1.2-11.1)) were associated with pregnancy loss. Diagnostic groups associated with pregnancy loss were HLHS and variants (aOR, 3.0 (95% CI, 1.7-5.3)), other single ventricles (aOR, 2.4 (95% CI, 1.1-4.9)) and other (aOR, 0.1 (95% CI, 0-0.97)). Time-to-pregnancy-loss analysis demonstrated a steeper survival curve for cases with an additional fetal diagnosis, indicating a higher rate of pregnancy loss compared to cases with isolated CHD (P < 0.0001). The risk of pregnancy loss is higher in cases with major fetal CHD compared with the general population and varies according to CHD type and presence of additional fetal diagnoses. Improved understanding of the incidence, risk factors and timing of pregnancy loss in CHD cases should inform patient counseling, antenatal surveillance and delivery planning. © 2023 International Society of Ultrasound in Obstetrics and Gynecology.

Epidemiological and clinical aspects of congenital heart disease in Fallujah Maternity and Children Hospital, Iraq

Objective: This study aimed to analyze the descriptive characteristics of children diagnosed with Congenital Heart Disease (CHD) at Fallujah Maternity and Children Hospital (FMCH).&#x0D; Methods: The prospective study included 1,025 pediatric patients born between 1st December 2018 and 30th September 2022, admitted to the newborns and children's wards, and diagnosed with CHD. Data were collected through interviews with patients' guardians using a registration and questionnaire form, capturing variables related to the child (e.g., name, sex, birth weight, type of CHD) and the mother (e.g., age, occupation, consanguinity, previous CHD births, chronic diseases during pregnancy). Residence (rural or urban) was also recorded.&#x0D; Results: Among the CHD cases, Atrial Septal Defect (ASD) was the most common (72%), followed by Ventricular Septal Defect (VSD) (25%) and Patent Ductus Arteriosus (PDA) (21%). Most diagnoses occurred in children aged 1-28 days. The male-to-female ratio was 1.2:1, and 0.2% had an ambiguous gender. About 58% of children weighed ≥ 2.5kg at birth, and 20% had a family history of CHDs.&#x0D; Conclusion: The increasing prevalence of congenital heart diseases in Fallujah requires preventive strategies targeting modifiable risk factors, better monitoring of high-risk pregnancies, and increased awareness of genetic counseling for families with congenital anomalies. The cardiology department should receive more attention and resources to improve diagnostic and therapeutic capabilities. Further research is needed to understand the reasons behind the recent rise in CHD cases in newborns.

ISUOG Practice Guidelines (updated): fetal cardiac screening.

ISUOG Practice Guidelines (updated): fetal cardiac screening.