Different Types Of Congenital Heart Disease Research Articles

Frontal Lobe Growth impairment in fetuses with congenital heart disease using Ultrasound parameters; A Comparative Cross-sectional study

Introduction and the Αim: 2D and 3D biometry measures can record the increased development of the fetal brain in the later part of gestation. The objective of this study is to determine whether or not the frontal brain regions of fetuses with congenital heart disease (CHD) are smaller than those of healthy controls. Patients and Μethods: The fetal Medicine unit of the Ain Shams University Maternity Hospital carried out a comparative cross-sectional study, including 140 normal fetuses and 140 fetuses with isolated CHD evaluated between 20 and 39 gestational weeks at our fetal medicine unit in the period November 2021–September 2023 Results: Our results showed the occipito-frontal diameter (OFD) and frontal antero-posterior diameter (FAPD) utilising ultrasonography. FAPD Mean±SD: 26.53 ± 10.44, OFD Mean±SD: 89.14 ± 28.34, and FAPD/OFD Mean±SD: 0.29 ± 0.05. The OFD Mean±SD was found to be 82.18 ± 18.42, the FAPD Mean±SD to be 31.68±6.58, and the FAPD/OFD Mean±SD to be 38.70±2.15. Our results showed that 0.7% of patients exhibited anomalies related to the central nervous system, such as dilated posterior fossa, vermis = 25 mm, and aberrant posterior cranial fossa. The percentage of those with a history of CHD was 3.9%.The history of CHD showed a statistically significant difference between the research groups. When examining comorbidities, surgical history, and the relationship between the other parameters and FAPD, OFD, and FAPD/OFD, there was no statistically significant difference between the groups under investigation, except for CNS abnormalities and gestational age. Conclusion, compared to normal fetuses, fetuses with CHD had shorter FAPDs and a lower FAPD/OFD ratio. Despite the fact that different types of CHD affect hemodynamics differently, there appears to be a common deficiency in the development of the frontal cortex.

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.

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.

Assessment of fetal heart aortic and pulmonary valve annulus area by three-dimensional ultrasonography: reference curves and applicability in congenital heart diseases.

To determine reference curves for fetal aortic and pulmonary valve annulus area by three-dimensional ultrasonography using the spatio-temporal image correlation (STIC) in the rendering mode, and to ascertain its applicability in congenital heart disease (CHD). We performed a retrospective cross-sectional study of 328 normal fetuses and 42 fetuses with CHD between 20 and 33weeks 6days of gestation. The outflow plane view of the great vessels was used to measure the areas of the valvar annuli, and the measurements were performed in systole. A linear regression model adjusted according to the determination coefficient (R2) was utilized to construct the reference intervals. The concordance correlation coefficient (CCC) was used to calculate the reproducibility of the mitral and tricuspid valve areas. The mean±standard deviation (SD) of the aortic and pulmonary valve annulus areas ranged from 6.6±1.2 to 32.9±1.1mm2 and 10.7±1.3 to 40.3±1.2mm2, respectively. We observed a linear relationship and strong positive correlation between the area of the aortic and pulmonary valve annuli with r=0.97 and 0.96, respectively. Good intra (CCC=0.99) and interobserver agreement (CCC=0.98) was observed for the measurement of the aortic valve annulus area. A good intra (CCC=0.99) and interobserver (CCC=0.97) agreement was also observed for the measurement of the pulmonary valve annulus area. The mean±SD of the difference of the areas of the aortic and pulmonary valve annuli between the normal fetuses and those with CHD were-1.801±1.429mm2 (p=0.208) and-1.033±1.467mm2 (p<0.0001), respectively. The reference curves for the areas of the aortic and pulmonary valve annuli of fetal hearts were determined, and showed good inter and intraobserver reproducibility. The constructed reference curves showed applicability in different types of CHD.

Optical coherence tomography of the pulmonary arteries in children with congenital heart diseases: A systematic review.

Optical coherence tomography (OCT) is a high-resolution intravascular imaging tool and has shown promise for providing real-time quantitative and qualitative descriptions of pulmonary vascular structures in vivo in adult pulmonary hypertension (PH), while not popular in pediatric patients with congenital heart diseases (CHD). The aim of this review is to summarize all the available evidence on the use of OCT for imaging pulmonary vascular remodeling in pediatric patients. We conducted the systematic literature resources (Cochran Library database, Medline via PubMed, EMBASE, and Web of Knowledge) from January 2010 to December 2021 and the search terms were "PH", "child", "children", "pediatric", "OCT", "CHD", "pulmonary vessels", "pulmonary artery wall". Studies in which OCT was used to image the pulmonary vessels in pediatric patients with CHD were considered for inclusion. Five studies met the inclusion criteria. These five papers discussed the study of OCT in the pulmonary vasculature of different types of CHD, including common simple CHD, complex cyanotic CHD, and Williams-Beuren syndrome. In biventricular anatomy, pulmonary vascular remodeling was primarily reflected by pulmonary intima thickening from two-dimensional OCT. In single-ventricle anatomy, due to the state of hypoxia, the morphology of pulmonary vessels was indirectly reflected by the number and shape of nourishing vessels from three-dimensional OCT. OCT may be an adequate imaging procedure for the demonstration of pulmonary vascular structures and provide additional information in pediatric patients.

Circulating Reactive Oxygen Species in Adults with Congenital Heart Disease.

Oxidative stress is an important pathophysiological mechanism in the development of numerous cardiovascular disorders, but few studies have examined the levels of oxidative stress in adults with congenital heart disease (CHD). The objective of this study was to investigate oxidative stress levels in adults with CHD and the association with inflammation, exercise capacity and endothelial function. To this end, 36 adults with different types of CHD and 36 age- and gender-matched healthy controls were enrolled. Blood cell counts, hs-CRP, NT-proBNP, fasting glucose, cholesterol levels, iron saturation and folic acid concentrations were determined in venous blood samples. Levels of superoxide anion radical in whole blood were determined using electron paramagnetic resonance spectroscopy in combination with the spin probe CMH. Physical activity was assessed with the IPAQ-SF questionnaire. Vascular function assessment (EndoPAT) and cardiopulmonary exercise testing were performed in the patient group. Superoxide anion radical levels were not statistically significantly different between adults with CHD and the matched controls. Moreover, oxidative stress did not correlate with inflammation, or with endothelial function or cardiorespiratory fitness in CHD; however, a significant negative correlation with iron saturation was observed. Overall, whole blood superoxide anion radical levels in adults with CHD were not elevated, but iron levels seem to play a more important role in oxidative stress mechanisms in CHD than in healthy controls. More research will be needed to improve our understanding of the underlying pathophysiology of CHD.

Abstract 11972: Economic Burden of Congenital Heart Surgery in China: Results From the National Center for Cardiovascular Diseases

Introduction: According to Reports on the Prevention and Control of Birth Defects in China, the total lifelong economic burden of new congenital heart diseases (CHD) cases was more than 12.6 billion Chinese Yuan. We aim at exploring the economic burden driven by congenital heart surgery and filling the gap in the health economics in China. Methods: We used data from CDCHS database (Registry No. ChiCTR1800014577) to prospectively analyze the economic burden of congenital heart surgery. The total expenditures were divided into eleven subjects and classified by STAT category, year, and different types of CHD. Stepwise multivariable linear regression model was utilized to analyze the contributing factor to the total expenditure. National economic data was accessed to better describe the burden. Results: A total of 6568 hospitalizations were enrolled from 2018 to 2020. The median of total expenditure of all enrolled patients during hospitalization was 63,129.14 (≈9,910.52 USD), and significant cost-related difference was defined among the five STAT categories (P&lt;0.001) with the lowest in STAT-1 (55,720.43, ≈8,747.46 USD) and highest in STAT-5 (190,587.01, ≈29,919.94 USD). The median total costs during the 3-year 2018 to 2020 period were 57,248.72 (≈8,987.38 USD), 63,423.45 (≈9,956.74 USD) and 67,867.37 (≈10,654.39 USD), respectively. Multivariable linear regression model showed that STAT category, complication, emergent operation and delay sternal closure were the major contributing factor to the total expenditure. Conclusions: For the first time, the economic burden of congenital heart surgery in China is delineated not only in terms of total expenditure but also the detail components. Also, the current healthcare policies in China were analyzed, and combined with the expenditure information, this analysis provides substantial information about specific factors driving to the economic burden with the purpose of aiding policy adjustments.

The risk spectrum of infective endocarditis among patients with congenital heart diseases

Abstract Background Patients with congenital heart disease (CHD) are considered at lifelong high risk of infective endocarditis (IE). The risk of IE presumably differs among different types of CHD, but little knowledge exists on the area. Purpose This study set out to investigate the long-term incidence of IE among CHD-patients and associated risk factors. Methods In this observational cohort study, all patients with CHD born in 1977–2018 were identified using Danish nationwide registries and followed from date of birth until occurrence of first-time IE, emigration, death, or end of study (December 31, 2018). The comparative risk of IE among patients with CHD versus age- and sex-matched controls from the background population was assessed. Moreover, the risk of IE was stratified according to type of CHD and risk factors associated with IE among patients with CHD were examined using Cox regression analysis including sex and relevant time-varying coefficients (i.e., cyanotic yet surgically untreated CHD, CHD repaired with prostheses, diabetes mellitus, chronic kidney disease, and presence of cardiac implantable devices). Results A total of 23,464 patients with CHD (50.0% men) were identified and matched with 93,856 controls from the background population. During a median follow-up of 17.7 years, 217 (0.9%) patients with CHD and 4 (0.0%) controls developed IE, corresponding to incidence rates of 5.2 (95% CI: 4.6–6.0) and 0.02 (95% CI: 0.01–0.1) per 10,000 person-years, respectively. The associated incidence of IE was greatest among patients with tetralogy of Fallot, malformations of the heart chambers (including transposition of the great arteries and truncus arteriosus), atrioventricular septal defects, and heart valve defects (Figure 1). Factors associated with IE among patients with CHD included male sex (HR 1.7 [95% CI, 1.3–2.2]), cyanotic yet surgically untreated CHD (HR 5.2 [95% CI, 3.5–7.7]), cardiac prostheses (HR 5.2 [95% CI, 3.6–7.5]), chronic kidney disease (HR 3.1 [95% CI, 1.3–7.6]), and presence of cardiac implantable electronic devices (HR 3.1 [95% CI, 1.5–6.2]). Conclusions Patients with CHD, especially those with tetralogy of Fallot and malformations of the heart chambers, have a substantially higher incidence of IE than the background population. With an increasing longevity of these patients, relevant guidelines concerning preventive measures are decisive to improve morbidity, mortality, and the quality of life among this growing group of patients. Funding Acknowledgement Type of funding sources: None.

Efficacy and Safety of Minimally Invasive Transcatheter Closure of Congenital Heart Disease under the Guidance of Transesophageal Ultrasound: A Randomized Controlled Trial.

Objective To investigate the efficacy of minimally invasive transcatheter closure of congenital heart disease (CHD) under the guidance of transesophageal ultrasound. Methods A total of 100 patients with CHD treated in our hospital from February 2019 to April 2020 were enrolled in the group. The patients were randomly divided into control group and research group. The control group received minimally invasive transcatheter closure under the guidance of X-ray, and the research group received minimally invasive transcatheter closure under the guidance of transesophageal ultrasound. The operative results, the intraoperative- and postoperative-related indexes, and the incidence of early postoperative complications and follow-up results were compared. Results First of all, we compared the results of the two groups: 48 cases of success, 2 cases of difficulty in the research group, 35 cases of success, 11 cases of difficulty, and 4 cases of failure in the control group. The success rate in the research group was higher than that in the control group (P < 0.05). Secondly, we compare the relevant indicators in the process of operation. The operation time, cardiopulmonary bypass time, upper and lower cavity obstruction time, and blood transfusion volume in the research group were lower than those in the control group (P < 0.05). In terms of postoperative-related indexes, the ventilator-assisted time, 24 h postoperative drainage, ICU time, and postoperative hospital stay in the research group were all lower than those in the control group (P < 0.05). The incidence of early postoperative complications in the research group was significantly lower than that in the control group such as secondary pleural hemostasis, pulmonary infection, pleural effusion, subcutaneous emphysema, poor incision healing, phrenic nerve loss, and right lower limb numbness (P < 0.05). All patients were followed up for 6 months, and the cardiac function of both groups returned to normal. There was no significant difference in the incidence of postoperative residual shunt and new tricuspid regurgitation. There was no significant difference in the data (P > 0.05). Considering abnormal ECG events, the incidence of abnormal ECG events (complete right bundle branch block, incomplete right bundle branch block, second- and third-degree block, left anterior branch block) in the research group was significantly lower than that in the control group (P < 0.05). Conclusion Minimally invasive transcatheter closure of CHD under the guidance of transesophageal ultrasound has the advantages of less trauma, less blood loss, short hospital stay, simple operation, less postoperative complications, and remarkable therapeutic effect. Minimally invasive transcatheter closure under the guidance of transesophageal ultrasound has the advantage of adapting to a wide range of syndromes and can be used for the closure of CHD in children. According to different types of CHD, registering the corresponding occlusive pathway can improve the success rate of operation. Through postoperative reexamination and regular follow-up, it is proved that minimally invasive transcatheter closure under the guidance of transesophageal ultrasound is safe, effective, and feasible.

High-risk phenotypes of genetic disease in a Neonatal Intensive Care Unit population.

High-risk phenotypes of genetic disease in a Neonatal Intensive Care Unit population.

Abnormal Extracardiac Development in Fetuses With Congenital Heart Disease

BackgroundKnowledge about extracardiac anomalies (ECA) in fetal congenital heart disease (CHD) can improve our understanding of the developmental origins of various outcomes in these infants. The prevalence and spectrum of ECA, including structural brain anomalies (SBA), on magnetic resonance imaging (MRI) in fetuses with different types of CHD and at different gestational ages, is unknown. ObjectivesThe purpose of this study was to evaluate ECA rates and types on MRI in fetuses with different types of CHD and across gestation. MethodsA total of 429 consecutive fetuses with CHD and MRI between 17 and 38 gestational weeks were evaluated. ECA and SBA rates were assessed for each type of CHD and classified by gestational age (<25 or ≥25 weeks) at MRI. ResultsOf all 429 fetuses with CHD, 243 (56.6%) had ECA on MRI, and 109 (25.4%) had SBA. Among the 191 fetuses with normal genetic testing results, the ECA rate was 54.5% and the SBA rate 19.4%. Besides SBA, extrafetal (21.2%) and urogenital anomalies (10.7%) were the most prevalent ECA on MRI in all types of CHD. Predominant SBA were anomalies of hindbrain-midbrain (11.0% of all CHD), dorsal prosencephalon (10.0%) development, and abnormal cerebrospinal fluid spaces (10.5%). There was no difference in the prevalence or pattern of ECA between early (<25 weeks; 45.7%) and late (≥25 weeks; 54.3%) fetal MRI. ConclusionsECA and SBA rates on fetal MRI are high across all types of CHD studied, and ECA as well as SBA are already present from midgestation onward.

Abstract 11567: Congenital Heart Disease Classification Improves SRTR Risk Estimation of Waitlist and Early Post-Transplant Mortality for Pediatric Heart Transplantation

Introduction: Risk estimation for waitlist and 1-year post-transplant mortality utilized by SRTR for pediatric heart transplantation dichotomizes patients based upon presence or absence of congenital heart disease (CHD). Hypothesis: Current SRTR risk estimations can be improved by adding further detail in categorization of CHD. Methods: A retrospective analysis of the Pediatric Heart Transplant Society (PHTS) database of pediatric (&lt;18yo) heart transplant listings from 2010 to 2020 was performed, excluding patients listed for retransplantation. Cox hazard models were developed for waitlist mortality during the first year following listing (WaitDeath) and one year post-transplant mortality (TxDeath). The initial model was constructed using covariates included in the current SRTR model and a second model was developed by adding CHD classifications (SRTR+) including: single ventricle vs. biventricular heart disease, ventricular morphology, and surgical interventions. Results: The WaitDeath model included 5,790 patients in which 722 of which died. In the SRTR WaitDeath model, CHD had a HR of 1.8 (95%CI of 1.4-2.3) compared to cardiomyopathy patients. Where as in SRTR+ model, patients with different types of CHD had HR’s that ranged from 1.5 to 2.1 (Table 1). The TxDeath model included 4,185 patients in which 304 which died. (Table 1) In the SRTR model of TxDeath, patients with CHD had a HR of 3.9 (2.9-5.1) compared to cardiomyopathy patients, whereas in the SRTR+ model patients with different types of CHD had HRs that ranged from 2.4-5.3 (Table 1). Conclusions: Inclusion of granular CHD data may improve waitlist and post-transplant mortality risk estimation in pediatric heart transplant patients, allowing for more refinement in allocation policies and understanding of difference in post-transplant outcomes across sites. This data supports further refinement of current SRTR risk adjustment models.

Abstract 11616: Seizures in Infants with Congenital Heart Disease

Background: Infants with congenital heart disease (CHD) may have an increased risk to develop seizures during hospitalization. Objectives: to examine the association of CHD with non-epileptic, non-traumatic seizures in hospitalized infants up to one year of age. Methods: We conducted a retrospective analysis of the U.S. National Inpatient Sample dataset (2015-2018). We used the international classification of disease-10 th version to identify infants with different types of CHD. We included infants up to one year of age. We excluded infants &lt;35 week gestational age, infants with central nervous system or with major congenital anomalies, common trisomy or genetic disorders. We used chi square testing to examine the association of subtypes of CHD with seizures excluding post traumatic and epileptic seizures. We calculated mortality in infants with CHD who developed seizures versus those who did not have seizures. Results: There was 12,023,781 infants in the weighted sample who met our inclusion criteria. Of them, 48.7% were females, 46.7% were Caucasians, 195,685 (1.63%) were identified with a CHD and 20,565 (0.17%) suffered at least one episode of seizure during hospitalization. Seizures occurred in 4225 (2.2%) of infants with any type of CHD compared to 16,340 (0.14%) in infants with no CHD, odds ratios for seizures is 15.6 (CI: 15.2-16.5, p &lt;0.001). The association of seizures with subtypes of CHD is shown in table-1. Among infants with CHD, 2205 infants expired during hospitalization. Mortality was higher in infants with CHD who suffered seizures (8.5%) vs. those who did not have seizures (1.0%), OR 9.6 (8.5-10.8, p &lt;0.001). Conclusion: Infants with CHD have a higher chance to experience seizure episodes during their hospitalization and those who have seizures have higher chances of mortality during hospitalization. Further analysis is required to understand the pathological correlation between seizures and CHD and its impact on long term developmental outcomes.

In silico analysis of GATA4 variants demonstrates main contribution to congenital heart disease.

Introduction: Congenital heart disease (CHD) is the most common congenital abnormality and the main cause of infant mortality worldwide. Some of the mutations that occur in the GATA4 gene region may result in different types of CHD. Here, we report our in silico analysis of gene variants to determine the effects of the GATA4 gene on the development of CHD. Methods: Online 1000 Genomes Project, ExAC, gnomAD, GO-ESP, TOPMed, Iranome, GME, ClinVar, and HGMD databases were drawn upon to collect information on all the reported GATA4 variations.The functional importance of the genetic variants was assessed by using SIFT, MutationTaster, CADD,PolyPhen-2, PROVEAN, and GERP prediction tools. Thereafter, network analysis of the GATA4protein via STRING, normal/mutant protein structure prediction via HOPE and I-TASSER, and phylogenetic assessment of the GATA4 sequence alignment via ClustalW were performed. Results: The most frequent variant was c.874T>C (45.58%), which was reported in Germany.Ventricular septal defect was the most frequent type of CHD. Out of all the reported variants of GATA4,38 variants were pathogenic. A high level of pathogenicity was shown for p.Gly221Arg (CADD score=31), which was further analyzed. Conclusion: The GATA4 gene plays a significant role in CHD; we, therefore, suggest that it be accorded priority in CHD genetic screening.

Pattern of paediatric congenital heart diseases.

Objective: To find out pattern along with age and gender wise distribution of congenital heart disease (CHD). Study Design: Cross Sectional study. Setting: Department of Pediatrics, Sughra Shafi Medical Complex, Narowal. Period: January 2019 to February 2020. Material &amp; Methods: During the study period, a total of 151 children aged between 1 month to 15 years as confirmed case of CHD according to echocardiography were enrolled for this study. Gender, age categories, area of residence and types of CHDs were represented as frequency and percentages. Patients with different types of CHDs were distributed between both gender and different age categories. SPSS version 26.0 was used to handle and analyze all study related data. Results: During the study period, a total of 151 confirmed cases of CHD were enrolled. There were 82 (54.3%) male and 69 (45.7%) female representing a male to female ratio of 1.2:1. Most of the cases were aged less than 1 year. There were 118 (78.1%) children below 1 year of age while 22 (14.6%) between 1 to 5 years of age and 11 (7.3%) above 5 years of age. Ventricular septal defect (VSD) and ASD were noted to be the most frequent types of acyanotic heart lesions in 41 (27.2%) and 29 (19.2%) cases respectively whereas TOF was the most frequent type of cyanotic heart lesion in 23 (15.2%) cases. Conclusion: VSD followed by ASD and TOF were noted to be the most frequent types of CHD.

Prognostic Value of CTA-Derived Left Ventricular Mass in Neonates with Congenital Heart Disease.

For therapeutic decisions regarding uni- or biventricular surgical repair in congenital heart disease (CHD), left ventricular mass (LVM) is an important factor. The aim of this retrospective study was to determine the LVM of infants with CHD in thoracic computed tomography angiographies (CTAs) and to evaluate its usefulness as a prognostic parameter, with special attention paid to hypoplastic left heart (HLH) patients. Manual segmentation of the left ventricular endo- and epicardial volumes was performed in CTAs of 132 infants. LVMs were determined from these volumes and normalized to body surface area. LVMs of patients with different types of CHD were compared to each other using analyses of variances (ANOVA). An LVM cutoff for discrimination between uni- and biventricular repair was determined using receiver operating characteristics. Survival rates were calculated using Kaplan–Meier statistics. Patients with a clinical diagnosis of an HLH had significantly lower mean LVM (21.88 g/m2) compared to patients without applicable disease (50.22 g/m2; p < 0.0001) and compared to other CHDs, including persistent truncus arteriosus, left ventricular outflow tract obstruction, transposition of the great arteries, pulmonary artery stenosis or atresia, and double-outlet right ventricle (all, p < 0.05). The LVM cutoff for uni- vs. biventricular surgery was 33.9 g/m2 (sensitivity: 82.3%; specificity: 73.7%; PPV: 94.9%). In a subanalysis of HLH patients, a sensitivity of 50.0%, specificity of 100%, PPV of 100%, and NPV of 83.3% was determined. Patient survival was not significantly different between the surgical approaches or between patients with LVM above or below the cutoff. LVM can be measured in chest CTA of newborns with CHD and can be used as a prognostic factor.

Patterns of Congenital Heart Disease Among Patients Attending in Specialized Hospitals

Background: Incidence of congenital heart disease is serious and common conditions that have significant impact on morbidity, mortality and healthcare costs in children and adults. The objective of this paper is socio-demographic characteristics, pattern, risk factors and geographical distribution of Congenital Heart Disease (CHD).&#x0D; Methods: This cross-sectional study was conducted throughout the year of 2013 in Dhaka city of Bangladesh. Among the children with confirmed diagnosis of CHD within the age ranging from birth to 18 years, admitted and treated at National Institute of Cardiovascular Disease (NICVD) and National Heart Foundation and Research Center (NHFRC) were included.&#x0D; Result: Out of 168 patients, 64% were living in rural area, 36% were living in urban. The study revealed that 58.9% of male were predominant whereas female was 41.1%. The mean (±SD) age of patients was 4.87(±2.83) years. 71.4% of the children were within 1 to 5 years of age. 36.9% CHD was diagnosed at the age of 1 - 5 years. Finding of the study revealed that there were different types of CHD namely Ventricular Septal Defect (VSD) 35.15%, Atrial Septal Defect (ASD) 27.4%, Patent Ductus Arteriosus (PDA) 13.7%, Coarctation of the aorta 4.8% and Tetralogy of Fallots 19%. In urban area, 37.4% patients had VSD while in rural area, 31.1% patients had ASD. This variation of CHD in relation to living place of the patients was statistically important (χ2 4 = 11.62, p=0.024). In female patients, VSD is more than 58%, however in male patients, ASD is more than 36.4%. This variation of CHD according to sex of the patients was statistically vital (χ2 4 = 27.85, p=0.001). Among the mothers, 39.8% utilized Ant-natal Care (ANC) had VSD while 24.0% having VSD did not utilized ANC. This variation was statistically significant (χ2 4 = 8.235, p=0.04). Among the patients, 41.7% were aged 1-5 years who had VSD while 50.0% were aged 6-10 years who had ASD. This variation was statistically important as it showed (χ2 8 = 43.601, p=0.000)&#x0D; Conclusions: As the most common type of congenital heart diseases are VSD and ASD, the findings of this study will contribute in early detection and proper management of CHD in the contexts to others and thus save lives.&#x0D; JOPSOM 2020; 39(2): 40-44

Somatic growth in children with congenital heart disease at 10 years of age: Risk factors and longitudinal growth

BackgroundChildren with congenital heart disease (CHD) are at risk of impaired growth. AimsTo describe height, weight, head circumference (HC), and body mass index (BMI) at 10 years and identify risk factors for altered longitudinal growth in children with CHD. Study designGrowth parameters were evaluated from birth until 10 years using z-scores. The impact of cardiac and noncardiac factors on longitudinal growth was investigated. SubjectsA total of 135 children with different types of CHD who underwent cardiopulmonary bypass surgery, no genetic disorder. Outcome measuresHead circumference, weight, height and BMI. ResultsAt 10 years, z-scores for height and BMI did not differ from the Swiss population (P > 0.1). Z-scores for weight and HC were significantly below the norm (−0.38 and − 0.71, P < 0.01). From 1 to 10 years, all growth parameters except BMI increased significantly (P ≤ 0.001, BMI: P = 0.14). Lower gestational age and longer length of hospitalization were associated with either impaired head circumference or length at 10 years, while lower socioeconomic status was associated with higher BMI and weight at 10 years (all P < 0.05). ConclusionDespite partial catch-up, somatic growth remains impaired in children with CHD with weight and HC below the norm at 10 years. The only cardiac factor associated with impaired longitudinal growth was duration of hospital stay. Furthermore, lower socioeconomic background may pose a risk of overweight at older age. Close monitoring of growth parameters and parental counselling in all CHD children is advisable beyond early childhood to ensure optimal somatic growth.

Reduced Parasympathetic Activity in Patients With Different Types of Congenital Heart Disease and Associations to Exercise Capacity

Current research indicates an imbalance in the autonomic nervous system (ANS) pathway activities in patients with congenital heart disease (CHD). The heart rate variability (HRV) is a measure to quantify activities of the parasympathetic and sympathetic branches. This study evaluated the parasympathetic activity by means of HRV in patients with CHD, regarding diagnostic subgroups and CHD severity, and the association to exercise capacity. From July 2016 to August 2018, a total of 222 patients with different types of CHD (28.4 ± 10.1 yr; 44% female) received breathing-controlled HRV measurement in the supine position. Based on 130 adjacent heartbeats, the root mean square of successive differences (RMSSD), a parasympathetic activity surrogate was estimated and log-transformed. Additionally, all patients underwent a cardiopulmonary exercise test. For comparison, a control group (CG) of 57 (29.0 ± 7.1 yr; 61% female) healthy volunteers was recruited. Patients with CHD exhibited reduced parasympathetic activity in comparison to the CG (lnRMSSD CHD: 3.55 ± 0.57 ms vs CG: 3.93 ± 0.55 ms; P < .001), with the lowest parasympathetic activity in patients with Fontan circulation (3.07 ± 0.54 ms; P < .001). Complex CHD (3.40 ± 0.54 ms) had worse values compared with patients with simple (3.87 ± 0.55 ms; P < .001) and moderate severity (3.74 ± 0.54 ms; P < .001). Better parasympathetic activity in CHD patients was associated with increased exercise capacity (r = 0.322; P < .001). Impaired parasympathetic activity suggests limited function of the ANS in patients with CHD. Further studies should focus on the association of exercise capacity and ANS to possibly improve parasympathetic activity and functional outcomes.

Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry.

Introduction: Pulmonary hypertension (PH) is a common complication in patients with congenital heart disease (CHD), aggravating the natural, post-operative, or post-interventional course of the underlying anomaly. The various CHDs differ substantially in characteristics, functionality, and clinical outcomes among each other and compared with other diseases with pulmonary hypertension. Objective: To describe current management strategies and outcomes for adults with PH in relation to different types of CHD based on real-world data. Methods and results: COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) is a prospective, international PH registry comprising, at the time of data analysis, >8200 patients with various forms of PH. Here, we analyzed a subgroup of 680 patients with PH due to CHD, who were included between 2007 and 2018 in 49 specialized centers for PH and/or CHD located in 11 European countries. At enrollment, the patients’ median age was 44 years (67% female), and patients had either pre-tricuspid shunts, post-tricuspid shunts, complex CHD, congenital left heart or aortic disease, or miscellaneous other types of CHD. Upon inclusion, targeted therapies for pulmonary arterial hypertension (PAH) included endothelin receptor antagonists, PDE-5 inhibitors, prostacyclin analogues, and soluble guanylate cyclase stimulators. Eighty patients with Eisenmenger syndrome were treatment-naïve. While at inclusion the primary PAH treatment for the cohort was monotherapy (70% of patients), with 30% of the patients on combination therapy, after a median observation time of 45.3 months, the number of patients on combination therapy had increased significantly, to 50%. The use of oral anticoagulants or antiplatelets was dependent on the underlying diagnosis or comorbidities. In the entire COMPERA-CHD cohort, after follow-up and receiving targeted PAH therapy (n = 511), 91 patients died over the course of a 5-year follow up. The 5-year Kaplan–Meier survival estimate for CHD associated PH was significantly better than that for idiopathic PAH (76% vs. 54%; p < 0.001). Within the CHD associated PH group, survival estimates differed particularly depending on the underlying diagnosis and treatment status. Conclusions: In COMPERA-CHD, the overall survival of patients with CHD associated PH was dependent on the underlying diagnosis and treatment status, but was significantly better as than that for idiopathic PAH. Nevertheless, overall survival of patients with PAH due to CHD was still markedly reduced compared with survival of patients with other types of CHD, despite an increasing number of patients on PAH-targeted combination therapy.