Congenital Heart Disease Group Research Articles

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

The yield of SNP microarray analysis for fetal ultrasound cardiac abnormalities.

Chromosomal microarray analysis (CMA) has emerged as a critical instrument in prenatal diagnostic procedures, notably in assessing congenital heart diseases (CHD). Nonetheless, current research focuses solely on CHD, overlooking the necessity for thorough comparative investigations encompassing fetuses with varied structural abnormalities or those without apparent structural anomalies. This study sought to assess the relation of single nucleotide polymorphism-based chromosomal microarray analysis (SNP-based CMA) in identifying the underlying causes of fetal cardiac ultrasound abnormalities. A total of 2092 pregnant women who underwent prenatal diagnosis from 2017 to 2022 were included in the study and divided into four groups based on the presence of ultrasound structural abnormalities and the specific type of abnormality. The results of the SNP-Array test conducted on amniotic fluid samples from these groups were analyzed. Findings from the study revealed that the non-isolated CHD group exhibited the highest incidence of aneuploidy, overall chromosomal abnormalities, and trisomy 18, demonstrating statistically significant differences from the other groups (p < 0.001). Regarding the distribution frequency of copy number variation (CNV) segment size, no statistically significant distinctions were observed between the isolated CHD group and the non-isolated CHD group (p > 0.05). The occurrence rates of 22q11.2 and 15q11.2 were also not statistically different between the isolated CHD group and the non-isolated congenital heart defect group (p > 0.05). SNP-based CMA enhances the capacity to detect abnormal CNVs in CHD fetuses, offering valuable insights for diagnosing chromosomal etiology and facilitating genetic counseling. This research contributes to the broader understanding of the utility of SNP-based CMA in the context of fetal cardiac ultrasound abnormalities.

Maternal and neonatal outcomes in women with congenital heart disease: A nationwide population-based study

BackgroundWe evaluated the outcomes of pregnancy in women with congenital heart disease (CHD) and their offspring in Taiwan. We also investigated how different severity levels may influence the outcomes. MethodsWe used data (2009–2017) from the Birth Certificate Application database in Taiwan, which is linked to the National Health Insurance Research Database and Taiwan Maternal and Child Health Database. We identified 2990 women with CHD who had 4227 births. Based on the CHD subtypes, patients were further divided into “severe CHD” and “simple CHD” groups. ResultsWomen with CHD have a significant risk of stillbirth. In maternal cardiac events, they had the highest risk of heart failure, followed by arrhythmia. The severity of CHD had a significant effect on the outcomes as well. The neonatal birth event that mothers with CHD have the highest risk of is preterm birth at < 32 weeks of gestation. The prominent difference in neonatal morbidities between mothers with severe and simple CHD is recurrent CHD in the offspring. The offspring of the severe CHD group had a higher risk of severe CHD, whereas those of the simple CHD group had a higher risk of simple CHD. ConclusionsDuring pregnancy, the monitoring of heart function and cardiac rhythm could be more intensive in mothers with CHD. In addition to accurately assessing fetal growth and development during antenatal care, mothers with severe CHD should be provided with careful fetal heart structure assessment and genetic testing along with counseling.

Survival does not differ by annual center transplant volume-A Pediatric Heart Transplant Society Registry study.

There are conflicting data regarding the relationship between center volume and outcomes in pediatric heart transplantation. Previous studies have not fully accounted for differences in case mix, particularly in high-risk congenital heart disease (CHD) groups. We aimed to evaluate the relationship between center volume and outcomes using the Pediatric Heart Transplant Society (PHTS) Registry and explore how case mix may affect outcomes. A retrospective cohort study of all pediatric patients in the PHTS Registry who received a heart transplant from 2009 to 2018 was performed. Centers were divided into 5 groups based on average yearly transplant volume. The primary outcome was time to death or graft loss and outcomes were compared using Kaplan-Meier analysis. There were 4583 cases among 55 centers included. There was no difference in time to death or graft loss by center volume in the entire cohort (p = .75), in patients with CHD (p = .79) or in patients with cardiomyopathy (p = .23). There was also no difference in time to death or graft loss by center size in patients undergoing transplant after Norwood, Glenn or Fontan (log rank p = .17, p = .31, and p = .10 respectively). There was a statistically significant difference in outcomes by center size in the positive crossmatch group (p < .0001), though no discernible pattern related to high or low center volume. Outcomes are similar among transplant centers of all sizes, including for high-risk patient groups with CHD. Future work is needed to understand how patient-specific risk factors may vary among centers of various sizes and whether this influences patient outcomes.

Impact of the Fetal Echocardiogram on Maternal Depression and Well-Being.

Fetal echocardiography allows for prenatal diagnosis of congenital heart disease (CHD). The objective of this study is to assess for the impact of fetal echocardiogram on levels of well-being and depression in pregnant women. Adult pregnant women carrying a fetus < 31-week gestational age were enrolled in the prospective observational study from February 2022 to June 2022. These subjects were split into two cohorts: those with CHD and those without CHD. Surveys were distributed prior to the fetal echocardiogram, six weeks later and six weeks after delivery. These surveys consisted of the Edinburgh Postpartum Depression Scale (EPDS) and questions about topics, such as anxiety and social support. Of the 152 subjects enrolled, 14 women had a fetus with CHD and 138 women had a fetus without CHD. Initial EPDS scores were elevated for the study population compared to the general population. Six weeks later, EPDS scores remained elevated. For the post-partum surveys, the EPDS scores were decreased below the rate of the general population for the group without CHD and similar to the general population rate for the CHD group. Anxiety, worry, and guilt remained low for both groups after the initial survey. Counseling from the pediatric cardiologist may help decrease anxiety and worry. Social support, like support groups, may be helpful for women carrying a fetus with CHD.

Coagulation Profile in Neonates with Congenital Heart Disease: A Pilot Study.

Background and Objectives: congenital heart disease (CHD), cyanotic and, to a lesser degree, acyanotic, often are accompanied by coagulation abnormalities, impacting substantially morbidity and mortality. Until now, no consistent hemostatic patterns have been demonstrated in neonates and children with CHD because they represent a variable and heterogenous population. The aim of the present study is to investigate the hemostatic profile, as well as the role of ADAMTS-13 (a disintegrin and metalloprotease with thrombospondin type-1 motives), the cleaving protein of von Willebrand factor (VWF) in neonates with CHD and compare them to healthy age-matched controls. Materials and Methods: twenty neonates with a mean gestational age of 37.1 ± 2.5 weeks were included in the CHD group, and 18 healthy neonates with a mean gestational age of 38.2 ± 1.5 weeks were in the control group. Results: prothrombin time was significantly prolonged, and accordingly, factor VII (FVII) levels were significantly decreased in the CHD group in comparison to controls. Factor VIII (FVIII), VWF, and ristocetin cofactor activity (Rcof) levels were significantly higher in the study vs. control group. Concentrations of ADAMTS-13 were decreased in the CHD vs. control group, but the difference was not statistically significant. Our results, in combination, indicate a balanced hemostatic mechanism, although with greater variability in neonates with CHD, while developmental aspects of coagulation are evident in the specific patient population. Conclusions: the coagulation profile is moderately impaired early in the course of CHD, though increased thrombogenicity is already present and should not be ignored.

Nucleated Red Blood Cell Counts Differentiate Cardiac from Respiratory Causes of Cyanosis at Birth.

Tissue hypoxia increases erythropoietin production and release of immature erythrocytes that can be measured using nucleated red blood cell counts (nRBC). We hypothesized that hypoxia due to congenital heart disease (CHD) is chronic and is better tolerated than hypoxia due to respiratory disease (RD), which is an acute stress in newborns leading to higher nRBC. This study assesses the utility of nRBC as a marker to differentiate hypoxia due to CHD vs RD in term neonates. This was a single-center, retrospective study of term neonates with cyanosis from 2015 to 2022. Neonates < 37weeks of gestation, with hypoxic-ischemic encephalopathy, and those with other causes of cyanosis were excluded. The patients were divided into 2 groups: cyanotic CHD and cyanotic RD. Clinical and laboratory data done within 12h and 24-36h after birth were collected. Data are represented as median and Interquartile range. Of 189 patients with cyanosis, 80 had CHD and 109 had RD. The absolute nRBC count at ≤ 12h of age was lower in the CHD (360 cells/mm3) compared to RD group (2340 cells/mm3) despite the CHD group having significantly lower baseline saturations. A value of 1070 cells/mm3 was highly sensitive and specific for differentiating CHD from RD. The positive predictive value for this cut-off value of 1070 cells/mm3 was 0.94 and the negative predictive value was 0.89. The absolute nRBC is a simple screening test and is available worldwide. A nRBC < 1070 cells/mm3 in cyanotic newborns should hasten the search for CHD etiology with the possible need for prostaglandin therapy.

Accuracy of Transthoracic Echocardiography in Detecting Persistent Left Superior Vena Cava against the Cardiac Computed Tomography Scan

Abstract Introduction: Detection of the persistent left superior vena cava (LSVC) is essential to diagnosing pediatric congenital heart diseases (CHDs). Transthoracic echocardiography is the most commonly used diagnostic tool for CHDs. This study compares transthoracic echocardiography and cardiac computed tomography (CT) to detect LSVC. Methods: Retrospective review of 49 pediatrics patients with CHDs who underwent echocardiography and cardiac CT. Results: Forty-nine pediatric patients with CHDs who underwent CT scans were included. In acyanotic CHD patients, CT identified four with bilateral superior vena cava (SVC), while echocardiography picked up three and missed one. In the remaining 16 patients with acyanotic CHDs, CT found single right-sided SVC; however, two of these were misidentified as having bilateral SVC during echocardiography. In the cyanotic CHD group, four patients had bilateral SVC. All four were picked up by echocardiography as well. However, echocardiography diagnosed one additional patient as having bilateral SVC, while the patient actually had a single SVC on CT scan. Conclusion: Transthoracic echocardiography can reasonably detect persistent LSVC in pediatric CHD patients.

Assessment of oral bacteria potentially associated with the mobile microbiome in children with congenital heart disease.

In this case-control study, we aimed to investigate the specific oral pathogens potentially associated with the mobile microbiome in children with congenital heart disease (CHD). Caries, oral hygiene and gingival indices were evaluated in 20 children with CHD and a healthy control group, and venous blood samples and saliva were collected. Using quantitative polymerase chain reaction (qPCR), blood samples were analyzed for the presence of bacterial DNA to determine the mobile microbiome, and saliva samples were analyzed to identify and quantify target microorganisms, including Streptococcus mutans (Sm) and its serotype k (Smk), Fusobacterium. nucleatum (Fn), Porphyromonas gingivalis (Pg), Scardovia wiggsiae (Sw) and Aggregitibacter actinomycetemcomitans (Aa) and its JP2 clone (JP2). The findings were analyzed by Mann Whitney U, chi-square, Fisher's exact and Spearman's Correlation tests. Bacterial DNA was identified in two blood samples. No significant differences were found between the groups regarding the presence and counts of bacteria in saliva. However, the CHD group exhibited significantly lower caries and higher gingival index scores than the control group. The presence of Pg and Aa were significantly associated with higher gingival index scores. Sm and Smk counts were significantly correlated with caries experience. A positive correlation was found between Fn and total bacteria counts. In conclusion, the mobile microbiome, which has been proposed as a potential marker of dysbiosis at distant sites, was very rare in our pediatric population. The counts of target microorganisms which are potentially associated with the mobile microbiome did not differ in children with CHD and healthy children.

The impact of pediatric congenital heart disease on primary teeth structure: a histological study.

Congenital heart disease (CHD) is an abnormality in the structure or function of the cardio-circulatory system present at birth and the ventricular septal defect (VSD) is the most common CHD in children. This study aimed to determine any differences in the histological structure of primary teeth between both healthy children and those children with ventricular septal defects in Erbil City. Methods enrolled children were divided into two groups. Group I (control) & group II (CHD) aged between 6-10 years old. A total of 44 children were collected, (22 children) in each group. Enamel, dentin, and odontoblast layers were examined histologically. Unpaired t-test used for statistical analysis. Results: The histopathological sections showed a significant difference in enamel, dentin, and odontoblast layer thickness (255.8 ± 41.68- 406.4 ±46.39), (1156 ± 116.0 - 1320 ± 117.4) and (29.74 ± 7.66 -41.38 ± 12.06) respectively, with p values (P < 0.0001) for enamel and dentin layer, and P < 0.0004 for odontoblast layer. A study of the images in the CHD group showed that the tooth tissue lost its integrity and cohesion in some places, and the thickness of the enamel and dentin layer in this group was significantly reduced compared to group I. Tissue loss in enamel, pulp, and dentin cell were observed. Also, connective tissue layers in the pulp were disrupted. Conclusions: CHD can alter the natural structure formation of primary teeth. Histologically, enamel, dentin, and odontoblasts layer thickness reduction are found in primary teeth in children with ventricular septal defects.

White matter microstructure is differently associated with executive functioning in youth born with congenital heart disease and youth born preterm.

Executive function deficits and adverse psychological outcomes are common in youth with congenital heart disease (CHD) or born preterm. Association white matter bundles play a critical role in higher order cognitive and emotional functions and alterations to their microstructural organization may result in adverse neuropsychological functioning. This study aimed to examine the relationship of myelination and axon density and orientation alterations within association bundles with executive functioning, psychosocial well-being, and resilience in youth with CHD or born preterm. Youth aged 16 to 26 years born with complex CHD or preterm at ≤33 weeks of gestational age and healthy controls completed a brain MRI and self-report assessments of executive functioning, psychosocial well-being, and resilience. Multicomponent driven equilibrium single-pulse observation of T1 and T2 and neurite orientation dispersion and density imaging were used to calculate average myelin water fraction (MWF), neurite density index (NDI), and orientation dispersion index values for eight bilateral association bundles. The relationships of bundle-average metrics with neuropsychological outcomes were explored with linear regression and mediation analyses. In the CHD group, lower MWF in several bundles was associated with poorer working memory and behavioral self-monitoring and mediated self-monitoring deficits relative to controls. In the preterm group, lower NDI in several bundles was associated with poorer emotional control and lower MWF in the left superior longitudinal fasciculus III mediated planning/organizing deficits relative to controls. No significant relationships were observed for psychosocial well-being or resilience. The findings of this study suggest that microstructural alterations to association bundles, including lower myelination and axon density, have different relationships with executive functioning in youth with CHD and youth born preterm. Future studies should aim to characterize other neurobiological, social, and environmental influences that may interact with white matter microstructure and neuropsychological functioning in these at-risk individuals.

Changes of serum TSH, FT3, and FT4 levels in infants received surgical correction of congenital heart disease under cardiopulmonary bypass

ObjectiveThis study aimed to explore the fluctuations and clinical relevance of serum thyrotropin (TSH), free triiodothyronine (FT3), and free thyroxine (FT4) levels in infants undergoing surgical correction for congenital heart disease (CHD) using cardiopulmonary bypass (CPB).MethodsIn a retrospective design, 58 infants who underwent CHD surgical correction under CPB between January 2021 and January 2022 at our institution were incorporated. These infants were categorized into two groups: simple CHD (n = 34) and complex CHD (n = 24). TSH, FT3, and FT4 serum concentrations were assessed at four intervals: 24 h pre-surgery (T0) and 24 h (T1), 48 h (T2), and 72 h (T3) post-surgery.ResultsThe simple CHD group displayed a significantly reduced CPB duration compared to the complex CHD group (P < 0.001). Both groups exhibited a notable decline in serum thyroid hormone concentrations at T1 compared to T0. However, from T1 to T3, an upward trend in hormone levels was observed. By T3, though the levels in both groups had risen notably from T1, they remained significantly diminished from T0 (P < 0.01). In both the simple and complex CHD cohorts, significant fluctuations in thyroid hormone levels (TSH, FT3, FT4) were noted across the different timepoints (T0, T1, T3) (P < 0.01). While no significant disparities were found between the two groups’ hormone concentrations at T0 and T1 (P > 0.05), at T2 and T3, the simple CHD group manifested higher TSH, FT3, and FT4 levels compared to the complex CHD group (P < 0.05).ConclusionsInfants undergoing CHD surgical correction under CPB experience significant declines in TSH, FT3, and FT4 serum levels. The post-surgery thyroid hormone recovery was more pronounced in infants with simple CHD compared to those with complex CHD. As such, vigilant monitoring of thyroid hormone levels during the perioperative phase is imperative, and timely intervention measures should be employed when necessary.

The Role of Speckle-Tracking Echocardiography in Predicting Mortality and Morbidity in Patients With Congenital Heart Disease: A Systematic Review and Meta-analysis

Speckle-tracking echocardiography (STE) is now routinely included in cardiac evaluations, but its role in predicting mortality and morbidity in congenital heart disease (CHD) is not well described. We conducted a systematic review to evaluate the prognostic value of STE in patients with CHD. The EMBASE, Medline, Web of Science, Scopus, and Cochrane Central Register of Controlled Trials (CENTRAL) databases were searched from inception to January 2023 for terms related to all CHD, STE, and prognosis. Meta-analysis of association of right ventricle and left ventricle strain (RV Sl and LV Sl, respectively) with major adverse cardiovascular events (MACEs) was performed in atrial switch transposition of the great arteries (asTGA)/congenitally corrected TGA (ccTGA), tetralogy of Fallot (ToF), and congenital aortic stenosis (cAS)/bicuspid aortic valve (BAV). P-value combination analysis was additionally performed for all CHD groups. A total of 33 studies (30 cohorts, n=8,619 patients, children, and adults) were included. Meta-analysis showed the following parameters as being associated with MACE: RV Sl in asTGA/ccTGA (hazard ratio [HR]=1.1/%; CI, [1.03; 1.18]), RV Sl and LV Sl in ToF (HR=1.14/%; CI, [1.03; 1.26] and HR=1.14/%; CI, [1.08; 1.2], respectively), and LV Sl in cAS/BAV (HR=1.19/%; CI, [1.15; 1.23]). The RV Sl and strain rate were associated with outcomes also in single ventricle/hypoplastic left heart syndrome (at all palliation stages except before Norwood stage 1) and LV Sl in Ebstein's anomaly. This systematic review and meta-analysis showed that biventricular strain and strain rate were associated with outcomes in a variety of CHD, highlighting the need for updated recommendations on the use of STE in the current guidelines, specific to disease types.

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.

Association Analysis between Maternal Serum Biomarkers and Fetal Congenital Heart Disease

Objective: To investigate the association of maternal serum biomarkers alpha fetal protein (AFP), β-human chorionic gonadotropin (HCG) and unconjugated estriol 3 (uE3) in the second trimester with fetal congenital heart disease (CHD) in low risk populations during the screening of Down's syndrome. Methods: 109 cases diagnosed with fetal CHD in the second trimester by fetal echocardiography were enrolled as the CHD group. Pregnancy- and gestational-age matched 344 cases without fetal CHD were used as the control group. The values of maternal serum biomarkers HCG, AFP and uE3 were tested and the association with CHD was analyzed. Results: The means of HCG multiple of median (MoM) and AFP MoM were higher, while the mean of uE3 MoM was lower in the CHD group than those of the control group (p &lt; 0.05). The number of cases with HCG MoM ≥85% quantile, AFP MoM ≥85% quantile was more, while that with uE3 MoM ≤15% quantile was less in the CHD group than that of the control group (p &lt; 0.05). The univariate logistic regression analysis showed that fetal CHD was associated with high values of HCG MoM and AFP MoM and low value of uE3 MoM as well as the HCG MoM ≥85% quantile, AFP MoM ≥85% quantile and the uE3 MoM ≤15% quantile. The multivariate logistic regression analysis showed that HCG MoM ≥85% quantile and AFP MoM ≥85% quantile or the uE3 ≤15% quantile were the independent factors of fetal CHD. In addition, the risk of fetal CHD was higher when one situation existed among the HCG MoM ≥85% quantile, AFP MoM ≥85% quantile and the uE3 MoM ≤15% quantile. The risk of fetal CHD was much higher when both the HCG MoM ≥85% quantile and AFP MoM ≥85% quantile existed or both the HCG MoM ≥85% quantile and the uE3 MoM existed ≤15% quantile. Conclusions: Second trimester maternal serum biomarkers may be useful indicators for fetal evaluation for CHD to screen positive pregnancies without identified chromosomal defects.

Activities of Daily Living, Physical Activity, Physical Fitness and Quality of Life in Children with Congenital Heart Disease: A Case-Control Study.

Despite reports of reduced physical fitness in children with congenital heart disease (CHD), no specific performance evaluations for activities of daily living have been conducted. The aim was to compare the activities of daily living, quality of life, posture, physical fitness and physical activity levels of children with CHD with healthy controls (HC). The study included 30 children aged 6-14 diagnosed with moderate or severe CHD and 30 age-sex-matched HC. The sociodemographic and clinical data of the participants were recorded. All participants went through several tests, namely the TGlittre-P test for activities of daily living, the 6-minute walk test (6MWT) for functional capacity, the Fitnessgram test battery for physical fitness, the hand dynamometer for measuring grip strength, the pedometer for measuring physical activity, and both the child and parents reported the Pediatric Quality of Life Inventory (PedsQL) for evaluating the quality of life, in addition to posture analyses. Values of p < 0.05 were considered statistically significant. Individuals with CHD had a longer TGlittre-P test completion time and a shorter 6MWT distance than HC (TGlittre-P: CHD 3.45 [3.24-4.02]min vs. HC 3.10 [2.57-3.23]min, 6MWT: CHD 514.00 [412.50-566.00]m vs. HC 591.50 [533.00-631.00]m). For the CHD group, sit-ups, push-ups, trunk lift, and sit-and-reach test scores within the Fitnessgram battery, grip strength, posture, and quality of life scores were lower than those for the HC group. Physical activity levels were similar in the groups. The performance of activities of daily living, functional capacity, physical fitness, posture, and quality of life of children with moderate and severe CHD were affected compared to healthy peers.

Microvascular Endothelial Function Assessed Using Peripheral Arterial Tonometry in Adolescents with Repaired Congenital Heart Disease.

Atherosclerosis can develop in adult patients with congenital heart disease (CHD) and should be given attention. Endothelial function is well known as a predictor of the development of atherosclerosis but has not been well investigated in patients with repaired CHD. This study aimed to clarify the endothelial function and its relationship with clinical backgrounds and parameters in adolescents with various types of repaired CHDs. Endothelial function was evaluated using peripheral arterial tonometry (PAT). The reactive hyperemia index (RHI) was evaluated and compared between adolescents with repaired CHD and those in the control group. The relationship between the clinical background and parameters was also investigated in patients with repaired CHD. Forty-eight patients with repaired CHD (age 14.0 ± 3.3 years) and 114 healthy volunteers were included in this study. Patients with repaired CHD comprised 16 with repaired non-cyanotic CHD, 14 with repaired tetralogy of Fallot, and 18 who underwent the Fontan procedure. RHI in the repaired CHD group was significantly lower than in the control group. There was no significant correlation between the RHI and blood biochemical markers, such as uric acid, creatine, and brain natriuretic peptide levels. The RHI was significantly higher in patients taking angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) than in those not taking them. Endothelial function was impaired in adolescents with repaired CHD compared to that in the control group. Microvascular endothelial dysfunction developed even in adolescents with simple non-cyanotic CHD.

SERUM ADAMTS9 LEVEL IN NEWBORN BABIES WITH CONGENITAL HEART DISEASE.

Introduction & Objective ADAMTS-9, one of the ADAMTS enzymes, is expressed in all fetal tissues, unlike other ADAMTS enzymes, and is thus thought to play a role in fetal development. In this context, the objective of this study is to investigate the relationship between ADAMTS-9 activity and the development of Congenital Heart Diseases (CHD) with a view to using ADAMTS-9 level as a biomarker for CHDs. Material & Method Newborns diagnosed with CHD and healthy newborns were included in the study as the CHD and control groups, respectively. Gestational age, maternal age, and mode of delivery information pertaining to the mothers and APGAR score and birth weight information pertaining to the newborns were recorded. Blood samples were taken from all newborns to determine their ADAMTS-9 levels in the first 24 hours of life. Results Fifty-eight newborns with CHD and 46 healthy newborns were included in the study. Median ADAMTS-9 levels were 46.57 [interquartile range(IQR):33.31 (min.26.92, max.124.25)] ng/ml and 23.36 [(IQR:5.48)(min.11.7, max.37.71)] ng/ml in the CHD and control groups, respectively. ADAMTS-9 levels in the CHD group were statistically significantly higher than in the control group (p=0.000). ADAMTS-9 levels of the CHD and control groups were analyzed by the receiver operating characteristics(ROC) curve. The area under the curve (AUC) value for ADAMTS-9levels of >27.86 ng/ml as the cut-off value for predicting the development of CHD in newborns was 0.836 [95% confidence interval (CI): 0.753-0.900, p=0.0001]. ADAMTS-9 levels of >27.86 ng/ml were determined to predict the development of CHD in newborns with a sensitivity of 77.78% [95% CI: 65.5-87.38] and a specificity of 84.78% [95% CI: 71.1-93.60]. Conclusion In conclusion, it was found that the serum ADAMTS-9 levels were significantly higher in newborns with CHD than in healthy newborns. In parallel, ADAMTS-9 levels above a certain cut-off value were associated with CHD.

Increased Cerebello-Prefrontal Connectivity Predicts Poor Executive Function in Congenital Heart Disease.

Children and adolescents with congenital heart disease (CHD) are at risk for cognitive impairments, such as executive function deficits and motor delays, which can impact their academic and adaptive functioning as well as their quality of life. We investigated whether alterations in connectivity between the prefrontal and cerebellar brain structures exist between CHD and control cohorts and if these alterations could predict cognitive or motor impairment among youths with CHD. 53 participants with CHD and 73 healthy control participants completed multi-modal magnetic resonance imaging (MRI) of the brain, including high-resolution diffusion tensor imaging at 3T. We measured connectivity from masked regions of interest in the cerebellum to the frontal cortex using a probabilistic tractography method. Participants also completed neuropsychological tests of cognitive and motor skills using the NIH Toolbox. In the CHD group, fractional anisotropy (FA) was increased in the cognitive loop connectivity pathways, including from the right cerebellum to the left thalamus (p = 0.0002) and from the left thalamus to the left medial frontal gyrus (MFG) (p = 0.0048) compared with the healthy control group. In contrast, there were no differences between CHD and controls in motor loop connectivity pathways. An increase in FA from the right thalamus to the MFG tract in the cognitive loop (posterior subdivision) predicted (p = 0.03) lower scores on the NIHTB tests, including those of executive functioning. A transient increase in connectivity of the cognitive loop in the adolescent group was observed relative to the child and adult groups. Our results suggest that selective alteration of cerebellum-cerebral connectivity circuitry within the cognitive loops predicts cognitive dysfunction in CHD youth. Our study suggests a critical period of cerebellar circuitry plasticity in the adolescent period in CHD subjects that drives neurocognitive function. Further replication and validation in other pediatric CHD cohorts is warranted for future work.

The Investigation of Cerebroplacental Ratio and Some Cranial Biometric Measurements in Fetuses with Isolated Congenital Heart Disease

Objective: In this study, we aimed to compare the measurements of some fetal cranial structures and Doppler parameters between the groups with congenital heart disease and healthy pregnant women. Materials and Methods: The study included 30 patients with intrauterine congenital heart disease and 30 healthy pregnant women. Fetuses with additional structural and genetic abnormalities were excluded from the study. In both groups, biparietal diameter (BPD), head circumference (HC), transcerebellar diameter (TCD), middle cerebral artery pulsatility index (MCA-PI), umbilical artery pulsatility index (UA-PI), umbilical artery resistance index (UA-RI), and umbilical artery systole/diastole ratio (UA S/D), as well as superior-inferior diameter of the cavum septum pellucidum (CSP) were measured by ultrasound. The cerebroplacental ratio (CPR), which is an indicator of the protective effect on the brain, was calculated. Data were statistically compared between the two groups, and P &lt; 0.05 was considered statistically significant. Results: A statistically significant difference was found between the groups in UA-RI and UA S/D values. The UA-RI and UA S/D values were significantly higher in the patient group than in the control group (P=0.048; P &lt; 0.001). HC values were lower in the congenital heart disease group, and this result was statistically significant (P=0.047). There was no statistically significant difference between groups in BPD, HC, and TCD Z-scores. There was no statistically significant difference between groups in MCA-PI &lt; 5th percentile, UA-PI &gt; 95th percentile, and CPR &lt; 1 scores. Conclusion: Congenital heart disease may cause chronic fetal hypoxia during the intrauterine process and lead to changes in the fetomaternal circulation. This study suggests that there may be relationships between fetal cranial biometric retardation and cerebral perfusion changes.