Congenital Heart Disease Group Research Articles

Abstract 14419: Cerebrovascular Stability Index is Associated With Motor Development Scores in 6-month Infants With Congenital Heart Disease

Introduction: Infants with congenital heart disease ( CHD ) have an increased risk for poorer neurodevelopmental outcomes ( NDO ) compared to healthy infants. Impaired cerebrovascular autoregulation ( CA ) may contribute to neurologic abnormalities in CHD infants. This study’s purpose is to assess cerebrovascular stability index ( CSI ), as a surrogate for CA, and its associations with NDO in infants with CHD compared to healthy control infants at 3 early-age timepoints. Hypothesis: We hypothesized that CHD infants who have poorer CSI will have significantly poorer neurodevelopmental outcomes than infants with better CSI. Methods: We conducted a prospective, longitudinal study in CHD infants and healthy controls ( HC ). We measured CSI and NDO at 3 ages (neonatal, 3-month, and 6-month). We performed 3 tilts while measuring cerebral oxygenation via near-infrared spectroscopy. CSI was determined by subtracting the average 2-minute sitting cerebral oxygenation from the 2-minute supine value for each tilt, then averaging the 3 tilt values for each age. The Einstein Neonatal Neurobehavioral Assessment Scale ( ENNAS ) measured neonatal NDO, while the Bayley-3 evaluated the 3 and 6-month NDO. Results: We assessed a total of 28 CHD and 40 HC infants. CHD infants had significantly lower ENNAS scores as neonates (68 CHD vs 83 HC, p<0.001), and at 6-months had lower cognitive (94 CHD vs 100 HC, p=0.04), and motor (86 CHD vs 98 HC, p<0.001) composite scores compared with the HC. However, the other domains did not significantly differ between groups. Linear regression analyses showed that motor development scores in the CHD group improved as CSI improved when adjusted for sex and ethnicity (β=3.64, SE=1.47, p=0.02). CSI did not significantly associate with the cognitive score or NDO for the other ages. Conclusion: We found better CSI significantly associated with higher motor development scores in 6-month old CHD infants, suggesting that impaired CSI may underlie abnormalities of motor development in CHD. Future studies are needed to better understand the mechanism of interaction between CSI and motor development.

Acute Hospital Outcomes for Renal Transplantation in Patients With Moderate or Severe Congenital Heart Disease

Children and adults with congenital heart disease (CHD) are increasingly recognized to be at risk for acute and chronic renal injury. Some of these may progress to the need for renal transplantation. We hypothesized that patients with underlying moderate or severe CHD who undergo renal transplantation will have worse acute hospital outcomes. Using a national administrative database, we queried for admissions aged 0 to 50 years with moderate or severe CHD and renal transplantation and compared these to admissions without CHD. There were 56 admissions for renal transplantation in the CHD group (0.04%) and 26,285 admissions in the group without CHD (0.21%, p<0.001). The CHD group were younger, had a higher proportion of Whites, longer length of stay, higher complication rates, higher in-hospital mortality, and higher costs. In conclusion, although renal transplantation is still relatively uncommon in the CHD population, there is an increasing recognition of severe chronic renal disease in the setting of CHD, making it important to understand the potential implications of these findings.

Cryopreserved human heart valve allografts: a ten-year single centre experience.

This study provides an overview of tissue banking activities at the Croatian Cardiovascular Tissue Bank (CTB) during past ten years and presents the outcomes of cryopreserved heart valve allografts (CHAs) use in different patient groups. From June 2011 until December 2021, 75 heart donations were referred to CTB: 41 recipient of heart transplant (RHT), 32 donors after brain death (DBD) and 2 donors after circulatory death (DCD) donations. Processing resulted in 103 valves of which 65 met quality requirements for clinical use. Overall tissue discard rate was 37%. The most frequent reasons for discard were inadequate morphology (12%) in RHT donations and microbiological contamination (19%) in DBD donations. Altogether, 38 CHAs were transplanted to 36 patients. Recipients were divided in three groups; infective endocarditis (IE), non-infectious heart disease and congenital heart disease group. In the IE group, the 30-day, 1-year and 3-year survival was 71%, 53% and 47%, respectively. Freedom from re-operation due to all graft-related causes was 76% and due to structural valve deterioration 88%. There were no cases of graft reinfection. In the congenital heart disease group CHAs were predominantly (94%) used for right ventricular outflow tract reconstruction and 88% of patients recovered without graft-related complications. At present, the number of demands for CHAs at CTB considerably outweighs their availability.

Executive functioning of patients with congenital heart disease: 45 years after surgery

Abstract Background For children with congenital heart disease (CHD), subtle neuropsychological deficits have been reported. However, very little is known about executive functioning in their adulthood. Purpose To investigate the self- and informant-reported executive functioning in adults with CHD operated in childhood (&amp;lt;15 years old). Material and methods A cohort study of 194 patients (age 50 [46–54] years), operated in childhood between 1968 and 1980 for one of the following diagnosis: atrial septal defect (ASD), ventricular septal defect (VSD), pulmonary stenosis (PS), tetralogy of Fallot (ToF) or transposition of the great arteries (TGA), were evaluated 40–53 years after surgery. The “Behavior Rating Inventory of Executive Functions – Adult version” (BRIEF-A) questionnaire was used to assess self- and informant-reported executive functioning and compared to the general population. Results The CHD group did not show worse executive functioning compared to normative data. In addition, no significant difference was found between simple CHD (ASD, VSD and PS) and moderate/complex CHD (ToF and TGA). Higher education and NYHA class 1 were associated with better self-reported executive functioning, while male patients and patients taking psychiatric or cardiac medications reported worse executive functioning. Conclusions Overall, our findings suggest reassuring outcomes regarding executive functioning in adults with CHD. However, these findings should be confirmed with neuropsychological assessment studies. Funding Acknowledgement Type of funding sources: Foundation. Main funding source(s): Thorax Foundation

Correlation between Mean Arterial Pressure and Regional Cerebral Oxygen Saturation on Cardiopulmonary Bypass in Pediatric Cardiac Surgery.

Some pediatric cardiac patients might experience low regional cerebral oxygen saturation (rSO2) during surgery. We investigated whether a pediatric patient's mean arterial pressure (MAP) can affect the rSO2 value during cardiopulmonary bypass (CPB). We retrospectively analyzed the cases of the pediatric patients who underwentcardiac surgery at our hospital (Jan. -Dec. 2019; n=141). At each MAP stage, we constructed line charts through the mean of the rSO2 values corresponding to each MAP and then calculated the correlation coefficients. We next divided the patients into age subgroups (neonates, infants, children) and into cyanotic congenital heart disease (CHD) and acyanotic CHD groups and analyzed these groups in the same way. The analyses of all 141 patients revealed that during CPB the rSO2 value increased with an increase in MAP (r=0.1626). There was a correlation between rSO2 and MAP in the children (r=0.2720) but not in the neonates (r=0.06626) or infants (r=0.05260). Cyanotic CHD or acyanotic CHD did not have a significant effect on the rSO2/MAP correlation. Our analysis demonstrated different patterns of a correlation between MAP and rSO2 in pediatric cardiac surgery patients, depending on age. MAP was positively correlated with rSO2 typically in children but not in neonate or infant patients.

Aerobic fitness change with time in children with congenital heart disease: A retrospective controlled cohort study

BackgroundTo evaluate the change in aerobic fitness (VO2max), measured by cardio-pulmonary exercise test (CPET), in children with congenital heart disease (CHD), compared to matched healthy controls, and identify predictors of VO2max change with time in this specific population. MethodThis longitudinal retrospective multicentre cohort study was carried out from 2010 to 2020. We included CHD paediatric patients from the cohort of a previous cross-sectional study, who had a second CPET at least 1 year after the first one, during their follow-up. ResultsWe included 936 children, 296 in the CHD group and 640 controls. Mean time between baseline and final CPET was 4.4 ± 1.7 years. After matching on age and gender and adjustment for age and BMI, the mean VO2max group difference was 10.5% ± 1.0% of percent-predict VO2max at baseline and increased to 19.1% ± 1.3% at final assessment. In the CHD group, the proportion of children with impaired aerobic fitness was significantly higher at final than at baseline CPET assessment (51.4% vs 20.3%; P < 0.01). The mean annual VO2max decrease was significantly worse in the CHD group than in controls (−1.88% ± 0.19% of percent-predict VO2max/year vs. -0.44% ± 0.27% of percent-predict VO2max/year, P < 0.01, respectively). In multivariate analyse, male gender, a high initial VO2max, a high BMI, and the number of cardiac surgical procedures ≥2, were predictors of the VO2max decrease with time. ConclusionThe VO2max decrease with time is more pronounced in children with CHD compared to healthy matched controls. This study highlighted the importance of serial CPET assessment in children with CHD.Trial registration NCT04815577.

COPHYD (Corona Owed Physical Deficiency): The Impact of Lockdown on Cardiopulmonary Function in Paediatric Cardiology Patients.

Due to the Corona-lockdowns the closure of sports facilities and schools has led to a decline in physical activity (PA) for months. PA is essential for maintaining cardiopulmonary function assessed by peak oxygen uptake. Since peak oxygen uptake represents the best predictor for mortality and morbidity in children with congenital heart disease the impact of lockdowns on this parameter is vital. We evaluated retrospectively cardiopulmonary exercise data from our patient collective from before lockdown with twin-paired data from during lockdown. The twin-pairing was achieved by matching patients with similar heart disease, age, sex, and test method. Cardiopulmonary exercise testing was achieved on either the treadmill or the bicycle. We were able to twin-pair 90 cases (26 twins with heart disease and 19 healthy twins). There was a significant decrease of cardiopulmonary function (V̇O2peak: 35.7±9.8 vs.30.4±10.6) in the heart disease cohort along with a significant decrease in peak oxygen pulse (13.3±4.1 vs. 11.4±4.5), and peak minute ventilation (V ̇E: 83.05±29.08 vs.71,49±24.96). The healthy children improved over the timeframe, but this only reached significance for peak minute ventilation (81.72±25.79 vs.97.94±31.4). We observed a significant decline of peak oxygen uptake during lockdown in the group of congenital heart disease (CHD) patients. This involved a loss of cardiac function as well as pulmonary function. This could be explained by limited access to sports facilities and restriction of regular daily movement due to school closure and overprotection. Healthy children improved their pulmonary function. This might be an indication of an improvement of exercise during confinement.Aufgrund des Corona-Lockdowns hat die Schließung von Sportanlagen und Schulen über Monate zu einem Rückgang der körperlichen Aktivität geführt. Körperliche Aktivität ist für die Aufrechterhaltung der kardiopulmonalen Funktion, bewertet durch die maximale Sauerstoffaufnahme, unerlässlich. Da die maximale Sauerstoffaufnahme der beste Prädiktor für Mortalität und Morbidität bei Kindern mit angeborenen Herzerkrankungen ist, sind die Auswirkungen des Lockdowns auf diesen Parameter von entscheidender Bedeutung. Spiroergometriedaten unseres Patientenkollektivs aus der Zeit vor dem Lockdown wurde mit Daten von statistischen Zwillingen aus dem gleichen Kollektiv während des Lockdowns verglichen. Die Zwillingspaarung wurde erreicht, indem Patienten mit ähnlichen Herzerkrankungen, Alter, Geschlecht und Testmodalitäten verglichen wurden. Die Spiroergometrie wurde entweder auf dem Laufband oder auf dem Fahrrad durchgeführt. Insgesamt wurden 90 Datensätze verbunden (26 Zwillinge mit Herzkrankheit und 19 gesunde Zwillinge). In der Kohorte der Herzerkrankten konnte eine signifikante Verschlechterung der kardiopulmonalen Funktion (V̇O2peak: 35,7±9,8 vs. 30,4±10,6), des Spitzensauerstoffpulses (O2puls: 13,3±4,1 vs. 11,4±4,5) und der Spitzenminutenventilation (VE: 83,05 ± 29,08 vs. 71,49 ± 24,96) beobachtet werden. Die gesunden Kinder verbesserten sich im Laufe der Zeit, wobei der Anstieg nur bei der Spitzenminutenventilation (81,72±25,79 vs. 97,94 ± 31,4) signifikant war. in der Kohorte der Patienten mit angeborenen Herzerkrankungen (CHD) beobachteten wir einen deutlichen Rückgang der maximalen Sauerstoffaufnahme während des Lockdowns. Dies liegt begründet in einer Verschlechterung der Herz-, sowie der Lungenfunktion. Erklärung dafür könnte der begrenzte Zugang zu Sporteinrichtungen, sowie Einschränkungen der täglichen Bewegungsfreiheit durch Schulschließungen und Überbehütung sein. Gesunde Kinder konnten ihr Atemminutenvolumen während dem Lockdown steigern und zeigten einen Trend zu besserer Belastbarkeit. Dies könnte auf eine Zunahme der körperlichen Bewegung während des Lockdowns hindeuten.

NMR-Based Metabolomic Analysis of Plasma in Patients with Adult Congenital Heart Disease and Associated Pulmonary Arterial Hypertension: A Pilot Study.

Patients with unrepaired congenital heart disease (CHD) are prone to pulmonary arterial hypertension (PAH). The ovine pulmonary arterial smooth muscle cells exposed to increased pulmonary blood flow (PBF) exhibited hyperproliferation and metabolic alterations, but the metabolic disorders of patients with CHD and associated PAH (PAH-CHD) have not yet been fully understood. Adult CHD patients were prospectively included and divided into the PAH-CHD group (n = 24) and CHD group (n = 38), while healthy adults were included as healthy control (HC) group (n = 29). Plasma from each subject was prepared for nuclear magnetic resonance (NMR) detection. 1H-NMR spectra were acquired using 850 MHz NMR spectrometer. A total of 28 metabolites were identified from the NMR spectra and their relative concentrations were calculated and analyzed by multivariate and univariate statistical analyses and metabolic pathway analysis. Receiver operating characteristic (ROC) curve analysis and correlation analysis were performed to identify potential biomarkers and assess their roles in clinical assessment. Multivariate statistical analysis showed that the metabolic profile of PAH-CHD was altered relative to CHD or HC, while that of CHD was altered relative to HC. The identified characteristic metabolites were alanine, glucose, glycine, threonine and lactate, and the areas under the ROC curves (AUCs) were 0.769, 0.808, 0.711, 0.842 and 0.817, respectively. Multivariate ROC curve analysis showed AUCs ranging from 0.895 to 0.955 for the combination of these characteristic metabolites. The correlation analysis indicated that lactate and threonine were significantly correlated with mean pulmonary arterial pressure, pulmonary vascular resistance and N-terminal pro-B-type natriuretic peptide. The increased PBF could trigger global metabolic alterations in patients with CHD, which were more severe in patients with PAH-CHD. The characteristic metabolites have the potential to be biomarkers of PAH-CHD, which could be used for its noninvasive diagnosis, severity and prognosis assessment, thereby improving the management of PAH-CHD.

Regional impairment of deep gray matter perfusion in neonates with congenital heart disease revealed by arterial spin labeling MRI.

The present study examined deep gray matter perfusion in neonates with congenital heart disease (CHD) with arterial spin labeling magnetic resonance imaging preoperatively. We found that neonates with cyanotic CHD showed lower right thalamus compared with controls and lower right basal ganglia perfusion compared with acyanotic CHD. When the CHD group was assessed as a whole, it showed slightly decreased left thalamus perfusion compared with controls. The results suggest that cardiac physiology plays a crucial part in changes in regional cerebral perfusion. Perfusion with arterial spin labeling may be a useful marker of high risk for impaired cerebral blood flow auto-regulation and cerebral hypoperfusion in neonates with CHD.

Physiological cost index: comparison between repaired congenital heart disease and healthy children

Children with congenital heart disease (CHD) have compromised physiological function compared to normal with the same age and anthropometric measure. Physiologic cost index (PCI) calculated the energy expenditure and gives an idea about the same during walking. So the present observational study calculates the energy expenditure in repaired congenital heart disease patient and provides the baseline for a goal to achieve during any rehabilitation program by comparing with the normal. To compare the PCI between repaired congenital heart disease patients and healthy children. The study design is observational. Total 40 participants were selected as per the criteria for inclusion and exclusion i.e. 20 healthy children and 20 with repaired congenital heart disease. Duration of the study will be for six months. The six-minute walk was performed according to guidelines, and PCI was calculated using the Macgregor’s formula. In all the variables found of 6MWT, statically significant difference was seen. The mean values for PCI for repaired CHD group was 13.84 and for normal children it was 8.6 using the unpaired T test p value was found to be p=0.0001 which is statically significant. We can conclude that the energy expenditure in children with repaired congenital heart disease is more as compared to the normal children with similar age group. PCI can be used as an effective tool for a physiotherapist in assessment and evaluation of functional performance and provide the baseline for repaired CHD children

Placental delayed villous maturation is associated with fetal congenital heart disease

The placenta is crucial for the overall development and lifelong health of the fetus. Abnormal placental development and function occur in pregnancies with fetal congenital heart disease. However, studies that use standardized diagnostic criteria and incorporate control populations are lacking. This limits the generalizability of current research and the ability to determine the specific placental abnormalities associated with congenital heart disease. This study applied consensus statement guidelines (known as the Amsterdam criteria) for placental pathology interpretation to compare the frequency and pattern of abnormalities in pregnancies with fetal congenital heart disease to demographically matched control pregnancies and evaluate for differences in placental abnormalities by cardiac physiology. A single-center retrospective cohort study was conducted from January 2013 to June 2019. Infants with a prenatal diagnosis of moderate-severe congenital heart disease who were born at ≥37 weeks of gestation were included. A control group born at ≥37 weeks of gestation but without fetal congenital heart disease or other major pregnancy complications was matched to the congenital heart disease group on maternal race and ethnicity and infant sex. Using the Amsterdam criteria, placental pathology findings were categorized as delayed villous maturation, maternal vascular malperfusion, fetal vascular malperfusion, and inflammatory lesions. The frequency of placental abnormalities was compared between groups, and logistic regression was performed to evaluate the association of clinical and sociodemographic factors with delayed villous maturation, maternal vascular malperfusion, and fetal vascular malperfusion. There were 194 pregnancies with fetal congenital heart disease and 105 controls included, of whom 83% in the congenital heart disease group and 82% in the control group were of non-Hispanic White race and ethnicity. Compared with controls, pregnancies with fetal congenital heart disease had higher rates of delayed villous maturation (6% vs 19%; P<.001) and maternal vascular malperfusion (19% vs 34%; P=.007) but not fetal vascular malperfusion (6% vs 10%; P=.23). Infants with congenital heart disease with 2-ventricle anatomy displayed the highest odds of delayed villous maturation compared with controls (odds ratio, 5.5; 95% confidence interval, 2.2-15.7; P<.01). Maternal vascular malperfusion was 2.2 times higher (P=.02) for infants with 2-ventricle anatomy and 2.9 times higher (P=.02) for infants with single-ventricle physiology with pulmonic obstruction. Within the congenital heart disease group, delayed villous maturation was associated with higher maternal body mass index, polyhydramnios, larger infant birth head circumference, and infant respiratory support in the delivery room, whereas maternal vascular malperfusion was associated with oligohydramnios. In multivariable models adjusting for cardiac diagnosis, associations of delayed villous maturation persisted for infant birth head circumference (odds ratio, 1.2; 95% confidence interval, 1.0-1.5; P=.02) and infant respiratory support in the delivery room (odds ratio, 3.0; 95% confidence interval, 1.3-6.5; P=.007). Pregnancies with fetal congenital heart disease displayed higher rates of delayed villous maturation and maternal vascular malperfusion than controls, suggesting that placental maldevelopment may relate to maternal factors. Future investigations are needed to determine the association of these abnormalities with postnatal infant outcomes.

Fetal congenital heart disease - mode of delivery and obstetrical complications

BackgroundThe optimal mode of delivery in cases of fetal congenital heart disease (CHD) is not established. The few relevant studies did not address operative vaginal delivery. The aim of this study was to assess the impact of fetal CHD on mode of delivery during a trial of labor, and to secondarily describe some obstetric complications.MethodsThe database of a tertiary medical center was searched for women who gave birth to a singleton, liveborn neonate in 2015–2018. Mode of delivery was compared between women carrying a fetus with known CHD and women with a healthy fetus matched 1:5 for maternal age, parity, body mass index, and gestational age.ResultsThe cohort included 616 women, 105 in the CHD group and 511 in the control group. The rate of operative vaginal delivery was significantly higher in the CHD group (18.09% vs 9.78%, OR 2.03, 95% CI 1.13–3.63, p = 0.01); the difference remained significant after adjustment for nulliparity and gestational age at delivery (aOR 2.58, 95% CI 1.36–4.9, p < 0.01). There was no difference between the CHD and control group in rate of intrapartum cesarean delivery (9.52% vs 10.76%, respectively, OR 0.97, 95% CI 0.47–1.98, p = 0.93). The most common indication for operative vaginal delivery was non-reassuring fetal heart rate (78.94% vs 64%, respectively). Median birth weight percentile was significantly lower in the CHD group (45th vs 53rd percentile, p = 0.04).ConclusionsOur findings suggest that operative vaginal delivery, performed mostly because of non-reassuring fetal heart rate, is more common in pregnancies complicated by a prenatal diagnosis of CHD than non-anomalous pregnancies.

Единственный желудочек сердца у кота породы мейн-кун

Univentricular heart (UH) is a rare group of congenital heart disease characterized by the atrial chambers connects with one ventricular chamber. The most frequent UH is double inlet morphologically left ventricle with the discordant ventriculo-arterial connection. The main diagnostic methods are echocardiography, angiocardiography and magnetic resonance imaging. In humans, heart transplantation is a radical method of surgical treatment. Palliative surgical management is different based on the underlying anatomy and include 2 or 3 stage defect correction with the exclusion of the rudimentary ventricle from the circulation. The focus of therapy is to management heart failure and pulmonary hypertension. The article describes the stages of diagnosis and treatment of UH in a Maine Coon.

Transfer of congenital heart patients from paediatric to adult services in England

ObjectiveThis study assessed the transfer of patients from paediatric cardiac to adult congenital heart disease (ACHD) services in England and the factors impacting on this process.MethodsThis retrospective cohort study used...

Serum miR-204 and miR-451 Expression and Diagnostic Value in Patients with Pulmonary Artery Hypertension Triggered by Congenital Heart Disease.

Objective To determine the level of expression and clinical importance of serum miR-204 and miR-451 in patients with pulmonary arterial hypertension caused by congenital heart disease (CHD-PAH). Methods From July 2019 to January 2021, 114 infants with congenital heart disease (CHD) were hospitalized at Qingdao's Fuwai Cardiovascular Hospital. They were grouped into categories: CHD (53 cases) and CHD-PAH (61 cases) based on whether they had pulmonary hypertension (PAH). In addition, 60 healthy children were selected as the control group. All children underwent routine biochemical examination, echocardiography, and pulmonary arterial pressure examination. By using an enzyme-linked immunosorbent assay (ELISA), the levels of brain natriuretic peptide (BNP) and asymmetric dimethylarginine (ADMA) in the blood were measured. Additionally, reverse transcription-polymerase chain reaction (RT-PCR) was used to determine the expression levels of miR-204 and miR-451 in peripheral blood. The correlation between miR-204, miR-451, BNP, ADMA, and mPAP was investigated using Pearson correlation analysis. Results Consequently, the TC, BNP, and ADMA serum levels were considerably higher in the CHD and CHD-PAH groups than in the control group (P < 0.05), whereas BNP and ADMA serum levels were significantly higher in the CHD-PAH group than in the CHD group (P < 0.05). According to RT-PCR data, the expression levels of miR-204 and miR-451 in the peripheral blood of children in the CHD and CHD-PAH groups were significantly lower (P < 0.05) when compared to the control group. The expression levels of miR-204 and miR-451 in the peripheral blood of children in the CHD-PAH group were substantially lower (P < 0.05) than in the CHD group. Significantly, the findings of the ROC curve revealed that the area under the curve (AUC) of CHD-PAH diagnosed by miR-204 and miR-451 alone was 0.737 and 0.725, respectively, and the AUC of joint diagnosis was 0.840, which was greater than that of single diagnosis (P < 0.05). Patients with CHD-PAH had lower levels of miR-204 and miR-451 in their blood, and this was found to be associated with BNP, ADMA, and mPAP, analyzed by Pearson correlation. Conclusions Children with CHD-PAH can be diagnosed based on aberrant expressions of miR-204 and miR-451 in their peripheral blood serum. Moreover, CHD-PAH can be diagnosed if the combination of miR-204 and miR-451 is detected as a biomarker, which has a higher diagnostic value.

Risk of maternal exposure to mixed air pollutants during pregnancy for congenital heart diseases in offspring

To explore the risk of maternal exposure to mixed air pollutants of particulate matter 1 (PM 1), particulate matter 2.5 (PM 2.5), particulate matter 10 (PM 10) and NO 2 for congenital heart disease (CHD) in offspring, and to estimate the ranked weights of the above pollutants. 6038 CHD patients and 5227 healthy controls from 40 medical institutions in 21 cities in Guangdong Registry of Congenital Heart Disease (GRCHD) from 2007 to 2016 were included. Logistic regression model was used to estimate the effect of maternal exposure to a single air pollutant on the occurrence of CHD in offspring. Spearman correlation coefficient was used to analyze the correlation between various pollutants, and Quantile g-computation was used to evaluate the joint effects of mixed exposure of air pollutants on CHD and the weights of various pollutants. The exposure levels of PM 1, PM 2.5, PM 10 and NO 2 in the CHD group were significantly higher than those in the control group (all P<0.01). The correlation coefficients among PM 1, PM 2.5, PM 10 and NO 2 were greater than 0.80. PM 1, PM 2.5, PM 10 and NO 2 exposure were associated with a significantly increased risk of CHD in offspring. Mixed exposure of these closely correlated pollutants presented much stronger effect on CHD than exposure of any single pollutants. There was a monotonic increasing relationship between mixed exposure and CHD risk. For each quantile increase in mixed exposure, the risk of CHD increased by 47% ( OR=1.47, 95% CI: 1.34-1.61). Mixed exposure had greater effect on CHD in the early pregnancy compared with middle and late pregnancy, but the greatest effect was the exposure in the whole pregnancy. The weight of PM 10 is the highest in the mixed exposure (81.3%). Maternal exposure to the mixture of air pollutants during pregnancy increases the risk of CHD in offspring, and the effect is much stronger than that of single exposure of various pollutants. PM 10 has the largest weights and the strongest effect in the mixed exposure.

Preliminary practice in the postoperative cardiopulmonary exercise testing for children with congenital heart disease

Objective: To investigate the postoperative cardiopulmonary fitness of children with congenital heart diseases (CHD). Methods: This is a retrospective study. A total of 136 children after cardiac correction undergoing cardiopulmonary exercise test (CPET) in Shanghai Children's Medical Center from March 1 to June 30, 2021 were selected. According to the surgical procedure, the children were divided into two groups: the biventricular correction group (BV group) (n=75) and single ventricular correction group (SV group) (n=61). The BV group was divided into two subgroups: simple congenital heart disease (S-CHD) group (n=35) and complex congenital heart disease (C-CHD) group (n=40). CPET parameters, including VO2 max, VO2/kg max, VO2/kg@AT, O2/HR max, HRR, PetCO2 max, CI, HRR at 1 min, VE/VCO2 slope, OUES/kg and EOV, were analyzed. CPET parameters of patients underwent different procedure were compared. In the subgroup analysis, CPET parameters of patients in S-CHD group and C-CHD group were compared. The parameters of the BV group were compared with the normal value. Linear correlation analysis was used to identify the correlation between the CPET parameters. Results: A total of 136 children with CHD after surgery were enrolled. The age was (9.4±3.1) (ranged 6.2-16.0) years, and there was 84 (61.8%) male. All the children completed CPET examination safely without experiencing serious circulation abnormalities. Compared with the SV group, the VO2/kg max ((32.6±6.9) ml·kg-1·min-1 vs. (23.5±5.9) ml·kg-1·min-1, P<0.001), O2/HR max ((7.24±2.93) ml/beat vs. (6.35±2.17)ml/beat, P=0.030), HRR at 1 min ((32.5±13.9) beat/min vs. (26.3±12.5) beat/min, P=0.036), OUES/kg (36.9±8.8 vs. 29.7±11.8, P=0.001) were significantly higher, VE/VCO2 slope (29.1±5.20 vs. 35.1±8.0, P<0.001) and incidence of EOV (32.0%(24/75) vs. 57.4%(31/61), P=0.027) were significantly lower in BV group. Compared with the simple CHD subgroup, VE/VCO2 slope and the incidence of EOV were higher, VO2/kg max, O2/HR max, HRR at 1 min and OUES/kg were lower in the complex CHD subgroup (all P<0.05). Cardiopulmonary function parameters of the BV group were lower than the normal value. Linear correlation analysis showed that VO2/kg@AT was strongly correlated with VO2/kg max (r=0.86, P<0.001), VO2/kg max was strongly correlated with OUES/kg (r=0.63, P<0.001), HRR was strongly correlated with CI (r=0.91, P<0.001), and VO2/kg max was strongly correlated with OUES/kg (r=0.63, P<0.001). VE/VCO2 slope was strongly correlated with PetCO2 max (r=1.00, P<0.001). Conclusions: The exercise cardiopulmonary function of children after single ventricular correction is weaker than that of biventricular correction, and the exercise tolerance of children after biventricular correction is lower than that of normal children. Among the children after biventricular correction, the exercise tolerance of children with complex CHD is lower than that of children with simple CHD. Postoperative CPET is of important realistic significance for CHD children.

Prevalence of Congenital Heart Disease in Chinese Children With Different Birth Weights and Its Relationship to the Neonatal Birth Weight.

ObjectiveThis study aimed to examine the prevalence and the related risk factors of congenital heart disease (CHD) in children with different birth weights in China and the relationship between the subtypes of CHD and birth weight (BW).MethodsThis study conducted a cross-sectional survey on the data collected in the children’s congenital heart disease database (CHDD) established in China. This database contained data from one Grade A, Level III Children’s Public Hospital in Zhengzhou, Henan. The study included all the children and their parents in the database from 2014 to 2020 as the study subjects, and the missing data were processed by means of imputation. Diagnoses of CHD were coded using the International Classification of Diseases version 10 (ICD-10), and subtypes were classified by the codes Q20 to Q26. We reported the prevalence of CHD based on birth weight and gestational age and analyzed the related risk factors for children with CHD in different birth weight groups and factors for children of the same birth weight groups between the CHD groups and the non-CHD groups. The generalized linear model was used to assess the association between the subtypes of CHD and BW by establishing three adjusting models, and the data were stratified for further analysis by urban-rural and infant gender.ResultsA total of 42,814 children were identified as having CHD among 5,071,799 live children; the overall prevalence of CHD was 8.44 per 1,000 live births during 2014–2020; and the three subtypes with the highest prevalence of CHD were atrial septal defect (ASD) (2.75‰), ventricular septal defect (VSD) (2.57‰), and patent foramen ovale (PFO) (1.12‰). The prevalence of CHD was 18.87‰ in the group with BW <1,500 g, 12.84‰ in the group with BW 1,500–2,500 g, 8.24‰ in the group with BW 2,500–4,000 g, and 4.80‰ in the group with BW ≥4,000 g. The prevalence of CHD was 16.62‰ in the small for gestational age (SGA) group, 6.99‰ in the appropriate for gestational age (AGA) group, and 6.40‰ in the larger for gestational age (LGA) group. Parental factors such as drinking, smoking, viral infections, peri-pregnancy exposure to radioactive substances, low family monthly expenditure, and low Apgar scores at 1 and 5 min were related to the increased risk of CHD in the offspring. Parental supplementation of folic acid and exercise during the peri-pregnancy period could reduce the risk of CHD in the offspring. The results of Model 3 adjusting for confounding variables showed that infants with ASD had a birth weight 461 g lower (95% CI: −1,085, –128), infants with VSD had a birth weight 426 g lower (95% CI: –932, –120), infants with tetralogy of Fallot (TOF) had a birth weight 532 g lower (95% CI: –987, –168), and without classification, infants with CHD had a birth weight 973 g lower (95% CI: –1,502, –204).ConclusionIn very low birth weight (VLBW) and low birth weight (LBW) infants, CHDs are more prevalent than in the general live-born population. Moreover, some peri-pregnancy factors of parents are closely related to the occurrence of CHD in offspring; different types of heart defects can lead to LBW. Therefore, if the fetus is found to have a heart defect during the prenatal examination, the mother should pay more attention to maintaining weight and ensuring that the fetus is within the normal weight range, thereby increasing the postpartum survival rate, reducing complications, and promoting children’s health.

Altered myelination in youth born with congenital heart disease.

Brain injury and dysmaturation is common in fetuses and neonates with congenital heart disease (CHD) and is hypothesized to result in persistent myelination deficits. This study aimed to quantify and compare myelin content in vivo between youth born with CHD and healthy controls. Youth aged 16 to 24 years born with CHD and healthy age‐ and sex‐matched controls underwent brain magnetic resonance imaging including multicomponent driven equilibrium single pulse observation of T1 and T2 (mcDESPOT). Average myelin water fraction (MWF) values for 33 white matter tracts, as well as a summary measure of average white matter MWF, the White Matter Myelination Index, were calculated and compared between groups. Tract‐average MWF was lower throughout the corpus callosum and in many bilateral association tracts and left hemispheric projection tracts in youth with CHD (N = 44) as compared to controls (N = 45). The White Matter Myelination Index was also lower in the CHD group. As such, this study provides specific evidence of widespread myelination deficits in youth with CHD, likely representing a long‐lasting consequence of early‐life brain dysmaturation in this population. This deficient myelination may underlie the frequent neurodevelopmental impairments experienced by CHD survivors and could eventually serve as a biomarker of neuropsychological function.

Utility of AlloMap® Scores in Younger Pediatric Heart Transplant Recipients

<h3>Purpose</h3> AlloMap® is used to predict acute cellular rejection (ACR) post heart transplant (HT) in pediatric patients > 15 years old. Data on its utility in a younger pediatric cohort are limited. We examine the correlation of AlloMap® score with hemodynamic, echocardiographic (echo), and endomyocardial biopsy (EMB) markers of rejection in HT recipients ≤ 15 years old. <h3>Methods</h3> Retrospective study of all pediatric patients ≤ 15 years old at our institution who had AlloMap® testing with routine cardiac catheterization and EMB, or when rejection was suspected based on clinical/echo evidence. <h3>Results</h3> 438 AlloMap® scores obtained from 57 children from 2010-2017. Median age at HT was 2.4 years (IQR 0.6-11.9 years) and 56% (n=32) were male. Indication for HT was cardiomyopathy (CM) in 56.1% (n=32) and congenital heart disease (CHD) in 43.9% (n=25) of patients. AlloMap® scores did not differ between CHD and CM groups (32.8 ± 3.5 vs 32.4 ± 3.7, p=.2). Scores were highest in those <2 years old. Scores differed between patients aged < 2 versus 2-10 years (p<.001) and between patients aged 2-10 and > 10 years old (p<.001). 32 rejection episodes were identified in 18 patients; 5 patients (27.8%) were < 1 year old. ACR grade 0/1R and 2R/3R were seen in 84.4% (n=27) and 15.6% (n=5), respectively. Those with ACR 0R had lower AlloMap® scores than those with ACR 1R (p=.024), but there was no difference between 1R and higher grade rejection. AlloMap® score weakly correlated with left ventricular end diastolic pressure (LVEDP) (r=.149, p=.026), but not with echo variables. <h3>Conclusion</h3> AlloMap® scores are similar in CM and CHD patients. Scores decrease with age, which may represent differing gene expression profiles over time. Scores are lower for those with ACR 0R versus 1R, but do not differ significantly between 1R and higher grade ACR. AlloMap® score correlates weakly with LVEDP, but not echo variables. Sample size is small with few rejection episodes. A larger study is needed to determine the utility of routine AlloMap® surveillance in HT recipients ≤ 15 years of age.