BACKGROUND Necrotizing enterocolitis(NEC)remains a prominent gastrointestinal emergency among infants,particularly term infants with congenital heart defects(CHD)being at high risk.The molecular processes that contrib...BACKGROUND Necrotizing enterocolitis(NEC)remains a prominent gastrointestinal emergency among infants,particularly term infants with congenital heart defects(CHD)being at high risk.The molecular processes that contribute to NEC have yet to be completely understood.The high mortality rates necessitate an active search for noninvasive biomarkers that can aid in the preclinical diagnosis and prognosis of NEC.MicroRNAs(miRs),which are involved in many biological processes in both health and disease,have been discovered to play an important role in regulating inflammation and immune responses via various signaling pathways.AIM To determine the plasma levels of miR-155,miR-221,miR-223,miR-320a,miR-451a as potential NEC biomarkers in term newborns with CHD.METHODS This prospective cohort study included twenty-tree term newborns with CHD who underwent cardiac surgery on the median day of life(DOL)=7.Nine of them developed NEC(Bell’s stage IIA and IIIA)within 1 week of cardiac surgery(NEC newborns).Blood samples were collected before(median DOL=5)and following(median DOL=13)cardiac surgery.Levels of plasma miR-155-5p,miR-221-3p,miR-223-3p,miR-320a-3p,and miR-451a were determined using real-time polymerase chain reaction.The functional analysis was executed using the DIANA-miRPath v4.0.RESULTS Preoperatively,NEC newborns had significantly lower plasma levels of miR-155(2.70-fold,P=0.020),miR-223(2.42-fold,P=0.030),and miR-320a(3.62-fold,P=0.006)than newborns without NEC.Postoperatively,miR-451a levels differed significantly between the newborn groups,showing a 4.70-fold decrease(P=0.014)in expression when clinical NEC symptoms appeared.According to receiver operating characteristic analysis,miR-320a was found to be the most effective predictive biomarker for NEC[area under the curve(AUC)=0.835,63%sensitivity,100%specificity],while miR-451a was identified as a NEC biomarker(AUC=0.835,85.7%sensitivity,76.9%specificity).Preoperatively,miR-155-5p,miR-223-3p,and miR-320a-3p were differentially expressed and targeted the forkhead box O and Hippo pathways(P<0.01).CONCLUSION Our study demonstrates,for the first time,that plasma miR-320a-3p levels can be used as a preclinical biomarker for NEC in term newborns with CHD.展开更多
BACKGROUND: Micro RNAs are small non-coding RNAs of approximately 22 nucleotides in length, and play important regulatory roles in normal heart development and the pathogenesis of heart diseases. Recently, a few prosp...BACKGROUND: Micro RNAs are small non-coding RNAs of approximately 22 nucleotides in length, and play important regulatory roles in normal heart development and the pathogenesis of heart diseases. Recently, a few prospective studies have implicated the diagnostic role of micro RNAs in congenital heart defects(CHD).DATA RESOURCES: This review retrieved the research articles in Pub Med focusing on the altered microR NAs in cardiac tissue or serum of patients with CHD versus healthy normal controls, as well as the studies exploring circulating microR NAs as potential biomarkers for(fetal) CHD.RESULTS: Most of the studies of interest were conducted in recent years, implicating that the topic in this review is a newly emerging field and is drawing much attention. Moreover, a number of differentially expressed microR NAs between CHD specimens and normal controls have been reported.CONCLUSION: Circulating micro RNAs may serve as potential biomarkers for diagnosis of CHD in the future, with more efforts paving the road to the aim.展开更多
Background:Given the pervasive issues of obesity and diabetes both in Puerto Rico and the broader United States,there is a compelling need to investigate the intricate interplay among body mass index(BMI),pregesta-tio...Background:Given the pervasive issues of obesity and diabetes both in Puerto Rico and the broader United States,there is a compelling need to investigate the intricate interplay among body mass index(BMI),pregesta-tional,and gestational maternal diabetes,and their potential impact on the occurrence of congenital heart defects(CHD)during neonatal development.Methods:Using the comprehensive System of Vigilance and Surveillance of Congenital Defects in Puerto Rico,we conducted a focused analysis on neonates diagnosed with CHD between 2016 and 2020.Our assessment encompassed a range of variables,including maternal age,gestational age,BMI,pregestational diabetes,gestational diabetes,hypertension,history of abortion,and presence of preeclampsia.Results:A cohort of 673 patients was included in our study.The average maternal age was 26 years,within a range of 22 to 32 years.The mean gestational age measured 39 weeks,with a median span of 38 to 39 weeks.Of the 673 patients,274(41%)mothers gave birth to neonates diagnosed with CHD.Within this group,22 cases were linked to pre-gestational diabetes,while 202 were not;20 instances were associated with gestational diabetes,compared to 200 without;and 148 cases exhibited an overweight or obese BMI,whereas 126 displayed a normal BMI.Conclusion:We identified a statistically significant correlation between pre-gestational diabetes mellitus and the occurrence of CHD.However,our analysis did not show a statistically significant association between maternal BMI and the likelihood of CHD.These results may aid in developing effective strategies to prevent and manage CHD in neonates.展开更多
Background:Research on fetal congenital heart defect(CHD)mostly focuses on etiology and mechanisms.However,studies on maternal complications or pathophysiology are limited.Our objective was to determine whether vascul...Background:Research on fetal congenital heart defect(CHD)mostly focuses on etiology and mechanisms.However,studies on maternal complications or pathophysiology are limited.Our objective was to determine whether vascular dysfunction exists in pregnant women carrying a fetus with congenital heart defects.Methods:We conducted a case-control study.27 cases of pregnant women carrying a fetus with major CHD admitted to our hospital for delivery between April 2021 and August 2022 were selected.Every case was matched with about 2 pregnant complication-free controls without fetal abnormalities.The proangiogenic and anti-angiogenic factors and pregnancy outcomes were compared.Results:The proangiogenic factors include vascular endothelial growth factor(VEGF)and placental growth factor(PlGF).The anti-angiogenic factors involve soluble fms-like tyrosine kinase 1(sFlt-1)and soluble endoglin(sEng).No differences were found in maternal plasma concentrations of PlGF,VEGF,and sFlt-1 between case-control groups when analyzed at 36 weeks≤gestational age(GA)<39 weeks and 39 weeks≤GA≤41 weeks.The concentrations of sEng in maternal plasma in the fetal CHD group were significantly higher than those in the control group:0.60(0.77)vs.0.32(0.26)ng/ml at 36 weeks≤GA<39 weeks,p=0.001 and 0.75(0.55)vs.0.28(0.27)ng/ml at 39 weeks≤GA≤41 weeks,p<0.001.Conclusion:Vascular dysfunction exists in pregnant women with fetal congenital heart defects,manifesting significantly elevated sEng concentration at delivery.展开更多
This review aims to sum up the improvements witnessed in the field of interventional cardiology during recent times. The last decade has witnessed significant technical advances in the field of radiological imaging an...This review aims to sum up the improvements witnessed in the field of interventional cardiology during recent times. The last decade has witnessed significant technical advances in the field of radiological imaging and also in interventional cardiology which has helped to offer more non-invasive solutions for the management of congenital heart defects. This has resulted from the use of advanced 3-dimensional fusion imaging instead of conventional 2-dimensional angiography, applying interactive real-time enhancement and using computed tomography and Magnetic Resonance Imaging for interventional procedures. Similarly the introduction of next generation devices, have not only improved the final outcome of the procedure but also has helped in reducing the challenges that were faced before and with the initial generation of devices. These advances have helped not only in reducing the radiation exposure, the use of contrast medium dose but also have resulted in improved early survival. The field of neonatal cardiology has advanced at an unprecedented pace. The transcatheter closure of patent ductus arteriosus has evolved over time and now it has been made possible at much lower body weight. Similarly, early use of stents for aortic coarctation has been found effective in some patients, especially when surgical intervention had been denied. The application of the hybrid approach for the management of complex congenital heart defects has also been effectively applied. More recently transcatheter placement of the pulmonary valve has been introduced for severely stenotic and/or regurgitant pulmonary valve in adolescents and adults. It is anticipated that in near future, this procedure would be available for relatively younger patients. In conclusion: last 2 decades have improvised pediatric interventional cardiology to incorporate less invasive solutions for CHD. The current advances in radio-diagnostic imaging, gadgetry and technical expertise have improved significantly and led to manage many of such defects by trans-catheter approach. This has led also, to replace the early surgical intervention with a more subtle hybrid approach, thus reducing not only the major surgical trauma but also been found to be cost-effective due to a shorter hospital stay. But a learning curve for performers is required for optimum results and also such procedures should be performed in a fully developed facility with an optimum surgical backup.展开更多
The objective of the work is to study the topographic and anatomical characteristics of the atrioventricular node’s artery in complex congenital heart defects.The material for the work was the following:44 specimens ...The objective of the work is to study the topographic and anatomical characteristics of the atrioventricular node’s artery in complex congenital heart defects.The material for the work was the following:44 specimens including hearts of fetuses and children of the first three years having congenital defects and 50 specimens of the hearts of a similar age with normal development as the control.A complex of generally accepted morphological methods was used to identify the conductive system of the heart,its blood supply.According to the data obtained given congenital heart defects,the artery of the atrioventricular node arises from an artery,that determines the type of blood supply to the heart.With the full form of the common atrioventricular canal,the presence of the“U”-shaped bend of the coronary artery at the point of departure of the atrioventricular artery from it is inconstant,which is apparently due to the localization of the atrioventricular node in the embryonic position.The intensity of the lateral branches from the artery of the atrioventricular node to the heart partitions is determined by the hemodynamic features with congenital heart defects.展开更多
Aim: To assess basic trends in epidemiology of congenital heart defects (CHDs). Method: Population based prospective observational study. Material: CHDs in infants born alive in a Norwegian county 1982-2011. Results: ...Aim: To assess basic trends in epidemiology of congenital heart defects (CHDs). Method: Population based prospective observational study. Material: CHDs in infants born alive in a Norwegian county 1982-2011. Results: In 828/71 217 infants (12 per 1000) a CHD was diagnosed. The prevalence increased from 8 to 12 per 1000 after introduction of early echocardiography in newborns with suspected CHD from 1986 (p = 0.0001). Ventricular septal defect (VSD) was the dominating CHD (474;57%). In 222 (27%) the CHDs were missed and diagnosed after discharge from hospital after birth. Twelve critical CHDs (causing death or requiring invasive procedures before 28 days of life) were missed. Prenatal diagnosis of critical CHDs increased from 4/67 (6%) born 1997-2006 to 4/11 (36%) born 2007-2011 (p = 0.01). In 177 (21%) a syndrome or extracardial defect occurred. The occurrence of CHDs associated with chromosomal disorders (60/73 (82%) trisomies) more than doubled from the cohort born in the first 10-year period 1982-1991 (0.6 per 1000) to the last 2002-2011 (1.4 per 1000) (p < 0.0001) in parallel with increasing births in women ≥ 35 years old in the population. 237 (29%) underwent therapeutic procedures (203 (86%) surgery, of whom 16 after initial catheter intervention, and 34 (14%) catheter intervention alone). 39/237 (16%) died, 101 (43%) were repaired and 97 (41%) had some minor residual defect. The death rate declined significantly from 65/532 (12%) born 1982-2001 to 11/296 (4%) born 2002-2011 (p = 0.0001). 37/76 (49%) deaths occurred within 28 days after birth. Conclusions: The rate of detection of CHDs increased substantially after introduction of echocardiography in newborns with suspected CHD, especially VSDs. Some critical CHDs were overlooked. The prenatal detection rate of such defects increased. The prevalence of CHDs with associated chromosomal disorders increased in parallel with increasing maternal age in the population. Most deaths occurred during the neonatal period. Increasing survival increases the load on long-term follow-up programmes.展开更多
Objective:Vertical right thoracotomy(VRAT)has become an alternative to sternotomy for the repair of non-complex congenital heart defects in our infants and children.Summary Background Data:Limited data exists on the c...Objective:Vertical right thoracotomy(VRAT)has become an alternative to sternotomy for the repair of non-complex congenital heart defects in our infants and children.Summary Background Data:Limited data exists on the comparison of the two approaches.Methods:The present study consisted of two groups;Group Ⅰ:(sternotomy;33 patients)and Group Ⅱ:(VRAT;35 patients).We compared the two groups on operative data,hours of invasive lines,narcotics used,length of stay,and total variable cost of stay.Results:The most frequent procedures were atrial and ventricular septal defect closure(25 patients,75.8%in Group Ⅰ)and(14 patients,40%in Group Ⅱ).The average age and weight were 33.43±53 months,and 14.7±16.9 kg for Group I,and 75.3±60.2 months and 24.9±18 kg for Group Ⅱ,respectively,(p<0.001).We found no differences in aortic cross-clamp/bypass times between groups(p=0.39 and 0.42,respectively).The use of narcotics was not significantly different between the two study groups(p=0.37)as was the total variable cost(p=0.115).Group Ⅱ had a lower time without invasive lines(p<0.001).In Group Ⅱ the total length of stay was significantly less as well(p<0.001).Conclusions:VRAT is a useful technique for repairing a wide range of heart defects and does not result in prolonged cardiopulmonary bypass or aortic cross-clamp times.Although total opioid use and total cost of stay are no different as with sternotomy,the shortened duration of invasive line use,and shorter length of stay make this approach worthy of consideration.展开更多
Background Di George syndrome（DGS） is the most common microdeletion syndrome in humans and a disorder caused by a defect in chromosome 22. Almost 80% of DGS patients manifest congenital heart defects（CHD）, which a...Background Di George syndrome（DGS） is the most common microdeletion syndrome in humans and a disorder caused by a defect in chromosome 22. Almost 80% of DGS patients manifest congenital heart defects（CHD）, which are highly variable and severe. However, the genetics of CHD in DGS remain elusive. This review concludes that the TBX1 gene plays a critical role in cardiovascular defects, involving many additional genes, such as Six1, Eya1, Fgf8, Fox, and Shh. Concerning the variable manifestations of CHD in DGS,additional modifiers have been shown of involvement, such as Wnt, MOZ, micro RNAs, VEGF, and CRK.Knowledge of the genetics underlying CHD in DGS has the potential to early detection and treatment of this disease.展开更多
Objective This study aimed to assess the associations between maternal drug use,cytochrome P450(CYP450)genetic polymorphisms,and their interactions with the risk of congenital heart defects(CHDs)in offspring.Methods A...Objective This study aimed to assess the associations between maternal drug use,cytochrome P450(CYP450)genetic polymorphisms,and their interactions with the risk of congenital heart defects(CHDs)in offspring.Methods A case-control study involving 569 mothers of CHD cases and 652 controls was conducted from November 2017 to January 2020.Results After adjusting for potential confounding factors,the results show that mothers who used ovulatory drugs(adjusted odds ratio[a OR]=2.12;95% confidence interval[CI]:1.08-4.16),antidepressants(a OR=2.56;95%CI:1.36-4.82),antiabortifacients(a OR=1.55;95%CI:1.00-2.40),or traditional Chinese drugs(a OR=1.97;95%CI:1.26-3.09)during pregnancy were at a significantly higher risk of CHDs in offspring.Maternal CYP450 genetic polymorphisms at rs1065852(A/T vs.A/A:OR=1.53,95%CI:1.10-2.14;T/T vs.A/A:OR=1.57,95%CI:1.07-2.31)and rs16947(G/G vs.C/C:OR=3.41,95%CI:1.82-6.39)were also significantly associated with the risk of CHDs in offspring.Additionally,significant interactions were observed between the CYP450 genetic variants and drug use on the development of CHDs.Conclusions In those of Chinese descent,ovulatory drugs,antidepressants,antiabortifacients,and traditional Chinese medicines may be associated with the risk of CHDs in offspring.Maternal CYP450 genes may regulate the effects of maternal drug exposure on fetal heart development.展开更多
Background:The management of suspected critical congenital heart defects(CCHD)relies on timely echocardiographic diagnosis.The availability of experienced echocardiographers is limited or even non-existent in many hos...Background:The management of suspected critical congenital heart defects(CCHD)relies on timely echocardiographic diagnosis.The availability of experienced echocardiographers is limited or even non-existent in many hospitals with obstetric units.This study evaluates remote-mentored echocardiography performed by physicians without experience in imaging of congenital heart defects(CHD).Methods:The setup included a pediatric cardiologist in a separate room,guiding a physician without experience in echocardiographic imaging of CHD in the examination of a symptomatic newborn.This remote-mentoring pair was blinded to the diagnosis of the newborn and presented with a simplified patient history.The echocardiographic images were streamed to the laptop of the mentor,along with a webcam feed showing the probe position.The task was to identify CCHD in need of immediate transfer to a pediatric cardiac surgical center.The result was compared to the previously completed echocardiographic report and the clinical decision of the patient-responsible pediatric cardiologist.Results:During 17 months,15 newborns were recruited.All six newborns with CCHD were correctly labeled by the remotementoring pair.One newborn with Tetralogy of Fallot was erroneously labeled as needing immediate transfer.Eight newborns without CCHD were correctly labeled.Conclusions:Remote-mentored echocardiography performed by examiners without experience in imaging CHD identified all newborns with CCHD in need of immediate transfer for specialist care.The setup shows promising results for improving the management of CCHD in hospitals without continuous pediatric cardiology service.展开更多
Objective To explore the multiple risk factors for family lifestyle of children with congenital heart defects(CHDs)in Shaanxi Province,China.Methods A 1∶1 case-control study was carried out to investigate 60 pairs of...Objective To explore the multiple risk factors for family lifestyle of children with congenital heart defects(CHDs)in Shaanxi Province,China.Methods A 1∶1 case-control study was carried out to investigate 60 pairs of children and their parents.The univariate and multivariable logistic regression models were used to analyze the influence of risk factors related to parents’lifestyle on CHDs.Results Several possible risk factors were found for CHDs,including fever(OR=4.465,P=0.017),pesticides contact(OR=2.234,P=0.083),passive smoking during pregnancy(OR=20.529,P=0.007)and father’s smoking(OR=3.342,P=0.005);fever(OR=2.428,P=0.012)and passive smoking during pregnancy(OR=1.201,P=0.037)were also correlated with ventricular sepal defect(VSD).Conclusion Fever,pesticides contact and passive smoking are associated with CHDs during pregnancy.We should focus our attention on health care during pregnancy to avoid the above-mentioned risk factors and call on parents to hold on to a good healthy lifestyle.展开更多
Background Cyanotic patients have potential growth retardation and malnutrition due to hypoxemia and other reasons. Ghrelin is a novel endogenous growth hormone secretagogue that has effects on growth and cardiovascul...Background Cyanotic patients have potential growth retardation and malnutrition due to hypoxemia and other reasons. Ghrelin is a novel endogenous growth hormone secretagogue that has effects on growth and cardiovascular activities. The aim of this study was to evaluate the plasma level and myocardial expression of ghrelin and insulin-like growth factor-1 （IGF-1） using an immature piglet model of chronic cyanotic congenital heart defects with decreased pulmonary blood flow. Methods Twelve weanling Chinese piglets underwent procedures of main pulmonary artery-left atrium shunt with pulmonary artery banding or sham operation as control. Four weeks later, hemodynamic parameters were measured. Enzyme-linked immunosorbent assay for plasma ghrelin and IGF-1 level measurement were performed. Ventricular ghrelin and IGF-1 mRNA expressions were measured by quantitative real-time polymerase chain reaction. Results Four weeks after surgical procedure, the cyanotic model produced lower arterial oxygen tension （（68.73±15.09） mmHg）, arterial oxygen saturation （（82.35±8.63）%）, and higher arterial carbon dioxide tension （（51.83±6.12） mmHg）, hematocrit （（42.67±3.83）%） and hemoglobin concentration （（138.17±16.73） g/L） than the control piglets （（194.08±98.79） mmHg, （96.43±7.91）%, （36.9±4.73） mmHg, （31.17±3.71）%, （109.83±13.75） g/L） （all P 〈0.05）. Plasma ghrelin level was significantly higher in the cyanotic model group in comparison to the control （P=0.004）, and the plasma IGF-1 level was significantly lower than control （P=0.030）. Compared with control animals, the expression of ghrelin mRNAs in the ventricular myocardium was significantly decreased in the cyanotic model group （P=0.000）, and the expression of IGF-1 mRNAs was elevated （P=0.001）. Conclusions Chronic cyanotic congenital heart defects model was successfully established. Plasma ghrelin level and myocardial IGF-1 mRNA expression were significantly up-regulated, while plasma iGF-1 level and myocardial ghrelin mRNA expression were down-regulated in the chronic cyanotic immature piglets. The ghrelin system may be an important part of the network regulating cardiac performance.展开更多
Congenital heart defect （CHD） is the most common fetal defects. Copy nmnber variations （CNVs） were demonstrated to be involved in the etiology of CHDs. We report three cases from a family diagnosed as CHDs with a ...Congenital heart defect （CHD） is the most common fetal defects. Copy nmnber variations （CNVs） were demonstrated to be involved in the etiology of CHDs. We report three cases from a family diagnosed as CHDs with a rare novel duplication ofXp22.33-p I 1.22. A 30-year-old woman, gmvida 2 para 0. Her first pregnancy at 2012 was diagnosed to be dichorionic twin pregnancy and her second pregnancy at 2014 was a singleton pregnancy. After a routine ultrasound scan at 22 week＇s gestation, all the fetuses were diagnosed with critical CHDs. The first fetus （male） exhibited tetralogy of Fallot, atrial septal defect, persistent left superior vena cava, and coronary sinus dilatation while the examination of the second fetus （male） revealed atrioventricular septal detect and hypoplastic left heart syndrome. The third fetus （t^male） was also diagnosed with an atrioventricular septaI defect and hypoplastic left heart syndrome. The parents decided to terminate the wegnancy.展开更多
Background Evidence remains limited on the association between maternal ozone(O,)exposure and congenital heart defects(CHDs)in offspring,and few studies have investigated the interaction and modification of paternal s...Background Evidence remains limited on the association between maternal ozone(O,)exposure and congenital heart defects(CHDs)in offspring,and few studies have investigated the interaction and modification of paternal smoking on this association.Methods Using a sample including pregnant women at high risk of fetal CHD(with metabolic disease,first-trimester viral infection,family history of CHD,etc.)from a maternal-fetal medicine study covering 1313 referral hospitals in China during 2013-2021,we examined the associations between maternal O_(3)exposure during 3-8 weeks of gestational age and fetal CHD in offspring and investigated the interaction and modification of paternal smoking on this association.CHD was diagnosed by fetal echocardiograms,maximum daily 8-hour average O,exposure data at a 10 km×10 km spatial resolution came from the Tracking Air Pollution in China dataset,and paternal smoking was collected using questionnaires.Logistic regression models were used to estimate adjusted odds ratios(ORs)and 95%confidence intervals(CIs).Results Among 27,834 pregnant women at high risk of fetal CHD,17.4%of fetuses were diagnosed with CHD.Each 10μg/m^(3)increase in maternal O_(3)exposure was associated with a 17%increased risk of CHD in offspring(OR=1.17,95%CI=1.14-1.20).Compared with paternal nonsmoking and maternal low O_(3)exposure,the ORs(95%CI)of CHD for smoking and low O_(3)exposure,nonsmoking and high O_(3)exposure,and smoking and high O_(3)exposure were 1.25(1.08-1.45),1.81(1.56-2.08),and 2.23(1.84-2.71),respectively.Paternal smoking cessation seemingly mitigated the increased risk of CHD.Conclusions Maternal O_(3)exposure and paternal smoking were interactively associated with an increased risk of fetal CHD in offspring,which calls for effective measures to decrease maternal exposure to O_(3)pollution and secondhand smoke for CHDprevention.展开更多
BACKGROUND Single-ventricle congenital heart disease often requires the Fontan procedure,which can lead to Fontan-associated liver disease(FALD)and multi-organ failure.Combined heart-liver transplantation(CHLT)is a po...BACKGROUND Single-ventricle congenital heart disease often requires the Fontan procedure,which can lead to Fontan-associated liver disease(FALD)and multi-organ failure.Combined heart-liver transplantation(CHLT)is a potential lifesaving option for these patients.AIMTo investigate the outcomes and complications of CHLT in patients with failing Fontan physiology.METHODSSeven retrospective studies of 121 patients undergoing CHLT were systematically reviewed. Quality was assessedwith the Newcastle-Ottawa Scale. A meta-analysis using random-effects models to calculate odds ratios (ORs) ormean differences (MDs) with 95% confidence intervals.RESULTSThe pooled 30-day, 1-year, 5-year, and 10-year survival rates after CHLT were 92.6%, 86.78%, 81.17%, and 77.8%,respectively. The mean intensive care unit and total hospital lengths of stay were 8.46 and 28.16 days. Meanischemic time was 267.29 minutes, while cardiopulmonary bypass time was 260.27 minutes. Infections (30%), renalreplacement therapy (36.84%), and graft rejection (12.34%) were notable complications. Compared to orthotopicheart transplantation (OHT), CHLT significantly reduced mortality (OR: 0.30, P = 0.009) and ischemic time (MD:–65.93 minutes), with no major differences in perioperative morbidity.CONCLUSIONCHLT offers a survival advantage over OHT for patients with FALD and failing Fontan physiology. Futureprospective studies are warranted to refine eligibility and improve long-term survival.展开更多
Objective:Many children with fully corrected congenital heart disease(CHD)avoid physicalactivity(PA).This descriptive study sought to determine child and parental factors that could predict PAlevels in Thai dhildren a...Objective:Many children with fully corrected congenital heart disease(CHD)avoid physicalactivity(PA).This descriptive study sought to determine child and parental factors that could predict PAlevels in Thai dhildren after corrective surgery.Methods:Ninety schoolaged children with fully correctedCHD were recruited from a cardiology clinic at a university hospital in northern Thailand.Data collectioninvolved five validated questionnaires:(1)the Modified Thai Adolescent's Fhysical Activity Questionnaire,(2)the Child Health Status Questionnaire-Forms I and II1,(3)the Parental Knowledge on School-agedChildrer's PhysicalActivity Scale,(4)the Perceived Self-efficacy to Physical Activity Questionnaire,and(5)the Parntal Bonding Instrument.The analysis included descriptive statistics and stepwise multipleregression.Results:Results showed that the physical activity(PA.)levels,assessed through total energyexpenditure,of child ran with CHD were below the recommended levels after treatment(t=-8.33,p<0.001).There were sigrificant correlations between PA and factors such as a child's perceived health status,PAself-efficacy,and parentaloverprotection(r=0.39,p<0.050;r=0.46,p<0.050;r=0.25,p<0.050,respectively).After adjustment for demographic data and CHD type,these factors had no association with a child's PA.Only two factors predicted childhood PA—the child's perceived health status and PA self-efficacy whichcould explain27.3%of the variance of PA.(p<0.001).Conclusion:The study highlights the necessity forinterventions to enhance childrer's perception of their health status and PA self-efficacy to increase theirphysical activity levels.展开更多
Background:The neutrophil-lymphocyte ratio(NLR)is a simple,cost-effective marker of systemic inflammation.This study aims to evaluate the association between perioperative NLR and postoperative outcomes in pediatric p...Background:The neutrophil-lymphocyte ratio(NLR)is a simple,cost-effective marker of systemic inflammation.This study aims to evaluate the association between perioperative NLR and postoperative outcomes in pediatric patients undergoing congenital heart surgery with cardiopulmonary bypass(CPB).Methods:We retrospectively reviewed 70 patients under 18 years of age who underwent surgery between 2018 and 2023.NLR was measured preoperatively and on postoperative days(POD)0,1,2,3,and 6.Receiver operating characteristic analysis identified optimal cutoffs,and associations with postoperative outcomes were assessed.Results:The preoperative NLR cutoff of 1.14(AUC=0.75)was associated with prolonged mechanical ventilation(>72 h)(p=0.02),extended intensive care unit(ICU)stay(p=0.004),and longer hospital stay(p=0.006).NLR values on POD3(AUC=0.74)and POD6(AUC=0.78)also demonstrated strong predictive ability for these outcomes.Conclusions:Elevated perioperative NLR-particularly preoperative values≥1.14-is associated with prolonged mechanical ventilation,longer ICU admission,and extended hospitalization in pediatric congenital heart surgery.NLR may serve as a practical biomarker for early risk stratification and postoperative management.展开更多
Background:Congenital heart disease(CHD)results from abnormal heart development during fetal development,leading to life-threatening complications.This study aimed to evaluate the feasibility of applying myocardial pa...Background:Congenital heart disease(CHD)results from abnormal heart development during fetal development,leading to life-threatening complications.This study aimed to evaluate the feasibility of applying myocardial parametric mapping in post-mortem magnetic resonance imaging and to examine differences in the left ventricular myocardium between fetuses with CHD and controls.Methods:This prospective case–control study was conducted on 14 deceased fetuses with CHD(CHD group)and 24 fetuses without CHD(control group).Fetuses with CHD were further stratified into the cyanotic(n=9)and non-cyanotic(n=5)CHD groups.T1,T2,and proton density relaxation times of the left ventricular myocardium were calculated and compared using multiple-dynamic multiple-echo post-mortem magnetic resonance imaging technology.Results:The myocardial T2 relaxation time was significantly different between the groups(p=0.033),with no difference in T1 or proton density relaxation times between the groups.A one-way analysis of variance with Tukey's test showed that the mean cyanotic CHD group showed a longer myocardial T2 relaxation time than the control group(98.00013.143 vs.83.5429.491 ms,p=0.003).Additionally,the correlation coefficient in the CHD group was significantly different between the myocardial T2 relaxation time and peak systolic velocity of pulmonary artery on a fetal echocardiogram(r2=0.681,p=0.010).Conclusions:These results suggest that using myocardial alterations in the T2 relaxation time may provide a accurate early warning for myocardial injury and enable noninvasive recognition of cardiac involvement in fetuses with CHD.展开更多
Objective:To perform a meta-analysis to evaluate the diagnostic performance of computed tomography(CT) and transthoracic echocardiography(TTE) in complex congenital heart diseases(CHD) in China.Methods:MEDLINE,Cochran...Objective:To perform a meta-analysis to evaluate the diagnostic performance of computed tomography(CT) and transthoracic echocardiography(TTE) in complex congenital heart diseases(CHD) in China.Methods:MEDLINE,Cochrane library and China National Knowledge Infrastructure(CNKI) database from January 1966 to October 2010,were searched for initial studies in China.All the studies,published in English or Chinese,used TTE,CT,or both as diagnostic tests for CHD and reported the rate of true-positive,true-negative,false-positive and false-negative diagnoses of CHD from TTE and CT findings with the surgical results as the 'gold-standard'(15 studies,XX patients) were collected.The statistic software package,'Meta-Disc 1.4',was used to conduct data analysis.A covariate analysis was used to evaluate the influence of patient or study-related factors on sensitivity.Results:Pooled sensitivity for diagnosis of CHD were 95% [95% confidence interval(CI):94%~96%] for CT studies and 87%(95% CI:85%~88%) for TTE studies.The difference between the pooled sensitivity of CT and that of TTE was statistically significant(P<0.001).TTE had higher sensitivity [0.96(95% CI:0.94~0.97)] for cardiac malformation but lower sensitivity [0.78(95% CI:0.76~0.81)] for extracardiac malformation than CT.Conclusion:CT can provide added diagnostic information compared with TTE in patients with CHD in China,especially for patients suspected of extracardiac malformation.展开更多
基金Supported by The Russian Science Foundation,No.19-75-20076.
文摘BACKGROUND Necrotizing enterocolitis(NEC)remains a prominent gastrointestinal emergency among infants,particularly term infants with congenital heart defects(CHD)being at high risk.The molecular processes that contribute to NEC have yet to be completely understood.The high mortality rates necessitate an active search for noninvasive biomarkers that can aid in the preclinical diagnosis and prognosis of NEC.MicroRNAs(miRs),which are involved in many biological processes in both health and disease,have been discovered to play an important role in regulating inflammation and immune responses via various signaling pathways.AIM To determine the plasma levels of miR-155,miR-221,miR-223,miR-320a,miR-451a as potential NEC biomarkers in term newborns with CHD.METHODS This prospective cohort study included twenty-tree term newborns with CHD who underwent cardiac surgery on the median day of life(DOL)=7.Nine of them developed NEC(Bell’s stage IIA and IIIA)within 1 week of cardiac surgery(NEC newborns).Blood samples were collected before(median DOL=5)and following(median DOL=13)cardiac surgery.Levels of plasma miR-155-5p,miR-221-3p,miR-223-3p,miR-320a-3p,and miR-451a were determined using real-time polymerase chain reaction.The functional analysis was executed using the DIANA-miRPath v4.0.RESULTS Preoperatively,NEC newborns had significantly lower plasma levels of miR-155(2.70-fold,P=0.020),miR-223(2.42-fold,P=0.030),and miR-320a(3.62-fold,P=0.006)than newborns without NEC.Postoperatively,miR-451a levels differed significantly between the newborn groups,showing a 4.70-fold decrease(P=0.014)in expression when clinical NEC symptoms appeared.According to receiver operating characteristic analysis,miR-320a was found to be the most effective predictive biomarker for NEC[area under the curve(AUC)=0.835,63%sensitivity,100%specificity],while miR-451a was identified as a NEC biomarker(AUC=0.835,85.7%sensitivity,76.9%specificity).Preoperatively,miR-155-5p,miR-223-3p,and miR-320a-3p were differentially expressed and targeted the forkhead box O and Hippo pathways(P<0.01).CONCLUSION Our study demonstrates,for the first time,that plasma miR-320a-3p levels can be used as a preclinical biomarker for NEC in term newborns with CHD.
文摘BACKGROUND: Micro RNAs are small non-coding RNAs of approximately 22 nucleotides in length, and play important regulatory roles in normal heart development and the pathogenesis of heart diseases. Recently, a few prospective studies have implicated the diagnostic role of micro RNAs in congenital heart defects(CHD).DATA RESOURCES: This review retrieved the research articles in Pub Med focusing on the altered microR NAs in cardiac tissue or serum of patients with CHD versus healthy normal controls, as well as the studies exploring circulating microR NAs as potential biomarkers for(fetal) CHD.RESULTS: Most of the studies of interest were conducted in recent years, implicating that the topic in this review is a newly emerging field and is drawing much attention. Moreover, a number of differentially expressed microR NAs between CHD specimens and normal controls have been reported.CONCLUSION: Circulating micro RNAs may serve as potential biomarkers for diagnosis of CHD in the future, with more efforts paving the road to the aim.
基金The San Juan Bautista School of Medicine’s Institutional Review Board approved the study(EMSJBIRB-7-2021).
文摘Background:Given the pervasive issues of obesity and diabetes both in Puerto Rico and the broader United States,there is a compelling need to investigate the intricate interplay among body mass index(BMI),pregesta-tional,and gestational maternal diabetes,and their potential impact on the occurrence of congenital heart defects(CHD)during neonatal development.Methods:Using the comprehensive System of Vigilance and Surveillance of Congenital Defects in Puerto Rico,we conducted a focused analysis on neonates diagnosed with CHD between 2016 and 2020.Our assessment encompassed a range of variables,including maternal age,gestational age,BMI,pregestational diabetes,gestational diabetes,hypertension,history of abortion,and presence of preeclampsia.Results:A cohort of 673 patients was included in our study.The average maternal age was 26 years,within a range of 22 to 32 years.The mean gestational age measured 39 weeks,with a median span of 38 to 39 weeks.Of the 673 patients,274(41%)mothers gave birth to neonates diagnosed with CHD.Within this group,22 cases were linked to pre-gestational diabetes,while 202 were not;20 instances were associated with gestational diabetes,compared to 200 without;and 148 cases exhibited an overweight or obese BMI,whereas 126 displayed a normal BMI.Conclusion:We identified a statistically significant correlation between pre-gestational diabetes mellitus and the occurrence of CHD.However,our analysis did not show a statistically significant association between maternal BMI and the likelihood of CHD.These results may aid in developing effective strategies to prevent and manage CHD in neonates.
基金supported by grants from the Guangzhou Municipal Science and Technology Bureau(Nos.202102080466,202201011423,202206010049,2023B03J0596,2023B03J1254,2023B03J1255)Department of Science and Technology of Guangdong Province(Nos.2020B1111170011,2023A1515012501)+1 种基金the Natural Science Foundation of Guangdong Province(Nos.2023A1515010801,2021A1515011445)the National Natural Science Foundation of China(Nos.82100371,81903287).
文摘Background:Research on fetal congenital heart defect(CHD)mostly focuses on etiology and mechanisms.However,studies on maternal complications or pathophysiology are limited.Our objective was to determine whether vascular dysfunction exists in pregnant women carrying a fetus with congenital heart defects.Methods:We conducted a case-control study.27 cases of pregnant women carrying a fetus with major CHD admitted to our hospital for delivery between April 2021 and August 2022 were selected.Every case was matched with about 2 pregnant complication-free controls without fetal abnormalities.The proangiogenic and anti-angiogenic factors and pregnancy outcomes were compared.Results:The proangiogenic factors include vascular endothelial growth factor(VEGF)and placental growth factor(PlGF).The anti-angiogenic factors involve soluble fms-like tyrosine kinase 1(sFlt-1)and soluble endoglin(sEng).No differences were found in maternal plasma concentrations of PlGF,VEGF,and sFlt-1 between case-control groups when analyzed at 36 weeks≤gestational age(GA)<39 weeks and 39 weeks≤GA≤41 weeks.The concentrations of sEng in maternal plasma in the fetal CHD group were significantly higher than those in the control group:0.60(0.77)vs.0.32(0.26)ng/ml at 36 weeks≤GA<39 weeks,p=0.001 and 0.75(0.55)vs.0.28(0.27)ng/ml at 39 weeks≤GA≤41 weeks,p<0.001.Conclusion:Vascular dysfunction exists in pregnant women with fetal congenital heart defects,manifesting significantly elevated sEng concentration at delivery.
文摘This review aims to sum up the improvements witnessed in the field of interventional cardiology during recent times. The last decade has witnessed significant technical advances in the field of radiological imaging and also in interventional cardiology which has helped to offer more non-invasive solutions for the management of congenital heart defects. This has resulted from the use of advanced 3-dimensional fusion imaging instead of conventional 2-dimensional angiography, applying interactive real-time enhancement and using computed tomography and Magnetic Resonance Imaging for interventional procedures. Similarly the introduction of next generation devices, have not only improved the final outcome of the procedure but also has helped in reducing the challenges that were faced before and with the initial generation of devices. These advances have helped not only in reducing the radiation exposure, the use of contrast medium dose but also have resulted in improved early survival. The field of neonatal cardiology has advanced at an unprecedented pace. The transcatheter closure of patent ductus arteriosus has evolved over time and now it has been made possible at much lower body weight. Similarly, early use of stents for aortic coarctation has been found effective in some patients, especially when surgical intervention had been denied. The application of the hybrid approach for the management of complex congenital heart defects has also been effectively applied. More recently transcatheter placement of the pulmonary valve has been introduced for severely stenotic and/or regurgitant pulmonary valve in adolescents and adults. It is anticipated that in near future, this procedure would be available for relatively younger patients. In conclusion: last 2 decades have improvised pediatric interventional cardiology to incorporate less invasive solutions for CHD. The current advances in radio-diagnostic imaging, gadgetry and technical expertise have improved significantly and led to manage many of such defects by trans-catheter approach. This has led also, to replace the early surgical intervention with a more subtle hybrid approach, thus reducing not only the major surgical trauma but also been found to be cost-effective due to a shorter hospital stay. But a learning curve for performers is required for optimum results and also such procedures should be performed in a fully developed facility with an optimum surgical backup.
文摘The objective of the work is to study the topographic and anatomical characteristics of the atrioventricular node’s artery in complex congenital heart defects.The material for the work was the following:44 specimens including hearts of fetuses and children of the first three years having congenital defects and 50 specimens of the hearts of a similar age with normal development as the control.A complex of generally accepted morphological methods was used to identify the conductive system of the heart,its blood supply.According to the data obtained given congenital heart defects,the artery of the atrioventricular node arises from an artery,that determines the type of blood supply to the heart.With the full form of the common atrioventricular canal,the presence of the“U”-shaped bend of the coronary artery at the point of departure of the atrioventricular artery from it is inconstant,which is apparently due to the localization of the atrioventricular node in the embryonic position.The intensity of the lateral branches from the artery of the atrioventricular node to the heart partitions is determined by the hemodynamic features with congenital heart defects.
基金the Vestfold Hospital Trust,supporting the study financially.
文摘Aim: To assess basic trends in epidemiology of congenital heart defects (CHDs). Method: Population based prospective observational study. Material: CHDs in infants born alive in a Norwegian county 1982-2011. Results: In 828/71 217 infants (12 per 1000) a CHD was diagnosed. The prevalence increased from 8 to 12 per 1000 after introduction of early echocardiography in newborns with suspected CHD from 1986 (p = 0.0001). Ventricular septal defect (VSD) was the dominating CHD (474;57%). In 222 (27%) the CHDs were missed and diagnosed after discharge from hospital after birth. Twelve critical CHDs (causing death or requiring invasive procedures before 28 days of life) were missed. Prenatal diagnosis of critical CHDs increased from 4/67 (6%) born 1997-2006 to 4/11 (36%) born 2007-2011 (p = 0.01). In 177 (21%) a syndrome or extracardial defect occurred. The occurrence of CHDs associated with chromosomal disorders (60/73 (82%) trisomies) more than doubled from the cohort born in the first 10-year period 1982-1991 (0.6 per 1000) to the last 2002-2011 (1.4 per 1000) (p < 0.0001) in parallel with increasing births in women ≥ 35 years old in the population. 237 (29%) underwent therapeutic procedures (203 (86%) surgery, of whom 16 after initial catheter intervention, and 34 (14%) catheter intervention alone). 39/237 (16%) died, 101 (43%) were repaired and 97 (41%) had some minor residual defect. The death rate declined significantly from 65/532 (12%) born 1982-2001 to 11/296 (4%) born 2002-2011 (p = 0.0001). 37/76 (49%) deaths occurred within 28 days after birth. Conclusions: The rate of detection of CHDs increased substantially after introduction of echocardiography in newborns with suspected CHD, especially VSDs. Some critical CHDs were overlooked. The prenatal detection rate of such defects increased. The prevalence of CHDs with associated chromosomal disorders increased in parallel with increasing maternal age in the population. Most deaths occurred during the neonatal period. Increasing survival increases the load on long-term follow-up programmes.
文摘Objective:Vertical right thoracotomy(VRAT)has become an alternative to sternotomy for the repair of non-complex congenital heart defects in our infants and children.Summary Background Data:Limited data exists on the comparison of the two approaches.Methods:The present study consisted of two groups;Group Ⅰ:(sternotomy;33 patients)and Group Ⅱ:(VRAT;35 patients).We compared the two groups on operative data,hours of invasive lines,narcotics used,length of stay,and total variable cost of stay.Results:The most frequent procedures were atrial and ventricular septal defect closure(25 patients,75.8%in Group Ⅰ)and(14 patients,40%in Group Ⅱ).The average age and weight were 33.43±53 months,and 14.7±16.9 kg for Group I,and 75.3±60.2 months and 24.9±18 kg for Group Ⅱ,respectively,(p<0.001).We found no differences in aortic cross-clamp/bypass times between groups(p=0.39 and 0.42,respectively).The use of narcotics was not significantly different between the two study groups(p=0.37)as was the total variable cost(p=0.115).Group Ⅱ had a lower time without invasive lines(p<0.001).In Group Ⅱ the total length of stay was significantly less as well(p<0.001).Conclusions:VRAT is a useful technique for repairing a wide range of heart defects and does not result in prolonged cardiopulmonary bypass or aortic cross-clamp times.Although total opioid use and total cost of stay are no different as with sternotomy,the shortened duration of invasive line use,and shorter length of stay make this approach worthy of consideration.
基金supported by the Major International(Regional)Joint Research Project of Ministry of Science and Technology of China(No.2010DFA32260/No.2008DFA31140)National Natural Science Foundation of China(No.81370230)+2 种基金Technology Foundation for Selected Overseas Chinese Scholar of Ministry of Human Resources and Social Security of China(Ping Zhu)Key Technologies Research and Development Program of China(No.2011BAI11B22)Guangdong Province Natural Science Fund(No.S2013010014009)
文摘Background Di George syndrome（DGS） is the most common microdeletion syndrome in humans and a disorder caused by a defect in chromosome 22. Almost 80% of DGS patients manifest congenital heart defects（CHD）, which are highly variable and severe. However, the genetics of CHD in DGS remain elusive. This review concludes that the TBX1 gene plays a critical role in cardiovascular defects, involving many additional genes, such as Six1, Eya1, Fgf8, Fox, and Shh. Concerning the variable manifestations of CHD in DGS,additional modifiers have been shown of involvement, such as Wnt, MOZ, micro RNAs, VEGF, and CRK.Knowledge of the genetics underlying CHD in DGS has the potential to early detection and treatment of this disease.
基金supported by the National Natural Science Foundation Program of China[82073653,81803313,and 81974019]China Postdoctoral Science Foundation[2020M682644]+6 种基金Hunan Provincial Science and Technology Talent Support Project(2020TJ-N07)Natural Science Foundation of Hunan Province[2018JJ2551]Hunan Provincial Key Research and Development Program[2018SK2063 and 2018SK2062]Open Project from NHC Key Laboratory of Birth Defect for Research and Prevention[KF2020006]National Key Research and Development Program of China[2018YFA0108700 and2017YFA0105602]Postgraduate Scientific Research Innovation Project of Hunan Province[grant number CX20200271]Fundamental Research Funds for the Central Universities of Central South University[grant number 2020zzts798]。
文摘Objective This study aimed to assess the associations between maternal drug use,cytochrome P450(CYP450)genetic polymorphisms,and their interactions with the risk of congenital heart defects(CHDs)in offspring.Methods A case-control study involving 569 mothers of CHD cases and 652 controls was conducted from November 2017 to January 2020.Results After adjusting for potential confounding factors,the results show that mothers who used ovulatory drugs(adjusted odds ratio[a OR]=2.12;95% confidence interval[CI]:1.08-4.16),antidepressants(a OR=2.56;95%CI:1.36-4.82),antiabortifacients(a OR=1.55;95%CI:1.00-2.40),or traditional Chinese drugs(a OR=1.97;95%CI:1.26-3.09)during pregnancy were at a significantly higher risk of CHDs in offspring.Maternal CYP450 genetic polymorphisms at rs1065852(A/T vs.A/A:OR=1.53,95%CI:1.10-2.14;T/T vs.A/A:OR=1.57,95%CI:1.07-2.31)and rs16947(G/G vs.C/C:OR=3.41,95%CI:1.82-6.39)were also significantly associated with the risk of CHDs in offspring.Additionally,significant interactions were observed between the CYP450 genetic variants and drug use on the development of CHDs.Conclusions In those of Chinese descent,ovulatory drugs,antidepressants,antiabortifacients,and traditional Chinese medicines may be associated with the risk of CHDs in offspring.Maternal CYP450 genes may regulate the effects of maternal drug exposure on fetal heart development.
基金This study was funded through a grant from the European Union's Project Horizon 2020 and 5G HEART,under Grant Agreement Number 857034[15]the Norwegian Association for Children with Congenital Heart Disease.
文摘Background:The management of suspected critical congenital heart defects(CCHD)relies on timely echocardiographic diagnosis.The availability of experienced echocardiographers is limited or even non-existent in many hospitals with obstetric units.This study evaluates remote-mentored echocardiography performed by physicians without experience in imaging of congenital heart defects(CHD).Methods:The setup included a pediatric cardiologist in a separate room,guiding a physician without experience in echocardiographic imaging of CHD in the examination of a symptomatic newborn.This remote-mentoring pair was blinded to the diagnosis of the newborn and presented with a simplified patient history.The echocardiographic images were streamed to the laptop of the mentor,along with a webcam feed showing the probe position.The task was to identify CCHD in need of immediate transfer to a pediatric cardiac surgical center.The result was compared to the previously completed echocardiographic report and the clinical decision of the patient-responsible pediatric cardiologist.Results:During 17 months,15 newborns were recruited.All six newborns with CCHD were correctly labeled by the remotementoring pair.One newborn with Tetralogy of Fallot was erroneously labeled as needing immediate transfer.Eight newborns without CCHD were correctly labeled.Conclusions:Remote-mentored echocardiography performed by examiners without experience in imaging CHD identified all newborns with CCHD in need of immediate transfer for specialist care.The setup shows promising results for improving the management of CCHD in hospitals without continuous pediatric cardiology service.
基金supported by the Tackling Project for Social Development of Committee of Science and Technology of Shaanxi Province(2003K10-G83)
文摘Objective To explore the multiple risk factors for family lifestyle of children with congenital heart defects(CHDs)in Shaanxi Province,China.Methods A 1∶1 case-control study was carried out to investigate 60 pairs of children and their parents.The univariate and multivariable logistic regression models were used to analyze the influence of risk factors related to parents’lifestyle on CHDs.Results Several possible risk factors were found for CHDs,including fever(OR=4.465,P=0.017),pesticides contact(OR=2.234,P=0.083),passive smoking during pregnancy(OR=20.529,P=0.007)and father’s smoking(OR=3.342,P=0.005);fever(OR=2.428,P=0.012)and passive smoking during pregnancy(OR=1.201,P=0.037)were also correlated with ventricular sepal defect(VSD).Conclusion Fever,pesticides contact and passive smoking are associated with CHDs during pregnancy.We should focus our attention on health care during pregnancy to avoid the above-mentioned risk factors and call on parents to hold on to a good healthy lifestyle.
基金This work was supported by a grant from the National Natural Science Foundation of China （No. 30972958）.
文摘Background Cyanotic patients have potential growth retardation and malnutrition due to hypoxemia and other reasons. Ghrelin is a novel endogenous growth hormone secretagogue that has effects on growth and cardiovascular activities. The aim of this study was to evaluate the plasma level and myocardial expression of ghrelin and insulin-like growth factor-1 （IGF-1） using an immature piglet model of chronic cyanotic congenital heart defects with decreased pulmonary blood flow. Methods Twelve weanling Chinese piglets underwent procedures of main pulmonary artery-left atrium shunt with pulmonary artery banding or sham operation as control. Four weeks later, hemodynamic parameters were measured. Enzyme-linked immunosorbent assay for plasma ghrelin and IGF-1 level measurement were performed. Ventricular ghrelin and IGF-1 mRNA expressions were measured by quantitative real-time polymerase chain reaction. Results Four weeks after surgical procedure, the cyanotic model produced lower arterial oxygen tension （（68.73±15.09） mmHg）, arterial oxygen saturation （（82.35±8.63）%）, and higher arterial carbon dioxide tension （（51.83±6.12） mmHg）, hematocrit （（42.67±3.83）%） and hemoglobin concentration （（138.17±16.73） g/L） than the control piglets （（194.08±98.79） mmHg, （96.43±7.91）%, （36.9±4.73） mmHg, （31.17±3.71）%, （109.83±13.75） g/L） （all P 〈0.05）. Plasma ghrelin level was significantly higher in the cyanotic model group in comparison to the control （P=0.004）, and the plasma IGF-1 level was significantly lower than control （P=0.030）. Compared with control animals, the expression of ghrelin mRNAs in the ventricular myocardium was significantly decreased in the cyanotic model group （P=0.000）, and the expression of IGF-1 mRNAs was elevated （P=0.001）. Conclusions Chronic cyanotic congenital heart defects model was successfully established. Plasma ghrelin level and myocardial IGF-1 mRNA expression were significantly up-regulated, while plasma iGF-1 level and myocardial ghrelin mRNA expression were down-regulated in the chronic cyanotic immature piglets. The ghrelin system may be an important part of the network regulating cardiac performance.
基金This work was supported by grants from the National Natural Science Foundation of China （N o. 81071159）, Beijing Municipal Science and Technology Commission （No. Z 141107002514006） and Beijing Municipal Administration of Hospital Clinical Medicine Development of Special Foundation （No, XMLX201310）.
文摘Congenital heart defect （CHD） is the most common fetal defects. Copy nmnber variations （CNVs） were demonstrated to be involved in the etiology of CHDs. We report three cases from a family diagnosed as CHDs with a rare novel duplication ofXp22.33-p I 1.22. A 30-year-old woman, gmvida 2 para 0. Her first pregnancy at 2012 was diagnosed to be dichorionic twin pregnancy and her second pregnancy at 2014 was a singleton pregnancy. After a routine ultrasound scan at 22 week＇s gestation, all the fetuses were diagnosed with critical CHDs. The first fetus （male） exhibited tetralogy of Fallot, atrial septal defect, persistent left superior vena cava, and coronary sinus dilatation while the examination of the second fetus （male） revealed atrioventricular septal detect and hypoplastic left heart syndrome. The third fetus （t^male） was also diagnosed with an atrioventricular septaI defect and hypoplastic left heart syndrome. The parents decided to terminate the wegnancy.
基金supported by the National Natural Science Foundation of China(82073573 to ZZY,U21A20523 to HYH)the Beijing Key Laboratory of Maternal-Fetal Medicine in Fetal Heart Disease(BZ0308 to HYH)the National Key Research and Development Program of China(2022YFC3703502 to LJ).
文摘Background Evidence remains limited on the association between maternal ozone(O,)exposure and congenital heart defects(CHDs)in offspring,and few studies have investigated the interaction and modification of paternal smoking on this association.Methods Using a sample including pregnant women at high risk of fetal CHD(with metabolic disease,first-trimester viral infection,family history of CHD,etc.)from a maternal-fetal medicine study covering 1313 referral hospitals in China during 2013-2021,we examined the associations between maternal O_(3)exposure during 3-8 weeks of gestational age and fetal CHD in offspring and investigated the interaction and modification of paternal smoking on this association.CHD was diagnosed by fetal echocardiograms,maximum daily 8-hour average O,exposure data at a 10 km×10 km spatial resolution came from the Tracking Air Pollution in China dataset,and paternal smoking was collected using questionnaires.Logistic regression models were used to estimate adjusted odds ratios(ORs)and 95%confidence intervals(CIs).Results Among 27,834 pregnant women at high risk of fetal CHD,17.4%of fetuses were diagnosed with CHD.Each 10μg/m^(3)increase in maternal O_(3)exposure was associated with a 17%increased risk of CHD in offspring(OR=1.17,95%CI=1.14-1.20).Compared with paternal nonsmoking and maternal low O_(3)exposure,the ORs(95%CI)of CHD for smoking and low O_(3)exposure,nonsmoking and high O_(3)exposure,and smoking and high O_(3)exposure were 1.25(1.08-1.45),1.81(1.56-2.08),and 2.23(1.84-2.71),respectively.Paternal smoking cessation seemingly mitigated the increased risk of CHD.Conclusions Maternal O_(3)exposure and paternal smoking were interactively associated with an increased risk of fetal CHD in offspring,which calls for effective measures to decrease maternal exposure to O_(3)pollution and secondhand smoke for CHDprevention.
文摘BACKGROUND Single-ventricle congenital heart disease often requires the Fontan procedure,which can lead to Fontan-associated liver disease(FALD)and multi-organ failure.Combined heart-liver transplantation(CHLT)is a potential lifesaving option for these patients.AIMTo investigate the outcomes and complications of CHLT in patients with failing Fontan physiology.METHODSSeven retrospective studies of 121 patients undergoing CHLT were systematically reviewed. Quality was assessedwith the Newcastle-Ottawa Scale. A meta-analysis using random-effects models to calculate odds ratios (ORs) ormean differences (MDs) with 95% confidence intervals.RESULTSThe pooled 30-day, 1-year, 5-year, and 10-year survival rates after CHLT were 92.6%, 86.78%, 81.17%, and 77.8%,respectively. The mean intensive care unit and total hospital lengths of stay were 8.46 and 28.16 days. Meanischemic time was 267.29 minutes, while cardiopulmonary bypass time was 260.27 minutes. Infections (30%), renalreplacement therapy (36.84%), and graft rejection (12.34%) were notable complications. Compared to orthotopicheart transplantation (OHT), CHLT significantly reduced mortality (OR: 0.30, P = 0.009) and ischemic time (MD:–65.93 minutes), with no major differences in perioperative morbidity.CONCLUSIONCHLT offers a survival advantage over OHT for patients with FALD and failing Fontan physiology. Futureprospective studies are warranted to refine eligibility and improve long-term survival.
基金the Chiang Mai University Junior ResearchFellowship Program.
文摘Objective:Many children with fully corrected congenital heart disease(CHD)avoid physicalactivity(PA).This descriptive study sought to determine child and parental factors that could predict PAlevels in Thai dhildren after corrective surgery.Methods:Ninety schoolaged children with fully correctedCHD were recruited from a cardiology clinic at a university hospital in northern Thailand.Data collectioninvolved five validated questionnaires:(1)the Modified Thai Adolescent's Fhysical Activity Questionnaire,(2)the Child Health Status Questionnaire-Forms I and II1,(3)the Parental Knowledge on School-agedChildrer's PhysicalActivity Scale,(4)the Perceived Self-efficacy to Physical Activity Questionnaire,and(5)the Parntal Bonding Instrument.The analysis included descriptive statistics and stepwise multipleregression.Results:Results showed that the physical activity(PA.)levels,assessed through total energyexpenditure,of child ran with CHD were below the recommended levels after treatment(t=-8.33,p<0.001).There were sigrificant correlations between PA and factors such as a child's perceived health status,PAself-efficacy,and parentaloverprotection(r=0.39,p<0.050;r=0.46,p<0.050;r=0.25,p<0.050,respectively).After adjustment for demographic data and CHD type,these factors had no association with a child's PA.Only two factors predicted childhood PA—the child's perceived health status and PA self-efficacy whichcould explain27.3%of the variance of PA.(p<0.001).Conclusion:The study highlights the necessity forinterventions to enhance childrer's perception of their health status and PA self-efficacy to increase theirphysical activity levels.
文摘Background:The neutrophil-lymphocyte ratio(NLR)is a simple,cost-effective marker of systemic inflammation.This study aims to evaluate the association between perioperative NLR and postoperative outcomes in pediatric patients undergoing congenital heart surgery with cardiopulmonary bypass(CPB).Methods:We retrospectively reviewed 70 patients under 18 years of age who underwent surgery between 2018 and 2023.NLR was measured preoperatively and on postoperative days(POD)0,1,2,3,and 6.Receiver operating characteristic analysis identified optimal cutoffs,and associations with postoperative outcomes were assessed.Results:The preoperative NLR cutoff of 1.14(AUC=0.75)was associated with prolonged mechanical ventilation(>72 h)(p=0.02),extended intensive care unit(ICU)stay(p=0.004),and longer hospital stay(p=0.006).NLR values on POD3(AUC=0.74)and POD6(AUC=0.78)also demonstrated strong predictive ability for these outcomes.Conclusions:Elevated perioperative NLR-particularly preoperative values≥1.14-is associated with prolonged mechanical ventilation,longer ICU admission,and extended hospitalization in pediatric congenital heart surgery.NLR may serve as a practical biomarker for early risk stratification and postoperative management.
基金supported by Zhejiang Provincial Natural Science Foundation of China(Grant/Award number:ZCLTGY24H0401)Education Department of Zhejiang Province(Grant/Award number:Y202352970).
文摘Background:Congenital heart disease(CHD)results from abnormal heart development during fetal development,leading to life-threatening complications.This study aimed to evaluate the feasibility of applying myocardial parametric mapping in post-mortem magnetic resonance imaging and to examine differences in the left ventricular myocardium between fetuses with CHD and controls.Methods:This prospective case–control study was conducted on 14 deceased fetuses with CHD(CHD group)and 24 fetuses without CHD(control group).Fetuses with CHD were further stratified into the cyanotic(n=9)and non-cyanotic(n=5)CHD groups.T1,T2,and proton density relaxation times of the left ventricular myocardium were calculated and compared using multiple-dynamic multiple-echo post-mortem magnetic resonance imaging technology.Results:The myocardial T2 relaxation time was significantly different between the groups(p=0.033),with no difference in T1 or proton density relaxation times between the groups.A one-way analysis of variance with Tukey's test showed that the mean cyanotic CHD group showed a longer myocardial T2 relaxation time than the control group(98.00013.143 vs.83.5429.491 ms,p=0.003).Additionally,the correlation coefficient in the CHD group was significantly different between the myocardial T2 relaxation time and peak systolic velocity of pulmonary artery on a fetal echocardiogram(r2=0.681,p=0.010).Conclusions:These results suggest that using myocardial alterations in the T2 relaxation time may provide a accurate early warning for myocardial injury and enable noninvasive recognition of cardiac involvement in fetuses with CHD.
文摘Objective:To perform a meta-analysis to evaluate the diagnostic performance of computed tomography(CT) and transthoracic echocardiography(TTE) in complex congenital heart diseases(CHD) in China.Methods:MEDLINE,Cochrane library and China National Knowledge Infrastructure(CNKI) database from January 1966 to October 2010,were searched for initial studies in China.All the studies,published in English or Chinese,used TTE,CT,or both as diagnostic tests for CHD and reported the rate of true-positive,true-negative,false-positive and false-negative diagnoses of CHD from TTE and CT findings with the surgical results as the 'gold-standard'(15 studies,XX patients) were collected.The statistic software package,'Meta-Disc 1.4',was used to conduct data analysis.A covariate analysis was used to evaluate the influence of patient or study-related factors on sensitivity.Results:Pooled sensitivity for diagnosis of CHD were 95% [95% confidence interval(CI):94%~96%] for CT studies and 87%(95% CI:85%~88%) for TTE studies.The difference between the pooled sensitivity of CT and that of TTE was statistically significant(P<0.001).TTE had higher sensitivity [0.96(95% CI:0.94~0.97)] for cardiac malformation but lower sensitivity [0.78(95% CI:0.76~0.81)] for extracardiac malformation than CT.Conclusion:CT can provide added diagnostic information compared with TTE in patients with CHD in China,especially for patients suspected of extracardiac malformation.
