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
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
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
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 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
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:Children with congenital heart disease(CHD),even after surgical approaches,and especially those who undergo staged procedures in thefirst months of life,remain vulnerable to readmissions and complications,re...Background:Children with congenital heart disease(CHD),even after surgical approaches,and especially those who undergo staged procedures in thefirst months of life,remain vulnerable to readmissions and complications,requiring very close monitoring and differentiated intervention strategies.Methods:Descriptive and exploratory study,of the experience report type,which presents the process of building the high-risk outpatient clinic for complex congenital heart diseases(AAR)at the Instituto do Coração(InCor).Results:Report of the path taken to structure the AAR,demonstrating the organization,interface with the multidisciplinary team,admission and discharge criteria,training,and patient profile.In thesefive years of care,275 patients were treated,59.65%with biventricular interstage physiology,followed by univentricular interstage physiology(34.55%),residual defects after surgical procedures(3.63%),tumors with risk of mechanical obstruction(1.45%)and patients with an intrauterine approach(0.72%).The significant number of critical patients who were successfully discharged from hospital(44.72%),the low mortality rate due to sudden deaths at home and the high adherence to follow-up cor-roborate the impact of this specialized assistance.Conclusions:Despite the limitations of the study,this experi-ence report showed that with few resources,there is the possibility of organizing an AAR with differentiated care,with the objective of early detection and treatment of residual injuries,identification of early interventions,edu-cation of parents for follow-up of their children,resulting in individualized treatment,promoting a better quality of life for this population.展开更多
Congenital ventricular aneurysm is a very rare cardiac anomaly.A diagnosis can be made during the prenatal period using fetal echocardiography.This study presents a very rare apically located left ventricular aneurysm...Congenital ventricular aneurysm is a very rare cardiac anomaly.A diagnosis can be made during the prenatal period using fetal echocardiography.This study presents a very rare apically located left ventricular aneurysm case,and the relevant literature was reviewed and discussed.In this case,a 35-year-old,gravida 2,parity 1 preg-nant woman at 24 weeks of gestation,displayed a wide aneurysmal image in the left ventricular apical wall on fetal echocardiography.There was a 1.79 mm muscular ventricular septal defect at the apical region of the interven-tricular septum.In the course of the color Doppler ultrasonography examination,an aberrantfibrous band within the left ventricle and consequent turbulentflow during systole were observed.The baby,born via cesarean section at 37 weeks of gestation,is now in its postnatal seventh month.However,during echocardiographic follow-ups,changes have been observed,including mild to moderate mitral insufficiency and a decrease in systolic function.Despite thesefindings,the clinical condition remains asymptomatic.It is of great importance to use a multidis-ciplinary approach in managing these rare cases that could lead to potential adverse outcomes during the antena-tal or postnatal periods.展开更多
Objective:The objective of this analysis is to describe the characteristics and morbidity during hospitalizations among adolescents with congenital heart disease(AdoCHD)from the Pediatric Health Information System(PHI...Objective:The objective of this analysis is to describe the characteristics and morbidity during hospitalizations among adolescents with congenital heart disease(AdoCHD)from the Pediatric Health Information System(PHIS)database.Methods:The PHIS database was queried for all AdoCHD admissions aged 12–18 years(1/1/2004–12/31/2013).Major forms of CHD were identified by their International Classification of Diseases,ninth revision codes,further verified based on their secondary diagnosis and/or procedure codes.Patient characteristics,diagnoses,procedures and vital status were assessed.Results:In total,there were 4,267 adolescents admitted to 42 Children’s Hospitals,58.3%were males,24.6%single ventricle(SV)patients,64.1%bi-ventricle(BV),and 11.3%could not be classified.They accounted for 8,512 hospitalizations(41,240 total hospital days),of which 31.6%were intensive care unit(ICU)stays.ICU stay was similar for the SVand BV patients with similar duration of mechanical ventilation between the two groups.Overall,the most common CHD among in-patients was tetralogy of Fallot(TOF,36.4%).Larger proportion of the BVAdoCHD admissions were for elective surgical and electrophysiological procedures.There were 109(2.5%)heart transplantations(1.3%SV vs.0.6%BV)and 120 in-hospital deaths(2.8%)(1.1%SV vs.1.3%BV).Hypoplastic left heart syndrome was the most common diagnosis in transplanted patients(46%)and those who died(28%);TOF(29%)was frequent in 91(2.1%)patients who had cardiac arrests.Conclusions:Different hospitalization patterns exist for BV and SV AdoCHD.Recognizing this risk may encourage directing resources toward optimizing long-term care of CHD patients.展开更多
BACKGROUND Pulmonary arterial hypertension(PAH)in pregnancy is one of the major obstetric complications and is considered a contraindication to pregnancy as it is classified as a class IV risk in the revised risk clas...BACKGROUND Pulmonary arterial hypertension(PAH)in pregnancy is one of the major obstetric complications and is considered a contraindication to pregnancy as it is classified as a class IV risk in the revised risk classification of pregnancy by the World Health Organisation.Pregnancy,with its adaptive and expectant mechanical and hormonal changes,negatively affects the cardiopulmonary circulation in pregnant women.Do patients with repaired simple congenital heart disease(CHD)develop other pulmonary and cardiac complications during pregnancy?Can pregnant women with sudden pulmonary hypertension be treated and managed in time?In this paper,we present a case of a 39-year-old woman who underwent cesarean section at 33 wk'gestation and developed PAH secondary to repaired simple CHD.Our research began by a PubMed search for"pulmonary hypertension"and"pregnancy"and"CHD"case reports.Three cases were selected to review PAH in pregnancy after correction of CHD defects.These studies were reviewed,coupled with our own clinical experience.CASE SUMMARY Herein,a case involving a woman who underwent atrial septal defect repair at the age of 34,became pregnant five years later,and had a sudden onset of PAH and right heart failure secondary to symptoms of acute peripheral edema in the third trimester of her pregnancy.As a result,the patient underwent a cesarean section and gave birth to healthy twins.Within three days after cesarean delivery,her cardiac function deteriorated as the pulmonary artery pressure increased.Effec-tive postpartum management,including diuresis,significant oxygen uptake,vasodilators,capacity and anticoagulants management,led to improvements in cardiac function and oxygenation.The patient was discharged from hospital with a stable recovery and transferred to local hospitals for further PAH treatment.CONCLUSION This case served as a reminder to obstetricians of the importance of pregnancy after repair of CHD.It is crucial for patients with CHD to receive early correction.It suggests doctors should not ignore edema of twin pregnancy.Also,it provides a reference for the further standardization of antenatal,in-trapartum and postpartum management for patients with CHD worldwide.展开更多
Early intervention to prevent premature mortality is vital for adults with congenital heart defects(CHD).Anatomic complexity and comorbid conditions are thought to contribute to CHD mortality.Since hypertension(HTN)an...Early intervention to prevent premature mortality is vital for adults with congenital heart defects(CHD).Anatomic complexity and comorbid conditions are thought to contribute to CHD mortality.Since hypertension(HTN)and heart failure(HF)are the comorbid conditions among the most prevalent causes of death in the United States,and commonly accompany CHD,it is crucial to evaluate whether they are reliable predictors of mortality for adults with CHD(ACHD)independent of anatomic CHD complexity.A retrospective cross-sectional analysis of ACHD,aged 18–64,with concomitant HTN and/or HF and at least one health care encounter during 2008–2010 were assessed.Of 5,397 ACHD patients(18.3%HTN without HF,4.4%HF without HTN,8.3%with both),3.0%died(n=163)during the study period.Overall,the sample was 45.1%white,61.4%female,and 29.0%had a complex CHD.Among those who died,23.3%had HTN without HF,17.2%had HF without HTN,and 42.3%had both.Crude analyses revealed that older age,male gender,black race,and having public health insurance were associated with increased mortality during the three-year study period compared to ACHD patients who were younger,female gender,white race,and covered by private health insurance.ACHD patients diagnosed with non-complex CHD lesions(i.e.,shunts,valves,or shunts+valves)were at greater risk of dying compared to those with severe complex CHDs.When CHD type was assessed separately,those with valve lesions were more likely to die compared to those with complex CHD lesions.After adjustment for age,gender,race,insurance and CHD complexity,ACHD patients with HF,with or without HTN,were equally likely to die during the study period.However,ACHD patients with HF,without or without HTN,who had valve defects were more likely to die during the three-year study period compared to patients with complex CHDs.展开更多
Congenital heart defect,accounting for about 30%of congenital defects,is the most common one.Data shows that congenital heart defects have seriously affected the birth rate of healthy newborns.In Fetal andNeonatal Car...Congenital heart defect,accounting for about 30%of congenital defects,is the most common one.Data shows that congenital heart defects have seriously affected the birth rate of healthy newborns.In Fetal andNeonatal Cardiology,medical imaging technology(2D ultrasonic,MRI)has been proved to be helpful to detect congenital defects of the fetal heart and assists sonographers in prenatal diagnosis.It is a highly complex task to recognize 2D fetal heart ultrasonic standard plane(FHUSP)manually.Compared withmanual identification,automatic identification through artificial intelligence can save a lot of time,ensure the efficiency of diagnosis,and improve the accuracy of diagnosis.In this study,a feature extraction method based on texture features(Local Binary Pattern LBP and Histogram of Oriented Gradient HOG)and combined with Bag of Words(BOW)model is carried out,and then feature fusion is performed.Finally,it adopts Support VectorMachine(SVM)to realize automatic recognition and classification of FHUSP.The data includes 788 standard plane data sets and 448 normal and abnormal plane data sets.Compared with some other methods and the single method model,the classification accuracy of our model has been obviously improved,with the highest accuracy reaching 87.35%.Similarly,we also verify the performance of the model in normal and abnormal planes,and the average accuracy in classifying abnormal and normal planes is 84.92%.The experimental results show that thismethod can effectively classify and predict different FHUSP and can provide certain assistance for sonographers to diagnose fetal congenital heart disease.展开更多
Development in medical intervention has significantly decreased the mortality rates for children with complex congenital heart disease(CHD)but among these survivors with complex heart disease there occurs a uniq...Development in medical intervention has significantly decreased the mortality rates for children with complex congenital heart disease(CHD)but among these survivors with complex heart disease there occurs a unique pattern of neuro-developmental and neuropsychology impairment characterized social interaction impairment,impulsive Behavior,and impaired executive functions.Presence of behavioral problem is found significantly high in pediatric population with chronic illness than children with absence of chronic illness.The sample of 200 children with congenital heart defect was selected between age 4-8 years using multistage stratified sampling.The childhood psychopathology measurement schedule(CPMS)by Dr.Savitha Malhotra was used for assessing Behavioral problems present in children with CHD.Pre-Post experimental design was used to investigate the study and the results were statistically analyzed using paired T test.The result revealed that the effectiveness of intervention program to retrain Behavior showed high significance.With increased survival rates,the aim of the intervention and research based on clinical practices gets a shift from short term medical assessment to long term assessment and intervention of morbidity.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
Congenital heart disease(CHD)is one of the most common causes of major birth defects,with a prevalence of 1%.Although an increasing number of studies have reported the etiology of CHD,the findings scattered throughout...Congenital heart disease(CHD)is one of the most common causes of major birth defects,with a prevalence of 1%.Although an increasing number of studies have reported the etiology of CHD,the findings scattered throughout the literature are difficult to retrieve and utilize in research and clinical practice.We therefore developed CHDbase,an evidence-based knowledgebase of CHD-related genes and clinical manifestations manually curated from 1114 publications,linking 1124 susceptibility genes and 3591 variations to more than 300 CHD types and related syndromes.Metadata such as the information of each publication and the selected population and samples,the strategy of studies,and the major findings of studies were integrated with each item of the research record.We also integrated functional annotations through parsing50 databases/tools to facilitate the interpretation of these genes and variations in disease pathogenicity.We further prioritized the significance of these CHD-related genes with a gene interaction network approach and extracted a core CHD sub-network with 163 genes.The clear genetic landscape of CHD enables the phenotype classification based on the shared genetic origin.Overall,CHDbase provides a comprehensive and freely available resource to study CHD susceptibilities,supporting a wide range of users in the scientific and medical communities.CHDbase is accessible at http://chddb.fwgenetics.org.展开更多
Pentalogy of Cantrell is a congenital anomaly characterized by a combination of birth defects involving the sternum,diaphragm,pericardium,abdominal wall,and heart.Pentalogy of Cantrell is a rare anomaly with high mort...Pentalogy of Cantrell is a congenital anomaly characterized by a combination of birth defects involving the sternum,diaphragm,pericardium,abdominal wall,and heart.Pentalogy of Cantrell is a rare anomaly with high mortality.This paper describes the treatment of a 9-month-old girl with pentalogy of Cantrell,pentalogy of Fallot,and left ventricular diverticulum.The patient is alive and well 13 years after surgery.展开更多
Right ventricular outflow tract(RVOT)obstruction is present in a variety of congenital heart disease states including tetralogy of Fallot,pulmonary atresia/stenosis and other conotruncal abnormalities etc.After surgic...Right ventricular outflow tract(RVOT)obstruction is present in a variety of congenital heart disease states including tetralogy of Fallot,pulmonary atresia/stenosis and other conotruncal abnormalities etc.After surgical repair,these patients develop RVOT residual abnormalities of pulmonic stenosis and/or insufficiency of their native outflow tract or right ventricle to pulmonary artery conduit.There are also sequelae of other surgeries like the Ross operation for aortic valve disease that lead to right ventricle to pulmonary artery conduit dysfunction.Surgical pulmonic valve replacement(SPVR)has been the mainstay for these patients and is considered standard of care.Transcatheter pulmonic valve implantation(TPVI)was first reported in 2000 and has made strides as a comparable alternative to SPVR,being approved in the United States in 2010.We provide a comprehensive review in this space–indications for TPVI,detailed procedural facets and up-to-date review of the literature regarding outcomes of TPVI.TPVI has been shown to have favorable medium-term outcomes free of reinterventions especially after the adoption of the practice of pre-stenting the RVOT.Procedural mortality and complications are uncommon.With more experience,recognition of risk of dreaded outcomes like coronary compression has improved.Also,conduit rupture is increasingly being managed with transcatheter tools.Questions over endocarditis risk still prevail in the TPVI population.Head-to-head comparisons to SPVR are still limited but available data suggests equivalence.We also discuss newer valve technologies that have limited data currently and may have more applicability for treatment of native dysfunctional RVOT substrates.展开更多
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.
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.
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.
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.
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 approved by the Ethics Committee,which waived the need for informed consent.Certificate of Presentation of Ethical Review:46190821.7.0000.0068.
文摘Background:Children with congenital heart disease(CHD),even after surgical approaches,and especially those who undergo staged procedures in thefirst months of life,remain vulnerable to readmissions and complications,requiring very close monitoring and differentiated intervention strategies.Methods:Descriptive and exploratory study,of the experience report type,which presents the process of building the high-risk outpatient clinic for complex congenital heart diseases(AAR)at the Instituto do Coração(InCor).Results:Report of the path taken to structure the AAR,demonstrating the organization,interface with the multidisciplinary team,admission and discharge criteria,training,and patient profile.In thesefive years of care,275 patients were treated,59.65%with biventricular interstage physiology,followed by univentricular interstage physiology(34.55%),residual defects after surgical procedures(3.63%),tumors with risk of mechanical obstruction(1.45%)and patients with an intrauterine approach(0.72%).The significant number of critical patients who were successfully discharged from hospital(44.72%),the low mortality rate due to sudden deaths at home and the high adherence to follow-up cor-roborate the impact of this specialized assistance.Conclusions:Despite the limitations of the study,this experi-ence report showed that with few resources,there is the possibility of organizing an AAR with differentiated care,with the objective of early detection and treatment of residual injuries,identification of early interventions,edu-cation of parents for follow-up of their children,resulting in individualized treatment,promoting a better quality of life for this population.
文摘Congenital ventricular aneurysm is a very rare cardiac anomaly.A diagnosis can be made during the prenatal period using fetal echocardiography.This study presents a very rare apically located left ventricular aneurysm case,and the relevant literature was reviewed and discussed.In this case,a 35-year-old,gravida 2,parity 1 preg-nant woman at 24 weeks of gestation,displayed a wide aneurysmal image in the left ventricular apical wall on fetal echocardiography.There was a 1.79 mm muscular ventricular septal defect at the apical region of the interven-tricular septum.In the course of the color Doppler ultrasonography examination,an aberrantfibrous band within the left ventricle and consequent turbulentflow during systole were observed.The baby,born via cesarean section at 37 weeks of gestation,is now in its postnatal seventh month.However,during echocardiographic follow-ups,changes have been observed,including mild to moderate mitral insufficiency and a decrease in systolic function.Despite thesefindings,the clinical condition remains asymptomatic.It is of great importance to use a multidis-ciplinary approach in managing these rare cases that could lead to potential adverse outcomes during the antena-tal or postnatal periods.
文摘Objective:The objective of this analysis is to describe the characteristics and morbidity during hospitalizations among adolescents with congenital heart disease(AdoCHD)from the Pediatric Health Information System(PHIS)database.Methods:The PHIS database was queried for all AdoCHD admissions aged 12–18 years(1/1/2004–12/31/2013).Major forms of CHD were identified by their International Classification of Diseases,ninth revision codes,further verified based on their secondary diagnosis and/or procedure codes.Patient characteristics,diagnoses,procedures and vital status were assessed.Results:In total,there were 4,267 adolescents admitted to 42 Children’s Hospitals,58.3%were males,24.6%single ventricle(SV)patients,64.1%bi-ventricle(BV),and 11.3%could not be classified.They accounted for 8,512 hospitalizations(41,240 total hospital days),of which 31.6%were intensive care unit(ICU)stays.ICU stay was similar for the SVand BV patients with similar duration of mechanical ventilation between the two groups.Overall,the most common CHD among in-patients was tetralogy of Fallot(TOF,36.4%).Larger proportion of the BVAdoCHD admissions were for elective surgical and electrophysiological procedures.There were 109(2.5%)heart transplantations(1.3%SV vs.0.6%BV)and 120 in-hospital deaths(2.8%)(1.1%SV vs.1.3%BV).Hypoplastic left heart syndrome was the most common diagnosis in transplanted patients(46%)and those who died(28%);TOF(29%)was frequent in 91(2.1%)patients who had cardiac arrests.Conclusions:Different hospitalization patterns exist for BV and SV AdoCHD.Recognizing this risk may encourage directing resources toward optimizing long-term care of CHD patients.
文摘BACKGROUND Pulmonary arterial hypertension(PAH)in pregnancy is one of the major obstetric complications and is considered a contraindication to pregnancy as it is classified as a class IV risk in the revised risk classification of pregnancy by the World Health Organisation.Pregnancy,with its adaptive and expectant mechanical and hormonal changes,negatively affects the cardiopulmonary circulation in pregnant women.Do patients with repaired simple congenital heart disease(CHD)develop other pulmonary and cardiac complications during pregnancy?Can pregnant women with sudden pulmonary hypertension be treated and managed in time?In this paper,we present a case of a 39-year-old woman who underwent cesarean section at 33 wk'gestation and developed PAH secondary to repaired simple CHD.Our research began by a PubMed search for"pulmonary hypertension"and"pregnancy"and"CHD"case reports.Three cases were selected to review PAH in pregnancy after correction of CHD defects.These studies were reviewed,coupled with our own clinical experience.CASE SUMMARY Herein,a case involving a woman who underwent atrial septal defect repair at the age of 34,became pregnant five years later,and had a sudden onset of PAH and right heart failure secondary to symptoms of acute peripheral edema in the third trimester of her pregnancy.As a result,the patient underwent a cesarean section and gave birth to healthy twins.Within three days after cesarean delivery,her cardiac function deteriorated as the pulmonary artery pressure increased.Effec-tive postpartum management,including diuresis,significant oxygen uptake,vasodilators,capacity and anticoagulants management,led to improvements in cardiac function and oxygenation.The patient was discharged from hospital with a stable recovery and transferred to local hospitals for further PAH treatment.CONCLUSION This case served as a reminder to obstetricians of the importance of pregnancy after repair of CHD.It is crucial for patients with CHD to receive early correction.It suggests doctors should not ignore edema of twin pregnancy.Also,it provides a reference for the further standardization of antenatal,in-trapartum and postpartum management for patients with CHD worldwide.
基金Cheryl Raskind-Hood and Wendy M.Book are supported by a Cooperative Agreement from the Centers for Disease Control and Prevention Cooperative Agreement,Public Health Pilot Project Surveillance of Congenital Heart Defects(CHDs)Focusing on Adolescents and AdultsFOA#DD12-1207.
文摘Early intervention to prevent premature mortality is vital for adults with congenital heart defects(CHD).Anatomic complexity and comorbid conditions are thought to contribute to CHD mortality.Since hypertension(HTN)and heart failure(HF)are the comorbid conditions among the most prevalent causes of death in the United States,and commonly accompany CHD,it is crucial to evaluate whether they are reliable predictors of mortality for adults with CHD(ACHD)independent of anatomic CHD complexity.A retrospective cross-sectional analysis of ACHD,aged 18–64,with concomitant HTN and/or HF and at least one health care encounter during 2008–2010 were assessed.Of 5,397 ACHD patients(18.3%HTN without HF,4.4%HF without HTN,8.3%with both),3.0%died(n=163)during the study period.Overall,the sample was 45.1%white,61.4%female,and 29.0%had a complex CHD.Among those who died,23.3%had HTN without HF,17.2%had HF without HTN,and 42.3%had both.Crude analyses revealed that older age,male gender,black race,and having public health insurance were associated with increased mortality during the three-year study period compared to ACHD patients who were younger,female gender,white race,and covered by private health insurance.ACHD patients diagnosed with non-complex CHD lesions(i.e.,shunts,valves,or shunts+valves)were at greater risk of dying compared to those with severe complex CHDs.When CHD type was assessed separately,those with valve lesions were more likely to die compared to those with complex CHD lesions.After adjustment for age,gender,race,insurance and CHD complexity,ACHD patients with HF,with or without HTN,were equally likely to die during the study period.However,ACHD patients with HF,without or without HTN,who had valve defects were more likely to die during the three-year study period compared to patients with complex CHDs.
基金supported by Fujian Provincial Science and Technology Major Project(No.2020HZ02014)by the grants from National Natural Science Foundation of Fujian(2021J01133,2021J011404)by the Quanzhou Scientific and Technological Planning Projects(Nos.2018C113R,2019C028R,2019C029R,2019C076R and 2019C099R).
文摘Congenital heart defect,accounting for about 30%of congenital defects,is the most common one.Data shows that congenital heart defects have seriously affected the birth rate of healthy newborns.In Fetal andNeonatal Cardiology,medical imaging technology(2D ultrasonic,MRI)has been proved to be helpful to detect congenital defects of the fetal heart and assists sonographers in prenatal diagnosis.It is a highly complex task to recognize 2D fetal heart ultrasonic standard plane(FHUSP)manually.Compared withmanual identification,automatic identification through artificial intelligence can save a lot of time,ensure the efficiency of diagnosis,and improve the accuracy of diagnosis.In this study,a feature extraction method based on texture features(Local Binary Pattern LBP and Histogram of Oriented Gradient HOG)and combined with Bag of Words(BOW)model is carried out,and then feature fusion is performed.Finally,it adopts Support VectorMachine(SVM)to realize automatic recognition and classification of FHUSP.The data includes 788 standard plane data sets and 448 normal and abnormal plane data sets.Compared with some other methods and the single method model,the classification accuracy of our model has been obviously improved,with the highest accuracy reaching 87.35%.Similarly,we also verify the performance of the model in normal and abnormal planes,and the average accuracy in classifying abnormal and normal planes is 84.92%.The experimental results show that thismethod can effectively classify and predict different FHUSP and can provide certain assistance for sonographers to diagnose fetal congenital heart disease.
文摘Development in medical intervention has significantly decreased the mortality rates for children with complex congenital heart disease(CHD)but among these survivors with complex heart disease there occurs a unique pattern of neuro-developmental and neuropsychology impairment characterized social interaction impairment,impulsive Behavior,and impaired executive functions.Presence of behavioral problem is found significantly high in pediatric population with chronic illness than children with absence of chronic illness.The sample of 200 children with congenital heart defect was selected between age 4-8 years using multistage stratified sampling.The childhood psychopathology measurement schedule(CPMS)by Dr.Savitha Malhotra was used for assessing Behavioral problems present in children with CHD.Pre-Post experimental design was used to investigate the study and the results were statistically analyzed using paired T test.The result revealed that the effectiveness of intervention program to retrain Behavior showed high significance.With increased survival rates,the aim of the intervention and research based on clinical practices gets a shift from short term medical assessment to long term assessment and intervention of morbidity.
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.
基金This work was supported by the Young Scientists Fund of the National Natural Science Foundation of China(Grant No.31801103)the Chinese Academy of Medical Sciences Initiative for Innovative Medicine(Grant No.2016-I2M-1-016).We acknowledge Dr.Adam Yongxin Ye at Harvard Medical School,Boston Children’s Hospital for his support and contribution to the project,and Dr.Ge Gao at Peking University for assistance with database comparison.
文摘Congenital heart disease(CHD)is one of the most common causes of major birth defects,with a prevalence of 1%.Although an increasing number of studies have reported the etiology of CHD,the findings scattered throughout the literature are difficult to retrieve and utilize in research and clinical practice.We therefore developed CHDbase,an evidence-based knowledgebase of CHD-related genes and clinical manifestations manually curated from 1114 publications,linking 1124 susceptibility genes and 3591 variations to more than 300 CHD types and related syndromes.Metadata such as the information of each publication and the selected population and samples,the strategy of studies,and the major findings of studies were integrated with each item of the research record.We also integrated functional annotations through parsing50 databases/tools to facilitate the interpretation of these genes and variations in disease pathogenicity.We further prioritized the significance of these CHD-related genes with a gene interaction network approach and extracted a core CHD sub-network with 163 genes.The clear genetic landscape of CHD enables the phenotype classification based on the shared genetic origin.Overall,CHDbase provides a comprehensive and freely available resource to study CHD susceptibilities,supporting a wide range of users in the scientific and medical communities.CHDbase is accessible at http://chddb.fwgenetics.org.
文摘Pentalogy of Cantrell is a congenital anomaly characterized by a combination of birth defects involving the sternum,diaphragm,pericardium,abdominal wall,and heart.Pentalogy of Cantrell is a rare anomaly with high mortality.This paper describes the treatment of a 9-month-old girl with pentalogy of Cantrell,pentalogy of Fallot,and left ventricular diverticulum.The patient is alive and well 13 years after surgery.
文摘Right ventricular outflow tract(RVOT)obstruction is present in a variety of congenital heart disease states including tetralogy of Fallot,pulmonary atresia/stenosis and other conotruncal abnormalities etc.After surgical repair,these patients develop RVOT residual abnormalities of pulmonic stenosis and/or insufficiency of their native outflow tract or right ventricle to pulmonary artery conduit.There are also sequelae of other surgeries like the Ross operation for aortic valve disease that lead to right ventricle to pulmonary artery conduit dysfunction.Surgical pulmonic valve replacement(SPVR)has been the mainstay for these patients and is considered standard of care.Transcatheter pulmonic valve implantation(TPVI)was first reported in 2000 and has made strides as a comparable alternative to SPVR,being approved in the United States in 2010.We provide a comprehensive review in this space–indications for TPVI,detailed procedural facets and up-to-date review of the literature regarding outcomes of TPVI.TPVI has been shown to have favorable medium-term outcomes free of reinterventions especially after the adoption of the practice of pre-stenting the RVOT.Procedural mortality and complications are uncommon.With more experience,recognition of risk of dreaded outcomes like coronary compression has improved.Also,conduit rupture is increasingly being managed with transcatheter tools.Questions over endocarditis risk still prevail in the TPVI population.Head-to-head comparisons to SPVR are still limited but available data suggests equivalence.We also discuss newer valve technologies that have limited data currently and may have more applicability for treatment of native dysfunctional RVOT substrates.
