Background: Congenital heart disease is a public health issue due to its incidence and mortality rate. The aim of this study was to investigate the long-term mortality of children with congenital heart disease admitte...Background: Congenital heart disease is a public health issue due to its incidence and mortality rate. The aim of this study was to investigate the long-term mortality of children with congenital heart disease admitted to the Departmental University Hospital of Borgou/Alibori (CHUD-B/A) from 2011 to 2022. Methods: This descriptive longitudinal study with analytical aims covered 11 years (April 1, 2011 to December 31, 2022). It consisted of a review of the records of children under 15 years of age with echocardiographically confirmed congenital heart disease. This was followed by an interview with the parents to assess the children’s current condition. Data were entered using Kobocollect software and analyzed using R Studio 4.2.2. software. Results: A total of 143 complete files were retained. The median age at diagnosis was 14 months (IIQ: Q1 = 4;Q3 = 60) with a range of 2 days and 175 months, and the sex-ratio (M/F) was 0.96. Left-to-right shunts were the most frequent cardiopathy group (62.9%). Only 35 children (24.5%) benefited from restorative treatment. The mortality rate was 31.5%. Median survival under the maximum bias assumption was 114 months and 216 months under the assumption of minimum bias. Survival was significantly better in children with right-to-left shunts (p = 0.0049) under the assumption of minimum bias. The death risk factors were: age at diagnosis less than 12 months (aHR = 7.58;95% CI = 3.36 - 17.24;p Conclusion: The long-term mortality of congenital heart disease is high and favoured by the absence of restorative treatment. Local correction of congenital heart disease and medical follow-up will help to reduce this mortality.展开更多
Objective:To analyze the perioperative respiratory care methods and application value in children with congenital heart disease.Methods:60 children with congenital heart disease(treated from January 2021 to October 20...Objective:To analyze the perioperative respiratory care methods and application value in children with congenital heart disease.Methods:60 children with congenital heart disease(treated from January 2021 to October 2023)were screened and divided into two groups randomly.Each group consisted of 30 cases.The perioperative routine was used in the control group.The observation group underwent the perioperative routine along with better respiratory care.Oxygenation indicators,surgical complications,and family satisfaction levels of the groups were compared.Results:There was no significant difference in the oxygenation index between the two groups of children at admission(P>0.05).At discharge,the oxygenation indicators in the observation group were better than those of the control group,and the incidence of surgical complications was lower than that of the control group.The total satisfaction of family members in the observation group was higher than that of the control group(P<0.05).Conclusion:During the perioperative period for children with congenital heart disease,the implementation of respiratory care,which mainly involves symptomatic care,catheter care,sputum suction care,etc.,can actively improve the oxygenation indicators,reduce surgical complications,and promote faster and better recovery,of children with congenital heart disease.展开更多
Objectives:To examine whether patients with congenital heart disease(CHD)are less likely to have a partner or children than individuals from the general population.Methods:Longitudinal study with two assessments of th...Objectives:To examine whether patients with congenital heart disease(CHD)are less likely to have a partner or children than individuals from the general population.Methods:Longitudinal study with two assessments of the same patients(n=244)from a hospital population and controls(n=238)from the German Socio-Economic Panel(GSOEP)using parental education,patients age,and sex as matching criteria.The first patient study was conducted between 5/2003 and 6/2004,the second one between 5/2017 and 4/2019.Controls were drawn from GSOEP-surveys 2004 and 2018.CHD-severity was classified according to type of surgery:curative,reparative,or palliative.Living single was used as outcome measure,for offspring the outcome was having children or not.Results:Among women with CHD the rate of those living single was higher than among controls with the differences depending on disease complexity(curative:OR=5.5;reparative:OR=1.9;palliative:OR=2.7).No statistically significant differences between patients and controls emerged in the male study population.With respect to children a marked difference emerged between women with CHD and controls.Among patients the odds of having children were lower than among controls(curative:OR=0.3;reparative:OR=0.3;palliative:OR=0.2).The rate of patients with children with CHD(women:5.6%;men:4.9%)was higher than expected(1%)if compared with the general population.Conclusions:Using partnership and children as outcome criteria,patients with CHD are disadvantaged if compared to subjects from the general population.In female patients the social consequences of the disease turned out as more pervasive than in women.展开更多
Objectives:To estimate the prevalence of Congenital Heart Disease(CHD)in school-age children,to identify the extent to which altitude affects the prevalence of the disease,and to examine trends in prevalence over time...Objectives:To estimate the prevalence of Congenital Heart Disease(CHD)in school-age children,to identify the extent to which altitude affects the prevalence of the disease,and to examine trends in prevalence over time in China.Methods:Seven databases were systematically searched and last retrieved on September 10,2021 for all studies reporting the prevalence of CHD in children after 1970 in China,which were then divided into high and low altitude regions based on 2500 meters above sea level.The random-effected model was used to combine prevalence data and subgroups analysis.The baseline data of all cases and individuals were used for comparison to calculate the odds ratio(OR)for overall and different altitude prevalence.Results:A total of 12,926,083 individuals(aged 3-18 years),with 31,835 cases from 86 studies,were included in the analysis.The pooled CHD prevalence of total children was 4.69[95%confidence interval(CI):4.10 to 5.29]per 1000 children.Overall,temporal trends analysis indicated that the prevalence of CHD in children continuously decreased with time,from 6.19(95%CI:4.50 to 7.88)per 1000 children in 1976-1985 to 3.30(95%CI:2.49;4.38)per 1000 children in 2016-2021.The OR for the prevalence of CHD in children from high and low altitudes with baseline data was 2.84(95%CI:2.48 to 3.27)and 1.31(95%CI:1.13 to 1.53)(χ^(2)=53.89,p<0.01),respectively.The OR of the prevalence of CHD in male children compared to females was 0.60(95%CI:0.53 to 0.68)at high altitudes and 0.79(95%CI:0.71 to 0.89)at low altitudes.Among the seven most common subtypes,patent ductus arteriosus was the most common at high altitudes,while atrial septal defects were the most common at low altitudes.Conclusion:This study provides valuable insights for further disease prevention and etiological exploration.The overall decreasing trend in the prevalence of CHD in children over time may indicate a positive effect of perinatal management and treatment during infancy.展开更多
The second edition of the expert consensus on pediatric nutrition was formed based on a global update of pedia-tric nutrition guidelines or consensus worldwide,the management of congenital heart disease,and the result...The second edition of the expert consensus on pediatric nutrition was formed based on a global update of pedia-tric nutrition guidelines or consensus worldwide,the management of congenital heart disease,and the results of multi-center clinical nutrition research for congenital heart disease following thefirst Chinese consensus edition of 2016.The consensus was also shaped by the results of three discussion sessions and two questionnaires con-ducted by the 13-member collaboration group.This process was informed by both clinical guidelines and expert consensus.The quality of literature,both in English and Chinese,and the level of recommendations were evaluated using the Grading of Recommendations Assessment,Development,and Evaluations(GRADE)system.展开更多
Background:In congenital heart disease(CHD)patients,detailed three-dimensional anatomy depiction plays a pivotal role for diagnosis and therapeutical decision making.Hence,the present study investigated the applicabil...Background:In congenital heart disease(CHD)patients,detailed three-dimensional anatomy depiction plays a pivotal role for diagnosis and therapeutical decision making.Hence,the present study investigated the applicability of an advanced cardiovascular magnetic resonance(CMR)whole heart imaging approach utilizing nonselective excitation and compressed sensing for anatomical assessment and interventional guidance of CHD patients in comparison to conventional dynamic CMR angiography.Methods:86 consecutive pediatric patients and adults with congenital heart disease(age,1 to 74 years;mean,35 years)underwent CMR imaging including a freebreathing,ECG-triggered 3D nonselective SSFP whole heart acquisition using compressed SENSE(nsWHcs).Anatomical assessability and signal intensity ratio(SIR)measurements were compared with conventional dynamic 3D-/4D-MR angiography.Results:The most frequent diagnoses were partial anomalous pulmonary venous drainage(17/86,20%),transposition of the great arteries(15/86,17%),tetralogy of Fallot(12/86,14%),and a single ventricle(7/86,8%).Image quality of nsWHcs was rated as excellent/good in 98%of patients.nsWHcs resulted in a reliable depiction of all large thoracic vessels(anatomic assessability,99%–100%)and the proximal segments of coronary arteries and coronary sinus(>90%).nsWHcs achieved a homogenously distributed SIR in all cardiac cavities and thoracic vessels without a significant difference between pulmonary and systemic circulation(10.9±3.5 and 10.6±3.4;p=0.15),while 3D angiography showed significantly increased SIR for targeted vs.non-targeted circulation(PA-angiography,15.2±8.1 vs.5.8±3.6,p<0.001;PV-angiography,7.0±3.9 vs.17.3±6.8,p<0.001).Conclusions:The proposed nsWHcs imaging approach provided a consistently high image quality and a homogeneous signal intensity distribution within the pulmonary and systemic circulation in pediatric patients and adults with a wide spectrum of congenital heart diseases.nsWHcs enabled detailed anatomical assessment and three-dimensional reconstruction of all cardiac cavities and large thoracic vessels and can be regarded particularly useful for preprocedural planning and interventional guidance in CHD patients.展开更多
Background:In patients with cyanotic congenital heart disease(CHD),cerebral oxygenation may be maintained by elevations in hematocrit(Hct).Hemodilution accompanying cardiopulmonary bypass(CPB),however,can disrupt cere...Background:In patients with cyanotic congenital heart disease(CHD),cerebral oxygenation may be maintained by elevations in hematocrit(Hct).Hemodilution accompanying cardiopulmonary bypass(CPB),however,can disrupt cerebral oxygen balance,leading to fluctuations in cerebral oxygen saturation(ScO_(2)).The present study investigated the effects of Hct changes on the fluctuation of ScO_(2)during CPB in cyanotic CHD using performance measurement(PM).Methods:Children with CHD(51 acyanotic and 46 cyanotic)who had undergone cardiac surgery using CPB were enrolled.Median performance error(MDPE),median absolute performance error(MDAPE),and wobble parameters of ScO_(2)were calculated before(reference value),during,and after CPB.Correlations of PM parameters with Hct and reductions in Hct(ΔHct)were also evaluated.Results:Before CPB,patients with cyanotic CHD had lower MDPE and larger wobble than those with acyanotic CHD,although mean ScO_(2)did not differ significantly between the two groups.During CPB,ScO_(2)of acyanotic CHD increased asΔHct increased,but PM variables were not associated withΔHct.In cyanotic CHD,MDPE(r=−0.324,p=0.032)and MDAPE(r=0.339,p=0.024)correlated significantly withΔHct during CPB.After CPB,MDPE(r=0.574,p=0.025)and MDAPE(r=−0.543,p=0.036)were significantly correlated with Hct in children with cyanotic CHD who underwent palliative surgery.Conclusion:Therefore,ScO_(2)fluctuation during CPB in children with cyanotic CHD may be affected by the decrease in Hct,suggesting that excessive hemodilution can negatively influence the maintenance of cerebral oxygenation in these patients.展开更多
BACKGROUND Congenital heart disease(CHD)is a cardiovascular malformation caused by abnormal heart and/or vascular development in the fetus.In children with CHD,abnormalities in the development and function of the nerv...BACKGROUND Congenital heart disease(CHD)is a cardiovascular malformation caused by abnormal heart and/or vascular development in the fetus.In children with CHD,abnormalities in the development and function of the nervous system are common.At present,there is a lack of research on the preoperative neurological development and injury in young children with non-cyanotic CHD.AIM To determine the changes in white matter,gray matter,and cerebrospinal fluid(CSF)by magnetic resonance imaging(MRI)in children with non-cyanotic CHD as compared with healthy controls.METHODS Children diagnosed with non-cyanotic CHD on ultrasonography(n=54)and healthy control subjects(n=35)were included in the study.All the subjects were aged 1-3 years.Brain MRI was performed prior to surgery for CHD.The SPM v12 software was used to calculate the volumes of the gray matter,white matter,CSF,and the whole brain(sum of the gray matter,white matter,and CSF volumes).Volume differences between the two groups were analyzed.Voxel-based morphometry was used to compare specific brain regions with statistically significant atrophy.RESULTS Compared with the control group,the study group had significantly reduced whole-brain white matter volume(P<0.05),but similar whole-brain gray matter,CSF,and whole-brain volumes(P>0.05).As compared with the healthy controls,children with non-cyanotic CHD had mild underdevelopment in the white matter of the anterior central gyrus,the posterior central gyrus,and the pulvinar.CONCLUSION Children with non-cyanotic CHD show decreased white matter volume before surgery,and this volume reduction is mainly concentrated in the somatosensory and somatic motor nerve regions.展开更多
BACKGROUND Parents of children with complicated congenital heart disease(CHD)have different needs after surgery.Little literature reports the impact factors for psychological needs of parents of children with complica...BACKGROUND Parents of children with complicated congenital heart disease(CHD)have different needs after surgery.Little literature reports the impact factors for psychological needs of parents of children with complicated CHD.AIM To investigate the status quo of the needs of parents of children after surgery for complex CHD,and analyze the influencing factors,in order to provide a theoretical basis for formulating corresponding nursing countermeasures.METHODS A modified Chinese version of the Critical Care Family Needs Inventory(MCCFNI)was used to select 200 parents of children with complex CHD after surgery within 72 h after admission to the intensive care unit in our hospital to conduct an online questionnaire survey.The aim was to understand the needs of parents in relation to the following five aspects:The support from medical staff,comfort of the parents themselves,the acquisition of information,their closeness to the children,and assurance of the child’s condition.RESULTS Parents of children with complex CHD had a higher degree of demand,especially in terms of condition assurance,acquisition of information,and closeness to the children.The age,education level,and residence of the parents were related to the five dimensions of the needs of parents of children with complex CHD who had undergone surgery.CONCLUSION In practice,nurses should formulate corresponding nursing strategies based on the different cultural and social backgrounds of parents of children after complex CHD surgery to meet their different needs,and improve satisfaction.These findings provide a theoretical basis for constructing a family participatory nursing model for children in the intensive care unit in the future.展开更多
Objective:To study the effect of dobutamine combined with meropenem on serum BNP, IGF-1, IGFBP-3, TNF-a, IL-6 and hs-CRP in children with congenital heart disease and pneumonia.Methods:A total of 70 children with cong...Objective:To study the effect of dobutamine combined with meropenem on serum BNP, IGF-1, IGFBP-3, TNF-a, IL-6 and hs-CRP in children with congenital heart disease and pneumonia.Methods:A total of 70 children with congenital heart disease and pneumonia in our hospital from June 2014 to Octomber 2016 were enrolled in this study. The subjects were divided into the control group (n=35) and the treatment group (n=35) randomly. The control group was treated with dobutamine, the treatment group were treated with dobutamine combined with meropenem. The two groups were treated for 10 days. The serum BNP, IGF-1, IGFBP-3, TNF-a, IL-6 and hs-CRP levels of the two groups before and after treatment were compared.Results: There were no significantly differences of the serum BNP, IGF-1, IGFBP-3, TNF-a, IL-6 and hs-CRP levels of the two groups before treatment. The serum BNP, TNF-a, IL-6 and hs-CRP levels of the two groups after treatment were significantly lower than before treatment, the serum IGF-1 and IGFBP-3 levels of the two groups after treatment were significantly higher than before treatment, and that of the treatment group were significantly better than the control group.Conclusion:Dobutamine combined with meropenem can significantly reduce the serum BNP, TNF-a, IL-6 and hs-CRP levels, improve serum IGF-1 and IGFBP-3 levels of children with congenital heart disease and pneumonia, and it was worthy clinical application.展开更多
COVID-19 pandemic continues to strike across the world with increasing number of infected patients,severe morbidity and mortality,social life and economy disruption.Universal access to vaccine prophylaxis will be pivo...COVID-19 pandemic continues to strike across the world with increasing number of infected patients,severe morbidity and mortality,social life and economy disruption.Universal access to vaccine prophylaxis will be pivotal in controlling this infection and providing individual level protection.However,mismatch between vaccine request and vaccine availability,as well as constraints in logistics of vaccine campaign is creating a transition phase of progressive but still incomplete inclusion of group of individuals in the vaccination process.Selected patients living with chronic and multisystemic disease may present increased propensity of adverse outcome,should Sars-Cov-2 infection develop.In these patients,expedite access to COVID-19 vaccination may be considered.The Italian Society of Pediatric Cardiology National Board is providing a viewpoint to inform policy makers and public authorities during vaccine allocation strategies development,to consider higher priority and expedite access for selected groups of patients living with congenital heart disease.展开更多
1 Background Congenital heart disease(CHD)is the most common major congenital anomaly,affecting approximately one in every 100 live births[1].Among congenital anomalies,66%of preventable deaths are due to CHD,and 58%o...1 Background Congenital heart disease(CHD)is the most common major congenital anomaly,affecting approximately one in every 100 live births[1].Among congenital anomalies,66%of preventable deaths are due to CHD,and 58%of the avertable morbidity and mortality due to congenital anomalies would result from scaling congenital heart surgery services[2].Every year,nearly 300,000 children and adults die from CHD,the majority of whom live in low-and middle-income countries(LMICs)[3].Approximately 49%of all individuals with CHD will require surgical or interventional care at some point in their lifetime[4];as a result of advances in access to and the delivery of such services,over 95%of children born with CHD in high-income countries now live into adulthood[3].Here,adults have surpassed children in the number of CHD cases at a ratio of 2:1[5].展开更多
Congenital heart disease(CHD),the most prevalent congenital ailment,has seen advancements in the“dual indi-cator”screening program.This facilitates the early-stage diagnosis and treatment of children with CHD,subse-...Congenital heart disease(CHD),the most prevalent congenital ailment,has seen advancements in the“dual indi-cator”screening program.This facilitates the early-stage diagnosis and treatment of children with CHD,subse-quently enhancing their survival rates.While cardiac auscultation offers an objective reflection of cardiac abnormalities and function,its evaluation is significantly influenced by personal experience and external factors,rendering it susceptible to misdiagnosis and omission.In recent years,continuous progress in artificial intelli-gence(AI)has enabled the digital acquisition,storage,and analysis of heart sound signals,paving the way for intelligent CHD auscultation-assisted diagnostic technology.Although there has been a surge in studies based on machine learning(ML)within CHD auscultation and diagnostic technology,most remain in the algorithmic research phase,relying on the implementation of specific datasets that still await verification in the clinical envir-onment.This paper provides an overview of the current stage of AI-assisted cardiac sounds(CS)auscultation technology,outlining the applications and limitations of AI auscultation technology in the CHD domain.The aim is to foster further development and refinement of AI auscultation technology for enhanced applications in CHD.展开更多
Aims:Although the application of ultrasound-guided vascular puncture and Z-stitch hemostasis to manage femoral access has been widely utilized,there is limited data on this combined application in adult congenital hea...Aims:Although the application of ultrasound-guided vascular puncture and Z-stitch hemostasis to manage femoral access has been widely utilized,there is limited data on this combined application in adult congenital heart disease(ACHD)patients undergoing electrophysiological(EP)procedures.We sought to evaluate the safety and efficacy of ultrasound-guided puncture and postprocedural Z-stitch hemostasis for ACHD patients under-going EP procedures.Methods and Results:The population of ACHD patients undergoing transfemoral EP pro-cedures at the University of Zurich Heart Center between January 2019 and December 2022 was observed and analyzed.During the study period,femoral access(left/right,arterial/venous)was performed under real-time ultrasound guidance.At the end of the procedure,a single Z-stitch was performed at the puncture site.We eval-uated the incidence of in-hospital complications associated with femoral access puncture in this population.Among 101 patients who had a total of 147 previous ipsilateral vascular punctures(mean 1.5 per person),100 patients underwent successful femoral vascular access for EP procedures.The median age of the patients was 47±15 years and 34(34%)were male.Z-stitches were performed after the procedure in 100 patients with 303 femoral vascular accesses(mean 3 punctures per person).No patient developed vascular puncture relevant inguinal hematoma,pseudo aneurysm,arteriovenousfistula,venous or arterial thrombosis.Conclusion:In ACHD patients undergoing EP procedures,optimal femoral access management can be achieved with ultra-sound-guided puncture and postprocedural Z-stitch hemostasis.展开更多
Aims:Multiple genes and environmental factors are known to be involved in congenital heart disease(CHD),but epigenetic variation has received little attention.Monozygotic(MZ)twins with CHD provide a unique model for e...Aims:Multiple genes and environmental factors are known to be involved in congenital heart disease(CHD),but epigenetic variation has received little attention.Monozygotic(MZ)twins with CHD provide a unique model for exploring this phenomenon.In order to investigate the potential role of Deoxyribonucleic Acid(DNA)methyla-tion in CHD pathogenesis,the present study examined DNA methylation variation in MZ twins discordant for CHD,especially ventricular septal defect(VSD).Methods and Results:Using genome-wide DNA methylation profiles,we identified 4004 differentially methylated regions(DMRs)in 18 MZ twin pairs discordant for CHD,and 2826 genes were identified.Gene Ontology(GO)and Kyoto Encyclopedia of Genes and Genomes(KEGG)analysis revealed a list of CHD-associated pathways.To further investigate the role of DNA methylation in VSD,data from 7 pairs of MZ twins with VSD were analyzed.We identified 1614 DMRs corresponding to 1443 genes associated with arrhythmogenic right ventricular cardiomyopathy,cyclic guanosine monopho-sphate-protein kinase G(cGMP-PKG)signaling pathway by KEGG analysis,and cell-cell adhesion,calcium ion transmembrane transport by GO analysis.A proportion of DMR-associated genes were involved in calcium signaling pathways.The methylation changes of calcium signaling genes might be related to VSD pathogenesis.Conclusion:CHD is associated with differential DNA methylation in MZ twins.CHD may be etiologically linked to DNA methylation,and methylation of calcium signaling genes may be involved in the development of VSD.展开更多
Background:Transcatheter closure(TCC)has emerged as the preferred treatment for selected congenital heart disease(CHD).While TCC offers benefits for patients with postoperative residual shunts,understanding its mid-an...Background:Transcatheter closure(TCC)has emerged as the preferred treatment for selected congenital heart disease(CHD).While TCC offers benefits for patients with postoperative residual shunts,understanding its mid-and long-term efficacy and safety remains crucial.Objective:This study aims to assess the mid-and long-term safety and efficacy of TCC for patients with residual atrial or ventricular septal shunts following CHD correction.Methods:In this consecutive retrospective study,we enrolled 35 patients with residual shunt who underwent TCC or surgical repair of CHD between June 2011 to October 2022.TCC candidacy was determined based on established criteria.Echocardiography and electrocardiogram were conducted during the perioperative period and continued as part of long-term follow-up.Results:Among the patients,5(14.3%)exhibited interatrial shunt-ing,while 30(85.7%)had interventricular shunting.TCC was successfully implemented in 33 of 35 patients,with exceptions in two cases of post-ventricular septal defect repair due to anatomical challenges involving the shape and aortic angulation.This resulted in a TCC success rate of 94.3%.Trace residual shunt was detected in two interventricular shunting cases and a mild residual shunt in one interventricular shunting case;all resolved by the three-month follow-up after TCC.Minor complications included one hematoma at the puncture site and one transient junctional rhythm during the perioperative period.During a median follow-up of 73 months,there were no instances of residual shunt,device embolization,occluder displacement,valve insufficiency,malignant arrhythmia,infective endocarditis,death,or other serious complications.Conclusion:TCC is an effective and safe therapy for patients with residual atrial or ventricular septal shunts following CHD correction.Thesefindings support the consideration of TCC as the preferred treatment option for appropriate patient populations.展开更多
Background:Patients with congenital heart disease(CHD)will transition to lifelong adult congenital cardiac care.However,their structural heart disease is challenging to convey via two-dimensional drawings.This study u...Background:Patients with congenital heart disease(CHD)will transition to lifelong adult congenital cardiac care.However,their structural heart disease is challenging to convey via two-dimensional drawings.This study utilized a tele-educational environment,with personalized three-dimensional(3D)modeling and health Details(3D+Details=“4D”),to improve actual and perceived knowledge,both important components of transition readiness in CHD patients.Methods:Participants aged≥13 years with a history of CHD and cardiac magnetic resonance imaging(MRI)studies were eligible.Cardiac MRI datasets were then used to segment and create 3D heart models(using Mimics,Materialize Inc.).Participantsfirst completed the MyHeart Questionnaire,a validated survey of actual knowledge.A tele-educational session was then scheduled,during which participants were shown a 3D model of a normal heart,followed by their personal 3D heart model and specific health details.Participants then repeated the actual knowledge survey,in addition to questionnaires assessing perceived knowledge pre-and post-session,as well as a satisfaction survey.Results:Twenty-two patients were included.Actual knowledge increased from 75%±15%to 89%±20%(p=0.00043)and perceived knowledge increased infive of seven questions.Actual knowledge correlated with perceived knowledge(r=0.608,p<0.0001).Ninety-one percent of participants ranked the 3D model as“very satisfactory”and ninety-five percent ranked the educational session as“very help-ful”or“extremely helpful.”Conclusions:The use of“4D”tele-education increased both actual and perceived knowledge and may help improve transition readiness in CHD patients.展开更多
Background:Current guidelines for managing pulmonary arterial hypertension(PAH)recommend a risk strati-fication approach.However,the applicability and accuracy of these strategies for PAH associated with congenital he...Background:Current guidelines for managing pulmonary arterial hypertension(PAH)recommend a risk strati-fication approach.However,the applicability and accuracy of these strategies for PAH associated with congenital heart disease(PAH-CHD)require further validation.This study aims to validate the reliability and predictive accuracy of a simplified stratification strategy for PAH-CHD patients over a three-year follow-up.Additionally,new prognostic variables are identified and novel risk stratification methods are developed for assessing and managing PAH-CHD patients.Methods:This retrospective study included 126 PAH-CHD patients.Clinical and biochemical variables across risk groups were assessed using Kruskal-Wallis and Fisher’s exact tests.Indepen-dent risk factors were identified using ordered logistic regression,while Kaplan-Meier and Cox proportional hazards regression analyses evaluated their impact on all-cause mortality.A new stratification model for the PAH-CHD population was constructed based on these analyses.Results:Significant survival differences across stratified risk groups were observed(p<0.001),validating the effectiveness of the simplified risk stratification method in PAH-CHD patients.Prothrombin activity was a strong independent predictor of adverse outcomes of PAH-CHD patients(Hazard ratio 0.95,p<0.001,C-index 0.70).A model combining N-terminal pro-brain natriuretic peptide,prothrombin activity,albumin,and right atrial area achieved an area under the curve of 0.89 and a C-index of 0.85.Conclusions:The simplified risk stratification method is applicable to PAH-CHD patients.Prothrombin activity is a strong independent predictor of adverse outcomes.A comprehensive risk stratification approach,incorporating both established and novel biomarkers,enhances accessibility and offers predictive efficacy during follow-up for PAH-CHD patients,comparable to established models.展开更多
Social determinants of health(SDOH)affect quality of life.We investigated SDOH impacts on self-perceived resilience among people with adult congenital heart disease(ACHD).Secondary analysis of data from two com-plemen...Social determinants of health(SDOH)affect quality of life.We investigated SDOH impacts on self-perceived resilience among people with adult congenital heart disease(ACHD).Secondary analysis of data from two com-plementary studies:a survey study conducted May 2021–June 2022 and a qualitative study conducted June 2020–August 2021.Resilience was assessed through CD-RISC10 score(range 0–40,higher scores reflect greater self-perceived resilience)and interview responses.Sociodemographic and SDOH(education,employment,living situa-tion,monetary stability,financial dependency,area deprivation index)data were collected by healthcare record review and self-report.We used linear regression with robust standard errors to analyze survey data and performed a thematic analysis of interview data.Survey participants(N=127)mean age was 42±14 years;51%were female,87%white.ACHD was moderate(75%)or complex(25%);41%functional class C or D.Resilience(mean 30±7)varied by monetary stability:compared to people with difficulty paying bills,resilience was 15.0 points higher(95%CI:6.9–23.1,p<0.001)for people reporting having enough money and 14.2 points higher(95%CI:5.9–22.4,p=0.001)for those reporting just enough money.Interview participants’(N=25)mean age was 32 years(range 22–44);52%were female,72%white.ACHD was moderate(56%)or complex(44%);76%functional class C or D.Participants discussed factors affecting resilience aligned with each of the major SDOH,prominently,economic stability and healthcare access and quality.Financial stability may be important for supporting self-perceived resi-lience in ACHD.This knowledge can inform the development of resilience interventions for this population.展开更多
Introduction: Congenital heart disease includes all cardiac and vascular malformations. It accounts for approximately one third of all congenital malformations and is a public health problem, particularly in developin...Introduction: Congenital heart disease includes all cardiac and vascular malformations. It accounts for approximately one third of all congenital malformations and is a public health problem, particularly in developing countries. The aim of this study was to analyze the epidemiological, clinical and paraclinical aspects of congenital heart disease. Methods: This was a retrospective descriptive and analytical study based on the records of 135 patients referred for surgery and followed up in the cardiology department of the Ignace Deen University Hospital, collected in November 2022. Results: Hospital prevalence was 5%. The mean age was 71 months, ranging from 1 month to 19 years. The age group over 24 months was the most represented (62%). The M/F sex ratio was 1.36. Urban origin was predominant (58%). The rate of children not attending school or dropping out was high (16%). Siblings with fewer than 4 children were the most common (88%). A heart murmur was the most frequent sign (78%), followed by cyanosis (36%) and heart failure (29%). The association between heart murmurs and CHD was proven with a p-value Conclusion: CHDs represent the main indication for paediatric cardiac surgery and follow-up (95%). We stress the importance of implementing a screening and management strategy for congenital heart disease.展开更多
文摘Background: Congenital heart disease is a public health issue due to its incidence and mortality rate. The aim of this study was to investigate the long-term mortality of children with congenital heart disease admitted to the Departmental University Hospital of Borgou/Alibori (CHUD-B/A) from 2011 to 2022. Methods: This descriptive longitudinal study with analytical aims covered 11 years (April 1, 2011 to December 31, 2022). It consisted of a review of the records of children under 15 years of age with echocardiographically confirmed congenital heart disease. This was followed by an interview with the parents to assess the children’s current condition. Data were entered using Kobocollect software and analyzed using R Studio 4.2.2. software. Results: A total of 143 complete files were retained. The median age at diagnosis was 14 months (IIQ: Q1 = 4;Q3 = 60) with a range of 2 days and 175 months, and the sex-ratio (M/F) was 0.96. Left-to-right shunts were the most frequent cardiopathy group (62.9%). Only 35 children (24.5%) benefited from restorative treatment. The mortality rate was 31.5%. Median survival under the maximum bias assumption was 114 months and 216 months under the assumption of minimum bias. Survival was significantly better in children with right-to-left shunts (p = 0.0049) under the assumption of minimum bias. The death risk factors were: age at diagnosis less than 12 months (aHR = 7.58;95% CI = 3.36 - 17.24;p Conclusion: The long-term mortality of congenital heart disease is high and favoured by the absence of restorative treatment. Local correction of congenital heart disease and medical follow-up will help to reduce this mortality.
文摘Objective:To analyze the perioperative respiratory care methods and application value in children with congenital heart disease.Methods:60 children with congenital heart disease(treated from January 2021 to October 2023)were screened and divided into two groups randomly.Each group consisted of 30 cases.The perioperative routine was used in the control group.The observation group underwent the perioperative routine along with better respiratory care.Oxygenation indicators,surgical complications,and family satisfaction levels of the groups were compared.Results:There was no significant difference in the oxygenation index between the two groups of children at admission(P>0.05).At discharge,the oxygenation indicators in the observation group were better than those of the control group,and the incidence of surgical complications was lower than that of the control group.The total satisfaction of family members in the observation group was higher than that of the control group(P<0.05).Conclusion:During the perioperative period for children with congenital heart disease,the implementation of respiratory care,which mainly involves symptomatic care,catheter care,sputum suction care,etc.,can actively improve the oxygenation indicators,reduce surgical complications,and promote faster and better recovery,of children with congenital heart disease.
基金Funding Statement:This report is based on two research projects.The first one was funded by the German Research Foundation(Deutsche Forschungsgemeinschaft-DFG)under Grant Numbers WE 2670/1-1 and GE1167/2-1 to SG(URL:https://www.dfg.de)The follow-up was funded by Stiftung Kinderherzen,Grant Number WGÖ-014/2016(URL:https://www.kinderherzen.de)TP,KN and SG.The funders had not been involved in the study design,in the collection,analysis and interpretation of data,and in writing the manuscript.
文摘Objectives:To examine whether patients with congenital heart disease(CHD)are less likely to have a partner or children than individuals from the general population.Methods:Longitudinal study with two assessments of the same patients(n=244)from a hospital population and controls(n=238)from the German Socio-Economic Panel(GSOEP)using parental education,patients age,and sex as matching criteria.The first patient study was conducted between 5/2003 and 6/2004,the second one between 5/2017 and 4/2019.Controls were drawn from GSOEP-surveys 2004 and 2018.CHD-severity was classified according to type of surgery:curative,reparative,or palliative.Living single was used as outcome measure,for offspring the outcome was having children or not.Results:Among women with CHD the rate of those living single was higher than among controls with the differences depending on disease complexity(curative:OR=5.5;reparative:OR=1.9;palliative:OR=2.7).No statistically significant differences between patients and controls emerged in the male study population.With respect to children a marked difference emerged between women with CHD and controls.Among patients the odds of having children were lower than among controls(curative:OR=0.3;reparative:OR=0.3;palliative:OR=0.2).The rate of patients with children with CHD(women:5.6%;men:4.9%)was higher than expected(1%)if compared with the general population.Conclusions:Using partnership and children as outcome criteria,patients with CHD are disadvantaged if compared to subjects from the general population.In female patients the social consequences of the disease turned out as more pervasive than in women.
基金supported by Qinghai Provincial Science and Technology Department(Grant No.2021-ZJ-751).
文摘Objectives:To estimate the prevalence of Congenital Heart Disease(CHD)in school-age children,to identify the extent to which altitude affects the prevalence of the disease,and to examine trends in prevalence over time in China.Methods:Seven databases were systematically searched and last retrieved on September 10,2021 for all studies reporting the prevalence of CHD in children after 1970 in China,which were then divided into high and low altitude regions based on 2500 meters above sea level.The random-effected model was used to combine prevalence data and subgroups analysis.The baseline data of all cases and individuals were used for comparison to calculate the odds ratio(OR)for overall and different altitude prevalence.Results:A total of 12,926,083 individuals(aged 3-18 years),with 31,835 cases from 86 studies,were included in the analysis.The pooled CHD prevalence of total children was 4.69[95%confidence interval(CI):4.10 to 5.29]per 1000 children.Overall,temporal trends analysis indicated that the prevalence of CHD in children continuously decreased with time,from 6.19(95%CI:4.50 to 7.88)per 1000 children in 1976-1985 to 3.30(95%CI:2.49;4.38)per 1000 children in 2016-2021.The OR for the prevalence of CHD in children from high and low altitudes with baseline data was 2.84(95%CI:2.48 to 3.27)and 1.31(95%CI:1.13 to 1.53)(χ^(2)=53.89,p<0.01),respectively.The OR of the prevalence of CHD in male children compared to females was 0.60(95%CI:0.53 to 0.68)at high altitudes and 0.79(95%CI:0.71 to 0.89)at low altitudes.Among the seven most common subtypes,patent ductus arteriosus was the most common at high altitudes,while atrial septal defects were the most common at low altitudes.Conclusion:This study provides valuable insights for further disease prevention and etiological exploration.The overall decreasing trend in the prevalence of CHD in children over time may indicate a positive effect of perinatal management and treatment during infancy.
基金supported by the National Natural Science Foundation of China(81970265,82270310)a Sub-Project of the National Key R&D Program“The recognition and Identification of Genetic Pathogenic Genes for Structural Birth Defects”(2021YFC2701002)+1 种基金Nanjing Science and Technology Development Project(2019060007)Jiangsu Provincial Key Research and Development Program(BE2023662).
文摘The second edition of the expert consensus on pediatric nutrition was formed based on a global update of pedia-tric nutrition guidelines or consensus worldwide,the management of congenital heart disease,and the results of multi-center clinical nutrition research for congenital heart disease following thefirst Chinese consensus edition of 2016.The consensus was also shaped by the results of three discussion sessions and two questionnaires con-ducted by the 13-member collaboration group.This process was informed by both clinical guidelines and expert consensus.The quality of literature,both in English and Chinese,and the level of recommendations were evaluated using the Grading of Recommendations Assessment,Development,and Evaluations(GRADE)system.
文摘Background:In congenital heart disease(CHD)patients,detailed three-dimensional anatomy depiction plays a pivotal role for diagnosis and therapeutical decision making.Hence,the present study investigated the applicability of an advanced cardiovascular magnetic resonance(CMR)whole heart imaging approach utilizing nonselective excitation and compressed sensing for anatomical assessment and interventional guidance of CHD patients in comparison to conventional dynamic CMR angiography.Methods:86 consecutive pediatric patients and adults with congenital heart disease(age,1 to 74 years;mean,35 years)underwent CMR imaging including a freebreathing,ECG-triggered 3D nonselective SSFP whole heart acquisition using compressed SENSE(nsWHcs).Anatomical assessability and signal intensity ratio(SIR)measurements were compared with conventional dynamic 3D-/4D-MR angiography.Results:The most frequent diagnoses were partial anomalous pulmonary venous drainage(17/86,20%),transposition of the great arteries(15/86,17%),tetralogy of Fallot(12/86,14%),and a single ventricle(7/86,8%).Image quality of nsWHcs was rated as excellent/good in 98%of patients.nsWHcs resulted in a reliable depiction of all large thoracic vessels(anatomic assessability,99%–100%)and the proximal segments of coronary arteries and coronary sinus(>90%).nsWHcs achieved a homogenously distributed SIR in all cardiac cavities and thoracic vessels without a significant difference between pulmonary and systemic circulation(10.9±3.5 and 10.6±3.4;p=0.15),while 3D angiography showed significantly increased SIR for targeted vs.non-targeted circulation(PA-angiography,15.2±8.1 vs.5.8±3.6,p<0.001;PV-angiography,7.0±3.9 vs.17.3±6.8,p<0.001).Conclusions:The proposed nsWHcs imaging approach provided a consistently high image quality and a homogeneous signal intensity distribution within the pulmonary and systemic circulation in pediatric patients and adults with a wide spectrum of congenital heart diseases.nsWHcs enabled detailed anatomical assessment and three-dimensional reconstruction of all cardiac cavities and large thoracic vessels and can be regarded particularly useful for preprocedural planning and interventional guidance in CHD patients.
文摘Background:In patients with cyanotic congenital heart disease(CHD),cerebral oxygenation may be maintained by elevations in hematocrit(Hct).Hemodilution accompanying cardiopulmonary bypass(CPB),however,can disrupt cerebral oxygen balance,leading to fluctuations in cerebral oxygen saturation(ScO_(2)).The present study investigated the effects of Hct changes on the fluctuation of ScO_(2)during CPB in cyanotic CHD using performance measurement(PM).Methods:Children with CHD(51 acyanotic and 46 cyanotic)who had undergone cardiac surgery using CPB were enrolled.Median performance error(MDPE),median absolute performance error(MDAPE),and wobble parameters of ScO_(2)were calculated before(reference value),during,and after CPB.Correlations of PM parameters with Hct and reductions in Hct(ΔHct)were also evaluated.Results:Before CPB,patients with cyanotic CHD had lower MDPE and larger wobble than those with acyanotic CHD,although mean ScO_(2)did not differ significantly between the two groups.During CPB,ScO_(2)of acyanotic CHD increased asΔHct increased,but PM variables were not associated withΔHct.In cyanotic CHD,MDPE(r=−0.324,p=0.032)and MDAPE(r=0.339,p=0.024)correlated significantly withΔHct during CPB.After CPB,MDPE(r=0.574,p=0.025)and MDAPE(r=−0.543,p=0.036)were significantly correlated with Hct in children with cyanotic CHD who underwent palliative surgery.Conclusion:Therefore,ScO_(2)fluctuation during CPB in children with cyanotic CHD may be affected by the decrease in Hct,suggesting that excessive hemodilution can negatively influence the maintenance of cerebral oxygenation in these patients.
文摘BACKGROUND Congenital heart disease(CHD)is a cardiovascular malformation caused by abnormal heart and/or vascular development in the fetus.In children with CHD,abnormalities in the development and function of the nervous system are common.At present,there is a lack of research on the preoperative neurological development and injury in young children with non-cyanotic CHD.AIM To determine the changes in white matter,gray matter,and cerebrospinal fluid(CSF)by magnetic resonance imaging(MRI)in children with non-cyanotic CHD as compared with healthy controls.METHODS Children diagnosed with non-cyanotic CHD on ultrasonography(n=54)and healthy control subjects(n=35)were included in the study.All the subjects were aged 1-3 years.Brain MRI was performed prior to surgery for CHD.The SPM v12 software was used to calculate the volumes of the gray matter,white matter,CSF,and the whole brain(sum of the gray matter,white matter,and CSF volumes).Volume differences between the two groups were analyzed.Voxel-based morphometry was used to compare specific brain regions with statistically significant atrophy.RESULTS Compared with the control group,the study group had significantly reduced whole-brain white matter volume(P<0.05),but similar whole-brain gray matter,CSF,and whole-brain volumes(P>0.05).As compared with the healthy controls,children with non-cyanotic CHD had mild underdevelopment in the white matter of the anterior central gyrus,the posterior central gyrus,and the pulvinar.CONCLUSION Children with non-cyanotic CHD show decreased white matter volume before surgery,and this volume reduction is mainly concentrated in the somatosensory and somatic motor nerve regions.
文摘BACKGROUND Parents of children with complicated congenital heart disease(CHD)have different needs after surgery.Little literature reports the impact factors for psychological needs of parents of children with complicated CHD.AIM To investigate the status quo of the needs of parents of children after surgery for complex CHD,and analyze the influencing factors,in order to provide a theoretical basis for formulating corresponding nursing countermeasures.METHODS A modified Chinese version of the Critical Care Family Needs Inventory(MCCFNI)was used to select 200 parents of children with complex CHD after surgery within 72 h after admission to the intensive care unit in our hospital to conduct an online questionnaire survey.The aim was to understand the needs of parents in relation to the following five aspects:The support from medical staff,comfort of the parents themselves,the acquisition of information,their closeness to the children,and assurance of the child’s condition.RESULTS Parents of children with complex CHD had a higher degree of demand,especially in terms of condition assurance,acquisition of information,and closeness to the children.The age,education level,and residence of the parents were related to the five dimensions of the needs of parents of children with complex CHD who had undergone surgery.CONCLUSION In practice,nurses should formulate corresponding nursing strategies based on the different cultural and social backgrounds of parents of children after complex CHD surgery to meet their different needs,and improve satisfaction.These findings provide a theoretical basis for constructing a family participatory nursing model for children in the intensive care unit in the future.
文摘Objective:To study the effect of dobutamine combined with meropenem on serum BNP, IGF-1, IGFBP-3, TNF-a, IL-6 and hs-CRP in children with congenital heart disease and pneumonia.Methods:A total of 70 children with congenital heart disease and pneumonia in our hospital from June 2014 to Octomber 2016 were enrolled in this study. The subjects were divided into the control group (n=35) and the treatment group (n=35) randomly. The control group was treated with dobutamine, the treatment group were treated with dobutamine combined with meropenem. The two groups were treated for 10 days. The serum BNP, IGF-1, IGFBP-3, TNF-a, IL-6 and hs-CRP levels of the two groups before and after treatment were compared.Results: There were no significantly differences of the serum BNP, IGF-1, IGFBP-3, TNF-a, IL-6 and hs-CRP levels of the two groups before treatment. The serum BNP, TNF-a, IL-6 and hs-CRP levels of the two groups after treatment were significantly lower than before treatment, the serum IGF-1 and IGFBP-3 levels of the two groups after treatment were significantly higher than before treatment, and that of the treatment group were significantly better than the control group.Conclusion:Dobutamine combined with meropenem can significantly reduce the serum BNP, TNF-a, IL-6 and hs-CRP levels, improve serum IGF-1 and IGFBP-3 levels of children with congenital heart disease and pneumonia, and it was worthy clinical application.
文摘COVID-19 pandemic continues to strike across the world with increasing number of infected patients,severe morbidity and mortality,social life and economy disruption.Universal access to vaccine prophylaxis will be pivotal in controlling this infection and providing individual level protection.However,mismatch between vaccine request and vaccine availability,as well as constraints in logistics of vaccine campaign is creating a transition phase of progressive but still incomplete inclusion of group of individuals in the vaccination process.Selected patients living with chronic and multisystemic disease may present increased propensity of adverse outcome,should Sars-Cov-2 infection develop.In these patients,expedite access to COVID-19 vaccination may be considered.The Italian Society of Pediatric Cardiology National Board is providing a viewpoint to inform policy makers and public authorities during vaccine allocation strategies development,to consider higher priority and expedite access for selected groups of patients living with congenital heart disease.
文摘1 Background Congenital heart disease(CHD)is the most common major congenital anomaly,affecting approximately one in every 100 live births[1].Among congenital anomalies,66%of preventable deaths are due to CHD,and 58%of the avertable morbidity and mortality due to congenital anomalies would result from scaling congenital heart surgery services[2].Every year,nearly 300,000 children and adults die from CHD,the majority of whom live in low-and middle-income countries(LMICs)[3].Approximately 49%of all individuals with CHD will require surgical or interventional care at some point in their lifetime[4];as a result of advances in access to and the delivery of such services,over 95%of children born with CHD in high-income countries now live into adulthood[3].Here,adults have surpassed children in the number of CHD cases at a ratio of 2:1[5].
基金supported by Jiangsu Provincial Health Commission(Grant No.K2023036).
文摘Congenital heart disease(CHD),the most prevalent congenital ailment,has seen advancements in the“dual indi-cator”screening program.This facilitates the early-stage diagnosis and treatment of children with CHD,subse-quently enhancing their survival rates.While cardiac auscultation offers an objective reflection of cardiac abnormalities and function,its evaluation is significantly influenced by personal experience and external factors,rendering it susceptible to misdiagnosis and omission.In recent years,continuous progress in artificial intelli-gence(AI)has enabled the digital acquisition,storage,and analysis of heart sound signals,paving the way for intelligent CHD auscultation-assisted diagnostic technology.Although there has been a surge in studies based on machine learning(ML)within CHD auscultation and diagnostic technology,most remain in the algorithmic research phase,relying on the implementation of specific datasets that still await verification in the clinical envir-onment.This paper provides an overview of the current stage of AI-assisted cardiac sounds(CS)auscultation technology,outlining the applications and limitations of AI auscultation technology in the CHD domain.The aim is to foster further development and refinement of AI auscultation technology for enhanced applications in CHD.
基金This study complied with the Declaration of Helsinki and was approved by the local Ethics Committee(Cantonal Ethics Committee Zurich,Nr.2016-00116).All patients signed informed consent for the procedure and the use of clinical data for scientific study.
文摘Aims:Although the application of ultrasound-guided vascular puncture and Z-stitch hemostasis to manage femoral access has been widely utilized,there is limited data on this combined application in adult congenital heart disease(ACHD)patients undergoing electrophysiological(EP)procedures.We sought to evaluate the safety and efficacy of ultrasound-guided puncture and postprocedural Z-stitch hemostasis for ACHD patients under-going EP procedures.Methods and Results:The population of ACHD patients undergoing transfemoral EP pro-cedures at the University of Zurich Heart Center between January 2019 and December 2022 was observed and analyzed.During the study period,femoral access(left/right,arterial/venous)was performed under real-time ultrasound guidance.At the end of the procedure,a single Z-stitch was performed at the puncture site.We eval-uated the incidence of in-hospital complications associated with femoral access puncture in this population.Among 101 patients who had a total of 147 previous ipsilateral vascular punctures(mean 1.5 per person),100 patients underwent successful femoral vascular access for EP procedures.The median age of the patients was 47±15 years and 34(34%)were male.Z-stitches were performed after the procedure in 100 patients with 303 femoral vascular accesses(mean 3 punctures per person).No patient developed vascular puncture relevant inguinal hematoma,pseudo aneurysm,arteriovenousfistula,venous or arterial thrombosis.Conclusion:In ACHD patients undergoing EP procedures,optimal femoral access management can be achieved with ultra-sound-guided puncture and postprocedural Z-stitch hemostasis.
基金China’s National Natural Science Foundation provided funding for this study(81900222)Guangzhou Science and Technology Program(SL2022A04J01269,202201020646)Guangzhou Health Science and Technology Program(20211A010026).
文摘Aims:Multiple genes and environmental factors are known to be involved in congenital heart disease(CHD),but epigenetic variation has received little attention.Monozygotic(MZ)twins with CHD provide a unique model for exploring this phenomenon.In order to investigate the potential role of Deoxyribonucleic Acid(DNA)methyla-tion in CHD pathogenesis,the present study examined DNA methylation variation in MZ twins discordant for CHD,especially ventricular septal defect(VSD).Methods and Results:Using genome-wide DNA methylation profiles,we identified 4004 differentially methylated regions(DMRs)in 18 MZ twin pairs discordant for CHD,and 2826 genes were identified.Gene Ontology(GO)and Kyoto Encyclopedia of Genes and Genomes(KEGG)analysis revealed a list of CHD-associated pathways.To further investigate the role of DNA methylation in VSD,data from 7 pairs of MZ twins with VSD were analyzed.We identified 1614 DMRs corresponding to 1443 genes associated with arrhythmogenic right ventricular cardiomyopathy,cyclic guanosine monopho-sphate-protein kinase G(cGMP-PKG)signaling pathway by KEGG analysis,and cell-cell adhesion,calcium ion transmembrane transport by GO analysis.A proportion of DMR-associated genes were involved in calcium signaling pathways.The methylation changes of calcium signaling genes might be related to VSD pathogenesis.Conclusion:CHD is associated with differential DNA methylation in MZ twins.CHD may be etiologically linked to DNA methylation,and methylation of calcium signaling genes may be involved in the development of VSD.
文摘Background:Transcatheter closure(TCC)has emerged as the preferred treatment for selected congenital heart disease(CHD).While TCC offers benefits for patients with postoperative residual shunts,understanding its mid-and long-term efficacy and safety remains crucial.Objective:This study aims to assess the mid-and long-term safety and efficacy of TCC for patients with residual atrial or ventricular septal shunts following CHD correction.Methods:In this consecutive retrospective study,we enrolled 35 patients with residual shunt who underwent TCC or surgical repair of CHD between June 2011 to October 2022.TCC candidacy was determined based on established criteria.Echocardiography and electrocardiogram were conducted during the perioperative period and continued as part of long-term follow-up.Results:Among the patients,5(14.3%)exhibited interatrial shunt-ing,while 30(85.7%)had interventricular shunting.TCC was successfully implemented in 33 of 35 patients,with exceptions in two cases of post-ventricular septal defect repair due to anatomical challenges involving the shape and aortic angulation.This resulted in a TCC success rate of 94.3%.Trace residual shunt was detected in two interventricular shunting cases and a mild residual shunt in one interventricular shunting case;all resolved by the three-month follow-up after TCC.Minor complications included one hematoma at the puncture site and one transient junctional rhythm during the perioperative period.During a median follow-up of 73 months,there were no instances of residual shunt,device embolization,occluder displacement,valve insufficiency,malignant arrhythmia,infective endocarditis,death,or other serious complications.Conclusion:TCC is an effective and safe therapy for patients with residual atrial or ventricular septal shunts following CHD correction.Thesefindings support the consideration of TCC as the preferred treatment option for appropriate patient populations.
文摘Background:Patients with congenital heart disease(CHD)will transition to lifelong adult congenital cardiac care.However,their structural heart disease is challenging to convey via two-dimensional drawings.This study utilized a tele-educational environment,with personalized three-dimensional(3D)modeling and health Details(3D+Details=“4D”),to improve actual and perceived knowledge,both important components of transition readiness in CHD patients.Methods:Participants aged≥13 years with a history of CHD and cardiac magnetic resonance imaging(MRI)studies were eligible.Cardiac MRI datasets were then used to segment and create 3D heart models(using Mimics,Materialize Inc.).Participantsfirst completed the MyHeart Questionnaire,a validated survey of actual knowledge.A tele-educational session was then scheduled,during which participants were shown a 3D model of a normal heart,followed by their personal 3D heart model and specific health details.Participants then repeated the actual knowledge survey,in addition to questionnaires assessing perceived knowledge pre-and post-session,as well as a satisfaction survey.Results:Twenty-two patients were included.Actual knowledge increased from 75%±15%to 89%±20%(p=0.00043)and perceived knowledge increased infive of seven questions.Actual knowledge correlated with perceived knowledge(r=0.608,p<0.0001).Ninety-one percent of participants ranked the 3D model as“very satisfactory”and ninety-five percent ranked the educational session as“very help-ful”or“extremely helpful.”Conclusions:The use of“4D”tele-education increased both actual and perceived knowledge and may help improve transition readiness in CHD patients.
基金This work was supported by the National Natural Science Foundation of China(82070052)the Joint Funds of the Natural Science Foundation of Gansu Province(23JRRA1544)granted to Yunshan Cao.
文摘Background:Current guidelines for managing pulmonary arterial hypertension(PAH)recommend a risk strati-fication approach.However,the applicability and accuracy of these strategies for PAH associated with congenital heart disease(PAH-CHD)require further validation.This study aims to validate the reliability and predictive accuracy of a simplified stratification strategy for PAH-CHD patients over a three-year follow-up.Additionally,new prognostic variables are identified and novel risk stratification methods are developed for assessing and managing PAH-CHD patients.Methods:This retrospective study included 126 PAH-CHD patients.Clinical and biochemical variables across risk groups were assessed using Kruskal-Wallis and Fisher’s exact tests.Indepen-dent risk factors were identified using ordered logistic regression,while Kaplan-Meier and Cox proportional hazards regression analyses evaluated their impact on all-cause mortality.A new stratification model for the PAH-CHD population was constructed based on these analyses.Results:Significant survival differences across stratified risk groups were observed(p<0.001),validating the effectiveness of the simplified risk stratification method in PAH-CHD patients.Prothrombin activity was a strong independent predictor of adverse outcomes of PAH-CHD patients(Hazard ratio 0.95,p<0.001,C-index 0.70).A model combining N-terminal pro-brain natriuretic peptide,prothrombin activity,albumin,and right atrial area achieved an area under the curve of 0.89 and a C-index of 0.85.Conclusions:The simplified risk stratification method is applicable to PAH-CHD patients.Prothrombin activity is a strong independent predictor of adverse outcomes.A comprehensive risk stratification approach,incorporating both established and novel biomarkers,enhances accessibility and offers predictive efficacy during follow-up for PAH-CHD patients,comparable to established models.
基金This study is supported by K23HL15180(NIH/NHLBI,Steiner)a grant from the American College of Cardiology Foundation.
文摘Social determinants of health(SDOH)affect quality of life.We investigated SDOH impacts on self-perceived resilience among people with adult congenital heart disease(ACHD).Secondary analysis of data from two com-plementary studies:a survey study conducted May 2021–June 2022 and a qualitative study conducted June 2020–August 2021.Resilience was assessed through CD-RISC10 score(range 0–40,higher scores reflect greater self-perceived resilience)and interview responses.Sociodemographic and SDOH(education,employment,living situa-tion,monetary stability,financial dependency,area deprivation index)data were collected by healthcare record review and self-report.We used linear regression with robust standard errors to analyze survey data and performed a thematic analysis of interview data.Survey participants(N=127)mean age was 42±14 years;51%were female,87%white.ACHD was moderate(75%)or complex(25%);41%functional class C or D.Resilience(mean 30±7)varied by monetary stability:compared to people with difficulty paying bills,resilience was 15.0 points higher(95%CI:6.9–23.1,p<0.001)for people reporting having enough money and 14.2 points higher(95%CI:5.9–22.4,p=0.001)for those reporting just enough money.Interview participants’(N=25)mean age was 32 years(range 22–44);52%were female,72%white.ACHD was moderate(56%)or complex(44%);76%functional class C or D.Participants discussed factors affecting resilience aligned with each of the major SDOH,prominently,economic stability and healthcare access and quality.Financial stability may be important for supporting self-perceived resi-lience in ACHD.This knowledge can inform the development of resilience interventions for this population.
文摘Introduction: Congenital heart disease includes all cardiac and vascular malformations. It accounts for approximately one third of all congenital malformations and is a public health problem, particularly in developing countries. The aim of this study was to analyze the epidemiological, clinical and paraclinical aspects of congenital heart disease. Methods: This was a retrospective descriptive and analytical study based on the records of 135 patients referred for surgery and followed up in the cardiology department of the Ignace Deen University Hospital, collected in November 2022. Results: Hospital prevalence was 5%. The mean age was 71 months, ranging from 1 month to 19 years. The age group over 24 months was the most represented (62%). The M/F sex ratio was 1.36. Urban origin was predominant (58%). The rate of children not attending school or dropping out was high (16%). Siblings with fewer than 4 children were the most common (88%). A heart murmur was the most frequent sign (78%), followed by cyanosis (36%) and heart failure (29%). The association between heart murmurs and CHD was proven with a p-value Conclusion: CHDs represent the main indication for paediatric cardiac surgery and follow-up (95%). We stress the importance of implementing a screening and management strategy for congenital heart disease.