Femoral Access with Ultrasound-Guided Puncture and Z-Stitch Hemostasis for Adults with Congenital Heart Diseases Undergoing Electrophysiological Procedures

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.

DNA Methylation Variation Is Identified in Monozygotic Twins Discordant for Congenital Heart Diseases

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.

Exploring kidney biopsy findings in congenital heart diseases:Insights beyond cyanotic nephropathy

BACKGROUND The association between congenital heart disease and chronic kidney disease is well known.Various mechanisms of kidney damage associated with congenital heart disease have been established.The etiology of kidneydisease has commonly been considered to be secondary to focal segmental glomerulosclerosis(FSGS),however,this has only been demonstrated in case reports and not in observational or clinical trials.AIM To identify baseline and clinical characteristics,as well as the findings in kidney biopsies of patients with congenital heart disease in our hospital.METHODS This is a retrospective observational study conducted at the Nephrology Depart-ment of the National Institute of Cardiology“Ignacio Chávez”.All patients over 16 years old who underwent percutaneous kidney biopsy from January 2000 to January 2023 with congenital heart disease were included in the study.RESULTS Ten patients with congenital heart disease and kidney biopsy were found.The average age was 29.00 years±15.87 years with pre-biopsy proteinuria of 6193 mg/24 h±6165 mg/24 h.The most common congenital heart disease was Fallot’s tetralogy with 2 cases(20%)and ventricular septal defect with 2(20%)cases.Among the 10 cases,one case of IgA nephropathy and one case of membranoproliferative glomerulonephritis associated with immune complexes were found,receiving specific treatment after histopathological diagnosis,delaying the initiation of kidney replacement therapy.Among remaining 8 cases(80%),one case of FSGS with perihilar variety was found,while the other 7 cases were non-specific FSGS.CONCLUSION Determining the cause of chronic kidney disease can help in delaying the need for kidney replacement therapy.In 2 out of 10 patients in our study,interventions were performed,and initiation of kidney replacement therapy was delayed.Prospective studies are needed to determine the usefulness of kidney biopsy in patients with congenital heart disease.

Noninherited Factors in Fetal Congenital Heart Diseases Based on Bayesian Network:A Large Multicenter Study

Background:Current studies have confirmed that fetal congenital heart diseases(CHDs)are caused by various factors.However,the quantitative risk of CHD is not clear given the combined effects of multiple factors.Objective:This cross-sectional study aimed to detect associated factors of fetal CHD using a Bayesian network in a large sample and quantitatively analyze relative risk ratios(RRs).Methods:Pregnant women who underwent fetal echocardiography(N=16,086 including 3,312 with CHD fetuses)were analyzed.Twenty-six maternal and fetal factors were obtained.A Bayesian network is constructed based on all variables through structural learning and parameter learning methods to find the environmental factors that directly and indirectly associated with outcome,and the probability of fetal CHD in the two groups is predicted through a junction tree reasoning algorithm,so as to obtain RR for fetal CHD under different exposure factor combinations.Taking into account the effect of gestational week on the accuracy of model prediction,we conducted sensitivity analysis on gestational week groups.Results:The single-factor analysis showed that the RRs for the numbers of births,spontaneous abortions,and parental smoking were 1.50,1.38,and 1.11(P<0.001),respectively.The risk gradually increased with the synergistic effect of ranging from one to more environmental factors above.The risk was higher among subjects with five synergistic factors,including the number of births,upper respiratory tract infection during early pregnancy,anemia,and mental stress as well as a history of spontaneous abortions or parental smoking,than in those with less than 5 factors(RR=2.62 or 2.28,P<0.001).This result was consistent across the participants grouped by GWs.Conclusion:We identified six factors that were directly associated with fetal CHD.A higher number of these factors led to a higher risk of CHD.These findings suggest that it is important to strengthen healthcare and prenatal counseling for women with these factors.

Specific Injuries Management in the Postoperative of Congenital Heart Diseases(Ⅱ):Univentricular Hearts

It is very important to understand that the univentricular heart surgery is just palliative, not being in anyway a definitive or curative surgery, but nowadays it’s the best initial treatment of this complex heart disease. The fundamental philosophy of treatment of every univentricular heart is to ensure the flow system and/or restrict the lung flow. Thus, initially a patient with univentricular heart who is undergoing surgery may need to ensure systemic flow (reconstruction of the aortic?arch type Norwood), to restrict the lung flow (pulmonary banding) or to provide enough?pulmonary flow (pulmonary-systemic fistulae). However, some heart diseases with univentricular physiology remain “balanced” autonomously, until the “second” stage of palliation is performed (cavo-pulmonary anastomosis type Glenn), but others require performance of pulmonary banding, if there’s no native lung protection and/or repair of the systemic circuit in a first stage, to reach next palliation steps in the best possible conditions.

Loss to Specialized Cardiology Follow-Up in Adults Living with Congenital Heart Disease

Background:Much has been written about the loss to follow-up in the transition between pediatric and adult Congenital Heart Disease(CHD)care centers.Much less is understood about the loss to follow-up(LTF)after a successful transition.This is critical too,as patients lost to specialised care are more likely to experience mor-bidity and premature mortality.Aims:To understand the prevalence and reasons for loss to follow-up(LTF)at a large Australian Adult Congenital Heart Disease(ACHD)centre.Methods:Patients with moderate or highly complex CHD and gaps in care of>3 years(defined as LTF)were identified from a comprehensive ACHD data-base.Structured telephone interviews examined current care and barriers to clinic attendance.Results:Overall,407(22%)of ACHD patients(n=1842)were LTF.The mean age at LTF was 31(SD 11.5)years and 54%were male;311(76%)were uncontactable.Compared to adults seen regularly,lost patients were younger,with a greater socio-economic disadvantage,and had less complex CHD(p<0.05 for all).We interviewed 59 patients(14%).The top 3 responses for care absences were“feeling well”(61%),losing track of time(36%),and not needing fol-low-up care(25%).Conclusions:A large proportion of the ACHD population becomes lost to specialised cardiac care,even after a successful transition.This Australian study reports younger age,moderate complexity defects,and socio-economic disadvantage as predictive of loss to follow-up.This study highlights the need for novel approaches to patient-centered service delivery even beyond the age of transition and resources to maintain patient engagement within the ACHD service.

Health Systems Strengthening to Tackle the Global Burden of Pediatric and Congenital Heart Disease: A Diagonal Approach

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].

Impact of Social Determinants of Health on Self-Perceived Resilience: An Exploratory Study of Two Cohorts of Adults with Congenital Heart Disease

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.

Machine Learning-Based Intelligent Auscultation Techniques in CongenitalHeart Disease: Application and Development

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.

Use of Patient-Specific “4D” Tele-Education to Enhance Actual and Perceived Knowledge in Congenital Heart Disease (CHD) Patients

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.

Congenital heart“Challenges”in Down syndrome

In this editorial,we comment on the article by Kong et al published in the recent issue of the World Journal of Cardiology.In this interesting case,the authors present the challenges faced in managing a 13-year-old patient with Down syndrome(DS)and congenital heart disease(CHD)associated with pulmonary arterial hypertension.In this distinct population,the Authors underscore the need for early diagnosis and management as well as the need of a multidisciplinary approach for decision making.It seems that the occurrence of CHD in patients with DS adds layers of complexity to their clinical management.This editorial aims to provide a comprehensive overview of the intricate interplay between DS and congenital heart disorders,offering insights into the nuanced diagnostic and therapeutic considerations for physicians.

Long-Term Mortality of Children with Congenital Heart Disease Admitted to the Departmental University Hospital of Borgou/Alibori from 2011 to 2022

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.

Congenital Heart Disease Referred for Surgery: Analysis and Epidemiological Description in the Cardiology Department of CHU Ignace Deen

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.

Effect of maternal pregestational diabetes mellitus on congenital heart diseases

Background The increasing population of diabetes mellitus in adolescent girls and women of childbearing age contributes to a large number of pregnancies with maternal pregestational diabetes mellitus.Congenital heart diseases are a common adverse outcome in mothers with pregestational diabetes mellitus.However,there is little systematic information between maternal pregestational diabetes mellitus and congenital heart diseases in the offspring.Data sources Literature selection was performed in PubMed.One hundred and seven papers were cited in our review,includ-ing 36 clinical studies,26 experimental studies,31 reviews,eight meta-analysis articles,and six of other types.Results Maternal pregestational diabetes mellitus poses a high risk of congenital heart diseases in the offspring and causes variety of phenotypes of congenital heart diseases.Factors such as persistent maternal hyperglycemia,oxidative stress,polymorphism of uncoupling protein 2,polymorphism of adiponectin gene,Notch 1 pathway,Nkx2.5 disorders,dysregula-tion of the hypoxia-inducible factor 1,and viral etiologies are associated with the occurrence of congenital heart diseases in the offspring of mothers with pregestational diabetes mellitus.Treatment options including blood sugar-reducing,anti-oxidative stress drug supplements and exercise can help to prevent maternal pregestational diabetes mellitus from inducing congenital heart diseases.Conclusions Our review contributes to a better understanding of the association between maternal pregestational diabetes mellitus and congenital heart diseases in the offspring and to a profound thought of the mechanism,preventive and therapeutic measurements of congenital heart diseases caused by maternal pregestational diabetes mellitus.

Epidemiological, Clinical Progress Aspects of Congenital Heart Disease with Neonatal Revelation at the Mother-Child Hospital of Bingerville (HME) Concerning 98 Cases from January 2021 to December 2022 (Côte d’Ivoire)

Introduction The distribution of congenital heart disease (CHD) in sub-Saharan Africa is highly imprecise and varies from one region to another due to the inequality of diagnostic facilities. The aim of this study was to determine the in-hospital prevalence of congenital heart disease in children at the Mother-Child hospital of Bingerville (HME) by specifying the diagnostic, therapeutic and evolutionary aspects. Materials and methods We conducted a retrospective, descriptive, cross-sectional study at HME of Bingerville from January 2021 to December 2022. All newborns with congenital heart disease confirmed by echocardiography were included in the study. Results Of 656 admissions to the neonatology department over the study period, congenital heart disease accounted for 14.9% (98/656) of cases. In our series, 76.7% were diagnosed before the 1st week of life, with a mean chronological age of 5.18 days and extremes of 0 and 46 days. There were as many male patients (50%) as female (50%), i.e. a sex ratio of 1. These newborns were premature in 60.2% of cases, with a mean and median gestational age of 34 weeks’ amenorrhea. Most were left-right shunts (90.8%). Persistent ductus arteriosus (PDA) (48.9%) predominated, followed by atrial septal defect (38.7%), ventricular septal defect (13.3%), common trunk artery (CTA) (3.1%) and open septal pulmonary atresia (OSPA) (1%) as the primary cyanogenic heart disease. Pulmonary arterial hypertension (PAH) (50%) was primary in 38.8% and secondary (61.2%). The mortality rate was 30.6%, and all CTA patients died (100%), with a significant statistical relationship (p = 0.027). Progression under treatment was marked by clinical stabilization (68/98) in 69.4% of cases. Conclusion: Congenital heart disease is relatively common at the Bingerville HME. Access to echocardiography should be facilitated in neonatology departments for rapid diagnosis and optimal management of congenital heart disease in newborns.

Analysis of Perioperative Respiratory Care Methods and their Application Value in Children with Congenital Heart Disease

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.

A novel and feasible way to cultivate and purify endothelial progenitor cells from bone marrow of children with congenital heart diseases

Background Endothelial progenitor cells （EPCs） are used in vascular tissue engineering and clinic therapy. Some investigators get EPCs from the peripheral blood for clinic treatment, but the number of EPCs is seldom enough. We have developed the cultivation and purification of EPCs from the bone marrow of children with congenital heart disease, to provide enough seed cells for a small calibre vascular tissue engineering study. Methods The 0.5-ml of bone marrow was separated from the sternum bone, and 5-ml of peripheral blood was collected from children with congenital heart diseases who had undergone open thoracic surgery. CD34＋ and CD34＋NEGFR＋ cells in the bone marrow and peripheral blood were quantified by flow cytometry. CD34＋/VEGFR＋ cells were defined as EPCs. Mononuclear cells in the bone marrow were isolated by Ficoll density gradient centrifugation and cultured by the EndoCult Liquid Medium KitTM. Colony forming endothelial cells was detected. Immunohistochemistry staining for Dil-ac-LDL and FITC-UEA-1 confirmed the endothelial lineage of these cells. Results CD34＋ and CD34＋NEGFR＋ cells in peripheral blood were （0.07±0.05）% and （0.05±0.02）%, respectively. The number of CD34＋ and CD34＋/VEGFR＋ cells in bone marrow were significantly higher than in blood, （4.41±1.47）% and （0.98±0.65）%, respectively （P 〈0.0001）. Many colony forming units formed in the culture. These cells also expressed high levels of Dil-ac-LDL and FITC-UEA-I. Conclusion This is a novel and feasible approach that can cultivate and purify EPCs from the bone marrow of children with congenital heart disease, and provide seed cells for small calibre vascular tissue engineering.

Incidence of congenital heart diseases in Chinese children with non-syndromic congenital blepharoptosis:a prospective observational study of 1053 patients

Background Congenital blepharoptosis(CBP)may be part of a large spectrum of birth defects presenting with other ocular or systemic conditions.Therefore,the aim of the study was to investigate the incidence of congenital heart diseases(CHD)in CBP children not associated with specific syndromes.Methods A total of 1053 Chinese children diagnosed with non-syndromic CBP were consecutively enrolled and their cardiac structure was evaluated by echocardiography.Results Forty children were identified with CHD.Twenty-four children had one type of structural malformation(simple CHD).Sixteen children had two or more types of structural malformation(complex CHD).CHD and complex CHD were more prevalent in patients with severe or bilateral ptosis.Multivariate analysis revealed that presence of severe ptosis and bilateral ptosis was independently associated with CHD occurrence.Conclusions We found an increased frequency of CHD in CBP children,suggesting a clinical need for routine echocardiography evaluation in CBP,especially in children with severe or bilateral ptosis.

Optical coherence tomography of the pulmonary arteries in children with congenital heart diseases:A systematic review

Importance:Optical coherence tomography(OCT)is a high-resolution intravascular imaging tool and has shown promise for providing real-time quantitative and qualitative descriptions of pulmonary vascular structures in vivo in adult pulmonary hypertension(PH),while not popular in pediatric patients with congenital heart diseases(CHD).Objective:The aim of this review is to summarize all the available evidence on the use of OCT for imaging pulmonary vascular remodeling in pediatric patients.Methods:We conducted the systematic literature resources(Cochran Library database,Medline via PubMed,EMBASE,and Web of Knowledge)from January 2010 to December 2021 and the search terms were“PH”,“child”,“children”,“pediatric”,“OCT”,“CHD”,“pulmonary vessels”,“pulmonary artery wall”.Studies in which OCT was used to image the pulmonary vessels in pediatric patients with CHD were considered for inclusion.Results:Five studies met the inclusion criteria.These five papers discussed the study of OCT in the pulmonary vasculature of different types of CHD,including common simple CHD,complex cyanotic CHD,and Williams-Beuren syndrome.In biventricular anatomy,pulmonary vascular remodeling was primarily reflected by pulmonary intima thickening from two-dimensional OCT.In single-ventricle anatomy,due to the state of hypoxia,the morphology of pulmonary vessels was indirectly reflected by the number and shape of nourishing vessels from three-dimensional OCT.Interpretation:OCT may be an adequate imaging procedure for the demonstration of pulmonary vascular structures and provide additional information in pediatric patients.

The association between SNPs of folate metabolism genes and congenital heart diseases:A systematic review and sequential meta-analysis

Background Aims:To summarize the epidemiologic evidence on the association between single nucleotide polymorphisms(SNPs)of folate metabolism genes from parents and children and risk of congenital heart diseases(CHDs)by a comprehensive systematic review and meta-analysis.Methods and results:Pub Med,Embase,Google Scholar,Cochrane Libraries,and Chinese databases were searched to identify potential studies through July2021 For mothers,the polymorphisms of Methylenetetrahydrofolate Reductase(MTHFR)at rs1801133 and rs1801131 were significantly associated with risk of CHDs in the homozygote comparisons(T/T vs C/C at rs1801133:OR:1.50,95%CI:1.31-1.71;C/C vs A/A at rs1801131:OR:1.39,95%CI:1.04-1.86).For fathers,the polymorphisms of MTHFR at rs1801133 were significantly associated with risk of CHDs in the heterozygote comparisons(C/T vs C/C:OR:1.26,95%CI:1.04-1.53).For children,the polymorphisms of MTHFR at rs1801133(T/T vs C/C:OR:2.05,95%CI:1.57-2.66),rs1801131(A/C vs A/A:OR:1.32,95%CI:1.06-1.63),and rs2274976(G/A vs G/G:OR:0.75,95%CI:0.61-0.92),and methionine synthase reductase(MSR)at rs1801394(G/G vs A/A:OR:1.85,95%CI:1.21-2.85)and rs1532268(T/T vs C/C:OR:2.44,95%CI:1.15-5.21;C/T vs C/C:OR:1.53,95%CI:1.11-2.10).This review also assessed the risk of specific CHD subtypes associated with folate metabolism gene SNPs of children.Relevant heterogeneity moderators have been identified by subgroup analysis.Sensitivity analysis yielded consistent results.No evidence of publication bias was observed.Conclusions:The present study indicates that polymorphisms of maternal MTHFR at rs1801133 and rs1801131,parental MTHFR at rs1801133,as well as children’s MTHFR at rs1801133,rs1801131 and rs2274976,and MSR at rs1801394 and rs1532268 are significantly associated with risk of CHDs.