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Preoperative serum cortisone levels are associated with cognition in preschool‑aged children with tetralogy of fallot after corrective surgery:new evidence from human populations and mice

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World Journal of Pediatrics 2024年第2期20卷 173-184页

作者： Si-Yu Ma Yu-Ting Liu Yue-Shuang Cun Qiang Wang Ming-Cui Fu Ke-De Wu Xin-Yu Cai Shu-Ting Cheng Nishant Patel Min Da Liang Hu Zhuoga Deqin Xue-Jun Kang Ming Yang Xu-Ming Mo Department of Cardiothoracic Surgery Children’s Hospital of Nanjing Medical University72 Guangzhou RoadNanjing 210008China Department of Radiology Children’s Hospital of Nanjing Medical University72 Guangzhou RoadNanjing 210008China Key Laboratory of Child Development and Learning Science Research Center For Learning ScienceSchool of Biological Sciences&Medical EngineeringMinistry of EducationSoutheast UniversityNanjing 210096China Medical School of Nanjing University Nanjing 210093China

Background tetralogy of fallot(TOF)is the most common cyanotic congenital heart *** with TOF would be confronted with neurological impairment across their *** study aimed to identify the risk factors for cerebral morphology changes and cognition in postoperative preschool-aged children with *** We used mass spectrometry(MS)technology to assess the levels of serum metabolites,Wechsler preschool and primary scale of intelligence-Fourth edition(WPPSI-Ⅳ)index scores to evaluate neurodevelopmental levels and multimodal magnetic resonance imaging(MRI)to detect cortical morphological *** Multiple linear regression showed that preoperative levels of serum cortisone were positively correlated with the gyrification index of the left inferior parietal gyrus in children with TOF and negatively related to their lower visual spaces index and nonverbal ***,preoperative SpO_(2) was negatively correlated with levels of serum cortisone after adjusting for all ***,after intervening levels of cortisone in chronic hypoxic model mice,total brain volumes were reduced at both postnatal(P)11.5 and P30 *** Our results suggest that preoperative serum cortisone levels could be used as a biomarker of neurodevelopmental impairment in children with *** study findings emphasized that preoperative levels of cortisone could influence cerebral development and cognition abilities in children with TOF.

关键词： Cognition Cortisone MRI Neurodevelopment tetralogy of fallot

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Severe hypoxemia after radiofrequency ablation for atrial fibrillation in palliatively repaired tetralogy of fallot: A case report

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World Journal of Cardiology 2024年第3期16卷 161-167页

作者： Zhi-Hang Li Lian Lou Yu-Xiao Chen Wen Shi Xuan Zhang Jian Yang Department of Cardiovascular Medicine The First Affiliated Hospital of Zhejiang University School of MedicineHangzhou 310000Zhejiang ProvinceChina Department of Cardiology The First Affiliated Hospital of Zhejiang University School of MedicineHangzhou 310003Zhejiang ProvinceChina

BACKGROUND Patients with tetralogy of fallot(TOF)often have arrhythmias,commonly being atrial fibrillation(AF).Radiofrequency ablation is an effective treatment for AF and does not usually cause severe postoperative hypoxemia,but the risk of complications may increase in patients with conditions such as *** SUMMARY We report a young male patient with a history of TOF repair who developed severe hypoxemia after radiofrequency ablation for AF and was ultimately confirmed to have a new right-to-left *** patient subsequently underwent atrial septal occlusion and eventually *** Radiofrequency ablation may cause iatrogenic atrial septal injury;thus possible complications should be predicted in order to ensure successful treatment and patient safety.

关键词： Atrial fibrillation Radiofrequency ablation tetralogy of fallot Right-to-left shunt Hypoxemia Medical decision Case report

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Single-Cell RNA Sequencing Reveals Potential for Endothelial-to-Mesenchymal Transition in tetralogy of fallot

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Congenital Heart Disease 2023年第6期18卷 611-625页

作者： Aisa Zulibiya Jing Wen Huiqing Yu Xiaoming Chen Lei Xu Xiao Ma Baojian Zhang State Key Laboratory of Pathogenesis Prevention and Treatment of High Incidence Diseases in Central AsiaUrumqi830002China Department 5 of Cardiology Affiliated Hospital of Traditional Chinese Medicine of Xinjiang Medical UniversityUrumqi830002China Department of Cardiology The First Affiliated Hospital of Jinan UniversityGuangzhou510630China

Background:tetralogy of fallot(TOF)is a very common cyanotic congenital heart ***-to-mesenchymal transition(EndoMT)is recognized as a physiological mechanism involved in embryonic heart development and endothelial ***,there is still a gap in the reports related to the mechanism of EndoMT development in ***:First,transcriptomic data of single cell nuclei of TOF and Donor were obtained based on the Gene Expression Omnibus(GEO)database,and the data were normalized and clus-tered by dimensionality reduction using the Seurat ***,differentially expressed genes(DEGs)between TOF and Donor were screened using the“FindMarkers”function,and the gene sets of interest were ***,to characterize the dynamics of EndoMT occurrence in TOF,we performed pseudotime cell tra-jectory inference as well as utilized SCENIC analysis to probe the gene regulatory networks(GRNs)dominated by transcription factors(TFs)in endothelial ***:We identiﬁed a total of six cell clusters based on single-cell nuclear transcriptome data from TOF and *** found that 611 genes with up-regulated expression within TOF showed conversion to *** subdividing endothelial cell subtypes,endothelial cells 2 were shown to be involved in cell adhesion,migration and extracellular matrix ***-time and SCENIC analyses showed that endothelial cell 2 has EndoMT *** addition,ERG and TEAD1 are TFs that play key reg-ulatory roles in this subtype,and both of their target genes are also highly expressed in *** demonstrates that ERG and TEAD1 effectively promote the EndoMT ***:Our study reveals the molecular mechanisms underlying the development of EndoMT in TOF,which demonstrates that manipulating the endothelial-to-mesenchymal transition may offer unprecedented therapeutic potential for the treatment of TOF.

关键词： tetralogy of fallot endothelial cells endothelial-mesenchymal transition congenital heart disease cellular subpopulation

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Growth of the Pulmonary Valve Annulus after the Modified Blalock-Taussig Shunt in Patients with tetralogy of fallot

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Congenital Heart Disease 2021年第5期16卷 433-441页

作者： Siraphop Thapmongkol Jarun Sayasathid Jessada Methrujpanont Kanthachat Thatsakorn Worawan Jittham Suwanna Puitm Methiniwiran Thapmongkol Jule Namchaisiri Division of Cardiothoracic Department of SurgeryFaculty of MedicineNaresuan University HospitalNaresuan UniversityPhitsanulok65000Thailand Division of Cardiothoracic Department of SurgeryBuddachinaraj HospitalPhitsanulok65000Thailand Division of Pediatric Cardiology Department of PediatricFaculty of MedicineNaresuan University HospitalNaresuan UniversityPhitsanulok65000Thailand Non-Infectious Ward Department of PediatricNaresuan University HospitalPhitsanulok65000Thailand Department of Adult Nursing Faculty of Nursing Naresuan UniversityNaresuan UniversityPhitsanulok65000Thailand Division of Cardiothoracic Department of SurgeryFaculty of MedicineKing Chulalongkorn Memorial HospitalChulalongkorn UniversityBangkok10330Thailand

Background:The surgical outcomes of tetralogy of fallot(TOF)have evolved dramatically and have resulted in lower mortality ***,the many cardiac centers have a trend to early single-stage complete repair more than a staged ***,the patients who have an early primary repair were required transannular patch augmentation of a pulmonary valve *** effect has been developed a chronic pulmonary insufficiency may lead to right ventricular dilation,*** this era,the aim of treatment of TOF is attempted to preserve pulmonary valve annulus for prevent right ventricular dysfunction in the *** systemic to pulmonary artery shunt is a palliative procedure or known as staged repair for symptomatic patients with *** modified Blalock-Taussig shunt(mBTS)is the most useful systemic to pulmonary shunt and perform as an initial procedure before complete *** mBTS can provide increase pulmonary blood flow as well as improve oxygenation and also promote pulmonary artery(PA)***,the effect of this procedure to promote growth of a pulmonary valve annulus is still ***:To compare a growth of pulmonary valve annulus between after staged repair and primary repair in patients with TOF(without pulmonary atresia).Methods:A retrospective case-control study,review of patients with TOF underwent total repair at our hospitals from January 2005 and December 2017 was performed,a total number of 112 patients underwent TOF ***-nine patients(26%)underwent a staged repair(mBTS group)and 83(74%)underwent total repair only or primary repair(PR group).We evaluated diameter of pulmonary valve annulus by using echocardiography at the time of first diagnosis and before complete repair on both ***:The age of diagnosis of mBTS group were younger than PR group(p=0.011).Therefore,pulmonary valve annuls were smaller in mBTS group.(Z-score,−2.93±1.42 vs.−1.89±0.97;p=0.001).However,the growth potential of pulmonary val

关键词： tetralogy of fallot systemic to pulmonary shunt modified Blalock-Taussig shunt pulmonary valve annulus pulmonic valve Z score

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Expression of Cx43-related microRNAs in patients with tetralogy of fallot

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World Journal of Pediatrics 2014年第2期10卷 138-144页

作者： Yao Wu Xiao-Jing Ma Hui-Jun Wang Wen-Can Li Long Chen Duan Ma Guo-Ying Huang Children's Hospital of Fudan University Key Laboratory of Molecular Medicine Ministry of EducationFudan UniversityShanghai 200032China Department of Forensic Medicine Fudan UniversityShanghai 200032China

Background:Abnormal expression of connexin 43(Cx43)has been reported to play an important role in the development of conotrunccal ***,less is known about the underlying reason for its abnormal ***(miRNAs),as an important part of gene expression regulation,have been implicated in some cardiac *** study aimed to investigate the expression of Cx43 and its related miRNAs in patients with tetralogy of fallot(TOF),and illustrate the potential role of abnormal miRNAs regulation to Cx43 expression in the pathology of ***:Real-time polymerase chain reaction was used to detect the expression of Cx43 and 10 Cx43-related miRNAs in the myocardium from 30 TOF patients and 10 normal *** was used to detect Cx43 protein *** miRNA binding sites in the 3'UTR of Cx43 were examined in 200 TOF patients and 200 healthy individuals,using Sanger sequencing,to exclude sequence variations resulting in binding diffi culties of ***:Cx43 mRNA and protein expression in the myocardium tissue was significantly increased in TOF *** expression of MiR-1 and 206 was significantly decreased in the TOF patients as compared with the controls(P0.05).No meaningful sequence variation was detected in the putative miR1/206 binding sites in the 3'UTR of ***:This study indicated that miR-1 and 206 is down-regulated in TOF patients,which may cause an up-regulation of Cx43 protein's *** provided a clue that miR-1 and 206 might be involved in the pathogenesis of TOF,additional experiments are needed to determine if in fact,miR-1 and 206 contribute substantially to TOF.

关键词： congenital heart disease Cx43 miRNA tetralogy of fallot

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Contemporary Patterns of Management of tetralogy of fallot:Data from a Single Center in China

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Congenital Heart Disease 2021年第1期16卷 53-64页

作者： Xianchao Jiang Jinyang Liu Bo Peng Heng Zhang Shoujun Li Jun Yan Qiang Wang Center for Pediatric Cardiac Surgery Fuwai HospitalNational Center for Cardiovascular DiseasesChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina Department of Cardiac Surgery Yunnan Fuwai Cardiovascular HospitalKunmingChina

Background:There is scarce research on large cohorts with tetralogy of fallot(TOF)from *** database in Fuwai Hospital was reviewed to ascertain current trends in the management of TOF and to determine the prevalence of various surgical techniques and the optimal early ***:This cross-sectional study included 1861 patients who underwent surgery between 2012 and 2017 and were aged 0-18 years old with a primary diagnosis of TOF.A total of 1760 eligible patients were included in the ***:A total of 1683 patients underwent repair of TOF as a one-stage operation(primary repair).Sixty-one patients underwent repair of TOF after prior *** patients who underwent one-stage repair(n=1683):858 were 6 months to 1 year old,421 were 1 to 2 years old,251 were 2-18 years old,and 145 were 3 to 6 months *** patients who underwent repair following prior palliation(n=61),58(95.1%)were older than 1 year of *** 1744 complete repairs,986(56.0%)had annulus-sparing(AS)*** in-hospital mortality was 15 of 1744(0.9%)for complete repair(including one-stage and staged repairs).The total incidence of the optimal early outcome was 78.2%in terms of a composite of the absence of death in the first year,significant right ventricular outflow tract obstruction,significant pulmonary valve insufficiency,or catheter or surgical ***:Surgical correction in patients with TOF can achieve an acceptable outcome in terms of death and *** repair at 6 months to 1 year of age is the most prevalent strategy in our ***,the relatively high incidence of early undesirable surgical adequacy of the pulmonary valve(PV)represents a wake-up call.

关键词： tetralogy of fallot chinese contemporary patterns of management cross-sectional study

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Relation or Influence of RVOTO in the Inflammatory Response to Reoxygenation in Patients with tetralogy of fallot

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Congenital Heart Disease 2021年第5期16卷 443-455页

作者： Hong Liu Luyao Ma Jinghang Li Bingqi Sun Siqiang Zheng Yongfeng Shao Department of Cardiovascular Surgery the First Affiliated Hospital of Nanjing Medical UniversityNanjing210029China Department of Cardiovascular Surgery Graduate SchoolTianjin Medical UniversityTianjin300070China Department of Cardiovascular Surgery Graduate SchoolChinese Academy of Medical Sciences&Peking Union Medical CollegeBeijing100730China

Background:This study evaluated differential inflammatory response to cardiopulmonary bypass reoxygenation in tetralogy of fallot ***:We performed a retrospective study at a cardiovascular center from 2012 to 2018,including 500 patients aged 1 week–18 years who received complete repair of tetralogy of *** were grouped according to tertiles of preoperative RVOT gradient on echocardiography into mild,moderate,and severe *** measured the highest perfusate oxygenation(PpO_(2))during aortic occlusion as independent *** outcome was systemic inflammatory response syndrome(SIRS)within 7 days postoperatively or the time of death or ***:Overall,rate of SIRS was 24.2% without significant differences among three groups(P>0.05).Older age,male,and smaller indexed left ventricular end-diastolic volume is independent risk factor of *** were significant interactions between RVOT stenosis and PpO2 on SIRS(P interaction=0.011):higher PpO_(2) was associated with a greater SIRS risk among combined moderate and severe stenotic children(OR 1.46395%CI[1.080,1.981]per-SD increase,P=0.014)but not among mild stenotic children(OR 0.900[0.608,1.333]per-SD increase;P=0.600),independent of ***:The association of PpO_(2) with SIRS was modified by RVOT obstruction severity in tetralogy of fallot repair.

关键词： Cardiopulmonary bypass tetralogy of fallot hypoxia/reoxygenation injury systemic inflammatory response syndrome

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Pulmonary Perfusion Asymmetry in Patients after Repair of tetralogy of fallot: A 4D Flow MRI-Based Study

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Congenital Heart Disease 2022年第2期17卷 117-128页

作者： Elena Panaioli Duarte Martins Marc Antoine Isorni Diala Khraiche Antoine Legendre Nathalie Boddaert Damien Bonnet Filippo Crea Francesca Raimondi Unite Medico-Chirurgicale de Cardiologie Congénitale et Pediatrique Centre de Référence des Maladies Cardiaques Congénitales ComplexesHopital Universitaire Necker-Enfants MaladesUniversitéde ParisParisFrance Department of Cardiovascular and Thoracic Sciences Fondazione Policlinico Universitario A.Gemelli IRCCSRomeItaly Catholic University of the Sacred Heart RomeItaly Pediatric Cardiology Department Hospital de Santa CruzCentro Hospitalar Lisboa OcidentalLisbonPortugal Centre de Cardiologie Evecquemont ParisFrance Pediatric Radiology Unit Hôpital Universitaire Necker-Enfants MaladesUniversitéde ParisParisFrance Decision and Bayesian Computation Computation Biology DepartmentNeuroscience DepartmentParisFrance School of Biomedical Engineering&Imaging Sciences King’s College LondonLondonUK

Background:Repaired tetralogy of fallot(rTOF)patients may have residual lesions such as main(MPA)and branch pulmonary artery stenosis(BPAS).While MPA stenosis is well studied,few data are available on BPAS in *** aimed to describe pulmonary perfusion in a large paediatric cohort of rTOF and its impact on right ventricular and outflow-tract hemodynamics using 4D flow ***:130 consecutive patients(mean age at CMR 14.3±4.6 years)were retrospectively reviewed.96 patients had transannular patch without valve preservation while 34 patients had conserved annulus or valved conduit.A pulmonary blood flow ratio(right pulmonary artery(RPA)/left pulmonary artery(LPA))between 0.75 and 1.56 was considered ***:Asymmetric pulmonary perfusion was present in 59/130 patients(45%),with 54/59(91%)having left lung hypoperfusion(blood flow ratio>1.56).RPA/LPA perfusion ratio in the whole cohort was independently associated with the LPA Z-score(−0.053,p=0.007),the RPA regurgitant fraction(RF)(0.013,p=0.011)and previous LPA stenting(0.648,p=0.004).Decreasing LPA%perfusion(and conversely increasing RPA%perfusion)was significantly associated with higher MPA diameter Z-score(−0.06,p=0.007).On multivariate analysis,MPA Z-score was independently associated with pulmonary RF(0.48,p<0.001)and with right ventricular indexed volumes(coefficient 3.6,p=0.023).In patients with transannular patch repair,asymmetric pulmonary flow was an independent predictor of right ventricular ejection fraction(RVEF)(−3.66,p=0.04).Conclusions:Pulmonary perfusion asymmetry is frequent in rTOF and is associated with abnormal right ventricular and outflow-tract hemodynamics,including MPA dilatation and decreased RVEF in patients after transannular patch.

关键词： tetralogy of fallot cardiac MRI 4D flow MRI pulmonary perfusion

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Pulmonary Hypertension Crisis in Patient with tetralogy of fallot and Mixed Total Anomalous Pulmonary Vein Connection after the Primary Correction:A Rare Case Report

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Congenital Heart Disease 2023年第6期18卷 671-678页

作者： Dian Kesumarini Yunita Widyastuti Cindy Elfira Boom Lucia Kris Dinarti Department of Anesthesia and Intensive Therapy National Cardiovascular Center Harapan KitaJakartaIndonesia Doctoral Program Faculty of Medicine and Public HealthUniversity of Gadjah MadaYogyakartaIndonesia Department of Anesthesia and Intensive Therapy Universitas Gadjah Mada/Dr.Sardjito HospitalYogyakartaIndonesia Department of Cardiology and Vascular Medicine Universitas Gadjah Mada/Dr.Sardjito HospitalYogyakartaIndonesia

tetralogy of fallot(TOF)with total anomalous pulmonary vein connections(TAPVC)is a rare type of complex congenital heart disease among all TOF ***-presentation of major aortopulmonary collateral arteries(MAPCAs)compensates for the lack of central pulmonary bloodﬂow and decreases the severity of right-to-left shunting in *** present a case of a 2-year-old child with complex diagnoses of TOF,TAPVC,a large secun-dum atrial septal defect(ASD),and intraoperatively identiﬁed *** underwent surgery to repair the TAPVC,valve-sparing reconstruction of the right ventricular outﬂow tract,interventricular defect closure,and the creation of patent foramen ovale(PFO).After the operation,hemodynamic instability happened along with sudden blood pressure drop,desaturation,and increased central venous pressure,which subsided after adminis-tering inhalational nitric oxide(NO).A postoperative pulmonary hypertension crisis was suggested when the patient experienced recurrent symptoms after the termination of ***ﬁndings of a D-shaped left ventricle(LV),right-to-left PFO shunt and high tricuspid valve gradientﬁrmly established the *** was subsequently managed with continuous NO inhalation and sildenaﬁl,which rendered a satisfactory *** TOF and TAPVC could be at particular risk of developing pulmonary hypertension crisis,especially in the presence of MAPCAs due to possible remodeling of the pulmonary ***,a relatively non-compliant LV function and small left atrial size may exacerbate the risk of developing postcapillary pulmonary hypertension after TAPVC repair.A successful postoperative outcome calls for a meticulous preoperative analysis of the anatomical lesions,as well as careful monitoring.

关键词： Pulmonary hypertension crisis major aortopulmonary collateral arteries tetralogy of fallot total anomalous pulmonary vein connection congenital heart disease case report

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Evaluation of Right Ventricular Global Longitudinal Function in Patients with tetralogy of fallot by Two-dimensional Ultrasound Speckle Tracking Imaging

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Journal of Huazhong University of Science and Technology(Medical Sciences) 2010年第1期30卷 126-131页

作者：李玉曼谢明星王新房吕清卢晓芳杨亚利马红方凌云张静李卫芹 Department of Ultrasonography Union HospitalTongji Medical CollegeHuazhong University of Science and Technology

Quantification of right ventricular(RV)volume and function remains a challenge because of RV complex geometry by conventional *** purpose of this study was to assess RV global longitudinal function in patients with tetralogy of fallot(TOF)by 2-dimensional ultrasound speckle tracking imaging(STI).Thirty-eight patients with TOF were enrolled in this study and divided into child group(n=25)and adult group(n=13)according to ***-eight age-and sex-matched normal subjects were selected as c...

关键词： echocardiography tetralogy of fallot ventricular function global right 2-dimensional speckle tracking

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