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Transcatheter Closure of Postoperative Residual Atrial or Ventricular Septal Shunts in Patients with congenital heart disease

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congenital heart disease 2024年第3期19卷 293-303页

作者： Jiawang Xiao Jianming Wang Zhongchao Wang Lili Meng Ming Zhao Qiguang Wang Department of congenital heart disease General Hospital of Northern Theater CommandShenyang110016China

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 ***: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 ***: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 *** candidacy was determined based on established *** and electrocardiogram were conducted during the perioperative period and continued as part of long-term ***:Among the patients,5(14.3%)exhibited interatrial shunt-ing,while 30(85.7%)had interventricular *** 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 *** 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 *** complications included one hematoma at the puncture site and one transient junctional rhythm during the perioperative *** 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 ***:TCC is an effective and safe therapy for patients with residual atrial or ventricular septal shunts following CHD *** support the consideration of TCC as the preferred treatment option for appropriate patient populations.

关键词： congenital heart disease residual shunt transcatheter closure surgical repair

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Venopulmonary ECMO Improved Hypoxemia and Supported the Right Ventricle in a Patient with Decompensated Eisenmenger Syndrome

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World Journal of Cardiovascular Surgery 2024年第1期14卷 7-19页

作者： Daniel Manzur-Sandoval Gian Manuel Jiménez-Rodríguez Edgar García-Cruz Ramón Espinosa-Soto Erika Yamali Ramirez-Marcano Yessenia Máyory Téllez-López José Luis Elizalde-Silva Gustavo Rojas-Velasco Cardiovascular Critical Care unit Ignacio Ch&#225vez National Institute of Cardiology Mexico City Mexico adult congenital heart disease unit Ignacio Ch&#225vez National Institute of Cardiology Mexico City Mexico Cardiology Department Ignacio Ch&#225vez National Institute of Cardiology Mexico City Mexico

Mechanical circulatory and/or respiratory assistance with extracorporeal membrane oxygenation (ECMO) has become a standard of care for patients with circulatory (venoarterial) and/or respiratory (venovenous) failure refractory to standard therapies. adult patients with congenital heart disease are an increasingly recognized and growing population and include various groups, such as undiagnosed cases in childhood and palliated and/or corrected cases, which require subsequent care because of residual lesions, cardiac arrest/insufficiency, and arrhythmias, among other conditions. In addition, these patients are prone to developing pathologies that are typical of adulthood with a generally increased risk of morbidity and mortality because of their low reserves and organic damage associated with the underlying heart disease, which makes them candidates for ECMO. These patients represent an additional challenge in this therapy because malformations and the presence of a shunt can generally affect the usual cannulation methods and hemodynamic and oximetry monitoring. Thus, the configuration decision must be made on a case-by-case basis. Here, we present a cannulation method, venopulmonary artery ECMO, which provides hemodynamic and respiratory support, and may be ideal for patients with shunts and/or right ventricular dysfunction. To our knowledge, this is the first report of this configuration in patients with congenital heart diseases.

关键词： Eisenmenger Syndrome Venopulmonary Artery ECMO Refractory Hypoxemia Right Ventricular Dysfunction

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heart Transplantation for adult congenital heart disease:Overview and Special Considerations

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Cardiovascular Innovations and Applications 2018年第B5期3卷 73-84页

作者： Dipankar Gupta Jana Reid Diego Moguillansky Renata Shih Mark S.Bleiweis Frederick J.Fricker Biagio A.Pietra congenital heart Center Department of PediatricsUF Health Shands Children’s HospitalUniversity of FloridaGainesvilleFLUSA

With improvements in their surgical and medical management,the number of patients with congenital heart disease(CHD)reaching adulthood has increased over the last *** the population of adult CHD patients continues to rise,an increasing number of these patients will require evaluation for heart *** is important to recognize advanced heart failure and other associated complications early in this cohort of complex patients for early referral to an adult CHD *** these patients present with unique challenges because of their multiple comorbidities and complex anatomy,there needs to be a careful selection process for transplantation to optimize the utilization of donor organs.

关键词： heart transplantation adult congenital heart disease congenital heart disease heart failure

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Comparing the efficacy and safety of low,medium,and high dosages of selexipag for treating pulmonary hypertension:A systematic review and meta-analysis

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Animal Models and Experimental Medicine 2024年第1期7卷 56-70页

作者： Shang Wang Yi Yan Jian Zhang Ping Yuan Ci-Jun Luo Hong-Ling Qiu Hui-Ting Li Jian Xu Lan Wang Tian-Lan Li Rong Jiang Department of Cardio-Pulmonary Circulation Shanghai Pulmonary HospitalSchool of MedicineTongji UniversityShanghaiChina heart Center and Shanghai Institute of Pediatric congenital heart disease Shanghai Children's Medical CenterNational Children's Medical CenterShanghai Jiaotong University School of MedicineShanghaiChina Department of Respiratory and Critical Care Medicine The 416 Hospital of Nuclear IndustryThe Second Affiliated Hospital of Chengdu Medical CollegeChengduChina Department of Hematology The Affiliated Hospital of Qingdao UniversityQingdaoChina

Background:The maintenance dosage of selexipag is categorized as low,medium or *** order to assess the efficacy and safety of different dosages of selexipag for the risk stratification of pulmonary arterial hypertension(PAH),we performed a sys-tematic review and ***:Studies assessing PAH risk stratification indices,such as the World Health Organization functional class(WHO-FC),six-minute walk distance(6MWD),N-terminal pro-B-type natriuretic peptide(NT-proBNP)level,right atrial pressure(RAP),cardiac index(CI)and mixed venous oxygen saturation(SvO2),were ***:Thirteen studies were *** led to improvements in the 6MWD(MD:24.20 m,95%CI:10.74-37.67),NT-proBNP(SMD:-0.41,95%CI:-0.79-0.04),CI(MD:0.47 L/min/m^(2),95%CI:0.17-0.77)and WHO-FC(OR:0.564,95%CI:0.457-0.697).Subgroup analysis demonstrated that all three dosages improved the 6MWD.A moderate dosage led to improvements in the CI(MD:0.30 L/min/m^(2),95%CI:0.15-0.46)and WHO-FC(OR:0.589,95%CI:0.376-0.922).Within 6 months of treatment,only the WHO-FC and CI were significantly improved(OR:0.614,95%CI:0.380-0.993;MD:0.30 L/min/m^(2),95%CI:0.16-0.45,respectively).More than 6 months of treatment significantly improved the 6MWD,WHO-FC and NT-proBNP(MD:40.87 m,95%CI:10.97-70.77;OR:0.557,95%CI:0.440-0.705;SMD:-0.61,95%CI:-1.17-0.05,respectively).Conclusions:Low,medium,and high dosages of selexipag all exhibited good *** treatment lasted for more than 6 months,selexipag exerted obvious effects,even in the low-dosage *** finding is important for guiding individualized treatments.

关键词： individualized treatments meta-analysis prostacyclin receptor agonist risk stratification systematic review

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Should we consider patent foramen ovale and secundum atrial septal defect as different steps of a single anatomo-clinical continuum？

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Journal of Geriatric Cardiology 2014年第3期11卷 177-179页

作者： Gianluca Rigatelli Section of adult congenital and adult heart disease Cardiovascular Diagnosis and Endoluminal Interventions Rovigo General Hospital Rovigo 45100

1 Introduction Isolated atrial septal defects （ASD） represent 7% of all cardiac anomalies and can present at any age. Adolescents and adults with simple congenital heart disease such as isolated atrial septal defects are more likely to reach adult age without being diagnosed. Secundum ASD （sASD） is by far the most common type, occurring in 1/1500 live births, with 65% to 75% involving females, On the other hand, patent foramen ovale （PFO） represents an endemic variant in the normal population with a prevalence of 25%-27%. These two entities appear so different that is difficult to fred a conjunction ring： nevertheless we use the same philosophy for the treatment, Indeed, device-based closure has been proved to be effective in both settings.

关键词： Anatomy Atrial septal defect Echocardiography Patent foramen ovale

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Optimized RNA interference therapeutics combined with interleukin-2 mRNA for treating hepatitis B virus infection

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Signal Transduction and Targeted Therapy 2024年第7期9卷 3063-3076页

作者： Wenjing Zai Min Yang Kuan Jiang Juan Guan Huijing Wang Kongying Hu Chao Huang Jieliang Chen Wei Fu Changyou Zhan Zhenghong Yuan Key Laboratory of Medical Molecular Virology(MOE/NHC/CAMS) Research Unit of Cure of Chronic Hepatitis B Virus Infection(CAMS)Shanghai Frontiers Science Center of Pathogenic Microbes and InfectionSchool of Basic Medical SciencesShanghai Medical CollegeFudan UniversityShanghaiP.R.China Department of Pharmacology School of Basic Medical SciencesFudan UniversityShanghaiP.R.China Shanghai Engineering Research Center for Synthetic Immunology Fudan UniversityShanghaiP.R.China Eye Institute and Department of Ophthamology Eye and ENT HospitalFudan UniversityShanghaiP.R.China Pharmacy Department of Huashan Hospital Fudan UniversityShanghaiP.R.China Institute of Pediatric Translational Medicine Shanghai Institute for Pediatric Congenital Heart DiseaseShanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong UniversityShanghaiChina Shanghai Institute of Infectious disease and Biosecurity ShanghaiP.R.China

This study aimed to develop a pan-genotypic and multifunctional small interfering RNA(siRNA)against hepatitis B virus(HBV)with an efficient delivery system for treating chronic hepatitis B(CHB),and explore combined RNA interference(RNAi)and immune modulatory modalities for better viral *** synthetic siRNAs targeting consensus motifs distributed across the whole HBV genome were designed and *** lipid nanoparticle(LNP)formulation was optimized by adopting HO-PEG_(2000)-DMG lipid and modifying the molar ratio of traditional polyethylene glycol(PEG)lipid in LNP *** efficacy and safety of this formulation in delivering siHBV(tLNP/siHBV)along with the mouse IL-2(mIL-2)mRNA(tLNP/siHBVIL2)were evaluated in the rAAVHBV1.3 mouse model.A siRNA combination(terms“siHBV”)with a genotypic coverage of 98.55%was selected,chemically modified,and encapsulated within an optimized LNP(tLNP)of high efficacy and security to fabricate a therapeutic formulation for *** results revealed that tLNP/siHBV significantly reduced the expression of viral antigens and DNA(up to 3log_(10)reduction;vs PBS)in dose-and time-dependent manners at single-dose or multi-dose frequencies,with satisfactory safety *** studies showed that tLNP/siHBVIL2 enables additive antigenic and immune control of the virus,via introducing potent HBsAg clearance through RNAi and triggering strong HBV-specific CD4^(+)and CD8^(+)T cell responses by expressed mIL-2 *** adopting tLNP as nucleic acid nanocarriers,the co-delivery of siHBV and mIL-2 mRNA enables synergistic antigenic and immune control of HBV,thus offering a promising translational therapeutic strategy for treating CHB.

关键词： formulation enable offering

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

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World Journal of Nephrology 2024年第1期13卷 25-32页

作者： Jose Daniel Juarez-Villa Iván Zepeda-Quiroz Sebastián Toledo-Ramírez Victor Hugo Gomez-Johnson Francisco Pérez-Allende Brian Ricardo Garibay-Vega Francisco E Rodríguez Castellanos Bernardo Moguel-González Edgar Garcia-Cruz Salvador Lopez-Gil Department of Nephrology Instituto Nacional de Cardiología Ignacio ChavezMexico City 14080Mexico congenital heart disease Instituto Nacional de Cardiología Ignacio ChavezMexico City 14080Mexico

BACKGROUND The association between congenital heart disease and chronic kidney disease is well *** mechanisms of kidney damage associated with congenital heart disease have been *** 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 *** To identify baseline and clinical characteristics,as well as the findings in kidney biopsies of patients with congenital heart disease in our *** 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 *** Ten patients with congenital heart disease and kidney biopsy were *** average age was 29.00 years±15.87 years with pre-biopsy proteinuria of 6193 mg/24 h±6165 mg/24 *** most common congenital heart disease was Fallot’s tetralogy with 2 cases(20%)and ventricular septal defect with 2(20%)*** 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 *** remaining 8 cases(80%),one case of FSGS with perihilar variety was found,while the other 7 cases were non-specific *** Determining the cause of chronic kidney disease can help in delaying the need for kidney replacement *** 2 out of 10 patients in our study,interventions were performed,and initiation of kidney replacement therapy was *** studies are needed to determine the usefulness of kidney biopsy in patients with congenital heart disease.

关键词： Renal biopsy congenital heart disease Chronic kidney disease Focal segmental glomerulosclerosis

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Risk Stratification and Prognosis of Pulmonary Arterial Hypertension Associated with congenital heart disease

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congenital heart disease 2024年第3期19卷 325-339页

作者： Wenjie Dong Zhibin Hong Aqian Wang Kaiyu Jiang Hai Zhu Fu zhang Zhaoxia Guo Hongling Su Yunshan Cao Department of Cardiology The First People’s Hospital of TianshuiTianshui741000China Department of Cardiology Pulmonary Vascular Disease CenterGansu Provincial HospitalLanzhou730000China heart Lung and Vessels CenterSichuan Provincial People’s HospitalUniversity of Electronic Science and Technology of ChinaChengdu610072China

Background:Current guidelines for managing pulmonary arterial hypertension(PAH)recommend a risk strati-fication ***,the applicability and accuracy of these strategies for PAH associated with congenital heart disease(PAH-CHD)require further *** study aims to validate the reliability and predictive accuracy of a simplified stratification strategy for PAH-CHD patients over a three-year ***,new prognostic variables are identified and novel risk stratification methods are developed for assessing and managing PAH-CHD ***:This retrospective study included 126 PAH-CHD *** and biochemical variables across risk groups were assessed using Kruskal-Wallis and Fisher’s exact ***-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 ***:Significant survival differences across stratified risk groups were observed(p<0.001),validating the effectiveness of the simplified risk stratification method in PAH-CHD *** 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 ***:The simplified risk stratification method is applicable to PAH-CHD *** 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.

关键词： Pulmonary hypertension pulmonary arterial hypertension congenital heart disease risk stratification

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Left atrial dysfunction in elderly patients with patent foramen ovale and atrial septal aneurysm

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Journal of Geriatric Cardiology 2009年第4期6卷 195-198页

作者： Gianluca Rigatelli Fabio Dell＇Avvocata Federico Ronco Massimo Giordan Paolo Cardaioli Section of adult congenital and adult heart disease Cardiovascular Diagnosis and Endoluminal Interventions Rovigo GeneralHospital Rovigo 45100 Italy

Objective Recently it has been suggested that, in patients with large patent foramen ovale （PFO） and atrial septal aneurysms （ASA）, a certain amount of left atrial （LA） dysfunction may be active as an alternate mechanism promoting arterial embolism. Following this hypothesis, elderly patients, being more susceptible to atrial chambers stiffness, should present a more severe LA dysfunction profile. We sought to evaluate the grade of LA dysfunction in elderly patients submitted to transcatheter PFO closure. Methods We retrospectively enrolled 28 consecutive patients with previous stroke （mean age 674-12.5 years, 18 females） referred to our centre for catheter-based PFO closure after recurrent stroke. Baseline values of LA passive and active emptying, LA conduit function, LA ejection fraction, and spontaneous echocontrast （SEC） in the LA and LA appendage were compared with those of 50 atrial fibrillation patients, as well as a sex/age/cardiac risk matched population of 70 healthy controls. Results Pre-closure elderly subjects demonstrated significantly greater reservoir function as well as passive and active emptying, with reduced conduit function and LA ejection fraction, when compared to healthy and younger patients. After closure in elderly patients, LAparameters did not return completely to the levels of healthy patients, whereas LA dysfunction in younger subjects returned normal. Conclusions This study suggests that elderly patients have more severe LA dysfunction than younger patients, which affects the LA remodelling after closure.

关键词： patent foramen ovale stroke embolism cardiac anatomy echocardiography

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Patent foramen ovale in the elderly:what to do?

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Journal of Geriatric Cardiology 2007年第4期4卷 254-256页

作者： Gianluca Rigatelli Fabio Dell’Avvocata Section of adult congenital and Structural heart disease Cardiovascular Diagnosis and Endoluminal Interventions Rovigo General Hospital Rovigo Italy

The increase in life expectance makes the diagnosis of PFO a possible and not easily manageable event in patients > 60-years-old due to the presence of different comorbidities and in particular of diastolic dysfunction which is considered as a contraindication to PFO closure. The literature review suggests that aged patients with PFO cannot be excluded"a priori"from PFO closure that should evaluated as therapeutic options in presence of anatomical and functional indications. Moreover in the elderly many other syndromes than paradoxical stroke mediated by PFO required full assessment and, if needed, transcather PFO closure:deoxygenating in obstructive sleeping apnoea, unexplained increased dyspnoea associated with hypoxemia after lung surgery, paralysis of the hemidiaphragm, and platypnea orthodeoxia. Differently from in the young and middle age, the management of PFO in aged patients should obligatory include the careful evaluation of potential comorbidities and eventual contraindications, such as severe diastolic dysfunction due to for example to hypertensive cardiomyopathy and coronary heart disease, the main causes of diastolic dysfunction.(J Geriatr Cardiol 2007;4:254-256.)

关键词： stroke patent foramen ovale elderly transcatheter closure

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