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Loss to Specialized Cardiology Follow-Up in Adults Living with Congenital Heart Disease
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作者 Cheryl Dickson Danielle Osborn +3 位作者 David Baker Judith Fethney David S.Celermajer Rachael Cordina congenital heart disease SCIE 2024年第1期49-63,共15页
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 succ... 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. 展开更多
关键词 Loss to follow-up FOLLOW-UP adult congenital heart disease lapse in care gaps in care care gaps care continuity ACHD predictors
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Femoral Access with Ultrasound-Guided Puncture and Z-Stitch Hemostasis for Adults with Congenital Heart Diseases Undergoing Electrophysiological Procedures
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作者 Fu Guan Matthias Gass +3 位作者 Florian Berger Heiko Schneider Firat Duru Thomas Wolber congenital heart disease SCIE 2024年第1期85-92,共8页
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
关键词 Congenital heart disease cardiac electrophysiology cardiac catheterization femoral access HEMOSTASIS
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Impact of Atrial Septal Defect Closure on Mortality in Older Patients
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作者 Sipawath Khamplod Yodying Kaolawanich +1 位作者 Khemajira Karaketklang Nithima Ratanasit congenital heart disease SCIE 2024年第1期93-105,共13页
Background:Atrial septal defect(ASD)is a common form of adult congenital heart disease that can lead to long-term adverse outcomes if left untreated.Early closure of ASD has been associated with excellent outcomes and... Background:Atrial septal defect(ASD)is a common form of adult congenital heart disease that can lead to long-term adverse outcomes if left untreated.Early closure of ASD has been associated with excellent outcomes and lower complication rates.However,there is limited evidence regarding the prognosis of ASD closure in older adults.This study aims to evaluate the mortality rates in older ASD patients with and without closure.Methods:A retrospective cohort study was conducted on patients aged 40 years or older with ASD between 2001 and 2017.Patients were followed up to assess all-cause mortality.Univariable and multivariable analyses were performed to identify the predictors of mortality.A p-value of<0.05 was considered statistically significant.Results:The cohort consisted of 450 patients(mean age 56.6±10.4 years,77.3%female),with 66%aged between 40 and 60 years,and 34%over 60 years.Within the cohort,299 underwent ASD closure(201 with transcatheter and 98 with surgical closure).During the median follow-up duration of 7.9 years,51 patients died.The unadjusted cumulative 10-year rate of mortality was 3%in patients with ASD closure,and 28%in patients without ASD closure(log-rank p<0.001).Multivariable analysis revealed that age(hazard ratio[HR]1.04,95%confidence interval[CI]1.006–1.06,p=0.01),NYHA class(HR 2.75,95%CI 1.63–4.62,p<0.001),blood urea nitrogen(BUN)(HR 1.07,95%CI 1.03–1.12,p<0.001),right ventricular systolic pressure(RVSP)(HR 1.07,95%CI 1.003–1.04,p=0.01),and lack of ASD closure(HR 15.12,95%CI 5.63–40.59,p<0.001)were independently associated with mortality.Conclusion:ASD closure demonstrated favorable outcomes in older patients.Age,NYHA class,BUN,RVSP,and lack of ASD closure were identified as independent factors linked to mortality in this population. 展开更多
关键词 Atrial septal defect congenital heart disease defect closure long-term survival MORTALITY
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Health Systems Strengthening to Tackle the Global Burden of Pediatric and Congenital Heart Disease: A Diagonal Approach
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作者 Dominique Vervoort Amy Verstappen +2 位作者 Sreehari Madhavankutty Nair Chong Chin Eu Bistra Zheleva congenital heart disease SCIE 2024年第2期131-138,共8页
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
关键词 Congenital heart disease pediatric heart disease global health health systems health policy
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Transcatheter Closure of Postoperative Residual Atrial or Ventricular Septal Shunts in Patients with Congenital Heart Disease
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作者 Jiawang Xiao Jianming Wang +3 位作者 Zhongchao Wang Lili Meng Ming Zhao Qiguang Wang congenital heart disease SCIE 2024年第3期293-303,共11页
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
关键词 Congenital heart disease residual shunt transcatheter closure surgical repair
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Risk Stratification and Prognosis of Pulmonary Arterial Hypertension Associated with Congenital Heart Disease
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作者 Wenjie Dong Zhibin Hong +6 位作者 Aqian Wang Kaiyu Jiang Hai Zhu Fu zhang Zhaoxia Guo Hongling Su Yunshan Cao congenital heart disease SCIE 2024年第3期325-339,共15页
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
关键词 Pulmonary hypertension pulmonary arterial hypertension congenital heart disease risk stratification
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Impact of Social Determinants of Health on Self-Perceived Resilience: An Exploratory Study of Two Cohorts of Adults with Congenital Heart Disease
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作者 Albert Osom Krysta S.Barton +5 位作者 Katie Sexton Lyndia Brumback Joyce P.Yi-Frazier Abby R.Rosenberg Ruth Engelberg Jill M.Steiner congenital heart disease SCIE 2024年第1期33-48,共16页
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
关键词 Social determinants of health adult congenital heart disease RESILIENCE financial stability
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A 63-Year-Old Male with D-Transposition of the Great Arteries Who Had an Early Form of the Arterial Switch Operation
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作者 Michael A.Rebolledo Jane S.Yao +2 位作者 Jason N.Johnson Umar S.Boston Benjamin R.Waller III congenital heart disease SCIE 2024年第1期65-68,共4页
We describe a 63-year-old male who appears to have undergone an early form of the arterial switch operation for D-transposition of the great arteries performed in the mid-1960s.We review the clinical and imaging data ... We describe a 63-year-old male who appears to have undergone an early form of the arterial switch operation for D-transposition of the great arteries performed in the mid-1960s.We review the clinical and imaging data that support our conclusion.He had a diagnostic cardiac catheterization which demonstrated severe pulmonary hypertension responsive to epoprostenol and oxygen.Our case may represent one example of the experimental surgical work done prior to Dr.Adibe Jatene’s description of thefirst successful arterial switch performed in 1975. 展开更多
关键词 Adult congenital heart disease arterial switch operation great vessel anomaly imaging(all modalities)
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Stubborn Hypoxia in Neonates with D-Transposition of the Great Arteries after Arterial Switch Operation: Central Sleep Apnea as the Cause and Potential Indicator of Brain Immaturity
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作者 Camden L.Hebson Kyle Bliton +4 位作者 Amr Y.Hammouda Kaitlyn Barr W.Hampton Gray Mohini Gunnett Waldemar F.Carlo congenital heart disease SCIE 2024年第2期185-195,共11页
D-transposition of the great arteries(d-TGA)is surgically repaired with the arterial switch operation(ASO)with excellent results,however short and long-term morbidities still develop including neurocognitive delay.Cli... D-transposition of the great arteries(d-TGA)is surgically repaired with the arterial switch operation(ASO)with excellent results,however short and long-term morbidities still develop including neurocognitive delay.Clinically significant central sleep apnea is uncommon in non-premature infants,but when present indicates immature autonomic control of respiration likely due to a neurologic disorder.We report the unanticipatedfinding of central sleep apnea in four-term neonates with d-TGA after uncomplicated ASO,with the short-term complication of delayed hospital discharge and long-term concerns regarding this early marker of brain immaturity and its hindrance to normal development.Within this report,we will review each patient’s clinical course and then examine the literature on pediatric central sleep apnea,neurodevelopmental outcomes after ASO,and the important overlap of these entities in the care of patients going forward. 展开更多
关键词 TRANSPOSITION congenital heart disease sleep apnea POLYSOMNOGRAPHY
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DNA Methylation Variation Is Identified in Monozygotic Twins Discordant for Congenital Heart Diseases
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作者 Shuliang Xia Huikang Tao +10 位作者 Shixin Su Xinxin Chen Li Ma Jianru Li Bei Gao Xumei Liu Lei Pi Jinqing Feng Fengxiang Li Jia Li Zhiwei Zhang congenital heart disease SCIE 2024年第2期247-256,共10页
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
关键词 Congenital heart disease monozygotic twins methylation modification EPIGENETICS
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Machine Learning-Based Intelligent Auscultation Techniques in Congenital Heart Disease:Application and Development
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作者 Yang Wang Xun Yang +6 位作者 Mingtang Ye Yuhang Zhao Runsen Chen Min Da Zhiqi Wang Xuming Mo Jirong Qi congenital heart disease SCIE 2024年第2期219-231,共13页
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
关键词 Congenital heart disease heart sound auscultation artificial intelligence machine learning
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Use of Patient-Specific “4D” Tele-Education to Enhance Actual and Perceived Knowledge in Congenital Heart Disease (CHD) Patients
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作者 Molly Clarke Karin Hamann +2 位作者 Nancy Klein Laura Olivieri Yue-Hin Loke congenital heart disease SCIE 2024年第1期5-17,共13页
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
关键词 Congenital heart disease tele-education transition
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Comparison of QT Correction Methods in the Pediatric Population of a Community Hospital: A Retrospective Study
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作者 Koren Hyogene Kwag Ibrahim Kak +5 位作者 Ying Li Walid Khass Alec McKechnie Oksana Nulman Brande Brown Manoj Chhabra congenital heart disease SCIE 2024年第1期107-121,共15页
Objective:Accurate measurement of QT interval,the ventricular action potential from depolarization to repolarization,is important for the early detection of Long QT syndrome.The most effective QT correction(QTc)formul... Objective:Accurate measurement of QT interval,the ventricular action potential from depolarization to repolarization,is important for the early detection of Long QT syndrome.The most effective QT correction(QTc)formula has yet to be determined in the pediatric population,although it has intrinsically greater extremes in heart rate(HR)and is more susceptible to errors in measurement.The authors of this study compare six dif-ferent QTc methods(Bazett,Fridericia,Framingham,Hodges,Rautaharju,and a computer algorithm utilizing the Bazett formula)for consistency against variations in HR and RR interval.Methods:Descriptive Retrospective Study.We included participants from a pediatric cardiology practice of a community hospital who had an ECG performed in 2017.All participants were healthy patients with no past medical history and no regular med-ications.Results:ECGs from 95 participants from one month to 21 years of age(mean 9.7 years)were included with a mean HR of 91 beats per minute(bpm).The two-sample paired t-test or Wilcoxon signed-rank test assessed for any difference between QTc methods.A statistically significant difference was observed between every combination of two QTc formulae.The Spearman’s rank correlation analysis explored the QTc/HR and QTc/RR relationships for each formula.Fridericia method was most independent of HR and RR with the lowest absolute value of correlation coefficients.Bazett and Computer had moderate correlations,while Framingham and Rautaharju exhibited strong correlations.Correlations were positive for Bazett and Computer,reflecting results from prior studies demonstrating an over-correction of Bazett at higher HRs.In the linear QTc/HR regression analysis,Bazett had the slope closest to zero,although Computer,Hodges,and Fridericia had comparable values.Alternatively,Fridericia had the linear QTc/RR regression coefficient closest to zero.The Bland-Altman method assessed for bias and the limits of agreement between correction formulae.Bazett and Computer exhibited good agreement with minimal bias along with Framingham and Rautaharju.To account for a possible skewed distri-bution of QT,all the above analyses were also performed excluding the top and bottom 2%of data as sorted by heart rate ranges(N=90).Results from this data set were consistent with those derived from all participants(N=95).Conclusions:Overall,the Fridericia correction method provided the best rate correction in our pedia-tric study cohort. 展开更多
关键词 Corrected QT interval QT prolongation long QT syndrome ELECTROCARDIOGRAM retrospective study bazett fridericia FRAMINGHAM hodges rautaharju computer algorithm
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Association of Congenital Heart Defects (CHD) with Factors Related to Maternal Health and Pregnancy in Newborns in Puerto Rico
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作者 Yamixa Delgado Caliani Gaytan +3 位作者 Naydi Perez Eric Miranda Bryan Colón Morales Mónica Santos congenital heart disease SCIE 2024年第1期19-31,共13页
Background:Given the pervasive issues of obesity and diabetes both in Puerto Rico and the broader United States,there is a compelling need to investigate the intricate interplay among body mass index(BMI),pregesta-tio... Background:Given the pervasive issues of obesity and diabetes both in Puerto Rico and the broader United States,there is a compelling need to investigate the intricate interplay among body mass index(BMI),pregesta-tional,and gestational maternal diabetes,and their potential impact on the occurrence of congenital heart defects(CHD)during neonatal development.Methods:Using the comprehensive System of Vigilance and Surveillance of Congenital Defects in Puerto Rico,we conducted a focused analysis on neonates diagnosed with CHD between 2016 and 2020.Our assessment encompassed a range of variables,including maternal age,gestational age,BMI,pregestational diabetes,gestational diabetes,hypertension,history of abortion,and presence of preeclampsia.Results:A cohort of 673 patients was included in our study.The average maternal age was 26 years,within a range of 22 to 32 years.The mean gestational age measured 39 weeks,with a median span of 38 to 39 weeks.Of the 673 patients,274(41%)mothers gave birth to neonates diagnosed with CHD.Within this group,22 cases were linked to pre-gestational diabetes,while 202 were not;20 instances were associated with gestational diabetes,compared to 200 without;and 148 cases exhibited an overweight or obese BMI,whereas 126 displayed a normal BMI.Conclusion:We identified a statistically significant correlation between pre-gestational diabetes mellitus and the occurrence of CHD.However,our analysis did not show a statistically significant association between maternal BMI and the likelihood of CHD.These results may aid in developing effective strategies to prevent and manage CHD in neonates. 展开更多
关键词 Congenital heart defects(CHD) obesisty maternal health DIABETES body mass index(BMI)
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Single-Nucleus RNA Sequencing Reveals Cardiac Macrophage Landscape in Hypoplastic Left Heart Syndrome
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作者 Xiaozhuo Xu Yilin Huang Xu Han congenital heart disease SCIE 2024年第2期233-246,共14页
Background:Hypoplastic left heart syndrome(HLHS)is one of the most challenging congenital heart diseases in clinical treatment.In cardiac tissues,resident macrophages fulfill critical functions in maintaining a stable... Background:Hypoplastic left heart syndrome(HLHS)is one of the most challenging congenital heart diseases in clinical treatment.In cardiac tissues,resident macrophages fulfill critical functions in maintaining a stable cardiac state and have strong regenerative capacity and organ specificity.However,the molecular mechanisms of macro-phages in HLHS remained unclear.Methods:Single-nucleus RNA sequencing(snRNA-seq)data of HLHS and healthy control(donors)samples obtained from the Gene Expression Omnibus(GEO)database were normalized and clustered using the Seurat package.The“FindMarkers”function was used to screen differentially expressed genes(DEGs)between the HLHS and donor groups and to analyze the functional enrichment of the set of genes of interest.Finally,cell-cell communication,pseudotime,and single-cell regulatory network inference and cluster-ing(SCENIC)analyses were used to study the mechanisms of macrophages in HLHS.Results:Based on the snRNA-seq data of HLHS and donors,we identified a total of 9 cell clusters,among which the proportion of macrophages was significantly less in the HLHS group than in the control group.Subdivision of macrophage subpopulations(Macrophages 1,2,and 3)showed that Macrophages 1 was mainly involved in nervous system development,angiogenesis,and apoptotic processes.In addition,analysis of communication between Macro-phages 1 and cardiomyocytes revealed that ligand-acceptor pairs such as GAS6/AXL,IL6,IGF1,THY1,and L1CAM were present only in the donor group.Finally,pesudotime and SCENIC analyses demonstrated that FOXO3 and ELF2 played a critical role for Macrophages 1 to maintain cardiac function in patients with HLHS.Conclusion:Our study improved the current understanding of the molecular mechanisms of macrophage devel-opment in HLHS,showing that manipulating the regulatory role of macrophages in the heart can be a novel treat-ment for HLHS. 展开更多
关键词 Hypoplastic left heart syndrome single-nucleus RNA sequencing MACROPHAGE cellular subpopulation transcription factor
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Treatment and Clinical Management of Chronic Thromboembolic Pulmonary Hypertension:An Update of Literature Review
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作者 Yuan Ren Yingxian Sun +1 位作者 Zhiguang Yang Yanli Chen congenital heart disease SCIE 2024年第2期157-176,共20页
Chronic thromboembolic pulmonary hypertension(CTEPH)is a chronic,progressive,debilitating,and life-threa-tening complication of pulmonary embolism(PE).Recent technological advances have permitted various treat-ment op... Chronic thromboembolic pulmonary hypertension(CTEPH)is a chronic,progressive,debilitating,and life-threa-tening complication of pulmonary embolism(PE).Recent technological advances have permitted various treat-ment options for the treatment of CTEPH,including surgery,angioplasty,and medical treatment,depending on the location and characteristics of lesions.Pulmonary endarterectomy(PEA)is the treatment of choice for CTEPH,as it offers excellent long-term outcomes and a high probability of recovery.Moreover,various medical and interventional therapies are currently being developed for patients with inoperable CTEPH.This review mainly summarizes the current treatment approaches of CTEPH,offering more options for specialist physicians to,thus,better manage chronic thromboembolic syndromes. 展开更多
关键词 Chronic thromboembolic pulmonary hypertension(CTEPH) chronic thromboembolic pulmonary disease(CTEPD) pulmonary endarterectomy(PEA) balloon pulmonary angioplasty(BPA) COVID-19
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Failure to Rescue as a Quality Metric in Congenital Heart Surgeries in a High-Complexity Service Provider Institution Located in a Middle-Income Country
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作者 Gustavo Cruz Santiago Pedroza +3 位作者 Juan F.Vélez Jessica Largo Juan F.Tejada Jorge H.Mejía-Mantilla congenital heart disease SCIE 2024年第2期207-218,共12页
Background:Failure to rescue has been an effective quality metric in congenital heart surgery.Conversely,mor-bidity and mortality depend greatly on non-modifiable individual factors and have a weak correlation with be... Background:Failure to rescue has been an effective quality metric in congenital heart surgery.Conversely,mor-bidity and mortality depend greatly on non-modifiable individual factors and have a weak correlation with better-quality performance.We aim to measure the complications,mortality,and risk factors in pediatric patients undergoing congenital heart surgery in a high-complexity institution located in a middle-income country and compare it with other institutions that have conducted a similar study.Methods:A retrospective observational study was conducted in a high-complexity service provider institution,in Cali,Colombia.All pediatric patients undergoing any congenital heart surgery between 2019 and 2022 were included.The main outcomes evaluated in the study were complication,mortality,and failure to rescue rate.Univariate and multivariate logistic regression analysis was performed with mortality as the outcome variable.Results:We evaluated 308 congenital heart sur-geries.Regarding the outcomes,201(65%)complications occurred,23(7.5%)patients died,and the FTR of the entire cohort was 11.4%.The presence of a postoperative complication(OR 14.88,CI 3.06–268.37,p=0.009),age(OR 0.79,CI 0.57–0.96,p=0.068),and urgent/emergent surgery(OR 8.14,CI 2.97–28.66,p<0.001)were the most significant variables in predicting mortality.Conclusions:Failure to rescue is an effective and comparable quality measure in healthcare institutions and is the major contributor to postoperative mortality in congenital heart surgeries.Despite our higher mortality and complication rate,we obtained a comparable failure to rescue rate to high-income countries’health institutions. 展开更多
关键词 Congenital heart surgery COMPLICATION mortality failure to rescue quality metric
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White Matter Lesions in Young-Middle Aged Migraineurs with Patent Foreman Ovale: A Case-Control Study
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作者 Yang Hua Jinyu Sun +3 位作者 Yuxuan Lou Hao Zhang Jing Shi Wei Sun congenital heart disease SCIE 2024年第3期279-291,共13页
Background:White matter lesion(WML)is common in aging brain and is associated with cognitive impairment and dementia.However,recent studies reported an association between patent foramen ovale(PFO)and WML in migraineu... Background:White matter lesion(WML)is common in aging brain and is associated with cognitive impairment and dementia.However,recent studies reported an association between patent foramen ovale(PFO)and WML in migraineurs,especially in young,middle-aged migraineurs.Our retrospective,case-control study aims to describe the clinical characteristics of WML in this population and to explore potential risk factors.Methods:226 patients with migraine and PFO were consecutively initially screened.Relevant factors were selected by the least absolute shrinkage and selection operator(LASSO)regression and multivariable logistic regression model.A Nomogram was employed to visualize the prediction model conveniently.The discrimination and calibration abilities were evaluated using the Receiver Operating Characteristic(ROC)curve,the Hosmer-Lemeshow test,and calibration curves.Results:One hundred and nineteen participants were ultimately enrolled in our study,with a median age of 36.9±12.7 years and 80.7%of females.Brain magnetic resonance imaging MRI showed 67(56.3%)patients had WML,whereas 52(43.7%)patients were categorized into the non-WML group.LASSO regression screened out potential variables and subsequent multivariate analysisfinally identified age,mean platelet volume,andfibri-nogen(FIB)as independent predictive factors of WML.The area under the ROC curve(AUC)was 0.807.Hos-mer-Lemeshow test and calibration curve verified a consistency between the predicted and actual probability.Conclusion:The predictive nomogram established and validated in our study may assist clinicians in screening WML among young middle-aged migraineurs with PFO and developing individualized preventive and treatment strategies. 展开更多
关键词 White matter lesion MIGRAINE patent foreman ovale NOMOGRAM predictive model
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Prenatal Diagnosis of an Apically Located Congenital Left Ventricular Aneurysm: A Rare Case
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作者 Yücel Kaya And Yavuz +3 位作者 Hasan Berkan Sayal Büşra Tsakir Gökalp Kabacaoğlu Kadriye NilayÖzcan congenital heart disease SCIE 2024年第1期123-129,共7页
Congenital ventricular aneurysm is a very rare cardiac anomaly.A diagnosis can be made during the prenatal period using fetal echocardiography.This study presents a very rare apically located left ventricular aneurysm... Congenital ventricular aneurysm is a very rare cardiac anomaly.A diagnosis can be made during the prenatal period using fetal echocardiography.This study presents a very rare apically located left ventricular aneurysm case,and the relevant literature was reviewed and discussed.In this case,a 35-year-old,gravida 2,parity 1 preg-nant woman at 24 weeks of gestation,displayed a wide aneurysmal image in the left ventricular apical wall on fetal echocardiography.There was a 1.79 mm muscular ventricular septal defect at the apical region of the interven-tricular septum.In the course of the color Doppler ultrasonography examination,an aberrantfibrous band within the left ventricle and consequent turbulentflow during systole were observed.The baby,born via cesarean section at 37 weeks of gestation,is now in its postnatal seventh month.However,during echocardiographic follow-ups,changes have been observed,including mild to moderate mitral insufficiency and a decrease in systolic function.Despite thesefindings,the clinical condition remains asymptomatic.It is of great importance to use a multidis-ciplinary approach in managing these rare cases that could lead to potential adverse outcomes during the antena-tal or postnatal periods. 展开更多
关键词 Heart aneurysm prenatal diagnosis ECHOCARDIOGRAPHY congenital heart defects left ventricle
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Bicuspidization Using the Open-Sleeve Technique for Congenital Aortic Stenosis during Infancy
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作者 Toshi Maeda Hiroki Ito +1 位作者 Keiichi Hirose Kisaburo Sakamoto congenital heart disease SCIE 2024年第2期177-183,共7页
Congenital aortic stenosis(cAS)frequently requires intervention during the neonatal or infantile period.However,surgical repair is challenging because of the narrow surgical space.We performed bicuspidization using th... Congenital aortic stenosis(cAS)frequently requires intervention during the neonatal or infantile period.However,surgical repair is challenging because of the narrow surgical space.We performed bicuspidization using the open-sleeve technique for cAS with a unicuspid aortic valve in two patients.Postoperatively,the patients were doing well without reintervention for the aortic valve for 8 and 6 years,respectively.Their aortic annular diameter increased along with somatic growth.Bicuspidization for neonates or infancy can be performed safely using the open-sleeve technique as its midterm results have been satisfactory. 展开更多
关键词 Aortic valve repair infant bicuspidization open-sleeve technique
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