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Perioperative extracorporeal membrane oxygenation in pediatric congenital heart disease:Chinese expert consensus

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World Journal of Pediatrics 2023年第1期19卷 7-19页

作者： Ru Lin Wei Wang Xu Wang Zhuo-Ming Xu Jin-Ping Liu Cheng-Bin Zhou Xiao-Yang Hong Xu-Ming Mo Shan-Shan Shi Li-Fen Ye Qiang Shu Department of Extracorporeal Life Support Heart InstituteNational Clinical Research Center for Child HealthChildren's HospitalZhejiang University School of MedicineHangzhouChina Department of Thoracic and Cardiovascular Surgery Shanghai Children's Medical CenterShanghai Jiao Tong University School of MedicineShanghaiChina Department of Pediatric Intensive Care Unit National Center for Cardiovascular Disease and Fuwai HospitalChinese Academy of Medical SciencesPeking Union Medical CollegeBeijingChina Department of Cardiopulmonary Bypass National Center for Cardiovascular Disease and Fuwai HospitalChinese Academy of Medical SciencesPeking Union Medical CollegeBeijingChina Department of Cardiovascular Surgery Guangdong Provincial Cardiovascular InstituteGuangdong Provincial People's HospitalGuangdong Academy of Medical SciencesGuangzhouChina Pediatric Intensive Care Unit Faculty of PediatricsChinese PLA General HospitalBeijingChina Department of Cardiothoracic Children's Hospital of Nanjing Medical UniversityNanjingChina Department of CICU Heart InstituteNational Clinical Research Center for Child HealthChildren's HospitalZhejiang University School of MedicineHangzhouChina Department of Cardiac Surgery National Clinical Research Center for Child HealthChildren's HospitalZhejiang University School of MedicineBinsheng Road 3333Hangzhou 310052China

Background congenital heart disease(CHD)is one of the main supportive diseases of extracorporeal membrane oxygena-tion in children.The management of extracorporeal membrane oxygenation(ECMO)for pediatric CHD faces more severe challenges due to the complex anatomical structure of the heart,special pathophysiology,perioperative complications and various concomitant malformations.The survival rate of ECMO for CHD was significantly lower than other classifica-tions of diseases according to the Extracorporeal Life Support Organization database.This expert consensus aims to improve the survival rate and reduce the morbidity of this patient population by standardizing the clinical strategy.Methods The editing group of this consensus gathered 11 well-known experts in pediatric cardiac surgery and ECMO field in China to develop clinical recommendations formulated on the basis of existing evidences and expert opinions.Results The primary concern of ECMO management in the perioperative period of CHD are patient selection,cannulation strategy,pump flow/ventilator parameters/vasoactive drug dosage setting,anticoagulation management,residual lesion screening,fluid and wound management and weaning or transition strategy.Prevention and treatment of complications of bleeding,thromboembolism and brain injury are emphatically discussed here.Special conditions of ECMO management related to the cardiovascular anatomy,haemodynamics and the surgical procedures of common complex CHD should be considered.Conclusions The consensus could provide a reference for patient selection,management and risk identification of periop-erative ECMO in children with CHD.

关键词： Circulatory support congenital heart disease Extracorporeal membrane oxygenation Pediatric Respiratory support

CHDbase:A Comprehensive Knowledgebase for congenital heart disease-related Genes and Clinical Manifestations

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Genomics, Proteomics & Bioinformatics 2023年第1期21卷 216-227页

作者： Wei-Zhen Zhou Wenke Li Huayan Shen Ruby W.Wang Wen Chen Yujing Zhang Qingyi Zeng Hao Wang Meng Yuan Ziyi Zeng Jinhui Cui Chuan-Yun Li Fred Y.Ye Zhou Zhou State Key Laboratory of Cardiovascular disease Beijing Key Laboratory for Molecular Diagnostics of Cardiovascular DiseasesCenter of Laboratory MedicineFuwai HospitalNational Center for Cardiovascular DiseasesChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100037China International Joint Informatics Laboratory&Jiangsu Key Laboratory of Data Engineering and Knowledge Service School of Information ManagementNanjing UniversityNanjing 210023China Institute of Molecular Medicine Peking UniversityBeijing 100871China

congenital heart disease(CHD)is one of the most common causes of major birth defects,with a prevalence of 1%.Although an increasing number of studies have reported the etiology of CHD,the findings scattered throughout the literature are difficult to retrieve and utilize in research and clinical practice.We therefore developed CHDbase,an evidence-based knowledgebase of CHD-related genes and clinical manifestations manually curated from 1114 publications,linking 1124 susceptibility genes and 3591 variations to more than 300 CHD types and related syndromes.Metadata such as the information of each publication and the selected population and samples,the strategy of studies,and the major findings of studies were integrated with each item of the research record.We also integrated functional annotations through parsing50 databases/tools to facilitate the interpretation of these genes and variations in disease pathogenicity.We further prioritized the significance of these CHD-related genes with a gene interaction network approach and extracted a core CHD sub-network with 163 genes.The clear genetic landscape of CHD enables the phenotype classification based on the shared genetic origin.Overall,CHDbase provides a comprehensive and freely available resource to study CHD susceptibilities,supporting a wide range of users in the scientific and medical communities.CHDbase is accessible at http://***.

关键词： congenital heart disease congenital heart defect Database Genetics Classification

贝叶斯推理和动态神经反馈促进先天性心脏病智能诊断的临床应用

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Engineering 2023年第4期23卷 90-102,M0005页

作者：谭伟敏曹银银马晓静茹港徽李吉春张璟高燕杨佳伦黄国英颜波李健 Shanghai Key Laboratory of Intelligent Information Processing School of Computer ScienceFudan UniversityShanghai 200433China Cardiovascular Center&Clinical Laboratory Center Children’s Hospital of Fudan UniversityNational Children’s Medical CenterShanghai 201102China

先天性心脏病(CHD)是婴幼儿死亡主要原因。基于人工智能的先天性心脏病诊断网络(CHDNet)是一种基于超声心动图视频的二分类模型,用于判别超声心动图视频是否包含心脏缺陷。现有的CHDNet模型表现出与医学专家相当甚至更好的判别性能,但... 详细信息

先天性心脏病(CHD)是婴幼儿死亡主要原因。基于人工智能的先天性心脏病诊断网络(CHDNet)是一种基于超声心动图视频的二分类模型,用于判别超声心动图视频是否包含心脏缺陷。现有的CHDNet模型表现出与医学专家相当甚至更好的判别性能,但它们在训练集之外的样本上的不可靠性已成为模型部署的关键瓶颈。而这是当前大多数基于AI诊断方法的共性问题。为了克服这一挑战,本文我们提出了两种基本机制——贝叶斯推理和动态神经反馈——分别用于衡量和提高人工智能诊断的可靠性。贝叶斯推理允许神经网络模型输出CHD判别的可靠性而不仅仅是单一的判别结果,而动态神经反馈是一个计算神经反馈单元,允许神经网络将知识从输出层反馈给浅层,使神经网络有选择地激活相关神经元。为了评估这两种机制的有效性,我们在包含三种常见CHD缺陷的4151个超声心动图视频上训练了CHDNet,并在1037个超声心动图视频的内部测试集和从其他心血管成像设备新收集的692个外部视频集上对其进行了测试。每个超声心动图视频对应于一位患者和一次就诊。我们在多种代表性神经网络架构上展示了贝叶斯推理获得的可靠性如何解释和量化神经网络内部与外部测试集之间的性能显著差异,以及尽管输入被噪声破坏或使用外部测试集时,设计的反馈单元如何帮助神经网络保持高精度和可靠性。

关键词： congenital heart disease Artificial intelligence Deep learning Model uncertainty

Machine Learning-Based Intelligent Auscultation Techniques in congenitalheart disease: Application and Development

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congenital heart disease 2024年第2期19卷 219-231页

作者： Yang Wang Xun Yang Mingtang Ye Yuhang Zhao Runsen Chen Min Da Zhiqi Wang Xuming Mo Jirong Qi Department of Cardiothoracic Surgery Children’s Hospital of Nanjing Medical UniversityNanjingChina

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.

关键词： congenital heart disease heart sound auscultation artiﬁcial intelligence machine learning

Expert Consensus on Nutritional Support for Children with congenital heart disease(2023 Edition)

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congenital heart disease 2023年第6期18卷 571-593页

作者： Xuming Mo Wei Cai Jirong Qi Zhuoming Xu Ying Wang Weihui Yan Shoujun Li Nianguo Dong Xinxin Chen Jinfen Liu Qiang Shu Jimei Chen Haibo Zhang Hao Zhang Quansheng Xing Qi An Xiaofeng Li Xu Wang Yan He Junwu Su Taibing Fan Teng Ming Weibing Tang Li Hong Jinghao Zheng Ming Ye Guocheng Sun Yiqun Ding Liang Tao Yifeng Yang Zhongshi Wu Hua Cao Qiang Wang Keming Yang Libing Zhang Ping Wen Yanqin Cui Bo Zhai Yong Zou Qingya Tang Rui Chen Chun Wu Zhiyu Feng Caixia Liu Yaping Mi Rufang Zhang Ke Lin Xin Li Mingan Pi Xiangming Fan Shanshan Shi Peng Huang Zhengxia Pan Jiafeng Qi Renwei Chen Shuguang Tao Yaqin Shu Huifeng Zhang Lan Jiang Min Da Nishant Patel Liang Hu Cardiac Surgery Group of Pediatric Surgery Society of Chinese Medical Association and Parenteral Enteral Nutrition Society of Chinese Medical Association Department of Cardiothoracic Surgery Children’s Hospital of Nanjing Medical UniversityNanjing210008China Department of Pediatric Surgery and Nutrition Xinhua HospitalSchool of MedicineShanghai Jiao Tong UniversityShanghai200092China Department of Cardiothoracic Surgery Shanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong UniversityShanghai200127China Division of Pediatric Gastroenterology and Nutrition Xinhua HospitalSchool of MedicineShanghai Jiao Tong UniversityShanghai200092China Pediatric Cardiac Surgery Center Fuwai HospitalNational Centre for Cardiovascular DiseasesState Key Laboratory of Cardiovascular DiseaseChinese Academy of Medical SciencesPeking Union Medical CollegeBeijing100037China Department of Cardiovascular Surgery Union HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan430022China Cardiovascular Center Guangzhou Women and Children’s Medical CenterGuangzhou Medical UniversityGuangzhou510623China Department of Cardiac Surgery Children’s HospitalZhejiang University School of MedicineNational Clinical Research Center for Child HealthHangzhou310052China Department of Cardiovascular Surgery Guangdong Cardiovascular InstituteGuangdong Provincial People’s HospitalGuangdong Academy of Medical SciencesSouthern Medical UniversityGuangzhou519041China heart Center and Shanghai Institue of Pediatric congenital heart disease Shanghai Children’s Medical CenterNational Children’s Medical CenterSchool of MedicineShanghai Jiao Tong UniversityShanghai200127China heart Center Qingdao Women and Children’s HospitalQingdao266034China Department of Cardiovascular Surgery West China HospitalSichuan UniversityChengdu610041China Department of Cardiac Surgery Beijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijing100045China Department of Pediatric Intensive Care Unit Fuwai HospitalChinese Academy of Medical SciencesPeking Union Medical CollegeBeijing100037China Depar

The second edition of the expert consensus on pediatric nutrition was formed based on a global update of pedia-tric nutrition guidelines or consensus worldwide,the management of congenital heart disease,and the results of multi-center clinical nutrition research for congenital heart disease following theﬁrst Chinese consensus edition of 2016.The consensus was also shaped by the results of three discussion sessions and two questionnaires con-ducted by the 13-member collaboration group.This process was informed by both clinical guidelines and expert consensus.The quality of literature,both in English and Chinese,and the level of recommendations were evaluated using the Grading of Recommendations Assessment,Development,and Evaluations(GRADE)system.

关键词： congenital heart disease nutrition enteral parenteral expert consensus

Multi-Institutional US Experience of the Occlutech AFR Device in congenital and Acquired heart disease

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congenital heart disease 2022年第1期17卷 107-116页

作者： Barry O’Callaghan Jenny Zablah Joseph Vettukattil Daniel Levi Morris Salem Allison Cabalka Jason Anderson Makram Ebeid Ryan Alexy Gareth J.Morgan The heart Institute Children’s Hospital ColoradoAuroraUSA Pediatric Cardiology Helen DeVos Children’s HospitalGrand RapidsUSA Pediatric Cardiology UCLA HealthMattel Children’s HospitalLos AngelesUSA Pediatric Cardiology UCLA Medical CentreLos AngelesUSA Pediatric Cardiology Mayo ClinicRochesterUSA Pediatric Cardiology University of Mississippi Medical CenterJacksonUSA Pediatric Cardiology Riley Hospital for Children at Indiana University HealthIndianapolisUSA

Objectives:To detail the US multi-institutional experience with the Occlutech©(Occlutech International AB,Helsingborg,Sweden)atrial flow regulator(AFR)in children and adults with acquired or congenital heart disease.Background:The creation of a long-term atrial communication is desirable in several cardiovascular disease phenotypes,most notably pulmonary arterial hypertension,disorders of increased left ventricular filling and increased cavopulmonary pressures in patients with a Fontan type circulation.Methods:Patients were identified for inclusion from the AFR device manufacturer database.Data was collected using a RedCap database following IRB approval.8 weeks of follow up data was sought for each patient based on available data.Data was analyzed and summarized using SPSS.Results:We report the experience of 6 US centers in the implantation of AFR devices in 15 patients,across a wide age range,with different disease phenotypes and a variety of indications.Implantation was technically successful in all patients and improvement was noted in both clinical and hemodynamic parameters.There were no immediate or intermediate term complications reported.3 patients died remote from implantation.Their deaths were not felt to be related to the AFR device or related procedural complications.Conclusion:Compassionate use of the AFR device in children and adults with congenital&acquired heart disease is technically feasible and produces beneficial short term hemodynamic and symptomatic improvement.Widespread uptake of this technique and treatment at specialist centers has the potential to provide significant benefits to a variety of complex patients with currently limited treatment options and indeterminate prognosis.

关键词： congenital heart disease pulmonary hypertension left atrial hypertension single ventricle palliation diastolic heart failure

Incidence and Related Risk Factors of Junctional Ectopic Tachycardia in Infants after Cardiac Surgery for congenital heart disease

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congenital heart disease 2022年第5期17卷 569-578页

作者： Jae Hee Seol Se Yong Jung Jae Young Choi Han Ki Park Young Hwan Park Nam Kyun Kim Department of Pediatrics Congenital Heart Disease CenterDivision of Pediatric CardiologySeverance Cardiovascular HospitalYonsei University College of MedicineSeoulKorea Department of Pediatrics Yonsei University Wonju College of MedicineWonjuKorea Department of Thoracic and Cardiovascular Surgery Division of Cardiovascular SurgeryCongenital Heart Disease CenterSeverance Cardiovascular HospitalYonsei University College of MedicineSeoulKorea Department of Pediatrics and Biomedical Engineering School of MedicineEmory UniversityAtlantaUSA

Objective:Junctional ectopic tachycardia is common after cardiac surgery for congenital heart disease.However,its incidence and related risk factors in infants after cardiac surgery are not well known.The objective of this study was to determine the overall incidence and related risk factors for junctional ectopic tachycardia in neonates and infants.Methods:We enrolled a total of 271 patients agedcongenital heart disease in infants.Occasionally,infants developing junctional ectopic tachycardia after cardiac surgery have specific preoperative electrocardiography findings.The risk factors for junctional ectopic tachycardia were associated not only with surgical procedural factors but also with preoperative electrocardiographic parameters.

关键词： Junctional ectopic tachycardia congenital heart disease early re-polarization

A New Three-Dimensional(3D)Printing Prepress Algorithm for Simulation of Planned Surgery for congenital heart disease

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congenital heart disease 2023年第5期18卷 491-505页

作者： Vitaliy Suvorov Olga Loboda Maria Balakina Igor Kulczycki Department of Surgical diseases of Children Saint Petersburg State Pediatric Medical UniversitySaint PetersburgRussia Higher School of Theoretical Mechanics Peter the Great Saint Petersburg Polytechnic UniversitySaint PetersburgRussia

Background:Three-dimensional printing technology may become a key factor in transforming clinical practice and in significant improvement of treatment outcomes.The introduction of this technique into pediatric cardiac surgery will allow us to study features of the anatomy and spatial relations of a defect and to simulate the optimal surgical repair on a printed model in every individual case.Methods:We performed the prospective cohort study which included 29 children with congenital heart defects.The hearts and the great vessels were modeled and printed out.Measurements of the same cardiac areas were taken in the same planes and points at multislice computed tomography images(group 1)and on printed 3D models of the hearts(group 2).Pre-printing treatment of the multislice computed tomography data and 3D model preparation were performed according to a newly developed algorithm.Results:The measurements taken on the 3D-printed cardiac models and the tomographic images did not differ significantly,which allowed us to conclude that the models were highly accurate and informative.The new algorithm greatly simplifies and speeds up the preparation of a 3D model for printing,while maintaining high accuracy and level of detail.Conclusions:The 3D-printed models provide an accurate preoperative assessment of the anatomy of a defect in each case.The new algorithm has several important advantages over other available programs.They enable the development of customized preliminary plans for surgical repair of each specific complex congenital heart disease,predict possible issues,determine the optimal surgical tactics,and significantly improve surgical outcomes.

关键词： 3D printing imaging in cardiac surgery congenital heart disease modelling in cardiac surgery pediatric cardiology algorithmic modelling of the heart medical imaging 3D modelling

Quality of Life in congenital heart disease Patients according to Their Anatomical and Physiological Classification

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congenital heart disease 2023年第2期18卷 197-206页

作者： Efrén Martínez-Quintana Hiurma Estupiñán-León Ana Beatriz Rojas-Brito Liuva Déniz-Déniz Alejandro Barreto-Martín Fayna Rodríguez-González Cardiology Service Complejo Hospitalario Universitario Insular-Materno InfantilLas Palmas de Gran Canaria35016Spain Department of Medical and Surgical Sciences Faculty of Health SciencesUniversidad de Las Palmas de Gran CanariaLas Palmas de Gran Canaria35016Spain Ophthalmology Service Hospital Universitario de Gran Canaria Dr.NegrínLas Palmas de Gran Canaria35012Spain

Background:Living well is as important as living longer.The objective of this study is to assess quality of life(QoL)in congenital heart disease(CHD)according to current AHA/ACC anatomical and physiological classification.Methods:Cross-sectional study examining the World Health Organization QoL Bref questionnaire(WHOQoL-Bref)in consecutive outpatient CHD patients from a single unit.Results:191 CHD patients were studied.Median age was 28±13 years and 59%were male.44(23%),115(60%)and 33(17%)CHD patients showed mild,moderate and great anatomical defects respectively while 69(36%)patients were in physiological Stage A,27(14%)in Stage B,84(44%)in Stage C and 11(6%)in Stage D.No significant differences were seen in relation the anatomical classification and the different sections of the WHOQoL-Bref questionnaire.CHD patients in Stages C and D had significant lower physical domain scores than patients in the Stage A(p<0.05).However,no significant differences were seen in the psychological,social relationships and environmental domains.The binary logistic regression analysis showed that having a higher educational level was a protective factor[OR 0.32(95%CI,0.12-0.87),p=0.026]while being married or cohabit was a risk factor[OR 3.46(95%CI,1.13-10.63),p=0.030]for having a worse rated QoL.Meanwhile,having a worse functional class(NYHA≥2)[OR 3.44(95%CI,1.20-9.81),p=0.021]was associated with dissatisfaction with health.Conclusion:Patients with advanced physiological stages scored lower on the physiological domain.No statistical significance was seen,according to the anatomical and physiological classification,in the psychological,social relationship and environmental domains.

关键词： Quality of life congenital heart disease complexity