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  • Open Access

    ARTICLE

    CHDTEPDB: Transcriptome Expression Profile Database and Interactive Analysis Platform for Congenital Heart Disease

    Ziguang Song1,2, Jiangbo Yu1, Mengmeng Wang3, Weitao Shen4, Chengcheng Wang1, Tianyi Lu1, Gaojun Shan1, Guo Dong1, Yiru Wang1, Jiyi Zhao1,*

    Congenital Heart Disease, Vol.18, No.6, pp. 693-701, 2023, DOI:10.32604/chd.2024.048081

    Abstract CHDTEPDB (URL: ) is a manually integrated database for congenital heart disease (CHD) that stores the expression profiling data of CHD derived from published papers, aiming to provide rich resources for investigating a deeper correlation between human CHD and aberrant transcriptome expression. The development of human diseases involves important regulatory roles of RNAs, and expression profiling data can reflect the underlying etiology of inherited diseases. Hence, collecting and compiling expression profiling data is of critical significance for a comprehensive understanding of the mechanisms and functions that underpin genetic diseases. CHDTEPDB stores the expression profiles of over 200 sets of 7… More >

  • Open Access

    ARTICLE

    Single-Cell RNA Sequencing Reveals Potential for Endothelial-to-Mesenchymal Transition in Tetralogy of Fallot

    Aisa Zulibiya1,2,#, Jing Wen3,#, Huiqing Yu3,#, Xiaoming Chen3, Lei Xu3, Xiao Ma1,2, Baojian Zhang1,2,*

    Congenital Heart Disease, Vol.18, No.6, pp. 611-625, 2023, DOI:10.32604/chd.2023.047689

    Abstract Background: Tetralogy of Fallot (TOF) is a very common cyanotic congenital heart disease. Endothelial-to-mesenchymal transition (EndoMT) is recognized as a physiological mechanism involved in embryonic heart development and endothelial formation. However, there is still a gap in the reports related to the mechanism of EndoMT development in TOF. Methods: 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 clustered by dimensionality reduction using the Seurat package. Subsequently, differentially expressed genes (DEGs) between TOF and Donor were screened using the “FindMarkers” function, and… More >

  • Open Access

    CASE REPORT

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

    Dian Kesumarini1,2, Yunita Widyastuti3, Cindy Elfira Boom1, Lucia Kris Dinarti4,*

    Congenital Heart Disease, Vol.18, No.6, pp. 671-678, 2023, DOI:10.32604/chd.2023.044746

    Abstract Tetralogy of Fallot (TOF) with total anomalous pulmonary vein connections (TAPVC) is a rare type of complex congenital heart disease among all TOF cases. Co-presentation of major aortopulmonary collateral arteries (MAPCAs) compensates for the lack of central pulmonary blood flow and decreases the severity of right-to-left shunting in TOF. We present a case of a 2-year-old child with complex diagnoses of TOF, TAPVC, a large secundum atrial septal defect (ASD), and intraoperatively identified MAPCAs. She 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).… More >

  • Open Access

    ARTICLE

    Risk Factors for Abuse in Children with Congenital Heart Disease Presenting at a Pediatric Tertiary Care Hospital

    Kristi K. Westphaln1,2,*, Karen Kay Imagawa2,3, Lorena Espinosa Smith1,4, Julia Srivastava5, Nancy A. Pike1,5

    Congenital Heart Disease, Vol.18, No.6, pp. 657-670, 2023, DOI:10.32604/chd.2023.044179

    Abstract Background: Congenital heart disease (CHD) is a chronic medical condition often diagnosed at birth and requires surgical intervention, multiple hospitalizations, and lifelong care. This can put significant stress on the family, leading to altered maternal mental health, bonding and attachment issues, and the potential for child abuse. The purpose of this study is to explore the characteristics of a sample of young children with CHD who experienced hospitalization with concurrent concern for child abuse in a free-standing pediatric tertiary care hospital. Methods: Electronic medical records were reviewed for children aged 0–5 years old who were hospitalized with concern for child… More >

  • Open Access

    ARTICLE

    Dynamic Changes in Left and Right Cerebral Oxygen Saturation during Selective Cerebral Perfusion in Young Infants

    Hwa-Young Jang1, Sang-Jun Beon2, Sung-Hoon Kim1, In-Kyung Song1, Won-Jung Shin1,*

    Congenital Heart Disease, Vol.18, No.6, pp. 639-647, 2023, DOI:10.32604/chd.2023.030065

    Abstract Objectives: We investigated whether the selective cerebral perfusion (SCP) technique causes differences in changes in cerebral perfusion between both hemispheres in young infants, using cerebral oxygen saturation (ScO2) as an index. Further, we determined the association between the discrepancy in ScO2 and cerebral perfusion pressure during SCP. Methods: The difference in ScO2 between the left and right cerebral hemispheres (ΔScO2 Rt-Lt) was calculated during clamping of the innominate artery (IA) and during SCP. Results: In 25 infants (aged 2 to 78 days), the left and right ScO2 were well maintained (median 63.2% and 60.9% during IA clamping, respectively; 64.0% and… More > Graphic Abstract

    Dynamic Changes in Left and Right Cerebral Oxygen Saturation during Selective Cerebral Perfusion in Young Infants

  • Open Access

    CASE REPORT

    Implementation of a High-Risk Outpatient Clinic for Children with Complex Congenital Heart Disease in a Reference Service in Brazil

    Gustavo Foronda1,2, Vanessa Ferreira Amorim de Melo2,3,*, Claudia Regina Pinheiro de Castro Grau4, Ingrid Magatti Piva1, Glaucia Maria Penha Tavares4, Ana Cristina Sayuri Tanaka1, Nana Miura1

    Congenital Heart Disease, Vol.18, No.6, pp. 649-656, 2023, DOI:10.32604/chd.2023.027987

    Abstract Background: Children with congenital heart disease (CHD), even after surgical approaches, and especially those who undergo staged procedures in the first months of life, remain vulnerable to readmissions and complications, requiring very close monitoring and differentiated intervention strategies. Methods: Descriptive and exploratory study, of the experience report type, which presents the process of building the high-risk outpatient clinic for complex congenital heart diseases (AAR) at the Instituto do Coração (InCor). Results: Report of the path taken to structure the AAR, demonstrating the organization, interface with the multidisciplinary team, admission and discharge criteria, training, and patient profile. In these five years… More >

  • Open Access

    ARTICLE

    Real-Time Remote-Mentored Echocardiography in Management of Newborns with Critical Congenital Heart Defects

    Håvard Bjerkeseth Solvin1,2,5,*, Simone Goa Diab1,4, Ole Jakob Elle2,3, Henrik Holmstrøm1,4, Henrik Brun2,4,*

    Congenital Heart Disease, Vol.18, No.5, pp. 551-559, 2023, DOI:10.32604/chd.2023.031537

    Abstract Background: The management of suspected critical congenital heart defects (CCHD) relies on timely echocardiographic diagnosis. The availability of experienced echocardiographers is limited or even non-existent in many hospitals with obstetric units. This study evaluates remote-mentored echocardiography performed by physicians without experience in imaging of congenital heart defects (CHD). Methods: The setup included a pediatric cardiologist in a separate room, guiding a physician without experience in echocardiographic imaging of CHD in the examination of a symptomatic newborn. This remote-mentoring pair was blinded to the diagnosis of the newborn and presented with a simplified patient history. The echocardiographic images were streamed to… More > Graphic Abstract

    Real-Time Remote-Mentored Echocardiography in Management of Newborns with Critical Congenital Heart Defects

  • Open Access

    ARTICLE

    Analysis of Risk Factors for Early Mortality in Surgical Shunt Palliation: Time for a Change?

    François-Xavier Van Vyve1, Karlien Carbonez2, Jelena Hubrechts2, Geoffroy de Beco1, Jean E. Rubay1, Mona Momeni3, Thierry Detaille4, Alain J. Poncelet1,*

    Congenital Heart Disease, Vol.18, No.5, pp. 539-550, 2023, DOI:10.32604/chd.2023.042344

    Abstract Objectives: Over the last decade, neonatal repair has been advocated for many congenital heart diseases. However, specific subgroups of complex congenital heart disease still require temporary palliation for which both surgical and endovascular techniques are currently available. We reviewed our institutional experience with shunt palliation with an emphasis on risk factors for early mortality. Methods: This is a single-center retrospective study on 175 patients undergoing surgery for central shunt or modified Blalock-Taussig shunt. All data were extracted from a prospectively collected computerized database. We identified risk factors for early mortality by uni- and multi-variable analysis. All data were censored at… More >

  • Open Access

    ARTICLE

    Analysis of Pulmonary Arteries Growth after Initial Shunt Palliation in Neonates and Infants

    François-Xavier Van Vyve1,#, Karlien Carbonez2,#, Geoffroy de Beco1, Stéphane Moniotte2, Jean E. Rubay1, Mona Momeni3, Laurent Houtekie4, Alain J. Poncelet1,*

    Congenital Heart Disease, Vol.18, No.5, pp. 525-537, 2023, DOI:10.32604/chd.2023.042341

    Abstract Objective: Despite increasing enthusiasm for neonatal repair, patients with ductal-dependent circulation (pulmonary/systemic) or restrictive pulmonary blood flow still require initial palliation. Ductal stenting has emerged as an endovascular approach whereas modified-Blalock-Taussig and central shunt remain surgical references. In this study, we analyzed the relationship between pulmonary artery growth, sites of shunt connection, or antegrade pulmonary blood flow in surgically placed shunts. The need for secondary catheter-based interventions or pulmonary arterioplasty was also investigated. Methods: A retrospective single-center study analyzing 175 patients undergoing surgery for a central or modified-Blalock-Taussig shunt. Outcome growth variables were right pulmonary artery/left pulmonary artery diameters/Z scores,… More > Graphic Abstract

    Analysis of Pulmonary Arteries Growth after Initial Shunt Palliation in Neonates and Infants

  • Open Access

    ARTICLE

    A New Three-Dimensional (3D) Printing Prepress Algorithm for Simulation of Planned Surgery for Congenital Heart Disease

    Vitaliy Suvorov1,2,*, Olga Loboda2, Maria Balakina1, Igor Kulczycki2

    Congenital Heart Disease, Vol.18, No.5, pp. 491-505, 2023, DOI:10.32604/chd.2023.030583

    Abstract 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… More > Graphic Abstract

    A New Three-Dimensional (3D) Printing Prepress Algorithm for Simulation of Planned Surgery for Congenital Heart Disease

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