Aisa Zulibiya^1,2,#, Jing Wen^3,#, Huiqing Yu^3,#, Xiaoming Chen³, Lei Xu³, Xiao Ma^1,2, Baojian Zhang^1,2,*

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 >

Pier Paolo Bassareo^1,2,3,*, Aurelio Secinaro⁴, Paolo Ciliberti⁵, Massimo Chessa^6,7, Marco Alfonso Perrone^5,8, Kevin Patrick Walsh^1,2,3, Colin Joseph Mcmahon^2,3

Congenital Heart Disease, Vol.18, No.6, pp. 595-610, 2023, DOI:10.32604/chd.2023.046229

Abstract Background: Congenital absence of pericardium (CAP), also known as pericardial agenesis, represents an uncommon cardiac abnormality and mostly incidental finding. It can be subdivided into complete and partial (left or right-sided) forms. Because of its infrequency, just case reports and a few case series have been released so far. This paper represents the largest systematic review in the field. Nine features (age at diagnosis, type, gender, clinical presentation, electrocardiography, imaging (ultrasounds, CT/MRI), concomitant cardiac defects, and outcome) were analysed. Methods: The electronic database PubMed was investigated from its establishment up to July 15^th, 2023. Just case reports and case series… More >

Dian Kesumarini^1,2, Yunita Widyastuti³, Cindy Elfira Boom¹, Lucia Kris Dinarti^4,*

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 >

Kristi K. Westphaln^1,2,*, Karen Kay Imagawa^2,3, Lorena Espinosa Smith^1,4, Julia Srivastava⁵, Nancy A. Pike^1,5

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 >

Hwa-Young Jang¹, Sang-Jun Beon², Sung-Hoon Kim¹, In-Kyung Song¹, Won-Jung Shin^1,*

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 (ΔScO_{2 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

Gustavo Foronda^1,2, Vanessa Ferreira Amorim de Melo^2,3,*, 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 >

Charis Tan^1,2 , Diana Zannino³, Carley Clendenning³, Sophie Offen⁴, Thomas L. Gentles⁵, Julian Ayer⁶, David Tanous⁷, Vishva Wijesekera⁸, Leeanne Grigg⁹, David Celermajer^2,4,10, Mark McGuire^2,4 , Yves d’Udekem^3,11,12, Rachael Cordina^2,4,10,*

Congenital Heart Disease, Vol.18, No.5, pp. 507-523, 2023, DOI:10.32604/chd.2023.028829

Abstract Objective: Sudden cardiac death (SCD) and malignant ventricular arrhythmia (VA) are increasingly recognized as important issues for people living with a Fontan circulation, but data are lacking. We sought to characterize the cohort who had sudden cardiac death, most likely related to VA and/or documented VA in the Australia and New Zealand Fontan Registry including risk factors and clinical outcomes. Methods: A retrospective cohort study was performed. Inclusion criteria were documented non-sustained ventricular tachycardia, sustained ventricular tachycardia, ventricular fibrillation, resuscitated cardiac arrest or SCD > 30 days post-Fontan completion. Results: Of 1611 patients, 20 (1.2%) had VA; 14 (1.0%) had… More >

Håvard Bjerkeseth Solvin^1,2,5,*, Simone Goa Diab^1,4, Ole Jakob Elle^2,3, Henrik Holmstrøm^1,4, Henrik Brun^2,4,*

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

François-Xavier Van Vyve¹, Karlien Carbonez², Jelena Hubrechts², Geoffroy de Beco¹, Jean E. Rubay¹, Mona Momeni³, Thierry Detaille⁴, Alain J. Poncelet^1,*

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 >

François-Xavier Van Vyve^1,#, Karlien Carbonez^2,#, Geoffroy de Beco¹, Stéphane Moniotte², Jean E. Rubay¹, Mona Momeni³, Laurent Houtekie⁴, Alain J. Poncelet^1,*

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