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 >

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 >

Aya Miyazaki^1,2,*, Hideki Uemura², Yasuyo Takeuchi³, Junya Tomida⁴, Yasuo Ono¹, Yoshifumi Fujimoto¹, Norie Mitsushita¹, Akio Ikai¹

Congenital Heart Disease, Vol.17, No.6, pp. 647-652, 2022, DOI:10.32604/chd.2022.021837

Abstract Appropriate heart rate in a failing pulmonary ventricle remains unknown, particularly in congenital heart disease with unique hemodynamics. A 71-year-old male with repaired tetralogy of Fallot and a pacemaker for a sinus node dysfunction suffered from heart failure symptoms with preserved left ventricular function. Simply changing the pacemaker’s lower rate from 60 to 75 bpm, New York Heart Association classification improved from III to II, and hemodynamic parameters drastically improved. We regarded this case as informative. Appropriate heart rate could be higher in congenital patients with failing right and non-failing left ventricles than in adults with malfunctioning LV. More > Graphic Abstract

Elena Panaioli^1,2,3,#, Duarte Martins^1,4,#, Marc Antoine Isorni⁵, Diala Khraiche¹, Antoine Legendre¹, Nathalie Boddaert⁶, Damien Bonnet¹, Filippo Crea^2,3, Francesca Raimondi^1,6,7,8,*

Congenital Heart Disease, Vol.17, No.2, pp. 117-128, 2022, DOI:10.32604/chd.2022.018779

Abstract Background: Repaired Tetralogy of Fallot (rTOF) patients may have residual lesions such as main (MPA) and branch pulmonary artery stenosis (BPAS). While MPA stenosis is well studied, few data are available on BPAS in rTOF. We aimed to describe pulmonary perfusion in a large paediatric cohort of rTOF and its impact on right ventricular and outflow-tract hemodynamics using 4D flow CMR. Methods: 130 consecutive patients (mean age at CMR 14.3 ± 4.6 years) were retrospectively reviewed. 96 patients had transannular patch without valve preservation while 34 patients had conserved annulus or valved conduit. A pulmonary blood flow ratio (right pulmonary artery (RPA)/left… More > Graphic Abstract

Hong Liu^1,#,*, Luyao Ma^1,#, Jinghang Li^1,#, Bingqi Sun², Siqiang Zheng³, Yongfeng Shao^1,*

Congenital Heart Disease, Vol.16, No.5, pp. 443-455, 2021, DOI:10.32604/CHD.2021.015588

Abstract Background: This study evaluated differential inflammatory response to cardiopulmonary bypass reoxygenation in tetralogy of Fallot repair. Methods: We performed a retrospective study at a cardiovascular center from 2012 to 2018, including 500 patients aged 1 week–18 years who received complete repair of tetralogy of Fallot. Patients were grouped according to tertiles of preoperative RVOT gradient on echocardiography into mild, moderate, and severe stenosis. We measured the highest perfusate oxygenation (PpO2) during aortic occlusion as independent variable. Primary outcome was systemic inflammatory response syndrome (SIRS) within 7 days postoperatively or the time of death or discharge. Results: Overall, rate of SIRS… More >

Siraphop Thapmongkol^1,*, Jarun Sayasathid¹, Jessada Methrujpanont², Kanthachat Thatsakorn¹, Worawan Jittham³, Suwanna Puitm⁴, Methiniwiran Thapmongkol⁵, Jule Namchaisiri⁶

Congenital Heart Disease, Vol.16, No.5, pp. 433-441, 2021, DOI:10.32604/CHD.2021.015770

Abstract Background: The surgical outcomes of tetralogy of Fallot (TOF) have evolved dramatically and have resulted in lower mortality rate. Currently, the many cardiac centers have a trend to early single-stage complete repair more than a staged repair. However, the patients who have an early primary repair were required transannular patch augmentation of a pulmonary valve frequently. This effect has been developed a chronic pulmonary insufficiency may lead to right ventricular dilation, dysfunction. In this era, the aim of treatment of TOF is attempted to preserve pulmonary valve annulus for prevent right ventricular dysfunction in the future. The systemic to pulmonary… More >

Xianchao Jiang¹, Jinyang Liu¹, Bo Peng¹, Heng Zhang², Shoujun Li¹, Jun Yan¹, Qiang Wang^1,2,*

Congenital Heart Disease, Vol.16, No.1, pp. 53-64, 2021, DOI:10.32604/CHD.2021.013233

Abstract Background: There is scarce research on large cohorts with tetralogy of Fallot (TOF) from China. The database in Fuwai Hospital was reviewed to ascertain current trends in the management of TOF and to determine the prevalence of various surgical techniques and the optimal early outcome. Methods: This cross-sectional study included 1861 patients who underwent surgery between 2012 and 2017 and were aged 0-18 years old with a primary diagnosis of TOF. A total of 1760 eligible patients were included in the analyses. Results: A total of 1683 patients underwent repair of TOF as a one-stage operation (primary repair). Sixty-one patients… More >

Gang Li, Han Zhang, Yao Yang, Yang Liu, Aijun Liu, Xiangming Fan, Pei Cheng, Junwu Su^*

Congenital Heart Disease, Vol.15, No.6, pp. 431-439, 2020, DOI:10.32604/CHD.2020.012249

Abstract Background: Pseudoaneurysm complicating right ventricular outflow tract (RVOT) with conduit placement was an infrequent complication but with potential for significant morbidity and mortality, and a more unusual pseudoaneurysm after RVOT patching was investigated here. Methods: Patients diagnosed as pseudoaneurysms at our institution from 2010 to 2019 were reviewed and their clinical characteristics were analyzed. Results: A total of seven patients developed pseudoaneurysm in RVOT were identified. One pseudoaneurysm arose after placement of a conduit between the right ventricle and the pulmonary artery, and the other six formed after RVOT patching. One patient presented with arrhythmia, one patient had the pseudoaneurysm… More >

Shivani M. Bhatt^1,*, Michael L. O’Byrne², Michael McBride², Stephen M. Paridon², Elizabeth Goldmuntz², Laura Mercer-Rosa²

Congenital Heart Disease, Vol.15, No.2, pp. 101-115, 2020, DOI:10.32604/CHD.2020.011287

Abstract Objective: The determinants of exercise capacity in repaired tetralogy of Fallot (rTOF) are multifactorial and remain incompletely understood. This study sought to evaluate the association of chronotropic response with exercise parameters and investigate the determinants of heart rate reserve (HRR) in a cohort of children and adolescents with rTOF. Design: We retrospectively analyzed patients with rTOF, age 8–18 years, who underwent cardiac magnetic resonance (CMR) and cardiopulmonary exercise test (CPET) for research purposes. Linear regression models were performed to test associations among clinical, CMR and CPET parameters. Outcomes included percent-predicted maximum VO₂ (%mVO₂) and HRR. Results: A total of 148… More >

Matthias Schneider^*, Miriam Moser, Varius Dannenberg, Andreas Mangold, Robert Schönbauer, Christian Hengstenberg, Harald Gabriel

Congenital Heart Disease, Vol.15, No.1, pp. 51-58, 2020, DOI:10.32604/CHD.2020.011712

Abstract Background: Predicting the probability for sudden cardiac death (SCD) and thus evaluation of patients for electrical device therapy and/or ablation is one of the main tasks in clinics for adults with congenital heart disease (ACHD) following repaired tetralogy of Fallot (rTOF) patients. Previous data suggests that QRS complex analysis can help identifying those patients who subsequently suffer from SCD. We hypothesized that a long QRS duration is associated with adverse rhythm events if caused by conduction abnormalities but not if caused by right ventricular remodeling. Methods: A retrospective analysis was performed entailing all rTOF patients who were seen at our… More >