Jae Gun Kwak¹, Eung Re Kim², Taeyoung Yun¹, Sungkyu Cho¹, Chang-Ha Lee², Woong-Han Kim^1,*

Congenital Heart Disease, Vol.18, No.3, pp. 325-336, 2023, DOI:10.32604/chd.2023.027758 - 09 June 2023

Abstract Objectives: To access the effectiveness of our modified right-ventricular overhauling procedure on tricuspid valve (TV) growth in patients with pulmonary atresia with intact ventricular septum (PAIVS). Methods: We retrospectively reviewed 21 patients with PAIVS who underwent modified right ventricular overhauling (mRVoh) between 2008 and 2019 at two institutions. Our mRVoh consisted of wide resection of hypertrophied infundibular and trabecular muscle, peeling off fibrotic endocardial tissue in the right ventricle (RV) cavity, surgical pulmonary valvotomy, and Blalock-Taussig shunt or banding of ductus arteriosus under cardiopulmonary bypass. The TV annulus sizes were measured and analyzed using echocardiography… More > Graphic Abstract

Wenlei Qian^1,#, Xinzhu Zhou^2,#, Ke Shi¹, Li Jiang¹, Xi Liu³, Liting Shen¹, Zhigang Yang^1,*

Congenital Heart Disease, Vol.18, No.1, pp. 113-125, 2023, DOI:10.32604/chd.2023.023542 - 09 January 2023

Abstract Background: Pulmonary atresia (PA) is a group of heterogeneous complex congenital heart disease. Only one study modality might not get a correct diagnosis. This study aims to investigate the diagnostic power of dual-source computed tomography (DSCT) for all intracardiac and extracardiac deformities in patients with PA compared with transthoracic echocardiography (TTE). Materials and Methods: This retrospective study enrolled 79 patients and divided them into three groups according to their main diagnosis. All associated malformations and clinical information, including treatments, were recorded and compared among the three groups. The diagnostic power of DSCT and TTE on all associated… More > Graphic Abstract

Sanam Safi^1,2, Takato Yamasaki^1,3, David J. Glidden⁴, Stephen P. Sanders^1,5, Chrystalle Katte Carreon^1,6,*

Congenital Heart Disease, Vol.17, No.2, pp. 141-146, 2022, DOI:10.32604/chd.2022.019743 - 26 January 2022

Abstract A maternally inherited 828 kb microdeletion of 2q37.3 manifested in a 3-week-old premature boy as left juxtaposition of the atrial appendages associated with tricuspid atresia, double outlet infundibulum, subvalvar pulmonary atresia, large secundum atrial septal defect, and right aortic arch with mirror-image branching, consistent with developmental arrest early in heart looping. To the best of our knowledge, no previous 2q37 deletion syndrome has been reported with such a severe cardiac dysmorphology. Hence, this case adds to the cardiac phenotypes identified in 2q37 deletion syndrome. More >

Muyu Qi^1,#, Xiaoping Lan^2,#, Jia Li¹, Junwen Ge¹, Li Shen^1,*, Rufang Zhang^1,*

Congenital Heart Disease, Vol.16, No.5, pp. 487-498, 2021, DOI:10.32604/CHD.2021.015887 - 03 June 2021

Abstract Objective: Pulmonary atresia (PA) is a rare type of complex cyanotic congenital heart defect characterized primarily by an undeveloped pulmonary valve or pulmonary artery. Therefore, defining a disease-causing gene mutation in a pulmonary atresia family is a possible method of genetic counseling, future prenatal diagnosis, and therapeutic approaches for pulmonary atresia. Methods: Blood samples were collected from six PA family members, and genomic DNA was extracted using the QIAamp DNA Blood Mini Kit. Gene detection was performed using a second-generation sequencing gene panel. Results: Genetic testing results indicated that a heterozygous mutation originating from maternal inheritance was detected… More >

Elio Caruso^*, Silvia Farruggio, Davide Calvaruso, David Fabio Petruccelli, David Angel Ortiz Ruiz, Corrado Di Mambro, Salvatore Agati

Congenital Heart Disease, Vol.16, No.3, pp. 205-210, 2021, DOI:10.32604/CHD.2021.015371 - 02 March 2021

Abstract We present a case of a one-day-old newborn, without prenatal diagnosis, referred to our cardiologic intensive care unit in critical condition presenting sub-cyanosis and peripheral oxygen saturation of 80%. Echocardiography diagnosis was tricuspid valve dysplasia with severe regurgitation, functional pulmonary valve atresia with intact ventricular septum and reversal flow in the large patent ductus arteriosus (PDA). Chest X-ray showed severe cardiomegaly and wall to wall heart. Prostaglandin E1 infusion was started once after birth. After few days, clinical conditions progressively worsened because of right heart failure; a first pharmacological approach to close PDA failed and More >

Jinyang Liu¹, Xianchao Jiang¹, Runsi Wang², Li Li³, Ju Zhao⁴, Fuxia Yan⁵, Run Yuan⁶, Qiang Wang^1,*

Congenital Heart Disease, Vol.16, No.3, pp. 245-254, 2021, DOI:10.32604/CHD.2021.014881 - 02 March 2021

Abstract Background: Never had literatures characterized the relationship between the property of major aortopulmonary collateral arteries (MAPCAs) and outcomes of selective unifocalization of pulmonary atresia with ventricular septal defects and MAPCAs. Methods: This is a case-series study. Thirteen patients were included. Angiography-based assessment was conducted to determine whether collateral arteries should be unifocalized or treated with intraoperative ligature. Specimens were collected and stained by HE and ET+VG. Results: Twelve patients underwent one-stage unifocalization at a median age of 37 months (range: 6–228 months) and a median weight of 14.0 kg (range: 5.0–49.0 kg), which produced a favorable right… More >

Jinyang Liu^1,#, Bo Peng^1,#, Xianchao Jiang^1,#, Simeng Zhang¹, Jiachen Li¹, Lizhi Lv¹, Qiang Wang^1,2,*, Xiang Li^3,*

Congenital Heart Disease, Vol.16, No.1, pp. 65-72, 2021, DOI:10.32604/CHD.2021.013462 - 23 December 2020

Abstract Background: This study aims to explore the efficacy of selective unifocalization (UF) for major aortopulmonary collateral arteries (MAPCAs) unifocalization in children with pulmonary atresia with ventricular septal defect (PA/VSD). Methods: A retrospective analysis of 13 patients with PA/VSD/MAPCAs who underwent surgery from June 2017 to December 2019. Sex, age, preoperative cardiovascular angiography test results and McGoon ratio were collected. The properties of the collateral arteries were evaluated by angiography, and selective UF for the “dendritic” MAPCAs and ligation of MAPCAs demonstrating distortion and resistance. Results: A total of 13 severe patients underwent one-stage repair, of which 1 More >

Pietro Paolo Tamborrino^1,2, Corrado Di Mambro^1,2,*, Cecilia Marcolin^1,2, Walter Vignaroli³, Giulia Cafiero⁴, Gianluca Brancaccio³, Sonia Albanese³, Massimo Stefano Silvetti^1,2, Adriano Carotti³, Fabrizio Drago^1,2

Congenital Heart Disease, Vol.16, No.1, pp. 85-94, 2021, DOI:10.32604/CHD.2021.013038 - 23 December 2020

Abstract Introduction: While previous studies only focused on the arrhythmic risk associated with specific correction strategies, this study evaluates this risk in a large cohort of paediatric patients with all phenotypes of PA-IVS after surgical repair. Methods: In this single centre observational cohort study, we retrospectively evaluated 165 patients with a diagnosis of PA-IVS and we excluded those with an exclusively percutaneous treatment, patients lost or with insufficient follow-up and those affected by other arrhythmic syndromes. Surgical history and clinical outcomes were reviewed. Results: 86 patients were included in the study (54 male [62.8%], mean age 16.4 ±… More >

Norihisa Toh^1,*, Yasuhiro Kotani², Teiji Akagi¹, Yosuke Kuroko², Kenji Baba³, Shin-ichi Otsuki³, Shingo Kasahara², Hiroshi Ito¹

Congenital Heart Disease, Vol.15, No.1, pp. 1-11, 2020, DOI:10.32604/CHD.2020.011579 - 17 June 2020

Abstract Background: There is limited information on outcomes of adult patients with pulmonary atresia with intact ventricular septum (PA-IVS) due to the low incidence of disease and the large variation of surgical histories. Methods: Among 58 patients with repaired PA-IVS, a total of 32 patients aged ≥16 years and who were followed at our institution between January 2003 and December 2018 were reviewed. Surgical history, clinical outcomes, and laboratory, echocardiographic and electrocardiographic data were obtained by chart review. Results: Follow-up was from the age of 16 years and the median age at the latest follow-up was 23.7 years.… More >

Bruno Lefort^1,2, Christophe Saint‐Etienne¹, Nathalie Soulé¹, Iris Ma¹, Fanny Dion¹, Alain Chantepie^1,2

Congenital Heart Disease, Vol.14, No.5, pp. 814-818, 2019, DOI:10.1111/chd.12812

Abstract Background and objective: Chronic total occlusion (CTO) guidewire have been recently reported as an alternative to radiofrequency for perforating atretic pulmonary valve. Since procedure failures or perforation of the right ventricle still occurred with CTO, we tried to enhance the stability, steering, and pushability of the wire using a microcatheter in order to improve the safety and efficacy of the procedure.
Methods: We performed pulmonary valve perforation with CTO guidewire and microcatheter in five consecutive newborns with pulmonary atresia with intact ventricular septum (PA‐IVS) under fluoroscopic and echocardiographic control.
Results: The valve was easily perforated at the first… More >