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

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 before and after mRVoh. Results:… More > Graphic Abstract

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

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 ± 6.1 years), with… 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

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. Twenty-four patients had… More >

Robin H.S. Chen¹, Adolphus K.T. Chau¹, Pak Cheong Chow¹, Tak Cheung Yung¹, Yiu Fai Cheung^1,2, Kin Shing Lun¹

Congenital Heart Disease, Vol.13, No.6, pp. 884-891, 2018, DOI:10.1111/chd.12658

Abstract Objective: Transcatheter valve perforation for pulmonary atresia intact ventricular septum is the standard of care for patients with mild right ventricular hypoplasia. However, its role in moderate right ventricular hypoplasia has been less well defined. We sought to report the long‐term outcome of patients with moderate hypoplastic right ventricle who had undergone the procedure.
Design, Settings, and Patients: We performed a retrospective analysis on patients who had undergone transcatheter pulmonary valve perforation from January 1996 to January 2015 at our institution. The procedures would be carried out irrespective of the right ventricular size, as long as there were no absolute… More >

Rohit S. Loomba^1,2, Andrew N. Pelech^1,2

Congenital Heart Disease, Vol.13, No.1, pp. 92-97, 2018, DOI:10.1111/chd.12510

Abstract Background: Pulmonary atresia with intact ventricular septum is a unique congenital malformation of the heart in which patients may undergo single-, two- or 1½- ventricle repair. Size of the tricuspid valve annulus, morphology of the right ventricle and presence of ventriculocoronary connections can all impact the selection of appropriate palliative strategy. We developed the aortic perfusion score, a novel scoring system based on anterograde coronary perfusion with the aim of being able to identify patients at risk for death or transplant.
Methods: A retrospective study was conducted. Patients were included if an initial catheterization was done prior to any intervention.… 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 attempt for… More >