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  • Open Access


    Role of Surgery on Growth of Tricuspid Valve in Pulmonary Atresia with Intact Ventricular Septum: Mid-Term Results of Modified Right-Ventricular Overhauling Procedure

    Jae Gun Kwak1, Eung Re Kim2, Taeyoung Yun1, Sungkyu Cho1, Chang-Ha Lee2, Woong-Han Kim1,*

    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… More > Graphic Abstract

    Role of Surgery on Growth of Tricuspid Valve in Pulmonary Atresia with Intact Ventricular Septum: Mid-Term Results of Modified Right-Ventricular Overhauling Procedure

  • Open Access


    Arrhythmic Risk in Paediatric Patients Undergoing Surgical Repair for Pulmonary Atresia with Intact Ventricular Septum

    Pietro Paolo Tamborrino1,2, Corrado Di Mambro1,2,*, Cecilia Marcolin1,2, Walter Vignaroli3, Giulia Cafiero4, Gianluca Brancaccio3, Sonia Albanese3, Massimo Stefano Silvetti1,2, Adriano Carotti3, Fabrizio Drago1,2

    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 ±… More >

  • Open Access


    Outcomes of Patients with Pulmonary Atresia with Intact Ventricular Septum Reaching Adulthood

    Norihisa Toh1,*, 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.… More >

  • Open Access


    Achieving biventricular circulation in patients with moderate hypoplastic right ventricle in pulmonary atresia intact ventricular septum after transcatheter pulmonary valve perforation

    Robin H.S. Chen1, Adolphus K.T. Chau1, Pak Cheong Chow1, Tak Cheung Yung1, Yiu Fai Cheung1,2, 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… More >

  • Open Access


    Aortic perfusion score for pulmonary atresia with intact ventricular septum: An antegrade coronary perfusion scoring system that is predictive of need for transplant and mortality

    Rohit S. Loomba1,2, Andrew N. Pelech1,2

    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… More >

  • Open Access


    Perforation of the atretic pulmonary valve using chronic total occlusion (CTO) wire and coronary microcatheter

    Bruno Lefort1,2, Christophe Saint‐Etienne1, Nathalie Soulé1, Iris Ma1, Fanny Dion1, Alain Chantepie1,2

    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 >

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