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  • Open Access


    Giant-Size Main Pulmonary Artery Aneurysm in an Adult Patient with Ebstein Anomaly and Dextrocardia

    Kristina Krzelj1,#,*, Irena Ivanac Vranesic2,#, Kristina Maric Besic2, Zeljko Duric1, Darko Anic1

    Congenital Heart Disease, Vol.18, No.2, pp. 207-212, 2023, DOI:10.32604/chd.2023.027453

    Abstract Main pulmonary artery aneurysms are rare, mostly asymptomatic and discovered accidentally. The main pulmonary artery aneurysms may be idiopathic or secondary to underlying diseases such as pulmonary hypertension, Behcet’s disease, connective tissue disorders, congenital heart disease, vasculitis, syphilis, tuberculosis and endocarditis. There are some indices that dextrocardia is associated with anomalies of the pulmonary arteries and pulmonary valve. A rare occurrence of main pulmonary artery aneurysms results in a lack of recommendations, so the remaining challenges are whether main pulmonary artery aneurysms should be treated, how, and when. The decision on surgical intervention or conservative treatment of the main pulmonary… More > Graphic Abstract

    Giant-Size Main Pulmonary Artery Aneurysm in an Adult Patient with Ebstein Anomaly and Dextrocardia

  • Open Access


    Health-Related Quality of Life, Emotional and Behavioral Problems in Children and Adolescents with Ebstein Anomaly

    Lianne M. Geerdink1,2,*, Malindi van der Mheen3,4, Gideon J. du Marchie Sarvaas5, Irene M. Kuipers6, Stefan Frerich7, Henriëtte ter Heide2, Willem A. Helbing8, Zina Feijzic1, Christian Schroer9, Chris L. de Korte10, Livia Kapusta1,11, Chris M. Verhaak12, Elisabeth M. W. J. Utens3,4

    Congenital Heart Disease, Vol.15, No.6, pp. 457-472, 2020, DOI:10.32604/CHD.2020.012994

    Abstract Background: Due to the improved survival rates of children and adolescents with congenital heart disease (CHD), more attention is now being directed towards their health-related quality of life (HRQoL), emotional and behavioral problems. Ebstein anomaly (EA) is a rare CHD with a broad clinical spectrum. The aim of the current study is to evaluate self- and proxy-reported HRQoL and emotional and behavioral problems in children and adolescents with EA. Methods: In this cross-sectional, multicenter study, we included EA patients (aged 8–17 years), who underwent routine clinical assessments in Dutch university hospitals between May 2017 and March 2019. The Generic Pediatric… More >

  • Open Access


    Contemporary outcomes and mortality risks of Ebstein anomaly: A single-center experience in Thailand

    Varisara Pornprasertchai, Chodchanok Vijarnsorn, Supaluck Kanjanauthai, Paweena Chungsomprasong, Prakul Chanthong, Kritvikrom Durongpisitkul, Jarupim Soongswang

    Congenital Heart Disease, Vol.14, No.4, pp. 619-627, 2019, DOI:10.1111/chd.12759

    Abstract Background: The increasing number of patients with Ebstein anomaly (EA) surviving into adulthood implies improvements in the treatments for the complex lesion. We revisited the clinical outcomes of patients with EA to demonstrate their “real world” survival.
    Objectives: To identify the survival and predictors of mortality in patients with EA who underwent medical or surgical management in the present era.
    Methods: All patients who had EA with atrioventricular concordance between 1994 and 2016 were retrospectively reviewed. Baseline characteristics, initial echocardio‐ graphic findings, treatments, and outcomes were explored. The survival analysis was performed at the end of 2017. A multivariate analysis… More >

  • Open Access


    Arrhythmia after cone repair for Ebstein anomaly: The Mayo Clinic experience in 143 young patients

    Philip Wackel1,2, Bryan Cannon1,2, Joseph Dearani3, Kristen Sessions1,2, Kimberly Holst3, Jonathan Johnson1,2, Frank Cetta1,2

    Congenital Heart Disease, Vol.13, No.1, pp. 26-30, 2018, DOI:10.1111/chd.12566

    Abstract Background: The increased incidence of preoperative and postoperative arrhythmia in Ebstein anomaly (EA) prompted some clinicians to perform an electrophysiology study (EPS) in all patients prior to surgery for EA. The cone repair (CR) is the current surgical option of choice for most young patients with EA but the effect of the CR on arrhythmia is not well established.
    Objectives: To assess the burden of arrhythmia in young patients after CR and to assess the utility of selective preoperative EPS.
    Materials and Methods: A retrospective review of all patients <21 years of age with EA who had a CR at… More >

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