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  • Open Access


    Long-Term Outcomes of Systemic-to-Pulmonary Artery Shunt in Patients with Functional Single Ventricle and Heterotaxy Syndrome

    Shota Kawai1, Takashi Kido1,*, Yuta Teguri1, Koji Miwa1, Tomomitsu Kanaya1, Yoichiro Ishii2, Hisaaki Aoki2, Futoshi Kayatani2, Sanae Tsumura1

    Congenital Heart Disease, Vol.18, No.4, pp. 399-411, 2023, DOI:10.32604/chd.2023.042243

    Abstract Objectives: We sought to determine the long-term outcomes and mortality-associated factors after systemic-to-pulmonary artery shunt (SPS) in patients with heterotaxy syndrome. Methods: We retrospectively analyzed all patients with a functional single ventricle and heterotaxy syndrome who underwent SPS at our center from January 2001 to April 2022. Results: This study involved 84 patients. Restrictive pulmonary blood flow requiring early modulation was presented in 34 (40%) patients. Compared with patients without restrictive pulmonary blood flow (N = 50 [60%]), the postnatal survival of these 34 patients was significantly lower at 10 years (log rank: p = 0.04), but the statistical significance… More >

  • Open Access


    One-Stage Correction with Intra- and Extraatrial Rerouting of Anomalous Systemic and Pulmonary Venous Return and Intraventricular Repair of Double Outlet Right Ventricle in a Patient with Heterotaxy Syndrome

    Helena Staehler1, Carina Hopfner2, Masamichi Ono1,*, Jürgen Hörer1

    Congenital Heart Disease, Vol.17, No.1, pp. 25-30, 2022, DOI:10.32604/CHD.2022.018366

    Abstract We report a very rare case of successful intracardiac correction in a patient with heterotaxy syndrome. The cardiac malformations included dextrocardia, double outlet right ventricle, pulmonary stenosis, interrupted inferior vena cava, hemiazygos continuation and total anomalous pulmonary venous return. One-stage correction was performed. The atrial procedure consisted of intra- and extraatrial rerouting of the anomalous systemic and pulmonary venous return. The hepatic veins were detached and diverted to the left atrium via an extracardiac conduit. The correction of the double outlet right ventricle was accomplished by intraventricular redirection of the blood flow from the left ventricle to the aorta. The… More >

  • Open Access


    Outcomes in adults with congenital heart disease and heterotaxy syndrome: A single‐center experience

    Christopher R. Broda, Katherine B. Salciccioli, Keila N. Lopez, Peter R. Ermis, Douglas S. Moodie, Heather A. Dickerson

    Congenital Heart Disease, Vol.14, No.6, pp. 885-894, 2019, DOI:10.1111/chd.12856

    Abstract Background: Heterotaxy syndrome (HS) is a condition in which the thoracoabdominal organs demonstrate an abnormal lateral arrangement and is often associated with con‐ genital heart disease (CHD). Little is known about the adult HS population with CHD.
    Objective: To describe the outcomes and sociodemographics of the adult CHD popu‐ lation with HS.
    Methods: Records of patients 18 years of age or older with diagnoses of both CHD and HS at Texas Children's Hospital from 1964 to 2018 were reviewed.
    Results: Sixty‐two patients metinclusion criteria.Median agewas 22.7 [IQR19.6‐30.0] years; 26 (42%) were female; and 13 (21%) of patients had a gap… More >

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