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    ARTICLE

    A population-based analysis of mortality in patients with Turner syndrome and hypoplastic left heart syndrome using the Texas Birth Defects Registry

    Diego A. Lara1, Mary K. Ethen2, Mark A. Canfield2, Wendy N. Nembhard3, Shaine A. Morris1

    Congenital Heart Disease, Vol.12, No.1, pp. 105-112, 2017

    Abstract Background: Hypoplastic left heart syndrome (HLHS) is strongly associated with Turner syndrome (TS); outcome data when these conditions coexist is sparse. We aimed to investigate long-term survival and causes of death in this population.
    Methods: The Texas Birth Defects Registry was queried for all live born infants with HLHS during 1999–2007. We used Kaplan–Meier and Cox regression analyses to compare survival among patients with HLHS with TS (HLHS/TS+) to patients who had HLHS without genetic disorders or extracardiac birth defects (HLHS/TS–).
    Results: Of the 542 patients with HLHS, 11 had TS (2.0%), 71 had other extracardiac birth defects… More >

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