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  • Open Access


    Prevalence of Bicuspid Aortic Valve in Turner Syndrome Patients Receiving Cardiac MRI and CT: A Meta-Analysis

    Pengzhu Li, Martina Bačová, Robert Dalla-Pozza, Nikolaus Alexander Haas, Felix Sebastian Oberhoffer*

    Congenital Heart Disease, Vol.17, No.2, pp. 129-141, 2022, DOI:10.32604/CHD.2022.018300

    Abstract Turner syndrome (TS) is a rare disorder affecting 25–50 in 100000 female newborns. Bicuspid aortic valve (BAV) is assumed to be the most common congenital heart defect (CHD) in TS. In literature, reported BAV prevalence in TS ranges between 14% and 34%. The specific BAV prevalence in TS is still unknown. The aim of this study was to give a more precise estimation of BAV prevalence in TS by conducting a meta-analysis of TS-studies, which detected BAV by either cardiac magnetic resonance imaging (MRI) or cardiac computed tomography (CT). We searched PubMed, Cochrane Library, and Web of Science databases to… More >

  • Open Access


    Systolic and Diastolic Strain Measurements Show Left Ventricular Dysfunction in Women with Turner Syndrome

    Allard T. van den Hoven1, Sultan Yilmazer1, Lidia R. Bons1, Roderick W. J. van Grootel1, L. W. Geenen1, An M. van Berendoncks1, Anthonie L. Duijnhouwer2, Hans-Marc J. Siebelink3, Jolien W. Roos-Hesselink1, Annemien E. van den Bosch1,*

    Congenital Heart Disease, Vol.16, No.4, pp. 357-368, 2021, DOI:10.32604/CHD.2021.014462

    Abstract Aim: This study aimed to describe the systolic left ventricular global longitudinal strain (LvGLS) and left ventricular peak early diastolic strain rate (Sre) in adult women with Turner syndrome (TS) and to determine its relationship with exercise capacity and clinical parameters. Methods: In this cross-sectional cohort study, consecutively included adult TS women underwent an electrocardiogram, transthoracic echocardiogram (TTE) and cardiopulmonary exercise test (CPET) on the same day. LvGLS and Sre were measured using 2D speckle tracking analysis (STE) and compared with age-matched healthy female controls. Results: Ninety-four adult women (age 36 ± 13 years) with TS and 32 healthy age-matched… More >

  • Open Access


    Congenital heart disease and cardiac procedural outcomes in patients with trisomy 21 and Turner syndrome

    Raysa Morales-Demori

    Congenital Heart Disease, Vol.12, No.6, pp. 820-827, 2017, DOI:10.1111/chd.12521

    Abstract Congenital heart disease (CHD) is present in approximately 50% of patients with trisomy 21 (T21) and Turner syndrome (TS). According to the American Academy of Pediatrics, every patient with these genetic disorders should have a postnatal echocardiogram. T21 is usually associated with atrioventricular (30%–60%), atrial (16%-21%), or ventricular septal defects (14%–27%). TS is usually associated with left-sided heart disease. However, the spectrum of CHD in these genetic disorders is wider than those mentioned lesions. More cardiac surgical procedures are offered to these patients and that has influenced positively their life expectancy for some CHD conditions. Single ventricular anatomy is associated… More >

  • Open Access


    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 or genetic… More >

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