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  • Open Access

    ARTICLE

    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

    ARTICLE

    Right ventricular and pulmonary vascular function indices for risk stratification of patients with pulmonary regurgitation

    Alexander C. Egbe, William R. Miranda, Patricia A. Pellikka, Sorin V. Pislaru, Barry A. Borlaug, Srikanth Kothapalli, Sindhura Ananthaneni, Harigopal Sandhyavenu, Maria Najam, Mohamed Farouk Abdelsamid, Heidi M. Connolly

    Congenital Heart Disease, Vol.14, No.4, pp. 657-664, 2019, DOI:10.1111/chd.12768

    Abstract Background: We hypothesized that echocardiographic indices of right ventricular to pulmonary artery (RV‐PA) coupling were comparable to cardiac magnetic resonance imaging (CMRI)‐derived RV volumetric indices in predicting disease severity in chronic pulmonary regurgitation (PR).
    Methods: Patients with ≥ moderate PR (2003‐2015) with and without prior CMRI scans were enrolled into the study cohort and validation cohort, respectively. Endpoint was to determine the association between noninvasive RV‐PA coupling in‐ dices (tricuspid annular plane systolic excursion/right ventricular systolic pressure [TAPSE/RVSP] and fractional area change [FAC]/RVSP ratio) and markers of disease severity, and compared this association to that of CMRI‐derived RV volumetric indi‐… More >

  • Open Access

    ARTICLE

    Intrauterine growth restriction is not associated with decreased exercise capacity in adolescents with congenital heart disease

    Andrew D. Spearman1, Rohit S. Loomba2, Michael Danduran1, Joshua Kovach1

    Congenital Heart Disease, Vol.13, No.3, pp. 369-376, 2018, DOI:10.1111/chd.12577

    Abstract Objective: Multiple studies demonstrate the association of intrauterine growth restriction (IUGR) with impaired aerobic fitness in adolescents and adults. To our knowledge, there are no studies including individuals with the history of both IUGR and congenital heart disease (CHD). Thus, we sought to evaluate the impact of IUGR on exercise capacity in adolescents with CHD.
    Study Design: We conducted a retrospective chart review of patients <18 years of age who underwent cardiopulmonary exercise testing (CPET) between August 1, 2003 and July 1, 2016. Individuals with birth weight <10th percentile for gestational age were defined as IUGR. Patients with IUGR were… More >

  • Open Access

    ARTICLE

    Small unrepaired atrial septal defects display impaired exercise capacity compared with healthy peers

    Sebastian Udholm, Christian Rex, Filip Eckerström, Mine Onat, Camilla Nyboe, Vibeke E. Hjortdal

    Congenital Heart Disease, Vol.14, No.3, pp. 372-379, 2019, DOI:10.1111/chd.12740

    Abstract Objective: Adult patients with small, unrepaired atrial septal defects have an in‐ creased risk of pneumonia, atrial fibrillation, and stroke. Furthermore, they have higher late mortality than the background population. The functional capacity is un‐ known in these patients. Therefore, our objective was to determine exercise capacity in adult patients diagnosed with an unrepaired atrial septal defect compared to healthy controls. Design: A cross‐sectional study. Patients: Adult patients with small, unrepaired atrial septal defects, aged 18‐65, di‐ agnosed between 1953 and 2011. Interventions: Cardiopulmonary exercise test was performed using an incremental bicycle test and gas exchange was measured using breath‐by‐breath… More >

  • Open Access

    ARTICLE

    A restrictive ventilatory pattern is common in patients with univentricular heart after Fontan palliation and associated with a reduced exercise capacity and quality of life

    Alessia Callegari1,2,4, Rhoia Neidenbach2, Ornella Milanesi1, Biagio Castaldi1, Martin Christmann4, Masamichi Ono3, Jan Müller2, Peter Ewert2, Alfred Hager2

    Congenital Heart Disease, Vol.14, No.2, pp. 147-155, 2019, DOI:10.1111/chd.12694

    Abstract Aim: The Fontan circulation is highly dependent on ventilation, improving pulmonary blood flow and cardiac output. A reduced ventilatory function is reported in these patients. The extent of this impairment and its relation to exercise capacity and qual‐ ity of life is unknown and objective of this study.
    Methods: This multicenter retrospective/cross‐sectional study included 232 patients (140 females, age 25.6 ± 10.8 years) after Fontan palliation (19.8% atrioventricular connection; 20.3% atriopulmonary connection; 59.9% total cavopulmonary connec‐ tion). Resting spirometry, cardiopulmonary exercise tests, and quality‐of‐life assess‐ ment (SF‐36 questionnaire) were performed between 2003 and 2015.
    Results: Overall, mean forced expiratory volume… More >

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