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  • Open Access

    ARTICLE

    Hand‐held echocardiography in children with hypoplastic left heart syndrome

    Alan F. Riley1, Elena C. Ocampo1, Joseph Hagan2, M. Regina Lantin‐Hermoso1

    Congenital Heart Disease, Vol.14, No.5, pp. 706-712, 2019, DOI:10.1111/chd.12774

    Abstract Background: When performed by cardiologists, hand‐held echocardiography (HHE) can assess ventricular systolic function and valve disease in adults, but its accuracy and utility in congenital heart disease is unknown. In hypoplastic left heart syndrome (HLHS), the echocardiographic detection of depressed right ventricular (RV) systolic function and higher grade tricuspid regurgitation (TR) can identify patients who are at increased risk of morbidity and mortality and who may benefit from additional imaging or medical therapies.
    Methods: Children with HLHS after Stage I or II surgical palliation (Norwood or Glenn procedures) were prospectively enrolled. Subjects underwent HHE by a pediatric cardiologist on the… More >

  • Open Access

    REVIEW

    Safety of contraceptive use among women with congenital heart disease: A systematic review

    Ginnie Abarbanell1, Naomi K. Tepper2, Sherry L. Farr3

    Congenital Heart Disease, Vol.14, No.3, pp. 331-340, 2019, DOI:10.1111/chd.12752

    Abstract Objective: Women with congenital heart disease (CHD) are at increased risk of preg‐ nancy complications and need information on safe, effective contraceptive methods to avoid unintended pregnancy. This systematic review examines evidence regarding safety of contraceptive use among women with CHD.
    Methods: The PubMed database was searched for any peer‐reviewed articles pub‐ lished through April 2018 that included safety outcomes associated with reversible contraceptive methods among women with CHD.
    Results: Five articles met inclusion criteria: three studies comparing contraceptive users to nonusers and two noncomparative studies. Sample sizes ranged from 65 to 505 women with CHD. Two studies found a… More >

  • Open Access

    ARTICLE

    Body mass index in adults with congenital heart disease

    Mahmoud Zaqout1,2, Kristof Vandekerckhove1, Nathalie Michels2, Laurent Demulier3, Thierry Bove4, Katrien François4, Julie De Backer3, Stefaan De Henauw2, Daniel De Wolf1

    Congenital Heart Disease, Vol.14, No.3, pp. 479-486, 2019, DOI:10.1111/chd.12751

    Abstract Objective: To investigate the status of body mass index (BMI) in adult people with congenital heart disease (ACHD).
    Methods: Five hundred thirty‐nine adults with CHD (53.8% men) were seen in the outpatient clinic from 2013 to 2015 and compared to a reference population (n = 1737). The severity of CHD was categorized as mild, moderate, and severe ac‐ cording to standard guidelines. Patients were categorized based on BMI as under‐ weight (<18.5), overweight (25‐30), or obese (>30). Echocardiography and magnetic resonance imaging were used to measure ventricular function while exercise capac‐ ity was estimated via cardiopulmonary exercise test.
    Results: Adults… More >

  • Open Access

    ARTICLE

    Accuracy of risk prediction scores in pregnant women with congenital heart disease

    Yuli Y. Kim1,2, Leah A. Goldberg2, Katherine Awh2, Tanmay Bhamare1,2, David Drajpuch2, Adi Hirshberg3, Sara L. Partington1,2, Rachel Rogers4, Emily Ruckdeschel1,2, Lynda Tobin1, Morgan Venuti2, Lisa D. Levine3

    Congenital Heart Disease, Vol.14, No.3, pp. 470-478, 2019, DOI:10.1111/chd.12750

    Abstract Objective: To assess performance of risk stratification schemes in predicting adverse cardiac outcomes in pregnant women with congenital heart disease (CHD) and to compare these schemes to clinical factors alone.
    Design: Single‐center retrospective study.
    Setting: Tertiary care academic hospital.
    Patients: Women ≥18 years with International Classification of Diseases, Ninth Revision, Clinical Modification codes indicating CHD who delivered between 1998 and 2014. CARPREG I and ZAHARA risk scores and modified World Health Organization (WHO) criteria were applied to each woman.
    Outcome Measures: The primary outcome was defined by ≥1 of the following: arrhyth‐ mia, heart failure/pulmonary edema, transient ischemic attack, stroke,… More >

  • Open Access

    ARTICLE

    Opportunities for training to advance the care for adults with congenital heart disease with advanced circulatory failure

    Christopher R. Broda

    Congenital Heart Disease, Vol.14, No.3, pp. 487-490, 2019, DOI:10.1111/chd.12748

    Abstract Heart failure is an emerging issue with important implications in adult patients with congenital heart disease. Practitioners with expertise in both adult congenital heart disease and heart failure are needed to manage this growing and often complex pop‐ ulation. In the United States, the optimal training pathway to enable practitioners to best care for these patients is ill‐defined. This article explores possibilities and issues that interested trainees may encounter during their training experience. More >

  • Open Access

    ARTICLE

    Education as important predictor for successful employment in adults with congenital heart disease worldwide

    Maayke A. Sluman1,2, Silke Apers3,4, Judith K. Sluiter1,*, Karen Nieuwenhuijsen1, Philip Moons4,5, Koen Luyckx6,7, Adrienne H. Kovacs8,9, Corina Thomet10, Werner Budts11, Junko Enomoto12, Hsiao‐Ling Yang13, Jamie L. Jackson14, Paul Khairy15, Stephen C. Cook16, Raghavan Subramanyan17, Luis Alday18, Katrine Eriksen19, Mikael Dellborg20,21, Malin Berghammer5,22, Eva Mattsson23, Andrew S. Mackie24, Samuel Menahem25, Maryanne Caruana26, Kathy Gosney27, Alexandra Soufi28, Susan M. Fernandes29, Kamila S. White30, Edward Callus31, Shelby Kutty32, Berto J. Bouma33, Barbara J.M. Mulder33

    Congenital Heart Disease, Vol.14, No.3, pp. 362-371, 2019, DOI:10.1111/chd.12747

    Abstract Background: Conflicting results have been reported regarding employment status and work ability in adults with congenital heart disease (CHD). Since this is an impor‐ tant determinant for quality of life, we assessed this in a large international adult CHD cohort.
    Methods: Data from 4028 adults with CHD (53% women) from 15 different countries were collected by a uniform survey in the cross‐sectional APPROACH International Study. Predictors for employment and work limitations were studied using general linear mixed models.
    Results: Median age was 32 years (IQR 25‐42) and 94% of patients had at least a high school degree. Overall employment rate… More >

  • Open Access

    ARTICLE

    Electronic health record associated stress: A survey study of adult congenital heart disease specialists

    Darcy N. Marckini1, Bennett P. Samuel1, Jessica L. Parker2, Stephen C. Cook1,3

    Congenital Heart Disease, Vol.14, No.3, pp. 356-361, 2019, DOI:10.1111/chd.12745

    Abstract Background: Physician burnout has many undesirable consequences, including nega‐ tive impact on patient care delivery and physician career satisfaction. Electronic health records (EHRs) may exacerbate burnout by increasing physician workload.
    Objective: To determine burnout in adult congenital heart disease (ACHD) specialists by assessing stress associated with EHRs.
    Design: Electronic survey study of ACHD providers.
    Setting: Canada and United States.
    Participants: Three hundred eighty‐three ACHD specialists listed on the Adult Congenital Heart Association directory between February and April 2017.
    Outcome Measures: Burnout was measured using the Maslach Burnout Inventory (MBI) to understand factors contributing to work life and EHR satisfaction. Chi‐… More >

  • Open Access

    ARTICLE

    S100B and its relation to cerebral oxygenation in neonates and infants undergoing surgery for congenital heart disease

    Jan Hinnerk Hansen1, Lydia Kissner1, Jana Logoteta1, Olaf Jung1, Peter Dütschke2, Tim Attmann3, Jens Scheewe3, Hans‐Heiner Kramer1,4

    Congenital Heart Disease, Vol.14, No.3, pp. 427-437, 2019, DOI:10.1111/chd.12741

    Abstract Objectives: Neonates and infants undergoing surgery for congenital heart disease are at risk for developmental impairment. Hypoxic‐ischemic brain injury might be one contributing factor. We aimed to investigate the perioperative release of the astro‐ cyte protein S100B and its relation to cerebral oxygenation.
    Methods: Serum S100B was measured before and 0, 12, 24, and 48 hours after sur‐ gery. Cerebral oxygen saturation was derived by near‐infrared spectroscopy. S100B reference values based on preoperative samples; concentrations above the 75th per‐ centile were defined as elevated. Patients with elevated S100B at 24 or 48 hours were compared to cases with S100B in… 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

    Comprehensive comparative outcomes in children with congenital heart disease: The rationale for the Congenital Catheterization Research Collaborative

    Christopher J. Petit1, Athar M. Qureshi2, Andrew C. Glatz3, Courtney E. McCracken1, Michael Kelleman1, George T. Nicholson4, Jeffery J. Meadows5, Shabana Shahanavaz6, Jeffrey D. Zampi7, Mark A. Law8, Joelle A. Pettus1, Bryan H. Goldstein9

    Congenital Heart Disease, Vol.14, No.3, pp. 341-349, 2019, DOI:10.1111/chd.12737

    Abstract Clinical research in the treatment of patients with congenital heart disease (CHD) is limited by the wide variety of CHD manifestations and therapeutic options as well as the generally low incidence of CHD. The availability of comprehensive, contemporary outcomes studies is therefore limited. This inadequacy may result in a lack of data‐driven medical decision making. In 2013, clinician scientists at two centers begana researchcollaboration,the Congenital Catheterization Research Collaborative (CCRC). Over time, the CCRC has grown to include nine cardiac centers from across the United States, with a common data coordinating center. The CCRC seeks to generate high‐quality, contemporary, statistically robust,… More >

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