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  • Open Access

    ARTICLE

    Adverse effects of amiodarone therapy in adults with congenital heart disease

    Benjamin M. Moore1,2, Rachael L. Cordina1,2, Mark A. McGuire1,2, David S. Celermajer1,2

    Congenital Heart Disease, Vol.13, No.6, pp. 944-951, 2018, DOI:10.1111/chd.12657

    Abstract Objective: Amiodarone is a highly effective antiarrhythmic therapy, however its tox‐ icity profile often limits treatment. This is particularly relevant in adults with congeni‐ tal heart disease (CHD), who are often young and in whom other antiarrhythmic agents commonly fail or are contraindicated. We sought to determine incidence and predictors of adverse effects caused by amiodarone in adult CHD (ACHD).
    Design: A retrospective review of patients with moderate to complex ACHD treated with amiodarone at our center between 2000 and 2017 was performed. Incidence and predictors of adverse effects were described. Efficacy of amiodarone therapy in controlling… More >

  • Open Access

    ARTICLE

    Is pulse oximetry helpful for the early detection of critical congenital heart disease at high altitude?

    Fabricio González‐Andrade, Daniel Echeverría, Valeria López, Michaela Arellano

    Congenital Heart Disease, Vol.13, No.6, pp. 911-918, 2018, DOI:10.1111/chd.12654

    Abstract Objective: To assess the pulse oximetry as a method for screening critical congenital heart disease (CHD) in newborns.
    Study design: This is an observational, transversal, descriptive simple study. The pre‐ ductal and postductal saturation were taken in term newborns thatfulfilled the criteria of inclusion and exclusion in the Hospital Gineco‐Obstetrico Isidro Ayora (HGOIA) in Quito. These measurements were performed between the 24 and 48 h after birth. Those new‐ borns that saturated less than 90% on initial pulse oxìmetry underwent 3 successive measurements at 1‐h intervals. Those who saturate less than 90% after 3 measurements or have… More >

  • Open Access

    ARTICLE

    Cardiac morphology for the millennial cardiology fellow: Nomenclature and advances in morphologic imaging

    Shankar Baskar, Seth B. Gray, Erica L. Del Grippo, Onyekachukwu Osakwe, Adam W. Powell, Justin T. Tretter

    Congenital Heart Disease, Vol.13, No.5, pp. 808-810, 2018, DOI:10.1111/chd.12675

    Abstract Cardiology fellows‐in‐training, both in adult and pediatric hospitals, need structured education in regards to congenital heart disease (CHD) nomenclature. With improved survival of patients with CHD, it is not uncommon for these patients to seek care in multiple adult and pediatric hospitals. A deep understanding of CHD nomenclature would aid in providing accurate medical and surgical care for these patients. In this forum, we share our experience with such structured education and also comment on recent advances in morphologic imaging that would aid in understanding the nomenclature. More >

  • Open Access

    ARTICLE

    Hemodynamic effects of ketamine in children with congenital heart disease and/or pulmonary hypertension

    Rohit S. Loomba1, Seth B. Gray2, Saul Flores3

    Congenital Heart Disease, Vol.13, No.5, pp. 646-654, 2018, DOI:10.1111/chd.12662

    Abstract Introduction: Ketamine is a drug often used for procedural sedation or as adjunct agent for general sedation in children with congenital heart disease. In the clinical realm, there is often confusion regarding the effects of ketamine on hemodynamics, particularly pulmonary vascular resistance and systemic vascular resistance. We per‐ formed a meta‐analysis of studies investigating the effects of ketamine on hemodynamics.
    Methods: A systematic review was conducted to identify studies characterizing the hemodynamic effects of ketamine in children with congenital heart disease. Studies were assessed for quality and those of satisfactory quality with pre‐ and postketa‐ mine hemodynamics… More >

  • Open Access

    ARTICLE

    Congenital coronary artery fistula: Presentation in the neonatal period and transcatheter closure

    Varun Aggarwal, Venkatachalam Mulukutla, Athar M. Qureshi, Henri Justino

    Congenital Heart Disease, Vol.13, No.5, pp. 782-787, 2018, DOI:10.1111/chd.12653

    Abstract Background: Congenital coronary artery fistula is a rare coronary anomaly. Most commonly, such fistulae drain into the right side of the heart or the pulmonary artery. Children with coronary artery fistulae are generally asymptomatic, although they may have left ventricular enlargement in the setting of a moderate sized left to right shunt. Symptoms of congestive heart failure or ischemia are very rare in neonatal period, and suggest the presence of a very large shunt and/or coronary steal.
    Methods: Single center retrospective review of transcatheter intervention on coro‐ nary artery fistulae presenting with symptoms in the neonatal period… More >

  • Open Access

    ARTICLE

    Narrative analysis of adults with complex congenital heart disease: Childhood experiences and their lifelong reverberations

    Michelle Keir1, Barbara Bailey1, Angela Lee1, Adrienne H. Kovacs1,2, S. Lucy Roche1,3

    Congenital Heart Disease, Vol.13, No.5, pp. 740-747, 2018, DOI:10.1111/chd.12647

    Abstract Background: With access to surgical care, >90% of today’s infants with congenital heart disease (CHD) will reach adulthood. During childhood, survivors accrue a wealth of health care experience and develop strategies for navigating life with a chronic disease.
    Methods: Seeking to learn from this individualized process, we invited adults with complex CHD to participate in narrative analysis—an established qualitative research method for studying how individuals derive meaning from their personal stories. Audio recordings of 2‐4 hour free‐form interviews were transcribed and iteratively analyzed to identify common themes and detect similarities or differences in language, viewpoint and interpretation.… More >

  • Open Access

    ARTICLE

    Identifying self‐reported neurocognitive deficits in the adult with congenital heart disease using a simple screening tool

    Ashley Brunmeier1,2, Michael P Reis3, Michael G. Earing1,2, Laura Umfleet 4, Salil Ginde1,2, Peter J. Bartz1,2, Scott Cohen1,2

    Congenital Heart Disease, Vol.13, No.5, pp. 728-733, 2018, DOI:10.1111/chd.12646

    Abstract Objective: Children with congenital heart disease (CHD) and adults with acquired heart disease are at an increased risk of neurocognitive impairment. The objective of this study was to determine the prevalence of self‐reported neurocognitive impair‐ ment and its risk factors in the adult congenital heart disease (ACHD) population.
    Design: The Wisconsin Adult Congenital Heart Disease Program recently began screening ACHD patients to identify those with significant self‐perceived neurocog‐ nitive impairments. Screening consists of using a validated neuro‐oncology screening instrument that has been modified for the ACHD population. Patients who answer this survey in a predetermined fashion consistent… More >

  • Open Access

    ARTICLE

    Hospital resource utilization and presence of advance directives at the end of life for adults with congenital heart disease

    Jill M. Steiner1, James N. Kirkpatrick1, Susan R. Heckbert2, James Sibley3, James A. Fausto3, Ruth A. Engelberg3, J. Randall Curtis3

    Congenital Heart Disease, Vol.13, No.5, pp. 721-727, 2018, DOI:10.1111/chd.12638

    Abstract Objective: Overall health care resource utilization by adults with congenital heart disease has increased dramatically in the past two decades, yet little is known about utilization patterns at the end of life. The objective of this study is to better under‐ stand the patterns and influences on end‐of‐life care intensity for adults with con‐ genital heart disease.
    Methods: We identified a sample of adults with congenital heart disease (n = 65), cancer (n = 10 784), or heart failure (n = 3809) who died between January 2010 and December 2015, cared for in one multi‐hospital health care… More >

  • Open Access

    ARTICLE

    Neurodevelopmental outcomes at 2 and 4 years in children with congenital heart disease

    Cheryl L. Brosig1,2, Laurel Bear1,2, Sydney Allen1, Pippa Simpson1, Liyun Zhang1, Michele Frommelt1,2, Kathleen A. Mussatto2

    Congenital Heart Disease, Vol.13, No.5, pp. 700-705, 2018, DOI:10.1111/chd.12632

    Abstract Background and Objectives: Children with congenital heart disease (CHD) are at risk for neurodevelopmental (ND) delays. The purpose of this study is to compare the ND testing results of children with CHD at 2 and 4 years of age and determine if rates of ND delays change over time.
    Methods: Children with CHD completed the Bayley Scales of Infant Development‐III (BSID‐III) at 2 years of age, and standardized neuropsychological measures at 4 years. Scores were compared with test norms and were classified as: average (within one SD of test mean); at risk (1‐2 SDs from the test… More >

  • Open Access

    ARTICLE

    Anticoagulation practices in adults with congenital heart disease and atrial arrhythmias in Switzerland

    Ketina Arslani1*, Lukas Notz1*, Marzena Zurek1, Matthias Greutmann2, Markus Schwerzmann3, Judith Bouchardy4, Reto Engel5, Christine Attenhofer Jost6, Daniel Tobler1

    Congenital Heart Disease, Vol.13, No.5, pp. 678-684, 2018, DOI:10.1111/chd.12627

    Abstract Background: In adults with congenital heart disease (CHD) and atrial arrhythmias, recommendations for thromboprophylaxis are vague and evidence is lacking. We aimed to identify factors that influence decision-making in daily practice.
    Methods: From the Swiss Adult Congenital HEart disease Registry (SACHER) we identified 241 patients with either atrial fibrillation (Afib) or atrial flutter/intraatrial reentrant tachycardia (Aflut/ IART). The mode of anticoagulation was reviewed. Logistic regression models were used to assess factors that were associated with oral anticoagulation therapy.
    Results: Compared with patients with Aflut/IART, patients with Afib were older (51 ± 16.1 vs 37 ± 16 years, P… More >

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