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  • Open Access


    Bioprosthetic pulmonary valve endocarditis: Incidence, risk factors, and clinical outcomes

    Brian Robichaud1, Garick Hill2, Scott Cohen1,3, Ronald Woods4, Michael Earing1,3, Peter Frommelt1, Salil Ginde1,3

    Congenital Heart Disease, Vol.13, No.5, pp. 734-739, 2018, DOI:10.1111/chd.12639

    Abstract Background: Pulmonary valve replacement (PVR) is a common operation in patients with congenital heart disease (CHD). As survival with CHD improves, infective endo‐ carditis (IE) is a growing complication after PVR. The aim of this study was to assess the incidence, risk factors, and clinical outcomes of IE after surgical PVR in patients with CHD at our institution.
    Methods: Retrospective analysis of all cases of surgical PVR performed at Children’s Hospital of Wisconsin between 1975 and 2016 was performed. All cases of IE after PVR were identified and clinical and imaging data were obtained by review of medical records.
    Results:More >

  • Open Access


    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 system. We used… More >

  • Open Access


    Diastolic velocity half time is associated with aortic coarctation gradient at catheterization independent of echocardiographic and clinical blood pressure gradients

    Adam B. Christopher1, Abraham Apfel2, Tao Sun2, Jackie Kreutzer1, David S. Ezon3

    Congenital Heart Disease, Vol.13, No.5, pp. 713-720, 2018, DOI:10.1111/chd.12637

    Abstract Objective: The most accurate noninvasive parameter to predict whether a patient with aortic coarctation will meet interventional criteria at catheterization remains elusive. We aim to determine the best independent echocardiographic predictors of a coarctation peak‐to‐peak pressure gradient ≥20 mm Hg at catheterization, the ac‐ cepted threshold for intervention.
    Design: Retrospective query of our catheterization database from 1/2007 to 7/2016 for the diagnostic code of aortic coarctation was performed. Multiple echocardio‐ graphic measurements and blood pressure gradients prior to cardiac catheterization were collected. Univariate correlation of variables with the continuous catheteriza‐ tion peak were calculated using Spearman’s rho. Univariate association with… More >

  • Open Access


    Incidence of aspiration in infants with single‐ventricle physiology following hybrid procedure

    Jennifer P. Lundine1,2, Robert Dempster3,4, Kirby Carpenito5, Holly Miller‐Tate5, Wendelin Burdo‐Hartman4,6, Elizabeth Halpin2, Omar Khalid4,5

    Congenital Heart Disease, Vol.13, No.5, pp. 706-712, 2018, DOI:10.1111/chd.12636

    Abstract Background: Swallowing dysfunction is a known complication for infants with complex congenital heart disease (CHD), but few studies have examined swallowing outcomes following the hybrid procedure for stage 1 palliation in children with single ventricle physiology.
    Objectives: (1) Identify the incidence of aspiration in all infants with single ventricle physiology who underwent the hybrid procedure and (2) Compare results of clinical bedside and instrumental swallowing evaluations to examine the predictive value of a less invasive swallowing assessment for this population of high‐risk infants.
    Methods: This was a retrospective cohort chart review study. All patients with single‐ventricle physiology who underwent the… More >

  • Open Access


    Mitochondrial DNA mutation “m.3243A>G”—Heterogeneous clinical picture for cardiologists (“m.3243A>G”: A phenotypic chameleon)

    Katharina Niedermayr1, Gerhard Pölzl2, Sabine Scholl‐Bürgi1, Christine Fauth3, Ulrich Schweigmann1, Edda Haberlandt1, Ursula Albrecht1, Manuela Zlamy1, Wolfgang Sperl4, Johannes A. Mayr4, Daniela Karall1

    Congenital Heart Disease, Vol.13, No.5, pp. 671-677, 2018, DOI:10.1111/chd.12634

    Abstract Objective: In general, a mitochondrial disorder is diagnosed on the basis of symptom combinations and confirmed by genetic findings. However, patients carrying the m.3243A>G mutation in the mitochondrial tRNA leucine 1 (MT‐TL1) do not always meet all the proposed criteria for the most frequently encountered mitochondrial syndrome “MELAS,” an acronym for Mitochondrial Encephalomyopathy, Lactic Acidosis, and at least one Stroke‐like episode. We here present various phenotypic characteristics of the mitochondrial mutation m.3243A>G with particular focus on cardiac manifestations.
    Methods and Results: We followed nine patients (1 month to 68 years old; median 42 years; four female and five male) from… More >

  • Open Access


    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 mean); and delayed… More >

  • Open Access


    Mortality trends from congenital malformations of the heart and the great vessels in children and adults in the seven socioeconomic regions of Mexico, 2000‐2015

    Juan Jesús Sánchez‐Barriga

    Congenital Heart Disease, Vol.13, No.5, pp. 690-699, 2018, DOI:10.1111/chd.12631

    Abstract Background: Congenital heart disease (CHD) represents a global health problem. In Mexico, in children <1 year of age it is the second cause of mortality. The aim was to determine mortality trends from CHD and the great vessels in children and adults nationwide, by state and socioeconomic region.
    Methods: Records of mortality associated to CHD and the great vessels for 2000‐2015 were obtained from the National Institute of Statistics and Geography. This information is collected from death certificates issued nationwide. International Classification of Diseases, 10th revision, codes corresponding to the basic cause of death from CHD and the great vessels.… More >

  • Open Access


    Patients with congenital heart defect and their families support genetic heart research

    Paul C. Helm1, Ulrike M. M. Bauer1,2, Hashim Abdul‐Khaliq2,3, Helmut Baumgartner1,4, Hans‐Heiner Kramer5, Christian Schlensak2,7, Thomas Pickardt1, Anne‐Karin Kahlert5,6*, Marc‐Phillip Hitz5*

    Congenital Heart Disease, Vol.13, No.5, pp. 685-689, 2018, DOI:10.1111/chd.12630

    Abstract Background: Congenital heart disease (CHD) affects up to 1% of live births the etiol‐ ogy remains relatively poorly understood. Thus, cardiac research is needed to under‐ stand the underlying pathomechanisms ofthe disease.About51 000CHDpatients are registered in the German National Register for Congenital Heart Defects (NRCHD). Patients and relatives were interviewed online about their willingness to support ge‐ netic heart research in order to donate a biological sample.
    Methods: Study participants were recruited via the database of the NRCHD. Seven thousand nine hundred eighty‐nine patients were invited to participate in the study. Participants have been asked to rate three questions on… More >

  • Open Access


    Coronary artery intimal thickening and ventricular dynamics in pediatric heart transplant recipients

    Anita T. Cote1,2, Martin Hosking3, Christine Voss1,3, Derek G. Human3, George G. S. Sandor1,3, Kevin C. Harris1,3

    Congenital Heart Disease, Vol.13, No.5, pp. 663-670, 2018, DOI:10.1111/chd.12629

    Abstract Objective: Pediatric heart transplant recipients are at risk of posttransplant coronary artery disease known as cardiac allograft vasculopathy (CAV), and also may develop diastolic dysfunction. As CAV begins with a process of progressive intimal thickening, these occult diffuse changes may be detected using optical coherence tomography (OCT). We hypothesized that the development of CAV, as identified via OCT, may be a mechanism of declining ventricular function. Accordingly, the purpose of this study was to assess coronary artery intimal thickening and LV strain in children who have undergone heart transplantation.
    Methods: In 17 children, we analyzed OCT images for coronary intima… More >

  • Open Access


    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 < .001)… More >

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