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  • Open Access


    Evaluation of systemic microvascular reactivity in adults with congenital heart disease

    Pablo Marino1,2, Gabriella de Oliveira Lopes1,3, Juliana Pereira Borges3, Maria Carolina Terra Cola1, Daniel Arkader Kopiler1, Eduardo Tibirica1

    Congenital Heart Disease, Vol.13, No.6, pp. 978-987, 2018, DOI:10.1111/chd.12660

    Abstract Objective: Adults with congenital heart disease share some features with those with chronic heart failure. Although microvascular endothelial dysfunction has been de‐ scribed in chronic heart failure, evaluation of the microcirculation in adults with con‐ genital heart disease is lacking. The present study aimed to investigate systemic microvascular reactivity in adults with congenital heart disease.
    Interventions: The patients initially underwent cardiopulmonary exercise testing. Then, the cutaneous microvascular reactivity was evaluated in these patients using a laser speckle contrast imaging system coupled with skin iontophoresis of endothelial‐de‐ pendent (acetylcholine) or ‐independent (sodium nitroprusside) vasodilators and pos‐ tocclusive reactive hyperemia (PORH) and… More >

  • Open Access


    Platelet parameters in children with chromosome 22q11 deletion and conotruncal heart defects

    Alka Anilkumar1, D. M. Vasudevan2, Mahesh Kappanayil3, K. R. Sundaram4, R. Krishna Kumar3, Sheela Nampoothiri5

    Congenital Heart Disease, Vol.13, No.3, pp. 483-487, 2018, DOI:10.1111/chd.12600

    Abstract Background and Objectives: The 22q11 deletion syndrome is associated with a wide spectrum of phenotypic features, hence clinical diagnosis is difficult. Individuals with this syndrome are found to have a risk of developing transfusion associated graft-versus-host reaction, if they are given nonirradiated blood. Our aim was to elucidate whether chromosome 22q11 deletion in children with syndromic conotruncal heart defects is associated with platelet abnormality.
    Materials and Methods: The genetic analysis was performed by standard cytogenetic and Fluorescence in situ hybridization technique. The platelet parameters in 39 patients with chromosome 22q11 deletion were compared with 154 cases without deletion.
    Results: In… More >

  • Open Access


    Association between maternal body mass index and congenital heart defects in infants: A meta-analysis

    Yu Zhu1*, Yong Chen1*, Yu Feng2, Di Yu1, Xuming Mo1

    Congenital Heart Disease, Vol.13, No.2, pp. 271-281, 2018, DOI:10.1111/chd.12567

    Abstract We conducted this meta-analysis to address the open question of a possible association between maternal body mass index (BMI) and congenital heart defects (CHDs) in infants. We conducted a comprehensive computerized search of PubMed, Web of Science, Medline, and Embase databased (January 1980 through August 2017). We assessed the association between maternal BMI and the risk for congenital heart defects in their offspring. Study-specific relative risk estimates were polled according to random-effect or fixed-effect models. From 2567 citations, a total of 13 case-control studies and 4 cohort studies were selected for a meta-analysis, including more than 1 150 000 cases.… More >

  • Open Access


    Screening performance of congenital heart defects in first trimester using simple cardiac scan, nuchal translucency, abnormal ductus venosus blood flow and tricuspid regurgitation

    Natasa Karadzov Orlic1, Amira Egic1, Barbara Damnjanovic‐Pazin MD2 | Relja Lukic1, Ivana Joksic3, Zeljko Mikovic1

    Congenital Heart Disease, Vol.14, No.6, pp. 1094-1101, 2019, DOI:10.1111/chd.12852

    Abstract Objective: The objective of this study was to analyze if the addition of simple cardiac scan in cases with increased nuchal translucency (NT) and/or abnormal ductus venosus (DV) blood flow, and/or tricuspid regurgitation (TCR) can improve detection of congenital heart defects (CHD) in chromosomally normal fetuses without non‐ cardiac defects at 11‐13 + 6 gestational weeks in a population of singleton pregnancies.
    Methods: During the 10 years period, all singleton pregnancies at 11‐13 + 6 weeks were routinely scanned for NT, DV blood flow and TCR assessment and, if a sin‐ gle of these parameters was abnormal, simple cardiac scan… More >

  • Open Access


    Utilization of inhaled nitric oxide after surgical repair of truncus arteriosus: A multicenter analysis

    Christine M. Riley1, Christopher W. Mastropietro2, Peter Sassalos3, Jason R. Buckley4, John M. Costello4, Ilias Iliopoulos5, Aimee Jennings6, Katherine Cashen7, Sukumar Suguna Narasimhulu8, Keshava M. N. Gowda9, Arthur J. Smerling10, Michael Wilhelm11, Aditya Badheka12, Adnan Bakar13,14, Elizabeth A. S. Moser15, Venu Amula16

    Congenital Heart Disease, Vol.14, No.6, pp. 1078-1086, 2019, DOI:10.1111/chd.12849

    Abstract Background: Elevated pulmonary vascular resistance (PVR) is common following repair of truncus arteriosus. Inhaled nitric oxide (iNO) is an effective yet costly therapy that is frequently implemented postoperatively to manage elevated PVR.
    Objectives: We aimed to describe practice patterns of iNO use in a multicenter cohort of patients who underwent repair of truncus arteriosus, a lesion in which recovery is often complicated by elevated PVR. We also sought to identify patient and center factors that were more commonly associated with the use of iNO in the postoperative period.
    Design: Retrospective cohort study.
    Setting: 15 tertiary care pediatric referral centers.
    Patients:More >

  • Open Access


    Three‐dimensional rotational angiography in congenital heart disease: Present status and evolving future

    Sok‐Leng Kang1, Aimee Armstrong2, Gregor Krings3, Lee Benson1

    Congenital Heart Disease, Vol.14, No.6, pp. 1046-1057, 2019, DOI:10.1111/chd.12838

    Abstract Three‐dimensional rotational angiography (3D‐RA) enables volumetric imaging through rotation of the C‐arm of an angiographic system and real‐time 3D recon‐ struction during cardiac catheterization procedures. In the field of congenital heart disease (CHD), 3D‐RA has gained considerable traction, owing to its capability for en‐ hanced visualization of spatial relationships in complex cardiac morphologies and real time image guidance in an intricate interventional environment. This review provides an overview of the current applications, strengths, and limitations of 3D‐RA acquisi‐ tion in the management of CHD and potential future directions. In addition, issues of dosimetry, radiation exposure, and optimization strategies will be… More >

  • Open Access


    Postoperative and long‐term outcomes in children with Trisomy 21 and single ventricle palliation

    Jennifer K. Peterson1, Shaun P. Setty1,2, Jessica H. Knight3, Amanda S. Thomas4, James H. Moller5, Lazaros K. Kochilas4,6

    Congenital Heart Disease, Vol.14, No.5, pp. 854-863, 2019, DOI:10.1111/chd.12823

    Abstract Objective: Patients with Trisomy 21 (T21) and single ventricle (SV) physiology present unique challenges compared to euploidic counterparts. This study reports postoperative and long‐term outcomes in patients with T21 and SV palliation.
    Design: This retrospective cohort study from the Pediatric Cardiac Care Consortium (PCCC) included patients with T21 (<21 years old) that underwent surgical palliation for SV between 1982 and 2008 and control patients without known genetic anom‐ aly following Fontan palliation for similar diagnoses. Kaplan‐Meier survival plots were created based on death events obtained from the PCCC and by linkage with the National Death Index (NDI) and the Organ… More >

  • Open Access


    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


    Use of 3D models of congenital heart disease as an education tool for cardiac nurses

    Giovanni Biglino1,2, Claudio Capelli2,3, Despina Koniordou3, Di Robertshaw2, Lindsay-Kay Leaver2, Silvia Schievano2,3, Andrew M. Taylor2,3, Jo Wray2

    Congenital Heart Disease, Vol.12, No.1, pp. 113-118, 2017

    Abstract Background: Nurse education and training are key to providing congenital heart disease (CHD) patients with consistent high standards of care as well as enabling career progression. One approach for improving educational experience is the use of 3D patient-specific models.
    Objectives: To gather pilot data to assess the feasibility of using 3D models of CHD during a training course for cardiac nurses; to evaluate the potential of 3D models in this context, from the nurses’ perspective; and to identify possible improvements to optimise their use for teaching.
    Design: A cross-sectional survey.
    Setting: A national training week for cardiac nurses.
    Participants: One… More >

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