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  • Open Access

    ARTICLE

    Atrial fibrillation in adults with congenital heart disease following cardiac surgery in a single center: Analysis of incidence and risk factors

    Michael A. Brock, John‐Anthony Coppola, Jana Reid, Diego Moguillansky

    Congenital Heart Disease, Vol.14, No.6, pp. 924-930, 2019, DOI:10.1111/chd.12857

    Abstract Objective: The primary aim of our work is to determine the incidence of atrial fibrillation following cardiac surgery in adults with congenital heart disease. Secondary aims include identifying risk factors predictive of developing early postoperative atrial fibrillation and morbidities associated with early postoperative atrial fibrillation.
    Design: Retrospective analysis.
    Setting: Single center, quaternary care children’s hospital.
    Patients: This review included patients at least 18 years of age with known congenital heart disease who underwent cardiac surgery requiring a median sternotomy at our congenital heart center from January 1, 2012 to December 31, 2016.
    Interventions: None.
    Outcome Measures: The primary outcome was… More >

  • Open Access

    ARTICLE

    Outcomes in adults with congenital heart disease and heterotaxy syndrome: A single‐center experience

    Christopher R. Broda, Katherine B. Salciccioli, Keila N. Lopez, Peter R. Ermis, Douglas S. Moodie, Heather A. Dickerson

    Congenital Heart Disease, Vol.14, No.6, pp. 885-894, 2019, DOI:10.1111/chd.12856

    Abstract Background: Heterotaxy syndrome (HS) is a condition in which the thoracoabdominal organs demonstrate an abnormal lateral arrangement and is often associated with con‐ genital heart disease (CHD). Little is known about the adult HS population with CHD.
    Objective: To describe the outcomes and sociodemographics of the adult CHD popu‐ lation with HS.
    Methods: Records of patients 18 years of age or older with diagnoses of both CHD and HS at Texas Children's Hospital from 1964 to 2018 were reviewed.
    Results: Sixty‐two patients metinclusion criteria.Median agewas 22.7 [IQR19.6‐30.0] years; 26 (42%) were female; and 13 (21%) of patients had a gap… More >

  • Open Access

    ARTICLE

    Surveillance and screening practices of New England congenital cardiologists for patients after the Fontan operation

    Brooke T. Davey1, Olga H. Toro‐Salazar1, Naomi Gauthier2,3, Anne Marie Valente2, Robert W. Elder4, Fred M. Wu2, Norman Berman3, Phyllis Pollack5, Ji Hyun Lee1, Rahul H. Rathod2

    Congenital Heart Disease, Vol.14, No.6, pp. 1013-1023, 2019, DOI:10.1111/chd.12854

    Abstract Introduction: Surveillance and management guidelines for Fontan patients are lacking due to the paucity of evidence in the literature of screening efficacy on outcome measures.
    Methods: The Fontan Working Group within the New England Congenital Cardiology Association designed an electronic survey to assess surveillance practices for pa‐ tients with Fontan procedures among New England congenital cardiologists and to explore variability in screening low‐risk vs high‐risk Fontan patients across regional programs.
    Results: Fifty‐six cardiologists representing 12 regional programs responded to the survey, comprising ~40% of the total New England congenital cardiac physicians. The majority of desired testing and consultation was available… More >

  • Open Access

    ARTICLE

    Kidney injury biomarkers after cardiac angiography in children with congenital heart disease

    Masashi Nishida, Shingo Kubo, Yuma Morishita, Kosuke Nishikawa, Kazuyuki Ikeda, Toshiyuki Itoi, Hajime Hosoi

    Congenital Heart Disease, Vol.14, No.6, pp. 1087-1093, 2019, DOI:10.1111/chd.12853

    Abstract Objective: This study aims to investigate the changes in renal function and levels of urinary biomarkers before and after cardiac angiography in children with congenital heart disease (CHD).
    Setting: Children with CHD are at a risk for kidney injury during contrast exposure in cardiac angiography.
    Outcome Measures: We measured urinary protein, albumin, N‐acetyl‐β‐D‐glucosaminidase (NAG), β2‐microglobulin (BMG), and liver‐type fatty acid‐binding protein (L‐FABP) levels, as well as serum creatinine and cystatin C levels, before and after cardiac angiography in 33 children with CHD.
    Results: No significant decrease was noted in either the creatinine‐based or cystatin C‐based estimated glomerular filtration rate at… More >

  • Open Access

    ARTICLE

    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

    ARTICLE

    Impact of durable ventricular assist devices on post‐transplant outcomes in adults with congenital heart disease

    Ari Cedars1, Luke Burchill2, S. Lucy Roche3, Jonathan Menachem4, Kelly Axsom5, Kristen Tecson6

    Congenital Heart Disease, Vol.14, No.6, pp. 958-962, 2019, DOI:10.1111/chd.12851

    Abstract Background: There are no published data on post‐transplant outcomes in durable ven‐ tricular assist device (VAD)‐supported adult congenital heart disease (ACHD) patients.
    Methods: We compared post‐transplant outcomes in VAD‐supported vs non‐VAD‐ supported ACHD patients using the Scientific Registry of Transplant Recipients.
    Results: At 1 year, there was no difference in post‐transplant mortality between VAD‐supported (12 patients) and non‐VAD‐supported (671 patients) ACHD patients.
    Conclusions: In appropriate ACHD patients, VAD use as a bridge to transplant is a reasonable strategy. More >

  • Open Access

    ARTICLE

    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

    ARTICLE

    Prolonged Tpeak‐Tend interval is a risk factor for sudden cardiac death in adults with congenital heart disease

    Jim T. Vehmeijer1, Zeliha Koyak1, A. Suzanne Vink1, Werner Budts2,3, Louise Harris4, Candice K. Silversides4, Erwin N. Oechslin4, Aeilko H. Zwinderman5, Barbara J.M. Mulder1,6, Joris R. de Groot1

    Congenital Heart Disease, Vol.14, No.6, pp. 952-957, 2019, DOI:10.1111/chd.12847

    Abstract Objective: Adult congenital heart disease (ACHD) patients are at risk of sudden cardiac death (SCD). However, methods for risk stratification are not yet well‐ defined. The Tpeak‐Tend (TpTe) interval, a measure of dispersion of ventricular repolari‐ zation, is a risk factor for SCD in non‐ACHD patients. We aim to evaluate whether TpTe can be used in risk stratification for SCD in ACHD patients.
    Design: From an international multicenter cohort of 25 790 ACHD patients, we iden‐ tified all SCD cases. Cases were matched to controls by age, gender, congenital de‐ fect, and (surgical) intervention.
    Outcome Measures: TpTe was measured on… More >

  • Open Access

    ARTICLE

    Risk stratification models for congenital heart surgery in children: Comparative single‐center study

    Sara Bobillo‐Perez1,2, Joan Sanchez‐de‐Toledo3,4, Susana Segura2, Monica Girona‐Alarcon2, Maria Mele5, Anna Sole‐Ribalta2, Debora Cañizo Vazquez6, Iolanda Jordan2,7, Francisco Jose Cambra1,2

    Congenital Heart Disease, Vol.14, No.6, pp. 1066-1077, 2019, DOI:10.1111/chd.12846

    Abstract Objective: Three scores have been proposed to stratify the risk of mortality for each cardiac surgical procedure: The RACHS‐1, the Aristotle Basic Complexity (ABC), and the STS‐EACTS complexity scoring model. The aim was to compare the ability to predict mortality and morbidity of the three scores applied to a specific population.
    Design: Retrospective, descriptive study.
    Setting: Pediatric and neonatal intensive care units in a referral hospital.
    Patients: Children under 18 years admitted to the intensive care unit after surgery.
    Interventions: None.
    Outcome measures: Demographic, clinical, and surgical data were assessed. Morbidity was considered as prolonged length of stay (LOS >… More >

  • Open Access

    ARTICLE

    Metabolic syndrome in adults with congenital heart disease and increased intima‐media thickness

    Anna‐Luisa Häcker1,2, Renate Oberhoffer1,2, Alfred Hager1, Peter Ewert1, Jan Müller1,2

    Congenital Heart Disease, Vol.14, No.6, pp. 945-951, 2019, DOI:10.1111/chd.12845

    Abstract Aims: Age‐related cardiovascular diseases are a relevant risk in the aging population of adults with congenital heart diseases (ACHD). Risk factors such as the metabolic syndrome (MetS) impact the risk of increased carotid intima‐media thickness (cIMT) and thereby long‐term cardiovascular diseases. The aim of the study was to assess MetS in ACHD and outline a possible association to cIMT.
    Methods and Results: In total, 512 ACHD (43.0 ± 9.6 years, 48.9% female) were screened for MetS by the standards of the International Diabetes Federation, and their cIMT by ultrasound from January 2017 to June 2019. MetS was prevalent in 72… More >

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