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  • Open Access

    ARTICLE

    RSV prophylaxis guideline changes and outcomes in children with congenital heart disease

    Adam S. Walpert1, Ian D. Thomas2, Merlin C. Lowe Jr1, Michael D. Seckeler3

    Congenital Heart Disease, Vol.13, No.3, pp. 428-431, 2018, DOI:10.1111/chd.12590

    Abstract Objective: The aim of this study was to compare inpatient outcomes and costs for children with respiratory syncytial virus and congenital heart disease before and after the change in management guidelines for respiratory syncytial virus prophylaxis.
    Design: Hospital discharge data from the Vizient (formerly University HealthSystem Consortium) were queried from October 2012 to June 2014 (Era 1) and July 2014 to April 2016 (Era 2) for patients aged <24 months with an any International Classification of Disease (ICD)-9 or ICD-10 code for congenital heart disease (745-747.49, Q20.0-Q26.4) and a primary or secondary admitting diagnosis of respiratory syncytial virus infection (079.6,… More >

  • Open Access

    ARTICLE

    Utility of incomplete right bundle branch block as an isolated ECG finding in children undergoing initial cardiac evaluation

    Omar Meziab, Dominic J. Abrams, Mark E. Alexander, Laura Bevilacqua, Vassilios Bezzerides, Doug Y. Mah, Edward P. Walsh, John K. Triedman

    Congenital Heart Disease, Vol.13, No.3, pp. 419-427, 2018, DOI:10.1111/chd.12589

    Abstract Objective: This study evaluates the ability of experienced pediatric electrophysiologists (EPs) to reliably classify incomplete right bundle branch block (IRBBB) and assesses its clinical utility as an isolated ECG finding in a group of healthy outpatient children without prior cardiac evaluation.
    Design: We performed a retrospective analysis of all electrocardiographic and echocardiographic records at Boston Children’s Hospital between January 1, 2005, and December 31, 2014. Echocardiographic diagnoses were identified if registered between the date of the index electrocardiogram and the ensuing year. A selected subset of 473 ECGs was subsequently reanalyzed in a blinded manner by six pediatric EPs to… More >

  • Open Access

    ARTICLE

    Role of virtual reality in congenital heart disease

    Chin Siang Ong1, Aravind Krishnan1, Chen Yu Huang1, Philip Spevak2, Luca Vricella1, Narutoshi Hibino1, Juan R. Garcia2, Lasya Gaur3

    Congenital Heart Disease, Vol.13, No.3, pp. 357-361, 2018, DOI:10.1111/chd.12587

    Abstract Objective: New platforms for patient imaging present opportunities for improved surgical planning in complex congenital heart disease (CHD). Virtual reality (VR) allows for interactive manipulation of high-resolution representations of patient-specific imaging data, as a supplement to traditional 2D visualizations and 3D printed heart models.
    Design: We present the novel use of VR for the presurgical planning of cardiac surgery in two infants with complex CHD to demonstrate interactive real-time views of complex intra and extracardiac anatomy.
    Results: The use of VR for cardiac presurgical planning is feasible using existing imaging data. The software was evaluated by both pediatric cardiac surgeons… More >

  • Open Access

    ARTICLE

    Decreased inappropriate shocks with new generation ICDs in children and patients with congenital heart disease

    Stephanie A. Goldstein, Martin J. LaPage, Brynn E. Dechert, Gerald A. Serwer, Sunkyung Yu, Ray E. Lowery, David J. Bradley

    Congenital Heart Disease, Vol.13, No.3, pp. 413-418, 2018, DOI:10.1111/chd.12585

    Abstract Objective: Inappropriate implantable cardioverter defibrillator (ICD) shocks in children and patients with congenital heart disease (CHD) remain a major complication of device therapy, occurring in as many as 50% of children with ICDs. New generation devices include algorithms designed to minimize inappropriate shocks. This study aimed to evaluate the effect of new generation ICDs on the incidence of inappropriate shocks in the pediatric and CHD population.
    Design: Retrospective study of patients with CHD or under age 25 receiving ICDs between 2000 and 2015. New generation ICDs were defined as those with Medtronic “SmartShock” algorithms.
    Results: Two hundred eight devices were… More >

  • Open Access

    ARTICLE

    Clinical yield of fetal echocardiography for suboptimal cardiac visualization on obstetric ultrasound

    Rick D. Vavolizza1,2, Pe’er Dar3,4, Barrie Suskin3,4, Robert M. Moore4,5, Kenan W.D. Stern1,4

    Congenital Heart Disease, Vol.13, No.3, pp. 407-412, 2018, DOI:10.1111/chd.12584

    Abstract Objective: Suboptimal cardiac imaging on obstetric ultrasound is a frequent referral indication for fetal echocardiography, even in the absence of typical risk factors for fetal cardiac disease. The clinical profile of patients and findings of examinations performed for such an indication are not well defined. Given the increased cost, time and resource utilization of fetal echocardiography, we sought to determine the clinical findings of such referrals.
    Study Design: We performed a single-center review of such referrals from January 2010 to June 2016. Patients with commonly accepted indications for fetal echocardiography were excluded. Demographic variables and echocardiogram findings were collected. Findings… More >

  • Open Access

    ARTICLE

    A multinational observational investigation of illness perceptions and quality of life among patients with a Fontan circulation

    Christina E. Holbein1, Nicholas D. Fogleman1,2, Kevin Hommel1, Silke Apers3, Jessica Rassart3, Philip Moons3,4, Koen Luyckx3, Maayke A. Sluman5, Junko Enomoto6, Bengt Johansson7, Hsiao-Ling Yang8, Mikael Dellborg4,9, Raghavan Subramanyan10, Jamie L. Jackson11, Werner Budts3,12, Adrienne H. Kovacs13, Stacey Morrison1, Martha Tomlin1, Kathy Gosney1, Alexandra Soufi14, Katrine Eriksen15, Corina Thomet3,16, Malin Berghammer4,17, Luis Alday18, Edward Callus19, Susan M Fernandes20, Maryanne Caruana21, Samuel Menahem22, Stephen C. Cook23, Gwen R. Rempel24, Kamila White25, Paul Khairy26, Shelby Kutty27, Gruschen Veldtman1

    Congenital Heart Disease, Vol.13, No.3, pp. 392-400, 2018, DOI:10.1111/chd.12583

    Abstract Objective: First, to compare QOL and illness perceptions between patients with a Fontan circulation and patients with anatomically simple defects (ie, atrial septal defects [ASD] or ventricular septal defects [VSD]). Second, to explore illness perceptions as a mediator of the association between congenital heart disease (CHD) diagnosis and QOL.
    Design: Cross-sectional observational study.
    Setting: Twenty-four cardiology centers from 15 countries across five continents.
    Patients: Four hundred thirty-five adult patients with congenital heart disease (177 Fontan and 258 ASD/VSD) ages 18-83 years.
    Outcome Measures: QOL and illness perceptions were assessed by the Satisfaction With Life Scale and the Brief Illness Perceptions… More >

  • Open Access

    ARTICLE

    Insurance access in adults with congenital heart disease in the Affordable Care Act era

    Chien-Jung Lin1, Eric Novak1, Michael W. Rich1, Joseph J. Billadello1,2

    Congenital Heart Disease, Vol.13, No.3, pp. 384-391, 2018, DOI:10.1111/chd.12582

    Abstract Background: Adults with congenital heart disease (ACHD) have traditionally been viewed as an underinsured population. Whether this is true in the Affordable Care Act era is unknown. We determined insurance patterns in ACHD patients compared to the non-ACHD cardiology population in a contemporary cohort.
    Methods: All cardiology outpatient visits between July 2016 and February 2017 to a large referral center in the United States were reviewed. The primary payer was categorized as health maintenance organization (HMO), preferred provider organization (PPO), Medicare, Medicaid, self-pay, or other. Diagnosis and lesion severity of ACHD were extracted from ICD-10 diagnostic codes and assigned according… More >

  • Open Access

    ARTICLE

    Congenital heart disease patients’ and parents’ perception of disease-specific knowledge: Health and impairments in everyday life

    Paul C. Helm1, Sebastian Kempert2, Marc-André Körten1, Wiebke Lesch3, Katharina Specht3, Ulrike M. M. Bauer1,3

    Congenital Heart Disease, Vol.13, No.3, pp. 377-383, 2018, DOI:10.1111/chd.12581

    Abstract Background: Children and adolescents with congenital heart disease (CHD) and their families require qualified combined medical and psychosocial information, care, and counseling. This study aimed to analyze CHD patients’ and parents’ perception of disease-specific knowledge, state of health, and impairments experienced in everyday life, as well as factors influencing these perceptions.
    Materials and Methods: Analyses were based on a survey among patients/parents recruited via the German National Register for Congenital Heart Defects (NRCHD). The total sample (N = 818) was divided into four groups: “Children” (176 patients), “Adolescents” (142 patients), “Adults” (269 patients), and “Parents” (231 parents). The patients were… More >

  • Open Access

    ARTICLE

    Improving outpatient advance care planning for adults with congenital or pediatric heart disease followed in a pediatric heart failure and transplant clinic

    Lindsay A. Edwards1,2, Christine Bui2,3, Antonio G. Cabrera1,2, Jill Ann Jarrell2,4,5

    Congenital Heart Disease, Vol.13, No.3, pp. 362-368, 2018, DOI:10.1111/chd.12579

    Abstract Objective: To improve outpatient advanced care planning (ACP) for adults with congenital/pediatric heart disease followed in a pediatric heart failure (HF) and transplant clinic through quality improvement (QI) methodology.
    Design: A one-year QI project was completed. We conducted quarterly chart reviews and incorporated feedback from the providers to direct subsequent interventions.
    Patients and Setting: Patients ≥18 years of age seen in the HF and Transplant Clinic for followup visit were included in analysis.
    Interventions: Interventions focused on five main areas: identifying and training providers to have ACP discussions, standardizing the ACP discussion, standardizing ACP and advance directive (AD) documentation in… More >

  • Open Access

    ARTICLE

    Analysis of DICER1 in familial and sporadic cases of transposition of the great arteries

    Nelly Sabbaghian1, Maria C. Digilio2, Gillian M. Blue3,4, Timothée Revil5, David S. Winlaw3,4, William D. Foulkes1,6

    Congenital Heart Disease, Vol.13, No.3, pp. 401-406, 2018, DOI:10.1111/chd.12578

    Abstract Objective: We previously identified a pathogenic germline DICER1 variant in a child with transposition of the great arteries who was a member of a family with DICER1 syndrome. In view of a report linking DICER1 knockout in murine cardiomyocytes to cardiac outflow defects, we investigated the involvement of DICER1 in transposition of the great arteries.
    Design: We used Fluidigm access array followed by next generation sequencing to screen for variants in the coding exons, their exon/intron boundaries and the 30 untranslated region of DICER1 in patient DNA.
    Cases: Germline DNA was collected from 129 patients with either sporadic or familial… More >

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