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  • Open Access

    ARTICLE

    Aortic perfusion score for pulmonary atresia with intact ventricular septum: An antegrade coronary perfusion scoring system that is predictive of need for transplant and mortality

    Rohit S. Loomba1,2, Andrew N. Pelech1,2

    Congenital Heart Disease, Vol.13, No.1, pp. 92-97, 2018, DOI:10.1111/chd.12510

    Abstract Background: Pulmonary atresia with intact ventricular septum is a unique congenital malformation of the heart in which patients may undergo single-, two- or 1½- ventricle repair. Size of the tricuspid valve annulus, morphology of the right ventricle and presence of ventriculocoronary connections can all impact the selection of appropriate palliative strategy. We developed the aortic perfusion score, a novel scoring system based on anterograde coronary perfusion with the aim of being able to identify patients at risk for death or transplant.
    Methods: A retrospective study was conducted. Patients were included if an initial catheterization was done prior to any intervention.… More >

  • Open Access

    EDITORIAL

    A fond farewell

    Douglas S. Moodie

    Congenital Heart Disease, Vol.14, No.6, pp. 884-884, 2019, DOI:10.1111/chd.12865

    Abstract This article has no abstract. More >

  • Open Access

    ARTICLE

    The incidence of arrhythmias during exercise stress tests among children with Kawasaki disease: A single-center case series

    Varun Aggarwal1,2, Kristen Sexson-Tejtal1, Wilson Lam1, Santiago O. Valdes1, Caridad M. de la Uz1, Jeffrey J. Kim1, Christina Y. Miyake1

    Congenital Heart Disease, Vol.14, No.6, pp. 1032-1036, 2019, DOI:10.1111/chd.12864

    Abstract Objective: Based on 2017 guidelines, participation in competitive sports with prior history of Kawasaki Disease (KD) requires those with coronary artery aneurysms (CAA) z score ≥ 5 to undergo evaluation for evidence of inducible ischemia or arrhythmias. The use of exercise stress testing (EST) to evaluate arrhythmias among KD patients has never been reported. This retrospective single-center case series study sought to describe the presence of inducible arrhythmias during EST in KD patients with or without CAA.
    Methods: Single-center retrospective review of medical records of patients diagnosed with KD between 1989-2015 at Texas Children’s Hospital, Houston, Texas who underwent EST… More >

  • Open Access

    ARTICLE

    Infundibular sparing versus transinfundibular approach to the repair of tetralogy of Fallot

    Mary K. Olive1, Charles D. Fraser2, Shelby Kutty3, Emmett D. McKenzie4, James M. Hammel5, Rajesh Krishnamurthy6, Nicolas A. Dodd7, Shiraz A. Maskatia8

    Congenital Heart Disease, Vol.14, No.6, pp. 1149-1156, 2019, DOI:10.1111/chd.12863

    Abstract Introduction: The right ventricular infundibular sparing approach (RVIS) to the repair of tetralogy of Fallot (TOF) avoids a full-thickness ventricular incision, typically utilized in the transinfundibular (TI) method.
    Methods: We performed a retrospective, age-matched cohort study of patients who underwent RVIS at Texas Children’s Hospital or TI at Children’s Hospital Medical Center in Nebraska and subsequently underwent cardiac magnetic resonance imaging (CMR). We compared right ventricular end-diastolic and systolic volumes indexed to body surface area (RVEDVi and RVESVi) and right ventricular ejection fraction (RVEF) as primary endpoints. Secondary endpoints were indexed left ventricular diastolic and systolic volume (LVEDVi and LVESVi),… More >

  • Open Access

    ARTICLE

    Baseline tubular biomarkers in young adults with congenital heart disease as compared to healthy young adults: Detecting subclinical kidney injury

    Dana Y. Fuhrman1,2, Lan Nguyen3, Morgan Hindes3, John A. Kellum2

    Congenital Heart Disease, Vol.14, No.6, pp. 963-967, 2019, DOI:10.1111/chd.12862

    Abstract Background: There are significant implications for kidney disease in young adults with congenital heart disease. Prior investigations have not focused on the use of urinary tubular biomarkers for the early identification of kidney disease in this growing patient group.
    Objective: Determine if young adults with congenital heart disease have differences in the baseline concentration of urinary tubular biomarkers when compared to healthy young adults.
    Design/Methods: In a pilot case control study, 30 patients from 18 to 35 years of age with congenital heart disease and a normal serum creatinine were recruited during a routine follow-up visit. In the same age… More >

  • Open Access

    ARTICLE

    Echocardiography vs cardiac magnetic resonance imaging assessment of the systemic right ventricle for patients with d-transposition of the great arteries status post atrial switch

    Margaret M. Samyn1,2, Ke Yan1, Conor Masterson3, Benjamin H. Goot1,2, David Saudek1,2, Julie Lavoie2, Aaron Kinney2, Mary Krolikowski1, Kan Hor4,5, Scott Cohen1,2

    Congenital Heart Disease, Vol.14, No.6, pp. 1138-1148, 2019, DOI:10.1111/chd.12861

    Abstract Objective: Patients with Dextro-transposition of the great arteries status post atrial switch (dTGA s/p atrial switch) are “at-risk” for systemic right ventricular (RV) dysfunction. Due to complex RV geometry, echocardiography (Echo) does not allow accurate determination of ejection fraction (EF), but cardiac magnetic resonance imaging (CMR) allows quantitative right ventricular assessment. Measures of ventricular deformation may be precursors to global ventricular dysfunction. The primary aim of this study was to characterize imaging and clinical findings for adult patients with dTGA s/p atrial switch.
    Design: This was a retrospective cohort study of patients with dTGA s/p atrial switch operation (February 1966… More >

  • Open Access

    ARTICLE

    Pulmonary artery pulsatility index predicts prolonged inotrope/ pulmonary vasodilator use after implantation of continuous flow left ventricular assist device

    Varun Aggarwal1,2, Sebastian C. Tume3, Marco Rodriguez1, Iki Adachi4, Antonio G. Cabrera1, Hari Tunuguntla1, Athar M. Qureshi1

    Congenital Heart Disease, Vol.14, No.6, pp. 1130-1137, 2019, DOI:10.1111/chd.12860

    Abstract Objective: Predictors of right ventricle (RV) dysfunction after continuous‐flow left ventricular assist device (CF‐LVAD) implantation in children are not well described. We explored the association of preimplantation Pulmonary Artery Pulsatility index (PAPi) and other hemodynamic parameters as predictors of prolonged postoperative inotropes/pulmonary vasodilator use after CF‐LVAD implantation.
    Design: Retrospective chart review.
    Setting: Single tertiary care pediatric referral center.
    Patients: Patients who underwent CF‐LVAD implantation from January 2012 to October 2017.
    Interventions: Preimplantation invasive hemodynamic parameters were analyzed to evaluate the association with post‐CF‐LVAD need for prolonged (>72 hours) use of inotropes/pulmonary vasodilators.
    Measurements and main results: Preimplantation cardiac catheterization data… More >

  • Open Access

    ARTICLE

    Concomitant pulmonary vein isolation and percutaneous closure of atrial septal defects: A pilot project

    Reinder Evertz1, Charlotte A. Houck2, Tim ten Cate1, Anthonie L. Duijnhouwer1, Rypko Beukema1, Sjoerd Westra1, Kevin Vernooy 1,3, Natasja M. S. de Groot2

    Congenital Heart Disease, Vol.14, No.6, pp. 1123-1129, 2019, DOI:10.1111/chd.12859

    Abstract Background: Patients with an atrial septal defect (ASD) are at increased risk of de‐ veloping atrial fibrillation (AF). Currently percutaneous ASD closure is the preferred therapeutic strategy and although pulmonary vein isolation (PVI) for AF is feasible after ASD closure, the transseptal puncture can be technically challenging and prob‐ ably increases the perioperative risk. A staged approach, with PVI several months be‐ fore ASD closure, has been recommended for patients already scheduled for closure, but no data are available on combined procedures.
    Purpose: This pilot study evaluates the feasibility of a combined procedure of PVI and ASD closure in patients… More >

  • Open Access

    ARTICLE

    Parenting stress trajectories during infancy in infants with congenital heart disease: Comparison of single‐ventricle and biventricular heart physiology

    Nadya Golfenshtein1, Alexandra L. Hanlon2, Janet A. Deatrick3, Barbara Medoff‐Cooper3,4

    Congenital Heart Disease, Vol.14, No.6, pp. 1113-1122, 2019, DOI:10.1111/chd.12858

    Abstract Objective: Parents of infants with congenital heart disease (CHD) experience increased parenting stress levels, potentially interfering with parenting practices and bear adverse family outcomes. Condition severity has been linked to parenting stress. The current study aimed to explore parenting stress trajectories over infancy in parents of infants with complex CHD, and to compare them by post‐operative cardiac physiology.
    Design: Data from a larger prospective cohort study was analyzed using longitudinal mixed‐effects regression modeling.
    Setting: Cardiac intensive care unit and outpatient clinic of a 480‐bed children's hospital in the American North‐Atlantic region.
    Participants: Parents of infants with complex CHD (n =… More >

  • 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 >

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