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  • Open Access


    Strength in numbers: Crowdsourcing the most relevant literature in pediatric cardiology

    Joseph J. Knadler1, Daniel J. Penny1, Tyler H. Harris2, Gary D. Webb3, Antonio G. Cabrera1,4, William B. Kyle1

    Congenital Heart Disease, Vol.13, No.5, pp. 794-798, 2018, DOI:10.1111/chd.12669

    Abstract Objective: The growing body of medical literature in pediatric cardiology has made it increasingly difficult for individual providers to stay abreast of the most current, meaningful articles to help guide practice. Crowdsourcing represents a collaborative process of obtaining information from a large group of individuals, typically from an online or web‐based community, and could serve a potential mechanism to pool indi‐ vidual efforts to combat this issue. This study aimed to utilize crowdsourcing as a novel way to generate a list of the most relevant, current publications in congenital heart disease, utilizing input from an international group of professionals in… More >

  • Open Access


    Hemodynamic effects of ketamine in children with congenital heart disease and/or pulmonary hypertension

    Rohit S. Loomba1, Seth B. Gray2, Saul Flores3

    Congenital Heart Disease, Vol.13, No.5, pp. 646-654, 2018, DOI:10.1111/chd.12662

    Abstract Introduction: Ketamine is a drug often used for procedural sedation or as adjunct agent for general sedation in children with congenital heart disease. In the clinical realm, there is often confusion regarding the effects of ketamine on hemodynamics, particularly pulmonary vascular resistance and systemic vascular resistance. We per‐ formed a meta‐analysis of studies investigating the effects of ketamine on hemodynamics.
    Methods: A systematic review was conducted to identify studies characterizing the hemodynamic effects of ketamine in children with congenital heart disease. Studies were assessed for quality and those of satisfactory quality with pre‐ and postketa‐ mine hemodynamics for each patient… More >

  • Open Access


    Congenital coronary artery fistula: Presentation in the neonatal period and transcatheter closure

    Varun Aggarwal, Venkatachalam Mulukutla, Athar M. Qureshi, Henri Justino

    Congenital Heart Disease, Vol.13, No.5, pp. 782-787, 2018, DOI:10.1111/chd.12653

    Abstract Background: Congenital coronary artery fistula is a rare coronary anomaly. Most commonly, such fistulae drain into the right side of the heart or the pulmonary artery. Children with coronary artery fistulae are generally asymptomatic, although they may have left ventricular enlargement in the setting of a moderate sized left to right shunt. Symptoms of congestive heart failure or ischemia are very rare in neonatal period, and suggest the presence of a very large shunt and/or coronary steal.
    Methods: Single center retrospective review of transcatheter intervention on coro‐ nary artery fistulae presenting with symptoms in the neonatal period from January 2000… More >

  • Open Access


    Incidence and factors influencing the spontaneous closure of Fontan fenestration

    Sudheer R. Gorla1, Nataley K. Jhingoeri1, Abhishek Chakraborty1, Kishore R. Raja1, Ashish Garg1, Satinder Sandhu1, Eliot R. Rosenkranz2, Sethuraman Swaminathan1

    Congenital Heart Disease, Vol.13, No.5, pp. 776-781, 2018, DOI:10.1111/chd.12652

    Abstract Introduction: The Fontan operation is the final stage of single ventricle palliation in patients with complex congenital heart disease. Fenestration in the Fontan conduit, providing an atrial level right to left shunt, has been shown to reduce early postoperative morbidity. However, there is limited data on the long‐term fate of this fenestration. The aim of this study is to define the rate of spontaneous closure of the fenestration in the Fontan conduit and factors predictive of the fate of the fenestration.
    Methods: This was a retrospective study reviewing the medical records of the patients who underwent fenestrated Fontan operation at… More >

  • Open Access


    Heart murmurs and echocardiography findings in the normal newborn nursery

    Michael E. Fenster1‡, John S. Hokanson2

    Congenital Heart Disease, Vol.13, No.5, pp. 771-775, 2018, DOI:10.1111/chd.12651

    Abstract Objective: To determine the clinical findings and management implications of echocardiograms performed on infants with murmurs in the nursery.
    Design: Retrospective cohort study conducted from January 2008 through December 2015. Patients in the study were followed by chart review for up to 5 years. In addition, a survey of nursery providers was conducted in February 2016.
    Setting: A single community hospital associated with a university.
    Patients: All 26 573 infants who received care in the normal newborn nursery were eligible for inclusion in the study. Infants with echocardiograms were analyzed. The survey was sent by e‐mail to all 135 physicians… More >

  • Open Access


    FibroSURE and elastography poorly predict the severity of liver fibrosis in Fontan-associated liver disease

    Jessica L. Schachter1, Manisha Patel2, Samuel R. Horton3, A. Mike Devane4, Alex Ewing5, Gary A. Abrams6

    Congenital Heart Disease, Vol.13, No.5, pp. 764-770, 2018, DOI:10.1111/chd.12650

    Abstract Objective: As the population of patients with Fontan circulation surviving into adult‐ hood increases, hepatic cirrhosis has grown to be a significant cause of morbidity and mortality. Early detection of advanced hepatic fibrosis is imperative for proper inter‐ vention and consideration for heart or combined heart/liver transplantation. Noninvasive biomarkers and elastography have been evaluated for their diagnostic utility with variable results in the Fontan population.
    Design: The cohort included 14 patients age 26.4 SD 7.5 who underwent Fontan surgery. All patients were evaluated with FibroSURE, shear wave elastography (SWE), hepatic duplex sonography, and liver biopsy. Liver fibrosis on biopsy was… More >

  • Open Access


    Interstage outcomes in single ventricle patients undergoing hybrid stage 1 palliation

    Janet M. Simsic, Christina Phelps, Kristin Kirchner, Kirby‐Rose Carpenito, Robin Allen, Holly Miller‐Tate, Karen Texter, Mark Galantowicz

    Congenital Heart Disease, Vol.13, No.5, pp. 757-763, 2018, DOI:10.1111/chd.12649

    Abstract Objective: Interstage readmissions are common in infants with single ventricle congenital heart disease undergoing staged surgical palliation. We retrospectively examined readmissions during the interstage period.
    Design: Retrospective analysis.
    Setting: The Heart Center at Nationwide Children’s Hospital, Columbus, Ohio.
    Patients: Newborns undergoing hybrid stage 1 palliation from January 2012 to December 2016 who survived to hospital discharge and were followed at our institution.
    Interventions: All patients underwent hybrid stage 1 palliation.
    Outcome Measures: Outcomes included (1) reason for interstage readmission; (2) feeding modality during interstage period; (3) major interstage adverse events; and (4) interstage mortality.
    Results: Study group comprised 57 patients.… More >

  • Open Access


    Prenatal detection of critical cardiac outflow tract anomalies remains suboptimal despite revised obstetrical imaging guidelines

    Heather Y. Sun1, James A. Proudfoot2, Rachel T. McCandless1

    Congenital Heart Disease, Vol.13, No.5, pp. 748-756, 2018, DOI:10.1111/chd.12648

    Abstract Background: Fetal echocardiography can accurately diagnose critical congenital heart disease prenatally, but relies on referrals from abnormalities identified on routine obstetrical ultrasounds. Critical congenital heart disease that is frequently missed due to inadequate outflow tract imaging includes anomalies such as truncus arteriosus, double outlet right ventricle, transposition of the great arteries, tetralogy of Fallot, pulmonary stenosis, and aortic stenosis.
    Objective: This study evaluated the prenatal detection rate of critical outflow tract anomalies in a single urban pediatric hospital before and after “AIUM Practice Guideline for the Performance of Obstetric Ultrasound Examinations,” which incorporated outflow tract imaging.
    Design: Infants with outflow… More >

  • Open Access


    Narrative analysis of adults with complex congenital heart disease: Childhood experiences and their lifelong reverberations

    Michelle Keir1, Barbara Bailey1, Angela Lee1, Adrienne H. Kovacs1,2, S. Lucy Roche1,3

    Congenital Heart Disease, Vol.13, No.5, pp. 740-747, 2018, DOI:10.1111/chd.12647

    Abstract Background: With access to surgical care, >90% of today’s infants with congenital heart disease (CHD) will reach adulthood. During childhood, survivors accrue a wealth of health care experience and develop strategies for navigating life with a chronic disease.
    Methods: Seeking to learn from this individualized process, we invited adults with complex CHD to participate in narrative analysis—an established qualitative research method for studying how individuals derive meaning from their personal stories. Audio recordings of 2‐4 hour free‐form interviews were transcribed and iteratively analyzed to identify common themes and detect similarities or differences in language, viewpoint and interpretation. Recruitment continued until… More >

  • Open Access


    Identifying self‐reported neurocognitive deficits in the adult with congenital heart disease using a simple screening tool

    Ashley Brunmeier1,2, Michael P Reis3, Michael G. Earing1,2, Laura Umfleet 4, Salil Ginde1,2, Peter J. Bartz1,2, Scott Cohen1,2

    Congenital Heart Disease, Vol.13, No.5, pp. 728-733, 2018, DOI:10.1111/chd.12646

    Abstract Objective: Children with congenital heart disease (CHD) and adults with acquired heart disease are at an increased risk of neurocognitive impairment. The objective of this study was to determine the prevalence of self‐reported neurocognitive impair‐ ment and its risk factors in the adult congenital heart disease (ACHD) population.
    Design: The Wisconsin Adult Congenital Heart Disease Program recently began screening ACHD patients to identify those with significant self‐perceived neurocog‐ nitive impairments. Screening consists of using a validated neuro‐oncology screening instrument that has been modified for the ACHD population. Patients who answer this survey in a predetermined fashion consistent with significant self‐perceived… More >

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