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  • Open Access

    ARTICLE

    Screening for thoracic aortic pathology: Clinical practice in a single tertiary center

    Lidia R. Bons1, Lucas Uchoa de Assis1, Silvy Dekker1, Robert M. Kauling1, Judith A.A.E. Cuypers1, Hence J.M. Verhagen2, Ricardo P.J. Budde3, Jolien W. Roos‐Hesselink1

    Congenital Heart Disease, Vol.13, No.6, pp. 988-996, 2018, DOI:10.1111/chd.12663

    Abstract Objective: The aim is to present current clinical practice of thoracic aorta screening in a tertiary referral center. We identified how often imaging techniques were used for screening and established the value of transthoracic echocardiography (TTE) in com‐ parison with computed tomography (CT) to detect aortic dilation. We also investigated which additional abnormalities of the heart, aorta or smaller arteries were discovered.
    Design: All patients ≥15 years who visited our tertiary center in 2012‐2016 for first thoracic aortic screening were retrospectively included. Diameters of the sinus of Valsalva (SoV) and maximum ascending aorta (AA) were compared between… More >

  • Open Access

    ARTICLE

    Achieving biventricular circulation in patients with moderate hypoplastic right ventricle in pulmonary atresia intact ventricular septum after transcatheter pulmonary valve perforation

    Robin H.S. Chen1, Adolphus K.T. Chau1, Pak Cheong Chow1, Tak Cheung Yung1, Yiu Fai Cheung1,2, Kin Shing Lun1

    Congenital Heart Disease, Vol.13, No.6, pp. 884-891, 2018, DOI:10.1111/chd.12658

    Abstract Objective: Transcatheter valve perforation for pulmonary atresia intact ventricular septum is the standard of care for patients with mild right ventricular hypoplasia. However, its role in moderate right ventricular hypoplasia has been less well defined. We sought to report the long‐term outcome of patients with moderate hypoplastic right ventricle who had undergone the procedure.
    Design, Settings, and Patients: We performed a retrospective analysis on patients who had undergone transcatheter pulmonary valve perforation from January 1996 to January 2015 at our institution. The procedures would be carried out irrespective of the right ventricular size, as long as there… More >

  • Open Access

    EDITORIAL

    From the Chair of the AAP Section on Cardiology & Cardiac Surgery

    Christopher S. Snyder

    Congenital Heart Disease, Vol.13, No.5, pp. 877-878, 2018, DOI:10.1111/chd.12697

    Abstract This article has no abstract. More >

  • Open Access

    REVIEW

    Efficacy of dexmedetomidine in prevention of junctional ectopic tachycardia and acute kidney injury after pediatric cardiac surgery: A meta‐analysis

    Xin Li MMed*, Chengxin Zhang*, Di Dai MMed, Haiyuan Liu, Shenglin Ge

    Congenital Heart Disease, Vol.13, No.5, pp. 799-807, 2018, DOI:10.1111/chd.12674

    Abstract Objective: We conducted a meta‐analysis to evaluate the effects of prophylactic perioperative dexmedetomidine administration on postoperative junctional ectopic tachycardia (JET) and acute kidney injury (AKI) in pediatric patients having under‐ gone cardiac surgery.
    Design: This systematic review was registered with PROSPERO (CRD42017083880). Databases including PubMed, Cochrane Central Register of Controlled Trials, and Web of Science were searched for randomized controlled trials (RCTs) and observa‐ tional cohort studies from its inception to March 2018. Two reviewers independently screened literature, extracted data, and assessed the quality of included studies using the Jadad scale and Newcastle‐Ottawa score. Meta‐analysis was then… More >

  • Open Access

    ARTICLE

    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… More >

  • Open Access

    ARTICLE

    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… More >

  • Open Access

    ARTICLE

    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… More >

  • Open Access

    ARTICLE

    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.… More >

  • Open Access

    ARTICLE

    Diastolic velocity half time is associated with aortic coarctation gradient at catheterization independent of echocardiographic and clinical blood pressure gradients

    Adam B. Christopher1, Abraham Apfel2, Tao Sun2, Jackie Kreutzer1, David S. Ezon3

    Congenital Heart Disease, Vol.13, No.5, pp. 713-720, 2018, DOI:10.1111/chd.12637

    Abstract Objective: The most accurate noninvasive parameter to predict whether a patient with aortic coarctation will meet interventional criteria at catheterization remains elusive. We aim to determine the best independent echocardiographic predictors of a coarctation peak‐to‐peak pressure gradient ≥20 mm Hg at catheterization, the ac‐ cepted threshold for intervention.
    Design: Retrospective query of our catheterization database from 1/2007 to 7/2016 for the diagnostic code of aortic coarctation was performed. Multiple echocardio‐ graphic measurements and blood pressure gradients prior to cardiac catheterization were collected. Univariate correlation of variables with the continuous catheteriza‐ tion peak were calculated using Spearman’s rho.… More >

  • Open Access

    ARTICLE

    Mitochondrial DNA mutation “m.3243A>G”—Heterogeneous clinical picture for cardiologists (“m.3243A>G”: A phenotypic chameleon)

    Katharina Niedermayr1, Gerhard Pölzl2, Sabine Scholl‐Bürgi1, Christine Fauth3, Ulrich Schweigmann1, Edda Haberlandt1, Ursula Albrecht1, Manuela Zlamy1, Wolfgang Sperl4, Johannes A. Mayr4, Daniela Karall1

    Congenital Heart Disease, Vol.13, No.5, pp. 671-677, 2018, DOI:10.1111/chd.12634

    Abstract Objective: In general, a mitochondrial disorder is diagnosed on the basis of symptom combinations and confirmed by genetic findings. However, patients carrying the m.3243A>G mutation in the mitochondrial tRNA leucine 1 (MT‐TL1) do not always meet all the proposed criteria for the most frequently encountered mitochondrial syndrome “MELAS,” an acronym for Mitochondrial Encephalomyopathy, Lactic Acidosis, and at least one Stroke‐like episode. We here present various phenotypic characteristics of the mitochondrial mutation m.3243A>G with particular focus on cardiac manifestations.
    Methods and Results: We followed nine patients (1 month to 68 years old; median 42 years; four female and five male) from… More >

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