Home / Journals / CHD / Vol.14, No.5, 2019
  • Open Access

    ARTICLE

    Long‐term cardiovascular outcome of Williams syndrome

    Seul Gi Cha1, Mi Kyung Song1, Sang Yun Lee1, Gi Beom Kim1, Jae Gun Kwak2, Woong Han Kim2, Eun Jung Bae1
    Congenital Heart Disease, Vol.14, No.5, pp. 684-690, 2019, DOI:10.1111/chd.12810
    Abstract Objective: Cardiovascular lesions are the leading cause of morbidity and mortality in patients with Williams syndrome. Recent studies have rebutted conventional reports about the natural course of cardiovascular anomalies in Williams syndrome.
    Design: Retrospective study.
    Setting: Single tertiary center.
    Patients: Eighty patients with Williams syndrome followed up for more than 5 years.
    Interventions: Not applicable.
    Outcome Measures: Long‐term outcome of cardiovascular lesions, peak velocity change in obstructive cardiovascular lesions over time, post‐interventional courses of disease‐specific intervention, and intervention‐free survival of obstructive cardio‐ vascular lesions.
    Results: The median follow‐up duration was 11.0 (5.1‐28.3) years. Among 80 pa‐ tients, supravalvular aortic stenosis… More >

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    ARTICLE

    Serial assessment of postoperative ventricular mechanics in young children with tetralogy of Fallot: Comparison of transannular patch and valve‐sparing repair

    Vidhya Annavajjhala1, Rajesh Punn1, Theresa A. Tacy1, Frank L. Hanley2, Doff B. McElhinney2
    Congenital Heart Disease, Vol.14, No.5, pp. 691-699, 2019, DOI:10.1111/chd.12772
    Abstract Background: Little is known about the early time course of biventricular function and mechanics after tetralogy of Fallot (TOF) repair. We sought to evaluate and describe the evolution of the right ventricle (RV) after TOF repair in young infants and children using conventional echocardiographic parameters and global longitudinal strain (GLS).
    Methods: A retrospective review was performed of all patients with TOF and pulmo‐ nary stenosis who underwent repair from January 2002 to September 2015 and had at least 3 serial postsurgical echocardiograms spanning from infancy to early child‐ hood (<8 years). Student’s t test was performed to compare patients who… More >

  • Open Access

    ARTICLE

    The role of echocardiography for quantitative assessment of right ventricular size and function in adults with repaired tetralogy of Fallot

    Alexander C. Egbe, Sorin V. Pislaru, Srikanth Kothapalli, Raja Jadav, Muhammad Masood, Mounika Angirekula, Patricia A. Pellikka
    Congenital Heart Disease, Vol.14, No.5, pp. 700-705, 2019, DOI:10.1111/chd.12783
    Abstract Background: Quantitative assessment of right ventricular (RV) systolic function by echocardiography is challenging in patients with congenital heart disease because of the complex geometry of the RV and the iatrogenic structural abnormalities resulting from prior cardiac surgeries. The purpose of this study was to determine the correla‐ tion between echocardiographic indices of RV systolic function and cardiac magnetic resonance imaging (CMRI) derived RV ejection fraction (RVEF) in adults with repaired tetralogy of Fallot (TOF).
    Methods: Quantitative assessment of RV function was performed with RV tissue Doppler systolic velocity (RV s'), tricuspid annular plane systolic excursion (TAPSE), and fractional area change… More >

  • Open Access

    ARTICLE

    Hand‐held echocardiography in children with hypoplastic left heart syndrome

    Alan F. Riley1, Elena C. Ocampo1, Joseph Hagan2, M. Regina Lantin‐Hermoso1
    Congenital Heart Disease, Vol.14, No.5, pp. 706-712, 2019, DOI:10.1111/chd.12774
    Abstract Background: When performed by cardiologists, hand‐held echocardiography (HHE) can assess ventricular systolic function and valve disease in adults, but its accuracy and utility in congenital heart disease is unknown. In hypoplastic left heart syndrome (HLHS), the echocardiographic detection of depressed right ventricular (RV) systolic function and higher grade tricuspid regurgitation (TR) can identify patients who are at increased risk of morbidity and mortality and who may benefit from additional imaging or medical therapies.
    Methods: Children with HLHS after Stage I or II surgical palliation (Norwood or Glenn procedures) were prospectively enrolled. Subjects underwent HHE by a pediatric cardiologist on the… More >

  • Open Access

    ARTICLE

    Role of Doppler echocardiography for assessing right ventricular cardiac output in patients with atrial septal defect

    Vidhushei Yogeswaran1, Rahul Kanade1, Camilo Mejia1, Ayotola Fatola1, Srikanth Kothapalli1, Maria Najam1, Harigopal Sandhyavenu1, Mounika Angirekula1, Karim Osma1, Mathews Jessey1, Donald Hagler2, Alexander C. Egbe1
    Congenital Heart Disease, Vol.14, No.5, pp. 713-719, 2019, DOI:10.1111/chd.12813
    Abstract Background: Although Doppler echocardiography is routinely used to assess left ventricle cardiac output, there are limited data about the feasibility of Doppler echo‐ cardiography for right ventricular (RV) cardiac output assessment in patients with left‐to‐right shunt. The purpose of the study was to determine the correlation be‐ tween Doppler‐derived and Fick‐derived RV cardiac index (CI), and the interobserver correlation in Doppler‐derived RV CI assessment.
    Methods: Retrospective study of patients (age ≥18 years) with unrepaired atrial septal defect who underwent cardiac catheterization and echocardiography (within 3 days), 2004‐2017. RV CI was calculated using the hydraulic orifice formula: [.785 × (right ventricle… More >

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    ARTICLE

    Echocardiographic assessment of single‐ventricle diastolic function and its correlation to short‐term outcomes after the Fontan operation

    Erin K. Davis1, Salil Ginde1, Jessica Stelter2, Peter Frommelt1, Garick D. Hill3
    Congenital Heart Disease, Vol.14, No.5, pp. 720-725, 2019, DOI:10.1111/chd.12814
    Abstract Background/Hypothesis/Objectives: Postoperative complications after the Fontan operation for single ventricle heart disease are common and include persistent pleural drainage and prolonged length of hospital stay (LOS). Diastolic ventricular dysfunction may increase risk for postoperative complications by raising central venous pressures. We sought to determine the relationship between preoperative echocardiographic measurements of diastolic function, including myocardial deformation imaging, on (a) preoperative invasive catheterization measurements and (b) postoperative outcomes after the Fontan procedure.
    Design/Methods: All patients that underwent Fontan procedure from 2011 to 2017 were included. Echocardiograms performed within 6 months prior to Fontan operation were evaluated. Measurements of ventricular global and… More >

  • Open Access

    ARTICLE

    Clinic nonattendance is associated with increased emergency department visits in adults with congenital heart disease

    Katherine Awh1,2, Morgan A. Venuti1,2, Lacey P. Gleason1,2, Rachel Rogers3, Srinivas Denduluri1, Yuli Y. Kim1,2
    Congenital Heart Disease, Vol.14, No.5, pp. 726-734, 2019, DOI:10.1111/chd.12784
    Abstract Objective: To determine the prevalence and predictors of nonattendance in an ACHD outpatient clinic, and to examine the relationship between nonattendance and emergency department (ED) visits, hospitalizations, and death.
    Methods: Patients ≥ 18 years who had scheduled appointments at an ACHD outpatient clinic between August 1, 2014 and December 31, 2014 were included. The primary outcome of interest was nonattendance of the first scheduled appointment of the study period, defined as “no-show” or “same-day cancellation.” Secondary outcomes of interest were ED visits, hospitalizations, and death until December 2017.
    Results: Of 527 scheduled visits, 55 (10.4%) were nonattended. Demographic and socioeconomic… More >

  • Open Access

    ARTICLE

    Secular trends in pregnancy rates, delivery outcomes, and related health care utilization among women with congenital heart disease

    Natalie Bottega1, Isabelle Malhamé2, Liming Guo1, Raluca Ionescu‐Ittu1, Judith Therrien1, Ariane Marelli1
    Congenital Heart Disease, Vol.14, No.5, pp. 735-744, 2019, DOI:10.1111/chd.12811
    Abstract Background: The number of women with congenital heart disease (CHD) of reproductive age is increasing, yet a description of trends in pregnancy and delivery outcomes in this population is lacking.
    Objective: To assess secular trends in pregnancy rates, delivery outcomes, and related health care utilization in the adult female CHD population in Quebec, Canada.
    Methods: The Quebec CHD database was used to construct a cohort with all women with CHD aged 18‐45 years between 1992 and 2004. Pregnancy and delivery rates were determined yearly and compared to the general population. Secular trends in pregnancy and delivery rates were assessed with… More >

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    ARTICLE

    Detection of arrhythmias in adult congenital heart disease patients with LINQTM implantable loop recorder

    Anudeep K. Dodeja1, Courtney Thomas1, Curt J. Daniels1,2, Naomi Kertesz1,2, Anna Kamp1,2
    Congenital Heart Disease, Vol.14, No.5, pp. 745-751, 2019, DOI:10.1111/chd.12815
    Abstract Background: Rhythm disorders are the leading cause of morbidity and mortality in adults with congenital heart disease (ACHD). Infrequent or asymptomatic arrhyth‐ mias may not be detected by routine monitoring. Implantable loop recorders (ILRs), such as the Reveal LINQTM, have been useful in long‐term monitoring for arrhythmias in adults with cryptogenic stroke.
    Objective: We propose the Reveal LINQTM will detect arrhythmias, not documented by other monitoring modalities, resulting in change in management in ACHD patients.
    Methods: This is a single center retrospective review of Reveal LINQTM use in ACHD patients from 2014‐2017. Medical records were reviewed to determine cardiac diag‐… More >

  • Open Access

    ARTICLE

    A model for geographic and sociodemographic access to care disparities for adults with congenital heart disease

    Katherine B. Salciccioli1, Abiodun Oluyomi2, Philip J. Lupo3, Peter R. Ermis1, Keila N. Lopez1
    Congenital Heart Disease, Vol.14, No.5, pp. 752-759, 2019, DOI:10.1111/chd.12819
    Abstract Background: Follow‐up at a regional adult congenital heart disease (ACHD) center is recommended for all ACHD patients at least once per the 2018 ACC/AHA guidelines. Other specialties have demonstrated poorer follow‐up and outcomes correlating with increased distance from health care providers, but driving time to regional ACHD centers has not been examined in the US population.
    Objective: To identify and characterize potential disparities in access to ACHD care in the US based on drive time to ACHD centers and compounding sociodemographic factors.
    Methods: Mid‐ to high‐volume ACHD centers with ≥500 outpatient ACHD visits and ≥20 ACHD surgeries annually were included… More >

  • Open Access

    ARTICLE

    Comparison of creatinine and cystatin C for estimation of glomerular filtration rate in pediatric patients after Fontan operation

    Danielle Kirelik1,2, Mark Fisher2, Michael DiMaria2, Danielle E. Soranno3, Katja M. Gist2
    Congenital Heart Disease, Vol.14, No.5, pp. 760-764, 2019, DOI:10.1111/chd.12776
    Abstract Background: There are several limitations when using creatinine to estimate glomerular filtration rate, especially in children with chronic medical conditions who are at high risk of kidney dysfunction. Cystatin C has been the recent focus of research as a replacement biomarker for creatinine. Our objective was to compare the 2 biomarkers in pediatric single‐ventricle heart disease patients who have undergone the Fontan operation. We hypothesized that there would be poor correlation and agreement between the 2 estimates of renal function.
    Methods: This was a single center retrospective chart review of 20 patients who had previously undergone Fontan operation. Demographic and… More >

  • Open Access

    ARTICLE

    More than the heart: Hepatic, renal, and cardiac dysfunction in adult Fontan patients

    Ryan D. Byrne1, Angela J. Weingarten2, Daniel E. Clark2, Shi Huang3, Roman E. Perri4, Andrew E. Scanga4, Jonathan N. Menachem2, Larry W. Markham5, Benjamin P. Frischhertz2
    Congenital Heart Disease, Vol.14, No.5, pp. 765-771, 2019, DOI:10.1111/chd.12820
    Abstract Setting: Fontan‐associated liver disease universally affects adults with single ventricle heart disease. Chronic kidney disease is also highly prevalent in adult Fontan patients. In this study, we evaluate the relationship of Fontan hemodynamics invasively and noninvasively with extra‐cardiac dysfunction as measured by MELD and MELD‐XI.
    Objective: We hypothesize that invasive and noninvasive measures of Fontan circuit congestion and ventricular dysfunction are associated with increased MELD and MELD‐XI scores.
    Design: Single‐center data from adults with Fontan palliation who had ongoing care, including cardiac catheterization, were retrospectively collected. Hemodynamic data from cardiac catheterization and echocardiographic assessment of ventricular and atrioventricular valve function… More >

  • Open Access

    ARTICLE

    The clinical characteristics of coronary artery fistula anomalies in children and adults: A 24‐year experience

    Kieu T. Huynh1, Vien T. Truong2,3, Tam N. M. Ngo3,4, Thao B. Dang5, Wojciech Mazur3, Eugene S. Chung3,6, Justin T. Tretter7, Dean J. Kereiakes3, Tuyen K. Le8, Vinh N. Pham1
    Congenital Heart Disease, Vol.14, No.5, pp. 772-777, 2019, DOI:10.1111/chd.12781
    Abstract Objectives: The aim of our work is to investigate the clinical characteristics of coro‐ nary artery fistula (CAF) anomalies in South Vietnam.
    Methods: This is a retrospective analysis of 119 patients with diagnosis of definite CAF between January 1992 and April 2016. The demographic, clinical, echocardio‐ graphic, and angiographic characteristics and management of CAF with short‐term outcomes are described.
    Results: The median age was 15 years (range, 1‐79 years), with 49 male (41%) and 70 female (59%). There were 77 symptomatic patients (64.7%) and 91 patients (76.5%) who presented with a murmur. The electrocardiogram was abnormal in 45.4% and cardiac… More >

  • Open Access

    ARTICLE

    Parental anxiety before invasive cardiac procedure in children with congenital heart disease: Contributing factors and consequences

    Oscar Werner1, Fedoua El Louali2, Virginie Fouilloux3, Pascal Amedro1,4, Caroline Ovaert2,5
    Congenital Heart Disease, Vol.14, No.5, pp. 778-784, 2019, DOI:10.1111/chd.12777
    Abstract Objective: Medical information provided to parents of a child with a congenital heart disease can induce major stress. Visual analog scales have been validated to assess anxiety in the adult population. The aim of this study was to analyze parental anxiety using a visual analog scale and to explore the influencing factors.
    Design: This prospective cross‐sectional study.
    Setting: Tertiary care regional referral center for congenital heart disease of Marseille—La Timone university hospital.
    Patients: Parents of children with a congenital heart disease, as defined by the ACC‐ CHD classification, referred for cardiac surgery or interventional cardiac catheterization, were offered to participate.
    More >

  • Open Access

    ARTICLE

    Health care‐associated infections are associated with increased length of stay and cost but not mortality in children undergoing cardiac surgery

    Sarah Tweddell, Rohit S. Loomba, David S. Cooper, Alexis L. Benscoter
    Congenital Heart Disease, Vol.14, No.5, pp. 785-790, 2019, DOI:10.1111/chd.12779
    Abstract Introduction: Health care‐associated infections (HAIs) increase mortality, length of stay, and cost in hospitalized patients. The incidence of and risk factors for developing HAIs in the pediatric population after cardiac surgery have been studied. This study evaluates the impact of HAIs on length of stay, inpatient mortality, and cost of hospitalization in the pediatric population after cardiac surgery.
    Methods: TheKids’InpatientDatabasewasqueriedforanalysis.Patientsunder18years of age who underwent cardiac surgery from 1997 to 2012 were included. HAIs were defined as central line‐associated blood stream infections, catheter‐associated urinary tract infections, ventilator‐associated pneumonias, and surgical wound infections. Univariate analysis compared admissions with and without a HAI.… More >

  • Open Access

    ARTICLE

    Stenting the vertical neonatal ductus arteriosus via the percutaneous axillary approach

    Jesse Lee1,2, Kanishka Ratnayaka1, John Moore1, Howaida El‐Said1
    Congenital Heart Disease, Vol.14, No.5, pp. 791-796, 2019, DOI:10.1111/chd.12786
    Abstract Background/Objective: Stenting the ductus arteriosus (DAS) has become an alternative to surgical systemic to pulmonary artery shunts in neonates with ductal‐ dependent pulmonary blood flow (PBF). Femoral approach for a vertical ductus can be difficult secondary to the acute angle and tortuous course, thus alternative ac‐ cess sites have been explored. Carotid access complications have been reported in 5%‐10%. The extensive use of an axillary arterial approach in the United States has not been reported. The aim of this study is to describe our experience with DAS using the axillary approach.
    Methods: We reviewed all patients with DAS with an… More >

  • Open Access

    ARTICLE

    Ancillary referral patterns in infants after initial assessment in a cardiac developmental outcomes clinic

    Sonia A. Monteiro1, Faridis Serrano1, Rocky Tsang1, Eboni Smith Hollier1, Danielle Guffey2, Lisa Noll1, Robert G. Voigt1, Nancy Ghanayem1, Lara Shekerdemian1
    Congenital Heart Disease, Vol.14, No.5, pp. 797-802, 2019, DOI:10.1111/chd.12789
    Abstract Objective: Neurodevelopmental impairment is common after surgery for congeni‐ tal heart disease (CHD) in infancy. While neurodevelopmental follow‐up of high‐risk patients has increased, the referral patterns for ancillary services following initial evaluation have not been reported. The aim of this study is to describe the rates and patterns of referral at the initial visit to our outcomes clinic of patients who under‐ went surgery for CHD during infancy.
    Outcomes Measures: The Cardiac Developmental Outcomes Program clinic at Texas Children’s Hospital provides routine longitudinal follow‐up with developmental pedi‐ atricians and child psychologists for children who required surgery for CHD within the… More >

  • Open Access

    ARTICLE

    Small atrial septal defects are associated with psychiatric diagnoses, emotional distress, and lower educational levels

    Sebastian Udholm1, Camilla Nyboe1, Thomas Meinertz Dantoft2, Torben Jørgensen2,3,4, Charlotte U. Rask5, Vibeke E. Hjortdal1
    Congenital Heart Disease, Vol.14, No.5, pp. 803-810, 2019, DOI:10.1111/chd.12808
    Abstract Objective: For the first time, we wish to assess the psychiatric burden in adult patients living with small, unrepaired atrial septal defects (ASD) using register‐based data, com‐ bined with self‐reported measures on levels emotional distress and educational status.
    Design: A descriptive study using both the unique Danish registries and validated psychiatric questionnaires and scales, including: The Symptom Checklist, Whiteley‐7, and Brief Illness Perception Questionnaire.
    Patients: Adult patients with small, unrepairedASD, diagnosed between 1953 and 2011.
    Outcome Measures: Number of register‐based psychiatric diagnoses. Additionally, symptoms of anxiety, depression, somatization, health anxiety, illness perception, and levels of educational attainment compared to age‐… More >

  • Open Access

    ARTICLE

    Catheter‐associated bloodstream infection incidence and outcomes in congenital cardiac surgery

    Brena Sue Haughey1, Shelby Coral White2, Michael David Seckeler2
    Congenital Heart Disease, Vol.14, No.5, pp. 811-813, 2019, DOI:10.1111/chd.12809
    Abstract Objective: Catheter‐associated bloodstream infections complicate and prolong hos‐ pitalizations. The incidence of catheter‐associated bloodstream infections in children undergoing congenital cardiac surgery has not been reported. This study sought to define the incidence of catheter‐associated bloodstream infections after congenital cardiac surgery in neonates and infants ≤12 months old and compare hospital out‐ comes and costs to those who underwent surgery and did not have a catheter‐associ‐ ated bloodstream infections.
    Design: Retrospective review of hospital admissions between October 2013 and November 2015 for neonates and infants ≤12 months old at admission with ICD‐9 codes for congenital cardiac surgery from discharge data from… More >

  • Open Access

    ARTICLE

    Perforation of the atretic pulmonary valve using chronic total occlusion (CTO) wire and coronary microcatheter

    Bruno Lefort1,2, Christophe Saint‐Etienne1, Nathalie Soulé1, Iris Ma1, Fanny Dion1, Alain Chantepie1,2
    Congenital Heart Disease, Vol.14, No.5, pp. 814-818, 2019, DOI:10.1111/chd.12812
    Abstract Background and objective: Chronic total occlusion (CTO) guidewire have been recently reported as an alternative to radiofrequency for perforating atretic pulmonary valve. Since procedure failures or perforation of the right ventricle still occurred with CTO, we tried to enhance the stability, steering, and pushability of the wire using a microcatheter in order to improve the safety and efficacy of the procedure.
    Methods: We performed pulmonary valve perforation with CTO guidewire and microcatheter in five consecutive newborns with pulmonary atresia with intact ventricular septum (PA‐IVS) under fluoroscopic and echocardiographic control.
    Results: The valve was easily perforated at the first attempt for… More >

  • Open Access

    ARTICLE

    Transvenous implantation of the Occlutech Atrial Flow Regulator: Preliminary results from swine models

    Daniel McLennan, Dunbar Ivy, Gareth J. Morgan
    Congenital Heart Disease, Vol.14, No.5, pp. 819-831, 2019, DOI:10.1111/chd.12816
    Abstract Aims: To evaluate in domestic pigs the histopathological processes after implanting the Occlutech Atrial Flow Regulator (AFR).
    Methods and Results: Eleven pigs were chosen and had successful implantation of the AFR. Five pigs were sacrificed at 28 days, and 5 pigs at 90 days. One pig was sacrificed at day 3 after device embolization. Each pig had echocardiography performed at 3 weeks to check patency. Post mortem evaluation included Gross evaluation, radiographic evaluation, histology, and electron microscopy. Nine of the 10 devices implanted remained patent at time of autopsy with no thrombus and minimal inflammation. One device placed in the… More >

  • Open Access

    ARTICLE

    Three year experience of a clinical cardiovascular genetics program for infants with congenital heart disease

    Gabrielle C. Geddes1,2, Erin Syverson1,2, Michael G. Earing1,2
    Congenital Heart Disease, Vol.14, No.5, pp. 832-837, 2019, DOI:10.1111/chd.12817
    Abstract Objective: To describe the first 3 years of experience of having an inpatient “cardiogenetics” program which involves medical geneticist assessment of infants with major congenital heart disease (CHD) requiring surgical intervention in the first year of life.
    Patients: Patients less than a year of age admitted to Children’s Hospital of Wisconsin’s Herma Heart Institute for surgical intervention for CHD seen by the cardiogenetics program. Patients with major trisomies (13, 18, and 21) were excluded.
    Outcome Measures: Utilization and yield of genetic testing, and diagnostic rate were assessed as outcome measures and compared to a baseline time period and a genetic… More >

  • Open Access

    ARTICLE

    Postoperative and short‐term atrial tachyarrhythmia burdens after transcatheter vs surgical pulmonary valve replacement among congenital heart disease patients

    Subeer K. Wadia1, Gentian Lluri1, Jamil A. Aboulhosn1, Hillel Laks2, Reshma M. Biniwale2, Glen S. Van Arsdell2, Daniel S. Levi3, Morris M. Salem3, Kevin M. Shannon1,3, Jeremy P. Moore1,3
    Congenital Heart Disease, Vol.14, No.5, pp. 838-845, 2019, DOI:10.1111/chd.12818
    Abstract Objective: We examined the atrial tachyarrhythmia (AT) burden among patients with congenital heart disease (CHD) following transcatheter (TC‐) or surgical (S‐) pulmo‐ nary valve replacement (PVR).
    Design/Setting: This was a retrospective observational study of patients who under‐ went PVR from 2010 to 2016 at UCLA Medical Center.
    Patients: Patients of all ages who had prior surgical repair for CHD were included. Patients with a history of congenitally corrected transposition of the great arteries, underwent a hybrid PVR procedure, or had permanent atrial fibrillation (AF) without a concomitant ablation were excluded.
    Outcome Measures: The primary outcome was a time‐to‐event analysis of… More >

  • Open Access

    ARTICLE

    32 year follow up of patients following atrial redirection surgery for transposition of the great arteries

    M. Louise Morrison1, Brian Grant1, Brian A. McCrossan1,2, Andrew J. Sands1,2, Colum G. Owens2, Mark S. Spence2, Frank A. Casey1, Brian G. Craig1,2, Christopher J. Lockhart2
    Congenital Heart Disease, Vol.14, No.5, pp. 846-853, 2019, DOI:10.1111/chd.12822
    Abstract Objective: A significant body of patients who have undergone Mustard or Senning procedure require lifelong follow up. In this retrospective review, we examined the cohort of such patients currently attending our center.
    Design: Patients who had undergone either Mustard or Senning procedure were identified. We retrospectively reviewed medical records, recorded demographic in‐ formation and data regarding the clinical state, NHYA class, cardiopulmonary exer‐ cise testing, NT‐proBNP measurement, and recent cardiac MRI findings.
    Results: Forty‐six patients were identified, the mean age was 32.2 years (± 6.1 years), 67.4% were male. Thirty‐two patients (69.6%) had undergone a Senning procedure. The median length… More >

  • Open Access

    ARTICLE

    Postoperative and long‐term outcomes in children with Trisomy 21 and single ventricle palliation

    Jennifer K. Peterson1, Shaun P. Setty1,2, Jessica H. Knight3, Amanda S. Thomas4, James H. Moller5, Lazaros K. Kochilas4,6
    Congenital Heart Disease, Vol.14, No.5, pp. 854-863, 2019, DOI:10.1111/chd.12823
    Abstract Objective: Patients with Trisomy 21 (T21) and single ventricle (SV) physiology present unique challenges compared to euploidic counterparts. This study reports postoperative and long‐term outcomes in patients with T21 and SV palliation.
    Design: This retrospective cohort study from the Pediatric Cardiac Care Consortium (PCCC) included patients with T21 (<21 years old) that underwent surgical palliation for SV between 1982 and 2008 and control patients without known genetic anom‐ aly following Fontan palliation for similar diagnoses. Kaplan‐Meier survival plots were created based on death events obtained from the PCCC and by linkage with the National Death Index (NDI) and the Organ… More >

  • Open Access

    ARTICLE

    Utilization of echocardiography in Ehlers‐Danlos syndrome

    Kristina K. Rauser‐Foltz1, Lois J. Starr2, Anji T. Yetman1
    Congenital Heart Disease, Vol.14, No.5, pp. 864-867, 2019, DOI:10.1111/chd.12824
    Abstract Objectives: This study assessed the frequency and utility of echocardiographic examination in patients with all forms of Ehlers‐Danlos syndrome and sought to iden‐ tify clinical variables associated with an abnormal echocardiogram.
    Design/setting: This was a retrospective study of all patients carrying a diagnosis of Ehlers‐Danlos syndrome of any type who were evaluated by a pediatrician or pediatric subspecialist at a single tertiary medical center with an affiliated children’s hospital during the period January 2013 to December 2018.
    Patients: Chart review was performed on all patients carrying a diagnosis of Ehlers‐ Danlos syndrome in the electronic medical record.
    Outcome Measures: Data… More >

  • Open Access

    REVIEW

    Myocarditis in the pediatric population: A review

    Soham Dasgupta, Glen Iannucci, Chad Mao, Martha Clabby, Matthew E. Oster
    Congenital Heart Disease, Vol.14, No.5, pp. 868-877, 2019, DOI:10.1111/chd.12835
    Abstract Myocarditis has a variable clinical presentation and there is still debate regarding accurate diagnostic criteria. Adding to the controversy surrounding this diagnosis, there is no clear consensus for the treatment or ongoing follow‐up of patients with myocarditis. All of this makes the diagnosis and management of myocarditis a par‐ ticular challenge in the pediatric population. Furthermore, the literature with respect to this topic is dynamic and ever‐changing. In this review article, we aim to review and summarize the common clinical presentations of myocarditis, along with the latest recommendations for diagnostic criteria, treatment, and follow‐up of patients with myocarditis. More >

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