Home / Journals / CHD / Vol.12, No.1, 2017
  • Open Access

    EDITORIAL

    Guest Editorials for the Coming Year

    Douglas S. Moodie
    Congenital Heart Disease, Vol.12, No.1, pp. 5-5, 2017, DOI:10.1111/chd.12444
    Abstract This article has no abstract. More >

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    ARTICLE

    “Frontiers in Fontan failure: A summary of conference proceedings”

    Camden Hebson1,2, Wendy Book1, Robert W. Elder3, Ryan Ford4, Maan Jokhadar1, Kirk Kanter5, Brian Kogon5, Adrienne H. Kovacs6, Rebecca D. Levit1, Michael Lloyd1, Kevin Maher2, Preeti Reshamwala4, Fred Rodriguez III1,2, Rene Romero7, Thor Tejada1, Anne Marie Valente8, Gruschen Veldtman9, Michael McConnell1,2
    Congenital Heart Disease, Vol.12, No.1, pp. 6-16, 2017
    Abstract “Frontiers in Fontan Failure” was the title of a 2015 conference sponsored by Children’s Healthcare of Atlanta and Emory University School of Medicine. In what is hoped to be the first of many such gatherings, speakers and attendees gathered to discuss the problem of long-term clinical deterioration in these patients. Specific focuses included properly defining the problem and then discussing different treatment strategies, both medical and surgical. The health of the liver after Fontan palliation was a particular point of emphasis, as were quality of life and future directions. More >

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    ARTICLE

    Sudden cardiac death and late arrhythmias after the Fontan operation

    Kavitha N. Pundi1, Krishna N. Pundi2, Jonathan N. Johnson1,3, Joseph A. Dearani4, Zhuo Li, BS5, David J. Driscoll1, Philip L. Wackel1, Christopher J. McLeod3, Frank Cetta1,3, Bryan C. Cannon1,3
    Congenital Heart Disease, Vol.12, No.1, pp. 17-23, 2017
    Abstract Objectives: We sought to examine the incidence and predictors of arrhythmias and sudden cardiac death (SCD) after Fontan operation.
    Background: Arrhythmias and SCD have been reported following operations for congenital heart disease, but the incidence and risk factors have not been well defined in patients after a Fontan operation.
    Methods: We reviewed records of all patients who had a Fontan operation from 1973 to 2012 (n 5 1052) at our institution. A questionnaire was mailed to patients who were not known to be deceased at the initiation of the study. Late arrhythmias were classified as bradyarrhythmias or tachyarrhythmias requiring treatment… More >

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    ARTICLE

    Bicuspid and Unicuspid Aortic Valve: Fate of Moderate/Severe Mixed Aortic Valve Disease

    Alexander C. Egbe*, Heidi M. Connolly*, Joseph T. Poterucha*,†, Carole A. Warnes*
    Congenital Heart Disease, Vol.12, No.1, pp. 24-31, 2017
    Abstract Objectives. There is a paucity of data about mixed aortic valve disease (MAVD) in patients with bicuspid/unicuspid aortic valve (BAV). This study sought to describe the outcomes of patients with moderate/severe MAVD.
    Methods. We queried our database for patients with BAV and moderate/severe MAVD seen between 1994 and 2013. We excluded patients with baseline New York Heart Association (NYHA) III/IV symptoms, left ventricular ejection fraction <50%, aortic dimension >50 mm, and significant disease of other valves. The purpose of the study was to determine the freedom from NYHA III/IV symptoms and aortic valve replacement (AVR).
    Results. We identified 138 patients… More >

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    ARTICLE

    Surgical outcome in pediatric patients with Ebstein’s anomaly: A multicenter, long-term study

    Lianne M. Geerdink1,2, Gideon J. du Marchie Sarvaas3, Irene M. Kuipers4, Willem A. Helbing5, Tammo Delhaas6, Henriette ter Heide7, Lieke Rozendaal8, Chris L. de Korte9, Sandeep K. Singh10, Tjark Ebels11, Mark G. Hazekamp12, Felix Haas13, Ad J. J. C. Bogers14, Livia Kapusta1,15
    Congenital Heart Disease, Vol.12, No.1, pp. 32-39, 2017
    Abstract Objective: Surgical outcomes of pediatric patients with Ebstein’s anomaly are often described as part of all-age-inclusive series. Our objective is to focus on patients treated surgically in childhood (0-18 y). We study the intended treatment (biventricular or 1.5 ventricle repair or univentricular palliation), freedom from unplanned reoperation and survival of this specific age group, in a nationwide study.
    Design: Records of all Ebstein’s anomaly patients born between 1980 and 2013 were reviewed. Demographic variables, intraoperative procedures and postoperative outcomes were analyzed.
    Results: Sixty-three patients underwent 109 operations. Median follow-up after diagnosis was 121 months (range 0-216 months). Twenty-nine (46%) patients… More >

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    ARTICLE

    Comparative Evaluation of Flow Quantification across the Atrioventricular Valve in Patients with Functional Univentricular Heart after Fontan’s Surgery and Healthy Controls: Measurement by 4D Flow Magnetic Resonance Imaging and Streamline Visualization

    Hoi Lam She*, Arno A.W. Roest, Emmeline E. Calkoen, Pieter J. van den Boogaard, Rob J. van der Geest, Mark G. Hazekamp§, Albert de Roos, Jos J.M. Westenberg
    Congenital Heart Disease, Vol.12, No.1, pp. 40-48, 2017
    Abstract Purpose. To evaluate the inflow pattern and flow quantification in patients with functional univentricular heart after Fontan’s operation using 4D flow magnetic resonance imaging (MRI) with streamline visualization when compared with the conventional 2D flow approach.
    Method. Seven patients with functional univentricular heart after Fontan’s operation and twenty-three healthy controls underwent 4D flow MRI. In two orthogonal two-chamber planes, streamline visualization was applied, and inflow angles with peak inflow velocity (PIV) were measured. Transatrioventricular flow quantification was assessed using conventional 2D multiplanar reformation (MPR) and 4D MPR tracking the annulus and perpendicular to the streamline inflow at PIV, and they… More >

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    ARTICLE

    Fused cardiac hybrid imaging with coronary computed tomography angiography and positron emission tomography in patients with complex coronary artery anomalies

    Christoph Gräni, Dominik C. Benz, Mathias Possner, Olivier F. Clerc, Fran Mikulicic, Jan Vontobel, Julia Stehli, Tobias A. Fuchs, Aju P. Pazhenkottil, Oliver Gaemperli, Philipp A. Kaufmann, Ronny R. Buechel
    Congenital Heart Disease, Vol.12, No.1, pp. 49-57, 2017
    Abstract Objective: To provide data on the value of fused cardiac hybrid imaging with coronary computed tomography angiography (CCTA) and positron emission tomography myocardial perfusion imaging (PET-MPI) in patients with complex coronary artery anomalies (CCAA).
    Design/setting: This is a retrospective, single-center study.
    Patients: Seven consecutive patients with CCAA (mean 57 ± 7 y, 86% were male) who underwent clinically indicated hybrid CCTA/PET-MPI between 2005 and 2015 in our clinic were included. The findings from both modalities and fused cardiac hybrid imaging were evaluated in these patients.
    Results: Out of the seven patients with CCAA, two patients had Bland–White–Garland anomaly, two patients… More >

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    ARTICLE

    Late gadolinium enhancement and adverse outcomes in a contemporary cohort of adult survivors of tetralogy of Fallot

    Richard J. Dobson1, Ify Mordi2, Mark H. Danton1, Niki L. Walker1, Hamish A. Walker1, Nikolaos Tzemos2
    Congenital Heart Disease, Vol.12, No.1, pp. 58-66, 2017
    Abstract Objective: Myocardial fibrosis has been associated with poorer outcomes in tetralogy of Fallot, however only a handful of studies have assessed its significance in the current era. Our aim was to quantify the amount of late gadolinium enhancement in both the LV and RV in a contemporary cohort of adults with surgically repaired tetralogy of Fallot, and assess the relationship with adverse clinical outcomes.
    Design: Single centre cohort study
    Setting: National tertiary referral center
    Patients: One hundred fourteen patients with surgically repaired tetralogy of Fallot with median age 29.5 years (range 17.5-64.2). Prospective follow-up for mean 2.4 years (SD 1.29).
    More >

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    ARTICLE

    Impact of changing indications and increased utilization of fetal echocardiography on prenatal detection of congenital heart disease

    Jonathan Komisar1, Shubhika Srivastava2, Miwa Geiger2, John Doucette3, Helen Ko2, Jay Shenoy2, Rajesh Shenoy2
    Congenital Heart Disease, Vol.12, No.1, pp. 67-73, 2017
    Abstract Background: Antenatal diagnosis of congenital heart defects (CHD) can impact outcomes in neonates with severe CHD. Obstetric screening guidelines and the indications for fetal echocardiography (FE) have evolved in an attempt to improve the early prenatal detection of CHD. Analyzing yield for specific indications will help clinicians better stratify at-risk pregnancies.
    Methods: Retrospective cohort study of all FE performed between 2000 and 2010 at a single tertiary care academic medical center in New York City. A total of 9878 FE met inclusion criteria for our study. In cases of multiple gestations (MG), each fetus was counted as a separate study.
    More >

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    ARTICLE

    Aortic stenting in the growing sheep causes aortic endothelial dysfunction but not hypertension: Clinical implications for coarctation repair

    Nicola Maschietto1, Luca Semplicini2, Giulio Ceolotto2, Arianna Cattelan2, Helen Poser3, Ilaria Iacopetti3, Gabriele Gerardi3, Giulia Maria De Benedictis3, Tommaso Pilla3, Daniele Bernardini3, Luca Aresu4, Stefania Rizzo5, Cristina Basso5, Andrea Semplicini2, Ornella Milanesi1
    Congenital Heart Disease, Vol.12, No.1, pp. 74-83, 2017
    Abstract Background: Stent implantation is the treatment of choice for adolescents and adults with aortic coarctation (CoAo). Despite excellent short-term results, 20%–40% of the patients develop arterial hypertension later in life, which was attributed to inappropriate response of the aortic baroreceptors to increased stiffness of the ascending aorta (ASAO), either congenital or induced by CoAo repair. In particular, it has been hypothesized that stent itself may cause or sustain hypertension. Therefore, we aimed to study the hemodynamic and structural impact following stent implantation in the normal aorta of a growing animal.
    Methods: Eight female sheep completed the study and a stent… More >

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    ARTICLE

    Rapid 3D imaging of the lower airway by MRI in patients with congenital heart disease: A retrospective comparison of delayed volume interpolated breath-hold examination (VIBE) to turbo spin echo (TSE)

    Benjamin H. Goot, Sonali Patel, Brian Fonseca
    Congenital Heart Disease, Vol.12, No.1, pp. 84-90, 2017
    Abstract Objective: When imaging the lower airway by MRI, the traditional technique turbo spin echo (TSE) results in high quality 2D images, however planning and acquisition times are lengthy. An alternative, delayed volume interpolated breath-holds examination (VIBE), is a 3D gradient echo technique that produces high spatial resolution imaging of the airway in one breath-hold. The objective of this study is to retrospectively evaluate the accuracy of lower airway measurements obtained by delayed VIBE when compared to TSE.
    Design: Patients with congenital heart disease who underwent a cardiac MRI (CMR) that included a delayed VIBE sequence from 5/2008 to 9/2013 were… More >

  • Open Access

    ARTICLE

    Neurocognitive and executive functioning in adult survivors of congenital heart disease

    Leda Klouda1, Wayne J. Franklin1, Anita Saraf1,2, Dhaval R. Parekh1, David D. Schwartz3
    Congenital Heart Disease, Vol.12, No.1, pp. 91-98, 2017
    Abstract Objective: Congenital heart disease (CHD) can affect the developing central nervous system, resulting in neurocognitive and behavioral deficits. Preoperative neurological abnormalities as well as sequelae of the open heart operations required to correct structural abnormalities of the heart contribute to these deficits. There are few studies examining the neurocognitive functioning of adults with CHD. This study sought to investigate multiple domains of neurocognitive functioning in adult survivors of CHD who had childhood cardiac surgery with either moderate or severe disease complexity.
    Design: A total of 48 adults (18–49 years of age) who had undergone cardiac surgery for CHD prior to… More >

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    ARTICLE

    Electrocardiographic early repolarization characteristics and clinical presentations in the young: a benign finding or worrisome marker for arrhythmias

    Raya Safa, Ronald Thomas, Peter P. Karpawich
    Congenital Heart Disease, Vol.12, No.1, pp. 99-104, 2017
    Abstract Background: The early ECG repolarization QRS pattern (ERp), with J-point elevation of 0.1 mV in two contiguous inferior and/or lateral leads, can be associated with ventricular arrhythmias among adults. The significance of an ERp in the young is unknown.
    Objective: The purpose of this study was to assess the prevalence of ERp among young patients (pts), describe and correlate the characteristics with clinical presentations and any arrhythmias.
    Methods: This was a 1 y retrospective review of ECGs obtained from patients referred specifically for documented arrhythmias, possible arrhythmia-related symptoms or sports clearance. ECGs were analyzed for ERp (J-point, ascending/horizontal patterns, location)… More >

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    ARTICLE

    A population-based analysis of mortality in patients with Turner syndrome and hypoplastic left heart syndrome using the Texas Birth Defects Registry

    Diego A. Lara1, Mary K. Ethen2, Mark A. Canfield2, Wendy N. Nembhard3, Shaine A. Morris1
    Congenital Heart Disease, Vol.12, No.1, pp. 105-112, 2017
    Abstract Background: Hypoplastic left heart syndrome (HLHS) is strongly associated with Turner syndrome (TS); outcome data when these conditions coexist is sparse. We aimed to investigate long-term survival and causes of death in this population.
    Methods: The Texas Birth Defects Registry was queried for all live born infants with HLHS during 1999–2007. We used Kaplan–Meier and Cox regression analyses to compare survival among patients with HLHS with TS (HLHS/TS+) to patients who had HLHS without genetic disorders or extracardiac birth defects (HLHS/TS–).
    Results: Of the 542 patients with HLHS, 11 had TS (2.0%), 71 had other extracardiac birth defects or genetic… More >

  • Open Access

    ARTICLE

    Use of 3D models of congenital heart disease as an education tool for cardiac nurses

    Giovanni Biglino1,2, Claudio Capelli2,3, Despina Koniordou3, Di Robertshaw2, Lindsay-Kay Leaver2, Silvia Schievano2,3, Andrew M. Taylor2,3, Jo Wray2
    Congenital Heart Disease, Vol.12, No.1, pp. 113-118, 2017
    Abstract Background: Nurse education and training are key to providing congenital heart disease (CHD) patients with consistent high standards of care as well as enabling career progression. One approach for improving educational experience is the use of 3D patient-specific models.
    Objectives: To gather pilot data to assess the feasibility of using 3D models of CHD during a training course for cardiac nurses; to evaluate the potential of 3D models in this context, from the nurses’ perspective; and to identify possible improvements to optimise their use for teaching.
    Design: A cross-sectional survey.
    Setting: A national training week for cardiac nurses.
    Participants: One… More >

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