Open Access

EDITORIAL

Congenital Heart Disease, Vol.13, No.5, pp. 645-645, 2018, DOI:10.1111/chd.12689
Abstract This article has no abstract. More >

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ARTICLE

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 >

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803

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ARTICLE

Juan Lizandro Rodríguez‐Hernández¹, Fayna Rodríguez‐González², Marta Riaño‐Ruiz³, Efrén Martínez‐Quintana^1,4
Congenital Heart Disease, Vol.13, No.5, pp. 655-662, 2018, DOI:10.1111/chd.12620
Abstract Introduction: Hyperuricemia has been associated with cardiovascular risk factors but it remains controversial if uric acid is an independent predictor of cardiac mortality.
Methods: A total of 503 CHD patients (457 nonhypoxemic and 46 hypoxemic) and 772 control patients fulfilled inclusion criteria. Demographic, clinical, and analytical data [serum uric acid and 24h urine uric acid levels, N‐terminal pro‐B‐type natriuretic peptide (NT‐pro‐BNP), and C‐reactive‐protein (CRP) concentrations] were studied. Survivals curves to determine cardiac death and arterial thrombosis in CHD patients were also examined.
Results: Noncyanotic and cyanotic CHD patients had significant higher serum uric acid concentration (5.2 ± 1.5 vs 4.9 ± 1.3mg/dL, P = .007 and… More >

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797

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ARTICLE

Anita T. Cote^1,2, Martin Hosking³, Christine Voss^1,3, Derek G. Human³, George G. S. Sandor^1,3, Kevin C. Harris^1,3
Congenital Heart Disease, Vol.13, No.5, pp. 663-670, 2018, DOI:10.1111/chd.12629
Abstract Objective: Pediatric heart transplant recipients are at risk of posttransplant coronary artery disease known as cardiac allograft vasculopathy (CAV), and also may develop diastolic dysfunction. As CAV begins with a process of progressive intimal thickening, these occult diffuse changes may be detected using optical coherence tomography (OCT). We hypothesized that the development of CAV, as identified via OCT, may be a mechanism of declining ventricular function. Accordingly, the purpose of this study was to assess coronary artery intimal thickening and LV strain in children who have undergone heart transplantation.
Methods: In 17 children, we analyzed OCT images for coronary intima… More >

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791

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ARTICLE

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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798

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ARTICLE

Ketina Arslani^1*, Lukas Notz^1*, Marzena Zurek¹, Matthias Greutmann², Markus Schwerzmann³, Judith Bouchardy⁴, Reto Engel⁵, Christine Attenhofer Jost⁶, Daniel Tobler¹
Congenital Heart Disease, Vol.13, No.5, pp. 678-684, 2018, DOI:10.1111/chd.12627
Abstract Background: In adults with congenital heart disease (CHD) and atrial arrhythmias, recommendations for thromboprophylaxis are vague and evidence is lacking. We aimed to identify factors that influence decision-making in daily practice.
Methods: From the Swiss Adult Congenital HEart disease Registry (SACHER) we identified 241 patients with either atrial fibrillation (Afib) or atrial flutter/intraatrial reentrant tachycardia (Aflut/ IART). The mode of anticoagulation was reviewed. Logistic regression models were used to assess factors that were associated with oral anticoagulation therapy.
Results: Compared with patients with Aflut/IART, patients with Afib were older (51 ± 16.1 vs 37 ± 16 years, P < .001)… More >

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744

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ARTICLE

Paul C. Helm¹, Ulrike M. M. Bauer^1,2, Hashim Abdul‐Khaliq^2,3, Helmut Baumgartner^1,4, Hans‐Heiner Kramer⁵, Christian Schlensak^2,7, Thomas Pickardt¹, Anne‐Karin Kahlert^5,6*, Marc‐Phillip Hitz^5*
Congenital Heart Disease, Vol.13, No.5, pp. 685-689, 2018, DOI:10.1111/chd.12630
Abstract Background: Congenital heart disease (CHD) affects up to 1% of live births the etiol‐ ogy remains relatively poorly understood. Thus, cardiac research is needed to under‐ stand the underlying pathomechanisms ofthe disease.About51 000CHDpatients are registered in the German National Register for Congenital Heart Defects (NRCHD). Patients and relatives were interviewed online about their willingness to support ge‐ netic heart research in order to donate a biological sample.
Methods: Study participants were recruited via the database of the NRCHD. Seven thousand nine hundred eighty‐nine patients were invited to participate in the study. Participants have been asked to rate three questions on… More >

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744

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ARTICLE

Juan Jesús Sánchez‐Barriga
Congenital Heart Disease, Vol.13, No.5, pp. 690-699, 2018, DOI:10.1111/chd.12631
Abstract Background: Congenital heart disease (CHD) represents a global health problem. In Mexico, in children <1 year of age it is the second cause of mortality. The aim was to determine mortality trends from CHD and the great vessels in children and adults nationwide, by state and socioeconomic region.
Methods: Records of mortality associated to CHD and the great vessels for 2000‐2015 were obtained from the National Institute of Statistics and Geography. This information is collected from death certificates issued nationwide. International Classification of Diseases, 10th revision, codes corresponding to the basic cause of death from CHD and the great vessels.… More >

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735

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ARTICLE

Cheryl L. Brosig^1,2, Laurel Bear^1,2, Sydney Allen¹, Pippa Simpson¹, Liyun Zhang¹, Michele Frommelt^1,2, Kathleen A. Mussatto²
Congenital Heart Disease, Vol.13, No.5, pp. 700-705, 2018, DOI:10.1111/chd.12632
Abstract Background and Objectives: Children with congenital heart disease (CHD) are at risk for neurodevelopmental (ND) delays. The purpose of this study is to compare the ND testing results of children with CHD at 2 and 4 years of age and determine if rates of ND delays change over time.
Methods: Children with CHD completed the Bayley Scales of Infant Development‐III (BSID‐III) at 2 years of age, and standardized neuropsychological measures at 4 years. Scores were compared with test norms and were classified as: average (within one SD of test mean); at risk (1‐2 SDs from the test mean); and delayed… More >

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774

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Jennifer P. Lundine^1,2, Robert Dempster^3,4, Kirby Carpenito⁵, Holly Miller‐Tate⁵, Wendelin Burdo‐Hartman^4,6, Elizabeth Halpin², Omar Khalid^4,5
Congenital Heart Disease, Vol.13, No.5, pp. 706-712, 2018, DOI:10.1111/chd.12636
Abstract Background: Swallowing dysfunction is a known complication for infants with complex congenital heart disease (CHD), but few studies have examined swallowing outcomes following the hybrid procedure for stage 1 palliation in children with single ventricle physiology.
Objectives: (1) Identify the incidence of aspiration in all infants with single ventricle physiology who underwent the hybrid procedure and (2) Compare results of clinical bedside and instrumental swallowing evaluations to examine the predictive value of a less invasive swallowing assessment for this population of high‐risk infants.
Methods: This was a retrospective cohort chart review study. All patients with single‐ventricle physiology who underwent the… More >

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978

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798

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ARTICLE

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. Univariate association with… More >

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738

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ARTICLE

Jill M. Steiner¹, James N. Kirkpatrick¹, Susan R. Heckbert², James Sibley³, James A. Fausto³, Ruth A. Engelberg³, J. Randall Curtis³
Congenital Heart Disease, Vol.13, No.5, pp. 721-727, 2018, DOI:10.1111/chd.12638
Abstract Objective: Overall health care resource utilization by adults with congenital heart disease has increased dramatically in the past two decades, yet little is known about utilization patterns at the end of life. The objective of this study is to better under‐ stand the patterns and influences on end‐of‐life care intensity for adults with con‐ genital heart disease.
Methods: We identified a sample of adults with congenital heart disease (n = 65), cancer (n = 10 784), or heart failure (n = 3809) who died between January 2010 and December 2015, cared for in one multi‐hospital health care system. We used… More >

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740

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ARTICLE

Ashley Brunmeier^1,2, Michael P Reis³, Michael G. Earing^1,2, Laura Umfleet ⁴, Salil Ginde^1,2, Peter J. Bartz^1,2, Scott Cohen^1,2
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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741

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ARTICLE

Brian Robichaud¹, Garick Hill², Scott Cohen^1,3, Ronald Woods⁴, Michael Earing^1,3, Peter Frommelt¹, Salil Ginde^1,3
Congenital Heart Disease, Vol.13, No.5, pp. 734-739, 2018, DOI:10.1111/chd.12639
Abstract Background: Pulmonary valve replacement (PVR) is a common operation in patients with congenital heart disease (CHD). As survival with CHD improves, infective endo‐ carditis (IE) is a growing complication after PVR. The aim of this study was to assess the incidence, risk factors, and clinical outcomes of IE after surgical PVR in patients with CHD at our institution.
Methods: Retrospective analysis of all cases of surgical PVR performed at Children’s Hospital of Wisconsin between 1975 and 2016 was performed. All cases of IE after PVR were identified and clinical and imaging data were obtained by review of medical records.
Results:… More >

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976

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797

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ARTICLE

Michelle Keir¹, Barbara Bailey¹, Angela Lee¹, Adrienne H. Kovacs^1,2, S. Lucy Roche^1,3
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 >

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808

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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 >

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1058

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762

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ARTICLE

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 >

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782

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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 >

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904

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ARTICLE

Michael E. Fenster^1‡, 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 >

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1037

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795

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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 >

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1053

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833

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ARTICLE

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 >

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1106

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759

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ARTICLE

Ashley E. Neal¹, Elizabeth Lehto¹, Karen Hughes Miller², Erin Davis³, Craig Ziegler²
Congenital Heart Disease, Vol.13, No.5, pp. 788-793, 2018, DOI:10.1111/chd.12626
Abstract Objective: The 2016 American Board of Pediatrics (ABP) content outline is comprehensive, including more than 50 cardiology-specific objectives within eight content areas. This study complements the quantitative analysis of a Kentucky-wide survey of trainees, pediatricians, and pediatric cardiologists asking them to identify “most important” cardiology content by analyzing their open-ended comments about “what should be added” and “why?” within these eight categories.
Design, Methods, Outcome Measures: This cross-sectional study used an original, online survey instrument based on the 2016 ABP cardiology-specific objectives. We began an initial analysis of the qualitative data using Pandit’s version of Glaser and Strauss Grounded theory… More >

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1050

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737

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ARTICLE

Joseph J. Knadler¹, Daniel J. Penny¹, Tyler H. Harris², Gary D. Webb³, Antonio G. Cabrera^1,4, 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 >

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REVIEW

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 conducted by RevMan… More >

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803

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ARTICLE

Shankar Baskar, Seth B. Gray, Erica L. Del Grippo, Onyekachukwu Osakwe, Adam W. Powell, Justin T. Tretter
Congenital Heart Disease, Vol.13, No.5, pp. 808-810, 2018, DOI:10.1111/chd.12675
Abstract Cardiology fellows‐in‐training, both in adult and pediatric hospitals, need structured education in regards to congenital heart disease (CHD) nomenclature. With improved survival of patients with CHD, it is not uncommon for these patients to seek care in multiple adult and pediatric hospitals. A deep understanding of CHD nomenclature would aid in providing accurate medical and surgical care for these patients. In this forum, we share our experience with such structured education and also comment on recent advances in morphologic imaging that would aid in understanding the nomenclature. More >

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ARTICLE

Anoymous
Congenital Heart Disease, Vol.13, No.5, pp. 811-876, 2018, DOI:10.1111/chd.12693
Abstract This article has no abstract. More >

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935

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EDITORIAL

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 >

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810