Open Access

ARTICLE

Tarek Alsaied¹, Jouke P. Bokma², Mark E. Engel³, Joey M. Kuijpers², Samuel P. Hanke¹, Liesl Zuhlke⁴, Bin Zhang⁵, Gruschen R. Veldtman⁶
Congenital Heart Disease, Vol.12, No.4, pp. 393-398, 2017, DOI:10.1111/chd.12468
Abstract Background: Reported long-term outcome measures vary greatly between studies in Fontan patients making comprehensive appraisal of mortality hazard challenging. We sought to create a clinical risk score to assist monitoring of Fontan patients in the outpatient setting.
Methods: A systematic review was conducted to evaluate risk factors for long-term (beyond the first postoperative year) mortality in Fontan patients. Studies were eligible for inclusion if ≥90 patients were included or ≥20 long-term mortalities we reported. Risk factors for long-term mortality were determined. The pooled hazard ratios were used to create components of a clinical score for long-term mortality… More >

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M. Elisabeth Heal¹, Lanier B. Jackson², Andrew M. Atz², Ryan J. Butts³
Congenital Heart Disease, Vol.12, No.4, pp. 399-402, 2017, DOI:10.1111/chd.12451
Abstract Cardiopulmonary exercise testing (CPET) aids in clinical assessment of patients with Fontan circulation. Effects of persistent fenestration on CPET variables have not been clearly defined. Associations between fenestration and CPET variables at anaerobic threshold (AT) and peak exercise were explored in the Pediatric Heart Network Fontan Cross-Sectional Study cohort. Fenestration patency was associated with a greater decrease in oxygen saturation from rest to peak exercise (fenestration -4.9 ± 3.8 v. nonfenestration -3 ± 3.5; P < .001). Physiological dead space at peak exercise was higher in fenestrated v. nonfenestrated (25.2 ± 16.1 v. 21.4 ± More >

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Karen Texter^1,2, Jo Ann M. Davis¹, Christina Phelps^1,2, Sharon Cheatham^1,2, John Cheatham^1,2, Mark Galantowicz^1,3, Timothy F. Feltes^1,2
Congenital Heart Disease, Vol.12, No.4, pp. 403-410, 2017, DOI:10.1111/chd.12459
Abstract Introduction: With increasing survival of children with HLHS and other single ventricle lesions, the complexity of medical care for these patients is substantial. Establishing and adhering to best practice models may improve outcome, but requires careful coordination and monitoring.
Methods: In 2013 our Heart Center began a process to build a comprehensive Single Ventricle Team designed to target these difficult issues.
Results: Comprehensive Single Ventricle Team in 2014 was begun, to standardize care for children with single ventricle heart defects from diagnosis to adulthood within our institution. The team is a multidisciplinary group of providers committed to improving… More >

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ARTICLE

Jeffrey A. Robinson, Jill K. Shivapour, Christopher S. Snyder
Congenital Heart Disease, Vol.12, No.4, pp. 411-416, 2017, DOI:10.1111/chd.12458
Abstract Objective: Pseudosyncope can be difficult to distinguish from true syncope. Often, pediatric patients with pseudosyncope undergo multiple tests and referrals before the appropriate diagnosis is reached. The purpose is to describe the utility of the head-up tilt table test to elicit the diagnosis of pseudosyncope in the pediatric population.
Design: Retrospective chart review from November 2012 to December 2015 of patients age ≤23 years referred for 30-minute, 80-degree tilt table test. Pretest probability for pseudosyncope was high if there was no response to traditional management, atypical episodes, occurrence during undesirable exercise, or prolonged episode duration. Inductive techniques… More >

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ARTICLE

Natalie M. Hoeting¹, Courtney E. McCracken¹, Michael McConnell^1,2, Denver Sallee^1,2, Glen J. Iannucci^1,2, Matthew E. Oster^1,2
Congenital Heart Disease, Vol.12, No.4, pp. 417-420, 2017, DOI:10.1111/chd.12460
Abstract Objective: Bicuspid aortic valve (BAV) disease is associated with potential lifetime complications, but auscultation of a BAV click is commonly missed or mistaken for a benign split first heart sound. Our objective was to determine whether pediatric cardiologists could reliably distinguish between BAV clicks and benign split first heart sounds.
Design: Quality evaluation project using de-identified recordings from an outpatient pediatric cardiology clinic.
Outcome Measures: Twenty-one cardiologists listened to five de-identified recordings of pediatric heart sounds (three with BAV clicks, two with mitral components of benign split first heart sounds) and indicated whether they believed each recording was… More >

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Jessica L. Bean Jaworski, Thomas Flynn, Nancy Burnham, Jesse L. Chittams, Therese Sammarco, Marsha Gerdes, Judy C. Bernbaum, Robert R. Clancy, Cynthia B. Solot, Elaine H. Zackai, Donna M. McDonald-McGinn, J. William Gaynor
Congenital Heart Disease, Vol.12, No.4, pp. 421-429, 2017, DOI:10.1111/chd.12461
Abstract Objective: Atypical development, behavioral difficulties, and academic underachievement are common morbidities in children with a history of congenital heart defects and impact quality of life. Language and social-cognitive deficits have been described, which are associated with autism spectrum disorders. The current study aimed to assess the rates of autism spectrum disorders in a large sample of children with a history of congenital heart defects and to assess medical, behavioral, and individual factors that may be associated with the risk of autism spectrum disorders.
Design: Participants included 195 children with a history of congenital heart defects, who are… More >

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Amitabh Thacoor
Congenital Heart Disease, Vol.12, No.4, pp. 430-434, 2017, DOI:10.1111/chd.12467
Abstract Marfan syndrome is a multisystemic genetic condition affecting connective tissue. It carries a reduced life expectancy, largely dependent on cardiovascular complications. More common cardiac manifestations such as aortic dissection and aortic valve incompetence have been widely documented in the literature. Mitral valve prolapse (MVP), however, has remained poorly documented. This article aims at exploring the existing literature on the pathophysiology and diagnosis of MVP in patients with Marfan syndrome, defining its current management and outlining the future developments surrounding it. More >

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Katie E. Cohen¹, Matthew W. Buelow¹, Jennifer Dixon¹, Ruta Brazauskas², Scott B. Cohen³, Michael G. Earing^1,3, Salil Ginde¹
Congenital Heart Disease, Vol.12, No.4, pp. 435-440, 2017, DOI:10.1111/chd.12470
Abstract Objective: Abnormal lung function characterized by a reduced forced vital capacity (FVC) is common in adults with repaired tetralogy of Fallot (TOF) and is associated with previous thoracotomies and sternotomies. The impact of abnormal lung function on clinical outcomes in adult patients with repaired TOF is unclear. The aim of this study was to determine the impact of abnormal lung function on the outcome of hospitalization and death in adults with repaired TOF when analyzed with other traditional cardiac risk factors.
Design: Retrospective study of adults with repaired TOF, who underwent spirometry between 2000 and 2014. FVC… More >

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Takaya Hoashi¹, Toru Iwasa², Koji Kagisaki¹, Masatoshi Shimada¹, Kenichi Kurosaki², Isao Shiraishi², Hajime Ichikawa¹
Congenital Heart Disease, Vol.12, No.4, pp. 441-447, 2017, DOI:10.1111/chd.12477
Abstract Objective: To review long-term respiratory outcomes for tetralogy of Fallot and absent pulmonary valve (TOF/APV) in respiratory symptomatic populations.
Methods: Of 25 consecutive patients undergoing primary total correction for TOF/APV between 1987 and 2016, Sixteen patients (64%) with a preoperative respiratory disturbance were enrolled. The median age at operation was 1.9 months old, including 4 neonates and 12 infants. Ten patients (62.5%) preoperatively necessitated mechanical ventilator support. During operation, dilated central pulmonary arteries (cPAs) were plicated and retracted anteriorly in all patients, except for the first patient of the study cohort. VSD was completely closed, and the… More >

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ARTICLE

Jana Rubáčková Popelová, MD1,2 | Markéta Tomková1,3 | Jakub Tomek1,4
Congenital Heart Disease, Vol.12, No.4, pp. 448-457, 2017, DOI:10.1111/chd.12466
Abstract Objective: The patients after Mustard and Senning corrections of transposition of the great arteries (TGA) are at an increased risk of unexpected death. The aim of this study was to identify markers allowing risk stratification of patients after atrial switch correction of TGA to provide them with optimum care.
Methods and Results: In this study, 87 patients were retrospectively evaluated after atrial switch correction of TGA followed-up between 2005 and 2015. The mortality during the follow-up was 9% (8 cardiac deaths). Markers significantly predictive of death using univariable Cox proportional hazard ratio survival analysis were: N-terminal pro-B-type… More >

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Magalie Ladouceur^1,2,3, Nadjia Kachenoura⁴, Gilles Soulat^1,3, Emilie Bollache⁴, Alban Redheuil⁴, Michel Azizi³, Christophe Delclaux³, Gilles Chatellier³, Pierre Boutouyrie^1,3, Laurence Iserin³, Damien Bonnet^2,3, Elie Mousseaux^1,3
Congenital Heart Disease, Vol.12, No.4, pp. 458-466, 2017, DOI:10.1111/chd.12472
Abstract Objectives: We aimed (1) determine if systemic right ventricle filling parameters influence systemic right ventricle stroke volume in adult patients with D-transposition of the great arteries (D-TGA) palliated by atrial switch, using cardiac magnetic resonance imaging and echocardiography, and (2) to study relationship of these diastolic parameters with exercise performance and BNP, in patients with preserved systolic systemic right ventricle function.
Design: Single-center, cross-sectional, prospective study.
Setting: In patients with D-TGA palliated by atrial switch, diastolic dysfunction of the systemic right ventricle may precede systolic dysfunction.
Methods: Forty-five patients with D-TGA and atrial switch and 45 age and sex-matched… More >

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ARTICLE

Paul Tannous^1,2, Sunil J Ghelani^1,2, Audrey C Marshall^1,2, Diego Porras^1,2
Congenital Heart Disease, Vol.12, No.4, pp. 467-474, 2017, DOI:10.1111/chd.12457
Abstract Objective: Angiographically detectable Thebesian veins (ThVs) are a rare finding sometimes associated with coronary steal and myocardial ischemia in adults, but there are limited data regarding prominent ThVs in the setting of complex congenital heart disease (CHD). This study represents the largest series to date describing the presence and temporal changes of angiographically detectable ThVs in children with CHD.
Methods: This is a single center case series describing the clinical characteristics and coronary anatomy in children with CHD and angiographicall detectable ThVs. After identification of the index case, additional patients were identified in a prospective manner during… More >

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Aaron R. Prosnitz¹, Jane Leopold², Mira Irons³, Kathy Jenkins¹, Amy E. Roberts¹
Congenital Heart Disease, Vol.12, No.4, pp. 475-483, 2017, DOI:10.1111/chd.12471
Abstract Objective: To describe a group of children with co-incident pulmonary vein stenosis and SmithLemli-Opitz syndrome and to generate hypotheses as to the shared pathogenesis of these disorders.
Design: Retrospective case series.
Patients: Five subjects in a pulmonary vein stenosis cohort of 170 subjects were diagnosed with Smith-Lemli-Opitz syndrome soon after birth.
Results: All five cases were diagnosed with Smith-Lemli-Opitz syndrome within 6 weeks of life, with no family history of either disorder. All cases had pathologically elevated 7-dehydrocholesterol levels and two of the five cases had previously reported pathogenic 7-dehydrocholesterol reductase mutations. Smith-Lemli-Opitz syndrome severity scores ranged from mild… More >

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Sarah A. Twichell¹, Corinna J. Rea¹, Patrice Melvin², Andrew J. Capraro¹, Joshua C. Mandel¹, Michael A. Ferguson¹, Daniel J. Nigrin¹, Kenneth D. Mandl¹, Dionne Graham², Justin P. Zachariah³
Congenital Heart Disease, Vol.12, No.4, pp. 484-490, 2017, DOI:10.1111/chd.12469
Abstract Background: Recognition of high blood pressure (BP) in children is poor, partly due to the need to compute age-sex-height referenced percentiles. This study examined the change in abnormal BP recognition before versus after the introduction of an electronic health record (EHR) app designed to calculate BP percentiles with a training lecture.
Methods and results: Clinical data were extracted on all ambulatory, non-urgent encounters for children 3–18 years old seen in primary care, endocrinology, cardiology, or nephrology clinics at an urban, academic hospital in the year before and the year after app introduction. Outpatients with at least 1… More >

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Lubica Kovacikova¹, Martin Zahorec¹, Peter Skrak¹, Brian D. Hanna², R. Lee Vogel²
Congenital Heart Disease, Vol.12, No.4, pp. 491-496, 2017, DOI:10.1111/chd.12480
Abstract Background: Telemedicine is a rapidly evolving form of modern information and communication technology used to deliver clinical services and educational activities.
Objective: The aim of this article is to report and analyze our experience with transatlantic consultation via videoconferencing in pediatric cardiology.
Methods: In February, 2013, videoconferencing project was launched between a medium-volume pediatric cardiac center in Bratislava, Slovakia and subspecialty experts from a high-volume pediatric cardiac program at The Children’s Hospital of Philadelphia (CHOP), USA. During 1.5–2 hours videoconferences, 2–3 patients with similar complex clinical scenarios were presented to CHOP experts. The main goal of the project… More >

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Karrie F. Downing^1,2, Matthew E. Oster^1,3, Sherry L. Farr¹
Congenital Heart Disease, Vol.12, No.4, pp. 497-506, 2017, DOI:10.1111/chd.12476
Abstract Objective: A substantial percentage of children with congenital heart disease (CHD) fail to transfer to adult care, resulting in increased risk of morbidity and mortality. Transition planning discussions with a provider may increase rates of transfer, yet little is known about frequency and content of these discussions. We assessed prevalence and predictors of transition-related discussions between providers and parents of children with special healthcare needs (CSHCN) and heart problems, including CHD.
Design: Using parent-reported data on 12- to 17-year-olds from the 2009–2010 National Survey of CSHCN, we calculated adjusted prevalence ratios (aPR) for associations between demographic factors… More >

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ARTICLE

Shaun Mohan¹, Brady S. Moffett², Wilson Lam², Caridad de la Uz², Christina Miyake², Santiago O. Valdes², Jeffrey J. Kim²
Congenital Heart Disease, Vol.12, No.4, pp. 507-511, 2017, DOI:10.1111/chd.12478
Abstract Objective: As survivors of congenital heart disease (CHD) continue to age, healthcare utilization by this population has increased. It is unknown how often these patients utilize the emergency department (ED) at children’s hospitals and how arrhythmias play a role in their utilization of care.
Design: Using a retrospective cohort design, the Pediatric Hospital Information System (PHIS) database was investigated and we studied adults (≥18 years) with CHD (ACHD) who presented to pediatric EDs from 2004 to 2014.
Setting: Tertiary care pediatric hospitals.
Results: Of the 6310 encounters to pediatric EDs, 1594 (25%) were for arrhythmias. The median age was… More >

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ARTICLE

Shankar Baskar¹, Philippa Horne², Samantha Fitzsimmons³, Philip R. Khoury¹, Joseph Vettukattill⁴, Koichiro Niwa⁵, Teiji Agaki⁶, Mark Spence⁷, Hisanori Sakazaki⁸, Gruschen Veldtman¹
Congenital Heart Disease, Vol.12, No.4, pp. 512-519, 2017, DOI:10.1111/chd.12481
Abstract Background: Patients with Eisenmenger syndrome have a shorter lifespan than the general population. A significant proportion develop arrhythmia and some, sudden death.
Objective: The aims of this study were to characterize the frequency, type and effects of arrhythmias in adult patients with Eisenmenger’s syndrome and to identify risk factors for arrhythmias.
Methods: This retrospective study included patients aged ≥18 years of age with Eisenmenger’s syndrome from three institutions. Arrhythmias were noted from electrocardiograms and Holter study reviews.
Results: A total of 167 patients, 96 females, 63 males (gender not available in 9 patients) were included in this study. The… More >

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Jennifer K. Peterson¹, Yanjun Chen², Danh V. Nguyen³, Shaun P. Setty¹
Congenital Heart Disease, Vol.12, No.4, pp. 520-532, 2017, DOI:10.1111/chd.12475
Abstract Objective: Despite overall improvements in congenital heart disease outcomes, racial and ethnic disparities have continued. The purpose of this study is to examine the effect of race and ethnicity, as well as other risk factors on congenital heart surgery length of stay and in-hospital mortality.
Design: From the 2012 Healthcare Cost and Utilization Project Kids Inpatient Database (KID), we identified 13 130 records with Risk Adjustment in Congenital Heart Surgery complexity scoreeligible procedures. Multivariate logistic and linear regression modeling with survey weights, stratification and clustering was used to examine the relationships between predictor variables and length of… More >

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Chih-Hsin Hsu^1,2, Jun-Neng Roan^1,3, Jieh-Neng Wang⁴, Chien-Chi Huang⁵, Chao-Jung Shih³, Jyh-Hong Chen^2,6, Jing-Ming Wu⁴, Chen-Fuh Lam^7,8
Congenital Heart Disease, Vol.12, No.4, pp. 533-539, 2017, DOI:10.1111/chd.12479
Abstract Objectives: Atrial septal defects may result in pulmonary hypertension and right heart remodeling. We analyzed improvements in patients with flow-induced pulmonary hypertension and the activation of endothelial progenitor cells after flow reduction.
Design: This prospective cohort study included 37 patients who were admitted for an occluder implantation. Blood samples were collected before and after the procedure. We determined the number of endothelial progenitor cells in outgrowth colonies and serum Hsp27 concentrations. Daily performance and cardiothoracic ratio were reevaluated later.
Results: Closure of the defect significantly reduced the pulmonary pressure and B-type natriuretic peptide levels. The cardiothoracic ratio and… More >

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Waldemar F. Carlo¹, Steven T. Clark², Santiago Borasino³, Jeffrey A. Alten³
Congenital Heart Disease, Vol.12, No.4, pp. 540-545, 2017, DOI:10.1111/chd.12482
Abstract Objective: Acute kidney injury (AKI) is a frequent complication after cardiopulmonary bypass (CBP) for cardiac surgery in neonates. It is unclear if exposure to computed tomography angiography (CTA) in the preoperative period increases the risk of AKI. We hypothesized a short interval between CTA and CPB surgery would be associated with higher rates of AKI in infants.
Design: In this single center retrospective review of patients between 2012 and 2015, neonates less than one month old were analyzed if they had CTA prior to cardiac surgery with CPB. Baseline, demographic, fluid balance, and laboratory data was analyzed.… More >

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Tyler H. Harris¹, Mark Adler², Sharon M. Unti³, Mary E. McBride⁴
Congenital Heart Disease, Vol.12, No.4, pp. 546-553, 2017, DOI:10.1111/chd.12483
Abstract Background: Training guidelines state that pediatricians should be able to diagnose, manage, and triage patients with heart disease. Acutely ill cardiac patients present infrequently and with high acuity, yet residents receive less exposure to acute cardiac conditions than previous generations. Trainees must learn to manage these situations despite this gap. Simulation has been used successfully to train learners to provide acute care. We hypothesized that a simulation-based cardiac curriculum would improve residents’ ability to manage cardiac patients.
Methods: Pediatric residents completed 4 simulation cases followed by debriefing and a computer presentation reviewing the learning objectives. Subjects returned… More >

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