Open Access

ARTICLE

Richard D. Mainwaring, Frank L. Hanley
Congenital Heart Disease, Vol.14, No.4, pp. 504-510, 2019, DOI:10.1111/chd.12826
Abstract Objective: Anomalous left main coronary artery (LMCA) with an intraconal course is a relatively rare form of anomalous aortic origin of a coronary artery (AAOCA) from the wrong sinus of Valsalva. There is currently a paucity of information regarding this entity. The purpose of this article is to review our surgical experience with repair of anomalous LMCA with an intraconal course.
Methods: This was a retrospective review of 12 patients with an anomalous LMCA and an intraconal course who underwent surgical repair. The median age at surgery was 15 years (range 2‐47). The seven oldest patients all… More >

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Fatima M. Ezzeddine¹, Tabitha Moe², Georges Ephrem³, William Aaron Kay³
Congenital Heart Disease, Vol.14, No.4, pp. 511-516, 2019, DOI:10.1111/chd.12771
Abstract Background: Delivery of care to the adult congenital heart disease (ACHD) popula‐ tion has been limited by a shortage in the ACHD physician resources. There is limited data regarding the adequacy of the ACHD physician resources in the United States and our population estimates are extrapolated from Canadian data. Therefore, we proposed to evaluate the adequacy of ACHD physician: patient ratios in the United States at both national and regional levels.
Methods: Data from the Adult Congenital Heart Association (ACHA) website along with metropolitan area and statewide population data from 2016 US Census Bureau estimates were analyzed.… More >

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ARTICLE

Jeremy M. Steele^1,2, Tamar J. Preminger⁴, Francine G. Erenberg², Lu Wang³, Katherine Dell², Tarek Alsaied¹, Kenneth G. Zahka²
Congenital Heart Disease, Vol.14, No.4, pp. 517-524, 2019, DOI:10.1111/chd.12754
Abstract Objectives: To determine the prevalence, age of onset, and risk factors for overweight and obesity in children with congenital heart disease (CHD).
Study Design: Children with CHD who were seen at our institution from 1996 to 2017 were studied. Patients were full-time residents of the United States and were receiving all cardiac care at our institution. Patients were categorized by age and CHD diagnosis. The date of last normal weight for age and the date of first recorded weight in the range of overweight and obese were documented.
Results: Nine hundred sixty-eight patients with CHD were included. The More >

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1126

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ARTICLE

Madalena Coutinho Cruz¹, André Viveiros Monteiro¹, Guilherme Portugal¹, Sérgio Laranjo², Ana Lousinha¹, Bruno Valente¹, Paulo Osório¹, Pedro Silva Cunha¹, Lídia de Sousa¹, José Alberto Oliveira¹, Ana Agapito¹, Mário Martins Oliveira¹, Fátima Pinto², Rui Cruz Ferreira¹
Congenital Heart Disease, Vol.14, No.4, pp. 525-533, 2019, DOI:10.1111/chd.12767
Abstract Objective: Sudden cardiac death is common in the adult congenital heart disease (ACHD) population. Knowledge and experience about the use of implantable cardio‐ verter defibrillators (ICD) in ACHD patients is very limited. We aimed to characterize a cohort of patients with ACHD and ICDs.
Design: Thirty consecutive ACHD patients submitted to an ICD implantation in a single tertiary center were evaluated. Data on baseline clinical features, heart defect, indication for ICD, type of device, appropriate therapies, ICD‐related complication, and mortality during follow‐up were collected.
Results: Of the 30 patients, 56.7% received appropriate therapies due to ventricular tachycardia (VT)… More >

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ARTICLE

Lisa X. Deng¹, Lacey P. Gleason², Katherine Awh², Abigail May Khan³, David Drajpuch^2,4, Stephanie Fuller⁵, Leah A. Goldberg², Christopher E. Mascio⁵, Sara L. Partington^2,4, Lynda Tobin^2,4, Adrienne H. Kovacs³, Yuli Y. Kim^2,4
Congenital Heart Disease, Vol.14, No.4, pp. 534-540, 2019, DOI:10.1111/chd.12778
Abstract Objective: To investigate the experiences and communication preferences of adult patients with congenital heart disease (CHD) in the domains of employment, insur‐ ance, and family planning.
Design: Patients ≥ 18 years of age completed a questionnaire about experiences and communication preferences regarding employment, health insurance, and family planning.
Results: Of 152 patients (median age = 33 years, 50% female, 35% with CHD of great complexity), one in four reported work‐related problems due to CHD and a quar‐ ter also recalled a previous gap in health insurance. Of females, 29% experienced an unplanned pregnancy. The median importance of discussion… More >

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ARTICLE

Jennifer Gerardin¹, Cheryl Raskind‐Hood², Fred H. Rodriguez^3,4, Trenton Hoffman², Andreas Kalogeropoulos⁵, Carol Hogue², Wendy Book³
Congenital Heart Disease, Vol.14, No.4, pp. 541-548, 2019, DOI:10.1111/chd.12780
Abstract Objective: Transfer of congenital heart disease care from the pediatric to adult set‐ ting has been identified as a priority and is associated with better outcomes. Our objective is to determine what percentage of patients with congenital heart disease transferred to adult congenital cardiac care.
Design: A retrospective cohort study.
Setting: Referrals to a tertiary referral center for adult congenital heart disease pa‐ tients from its pediatric referral base.
Patients: This resulted in 1514 patients age 16‐30, seen at least once in three pediat‐ ric Georgia health care systems during 2008‐2010.
Interventions: We analyzed for protective factors associated with age‐appropriate… More >

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ARTICLE

Maria Fedchenko, Zacharias Mandalenakis, Helena Dellborg, Görel Hultsberg‐Olsson, Anna Björk, Peter Eriksson, Mikael Dellborg
Congenital Heart Disease, Vol.14, No.4, pp. 549-558, 2019, DOI:10.1111/chd.12785
Abstract Background: The aging patient with adult congenital heart disease (ACHD) faces the risk of developing atherosclerotic disease. Patients with coarctation of the aorta (CoA) are especially vulnerable because of an inherent high risk of developing hy‐ pertension. However, data on the prevalence of other cardiovascular risk factors are scarce. Therefore, this study aimed to describe the prevalence of traditional cardio‐ vascular risk factors (diabetes, hypertension, hyperlipidemia, smoking, obesity, and sedentary lifestyle) in adult patients with CoA.
Methods: Patients with CoA who were registered at the ACHD clinic in Gothenburg were asked to participate in a comprehensive cardiovascular… More >

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1183

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ARTICLE

Stephen J. Dolgner^1,2,3, Eric V. Krieger^1,3, Jacob Wilkes⁴, Susan L. Bratton⁵, Ravi R. Thiagarajan^6,7, Cindy S. Barrett⁸, Titus Chan^1,2,9
Congenital Heart Disease, Vol.14, No.4, pp. 559-570, 2019, DOI:10.1111/chd.12758
Abstract Objective: Adult congenital heart disease (ACHD) patients who undergo cardiac surgery are at risk for poor outcomes, including extracorporeal membrane oxygenation support (ECMO) and death. Prior studies have demonstrated risk factors for mortality, but have not fully examined risk factors for ECMO or death without ECMO (DWE). We sought to identify risk factors for ECMO and DWE in adults undergoing congenital heart surgery in tertiary care children’s hospitals.
Design: All adults (≥18 years) undergoing congenital heart surgery in the Pediatric Health Information System (PHIS) database between 2003 and 2014 were included. Patients were classified into three groups:… More >

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1165

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ARTICLE

Donald J. Hagler^1,2, William R. Miranda², Brielle J. Haggerty¹, Jason H. Anderson¹, Jonathan N. Johnson¹, Frank Cetta^1,2, Sameh M. Said^1,3, Nathaniel W. Taggart¹
Congenital Heart Disease, Vol.14, No.4, pp. 571-581, 2019, DOI:10.1111/chd.12757
Abstract Background: Stenosis of the venous connections and conduits is a well‐known late complication of the Fontan procedure. Currently, data on the outcomes of percuta‐ neous intervention for the treatment of extra‐ or intracardiac conduits and lateral tunnel baffles obstruction are limited. In an attempt to better define the nature and severity of the stenosis and the results of catheter interventional management, we reviewed Fontan patients with obstructed extra‐ or intracardiac conduits and lateral tunnel baffles.
Methods: Retrospective review of all Fontan patients who had cardiac catheteriza‐ tion from January 2002 to October 2018 was performed. Hemodynamic and… More >

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ARTICLE

Jenny Alenius Dahlqvist¹, Jan Sunnegårdh², Katarina Hanséus³, Eva Strömvall Larsson², Anders Nygren², Magnus Dalén^4,5, Håkan Berggren⁶, Jens Johansson Ramgren⁷, Urban Wiklund⁸, Annika Rydberg¹
Congenital Heart Disease, Vol.14, No.4, pp. 582-589, 2019, DOI:10.1111/chd.12766
Abstract Objective: Fontan surgery is performed in children with univentricular heart defects. Previous data regarding permanent pacemaker implantation frequency and indica‐ tions in Fontan patients are limited and conflicting. We examined the prevalence of and risk factors for pacemaker treatment in a consecutive national cohort of patients after Fontan surgery in Sweden.
Methods: We retrospectively reviewed all Swedish patients who underwent Fontan surgery from 1982 to 2017 (n = 599).
Results: After a mean follow‐up of 12.2 years, 13% (78/599) of the patients with Fontan circulation had received pacemakers. Patients operated with the extracardiac conduit (EC) type of total… More >

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1216

Open Access

ARTICLE

Michael V. Di Maria¹, Cindy Barrett¹, Carey Rafferty¹, Kelly Wolfe¹, Sarah L. Kelly¹, Deborah R. Liptzin², Dania Brigham³, Adel Younoszai¹
Congenital Heart Disease, Vol.14, No.4, pp. 590-599, 2019, DOI:10.1111/chd.12769
Abstract Background: Children with single ventricle (SV) heart disease who undergo Fontan operation are at risk for developing multiorgan dysfunction. Although survival has improved, significant comorbidities involving multiple organ systems may develop, requiring evaluation and management by many subspecialists. Using data from an internal survey, we documented high care variability for our Fontan population. We then developed a multidisciplinary clinic, designed and implemented a clinical care pathway to decrease variability of patient assessment.
Methods: After creating a multidisciplinary team and a clinical care pathway, we initiated a multidisciplinary clinic (MDC) where patients could see multiple subspecialists during a… More >

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1180

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ARTICLE

Jose A. Silva‐Sepulveda¹, Yudy Fonseca², Irine Vodkin³, Gabrielle Vaughn¹, Robert Newbury⁴, Vera Vavinskaya⁵, Jerry Dwek⁶, James C. Perry¹, Preeti Reshamwala⁷, Cynthia Baehling⁸, James Lyon⁹, Christopher Davis¹, Jesse W. Lee¹, Hannah El‐Sabrout¹⁰, Doaa Shahbah¹¹, Laith Alshawabkeh¹², John W Moore¹, Howaida El‐Said¹
Congenital Heart Disease, Vol.14, No.4, pp. 600-608, 2019, DOI:10.1111/chd.12770
Abstract Introduction: Liver fibrosis and cirrhosis are late complications in Fontan palliation. Liver biopsy is the gold standard. The goal of this study is to correlate transjugular liver biopsy (TJLB) in the setting of Fontan palliation with noninvasive testing and hemodynamics.
Methods: Between August 2014 and July 2017, 49 Fontan patients underwent TJLB. All the patients had hemodynamic evaluation, 28 patients had MRE (magnetic reso‐ nance elastography) and 40 patients had cardiopulmonary exercise test. Histologic liver fibrosis was quantitated using traditional histologic scoring systems and a modi‐ fied Ishak congestive hepatic fibrosis score.
Results: Median age 17.8 years, median… More >

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ARTICLE

Clifford L. Cua¹, Melissa Moore‐Clingenpeel¹, Nazia Husain², Ralf Holzer³, John P. Cheatham¹, Janaki Gokhale⁴
Congenital Heart Disease, Vol.14, No.4, pp. 609-613, 2019, DOI:10.1111/chd.12755
Abstract Background: Increased ventricular end‐diastolic pressure (VEDP) is a known risk fac‐ tor for morbidity and mortality in patients with single right ventricle (RV) physiology. Previous studies have shown mixed results correlating echocardiographic measure‐ ments with catheter‐derived VEDP in this population. Goal of this study was to eval‐ uate if echocardiographic systolic/diastolic ratio (S/D) correlated with VEDP.
Methods: Patients with single RV physiology who underwent simultaneous echocar‐ diography and catheterization were evaluated. Systolic and diastolic durations were measured using tricuspid inflow durations from Doppler analysis to calculate the S/D ratio. VEDP was obtained from the catheterization report.
Results: Twenty‐seven… More >

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1093

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ARTICLE

Amy Sanyahumbi¹, Andrea Beaton², Danielle Guffey³, Mina C. Hosseinipour⁴, Melissa Karlsten¹, Charles G. Minard³, Daniel J. Penny¹, Craig A. Sable⁵, Peter N. Kazembe⁶
Congenital Heart Disease, Vol.14, No.4, pp. 614-618, 2019, DOI:10.1111/chd.12756
Abstract Background: In asymptomatic children, screening echocardiography has been used to attempt to diagnose rheumatic heart disease (RHD) at an early stage (latent RHD). World Heart Federation guidelines have standardized categorization of “definite,” “borderline,” or no RHD by echo findings. The progression of RHD diagnosed through echo screening is not known. In 2014, we screened 1450 schoolchildren in Malawi.
Objective: Our objective was to evaluate 2‐year RHD evolution among those diag‐ nosed through screening.
Methods: Two‐year follow‐up echocardiograms of those diagnosed with latent RHD were read by a primary, secondary, then third reader if there was disagreement. Progression or… More >

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1113

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ARTICLE

Varisara Pornprasertchai, Chodchanok Vijarnsorn, Supaluck Kanjanauthai, Paweena Chungsomprasong, Prakul Chanthong, Kritvikrom Durongpisitkul, Jarupim Soongswang
Congenital Heart Disease, Vol.14, No.4, pp. 619-627, 2019, DOI:10.1111/chd.12759
Abstract Background: The increasing number of patients with Ebstein anomaly (EA) surviving into adulthood implies improvements in the treatments for the complex lesion. We revisited the clinical outcomes of patients with EA to demonstrate their “real world” survival.
Objectives: To identify the survival and predictors of mortality in patients with EA who underwent medical or surgical management in the present era.
Methods: All patients who had EA with atrioventricular concordance between 1994 and 2016 were retrospectively reviewed. Baseline characteristics, initial echocardio‐ graphic findings, treatments, and outcomes were explored. The survival analysis was performed at the end of 2017. A… More >

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1163

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ARTICLE

An Van Berendoncks^1,2, Roderick Van Grootel¹, Jackie McGhie¹, Matthijs van Kranenburg¹, Myrthe Menting¹, Judith A.A.E. Cuypers¹, Ad J.J.C. Bogers³, Maarten Witsenburg¹, Jolien W. Roos‐Hesselink¹, Annemien E. van den Bosch¹
Congenital Heart Disease, Vol.14, No.4, pp. 628-637, 2019, DOI:10.1111/chd.12762
Abstract Aims: Reliable evaluation of the severity and consequences of pulmonary regurgita‐ tion (PR) in patients with repaired tetralogy of Fallot (TOF) is crucial to timely identify the need for pulmonary valve intervention. We aimed to identify the accuracy of echocardiographic parameters to differentiate between moderate and severe PR, using phase contrast cardiac magnetic resonance imaging (CMR) as gold standard.
Methods and results: In this cross‐sectional study, 45 TOF patients with both echo‐ cardiographic and CMR measurements of PR were enrolled. All quantitative and semiquantitative echocardiographic measurements such as pressure half time (PHT), Color flow jet width (CFJW),… More >

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Rachel Wong¹, Mohammed Al‐Omary², David Baker², Neil Spratt^1,2, Andrew Boyle^1,2, Natasha Baker Cert¹, Peter Howe¹, Nicholas Collins²
Congenital Heart Disease, Vol.14, No.4, pp. 638-644, 2019, DOI:10.1111/chd.12763
Abstract Objectives: Improvements in the management of complex congenital heart disease, including those with single ventricle physiology, have resulted in increased survival. As this population ages, the recognition of cognitive impairment is increasingly im‐ portant. At present, little is known about the potential mechanisms of cognitive dys‐ function. In this cross‐sectional study, we aimed to characterize the nature of abnormalities in cerebral blood flow and the relationship to cognitive deficits in adults with single ventricular physiology.
Patients: Ten adults with single ventricular physiology (age 18‐40 years) and 12 age‐ and gender‐matched controls underwent transcranial Doppler ultrasound and ac‐… More >

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1122

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ARTICLE

Hongyun Ruan¹, Yigang Zhang², Ru Liu³, Xiangjun Yang¹
Congenital Heart Disease, Vol.14, No.4, pp. 645-650, 2019, DOI:10.1111/chd.12764
Abstract Objective: The optimal dose of Fasudil is still controversial in congenital heart disease accompanied with severe pulmonary hypertension (CHD‐PAH). This study aimed to compare acute hemodynamic changes after different doses of Fasudil in 60 consecu‐ tive adult patients with CHD‐PAH.
Design: Prospective randomized controlled trial.
Setting: Tertiary cardiology center.
Patients: Adult patients with CHD‐PAH.
Interventions: Patients were randomized to Fasudil 30 or 60 mg.
Outcome Measures: The hemodynamic parameters were measured at baseline and after 30 minutes of Fasudil through right cardiac catheterization. Blood gas results were obtained from the pulmonary artery, right ventricle, right atrium, superior and inferior vena cava,… More >

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1162

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James A. Kuo¹, Tyler Hamby², Maham N. Munawar³, Eldad Erez⁴, Vincent K. H. Tam⁵
Congenital Heart Disease, Vol.14, No.4, pp. 651-656, 2019, DOI:10.1111/chd.12765
Abstract Objective: Various options exist for right ventricular outflow tract (RVOT) recon‐ struction in congenital heart disease. The Freestyle porcine aortic root may be used but its longevity is not well defined.
Design: We performed a retrospective review of all non‐Ross RVOT reconstructions using the Freestyle root in our institution. Survival and reintervention, either by sur‐ gery, transcatheter valve implantation, balloon valvuloplasty, or bare metal stent placement, were recorded. Factors associated with reintervention were assessed using Cox regression.
Results: Between January 2002 and December 2015, there were 182 patients identi‐ fied. Sixteen patients were lost to follow‐up and 3… More >

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1164

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Alexander C. Egbe, William R. Miranda, Patricia A. Pellikka, Sorin V. Pislaru, Barry A. Borlaug, Srikanth Kothapalli, Sindhura Ananthaneni, Harigopal Sandhyavenu, Maria Najam, Mohamed Farouk Abdelsamid, Heidi M. Connolly
Congenital Heart Disease, Vol.14, No.4, pp. 657-664, 2019, DOI:10.1111/chd.12768
Abstract Background: We hypothesized that echocardiographic indices of right ventricular to pulmonary artery (RV‐PA) coupling were comparable to cardiac magnetic resonance imaging (CMRI)‐derived RV volumetric indices in predicting disease severity in chronic pulmonary regurgitation (PR).
Methods: Patients with ≥ moderate PR (2003‐2015) with and without prior CMRI scans were enrolled into the study cohort and validation cohort, respectively. Endpoint was to determine the association between noninvasive RV‐PA coupling in‐ dices (tricuspid annular plane systolic excursion/right ventricular systolic pressure [TAPSE/RVSP] and fractional area change [FAC]/RVSP ratio) and markers of disease severity, and compared this association to that of CMRI‐derived… More >

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1215

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Amy E. Delaney¹, Nina M. Dadlez², Audrey C. Marshall²
Congenital Heart Disease, Vol.14, No.4, pp. 665-670, 2019, DOI:10.1111/chd.12821
Abstract Background: In pediatric cardiac care, many centers participate in multiple, national, domain‐specific registries, as a major component of their quality assessment and im‐ provement efforts. Small cardiac programs, whose clinical activities and scale may not be well‐suited to this approach, need alternative methods to assess and track quality.
Methods: We conceived of and piloted a rapid‐approach cardiac quality assessment, intended to encompass multiple aspects of the service line, in a low‐volume program. The assessment incorporated previously identified measures, drawn from multiple sources, and ultimately relied on retrospective chart review.
Results: A collaborative, multidisciplinary team formed and came to… More >

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1105

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Alessia Callegari¹, Barbara Burkhardt¹, Christa Relly², Walter Knirsch¹, Martin Christmann¹
Congenital Heart Disease, Vol.14, No.4, pp. 671-677, 2019, DOI:10.1111/chd.12830
Abstract Background: Since routine clinical use of antibiotics as well as surgical and catheter‐ based closure of a patent arterial duct (PDA), PDA‐associated infective endarteritis (PDA‐IE) is rare but can still occur when the ductus is still open or as it closes. Thus, clinicians should maintain a high index of concern for patients with unexplained fever.
Methods: We report on a PDA‐IE in a young infant shortly after potentially delayed obliteration of a PDA. We discuss this case report by reviewing the literature in regard to the pathogenesis (infection primary or secondary to PDA thrombus formation), clinical (new… More >

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