Open Access

EDITORIAL

Douglas S. Moodie
Congenital Heart Disease, Vol.14, No.6, pp. 884-884, 2019, DOI:10.1111/chd.12865
Abstract This article has no abstract. More >

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1136

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ARTICLE

Christopher R. Broda, Katherine B. Salciccioli, Keila N. Lopez, Peter R. Ermis, Douglas S. Moodie, Heather A. Dickerson
Congenital Heart Disease, Vol.14, No.6, pp. 885-894, 2019, DOI:10.1111/chd.12856
Abstract Background: Heterotaxy syndrome (HS) is a condition in which the thoracoabdominal organs demonstrate an abnormal lateral arrangement and is often associated with con‐ genital heart disease (CHD). Little is known about the adult HS population with CHD.
Objective: To describe the outcomes and sociodemographics of the adult CHD popu‐ lation with HS.
Methods: Records of patients 18 years of age or older with diagnoses of both CHD and HS at Texas Children's Hospital from 1964 to 2018 were reviewed.
Results: Sixty‐two patients metinclusion criteria.Median agewas 22.7 [IQR19.6‐30.0] years; 26 (42%) were female; and 13 (21%) of patients had a gap… More >

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1207

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ARTICLE

Bradley Johnson^1,2, Matthew Buelow^1,2, Michael Earing^1,2, Scott Cohen^1,2, Peter Bartz^1,2, Salil Ginde^1,2
Congenital Heart Disease, Vol.14, No.6, pp. 895-900, 2019, DOI:10.1111/chd.12839
Abstract Objective: As adults with congenital heart disease (CHD) grow older, preoperative screening for coronary artery disease (CAD) may be indicated prior to CHD surgery. Data regarding the indications for preoperative CAD screening in this population are limited. Current practice is to follow guidelines for patients with valvular heart dis‐ ease; however, the risk for CAD in certain congenital heart diagnoses may be higher than the general population. This study aimed to assess the results of preoperative CAD screening in patients prior to CHD surgery.
Design: Retrospective study.
Setting: Single tertiary center.
Patients: Patients ≥35 years that had CHD surgery from… More >

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1037

Open Access

REVIEW

Mahmoud Alsalehi¹, Aamir Jeewa¹, Andrea Wan¹, Juan Contreras², Shi‐Joon Yoo³, Jessica A. Laks¹
Congenital Heart Disease, Vol.14, No.6, pp. 901-923, 2019, DOI:10.1111/chd.12842
Abstract Left main coronary artery ostial atresia (LMCAOA) is a rare congenital anomaly of the coronary arteries. The published literature regarding the current diagnostic and manage‐ ment recommendations are limited. We present three case series of LMCAOA from our institution, including one with a unique association with anomalous origin of left coronary artery (LCA) from pulmonary artery. In addition, this report includes a review of 50 pedi‐ atric and 43 adult cases from literature. The majority of the patients were symptomatic. Sudden cardiac death occurred in 10% of pediatric patients and 7% of adult patients. Almost half of pediatric patients had… More >

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1123

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ARTICLE

Michael A. Brock, John‐Anthony Coppola, Jana Reid, Diego Moguillansky
Congenital Heart Disease, Vol.14, No.6, pp. 924-930, 2019, DOI:10.1111/chd.12857
Abstract Objective: The primary aim of our work is to determine the incidence of atrial fibrillation following cardiac surgery in adults with congenital heart disease. Secondary aims include identifying risk factors predictive of developing early postoperative atrial fibrillation and morbidities associated with early postoperative atrial fibrillation.
Design: Retrospective analysis.
Setting: Single center, quaternary care children’s hospital.
Patients: This review included patients at least 18 years of age with known congenital heart disease who underwent cardiac surgery requiring a median sternotomy at our congenital heart center from January 1, 2012 to December 31, 2016.
Interventions: None.
Outcome Measures: The primary outcome was… More >

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1020

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ARTICLE

Benjamin Zielonka¹, Yuli Y. Kim^2,3, Gregory E. Supple², Sara L. Partington^2,3, Emily S. Ruckdeschel^2,3, Francis E. Marchlinski², David S. Frankel²
Congenital Heart Disease, Vol.14, No.6, pp. 931-938, 2019, DOI:10.1111/chd.12833
Abstract Objective: Atrial arrhythmias and atrioventricular valve regurgitation (AVVR) are common causes of morbidity among adults with congenital heart disease (ACHD). The impact of rhythm control on AVVR in this population is unknown. We sought to determine whether a rhythm control strategy is associated with greater freedom from AV valve surgery than a rate control strategy.
Design: Patients evaluated by both ACHD and electrophysiology specialists at a single academic center were screened for atrial arrhythmias and at least moderate‐severe AVVR. Clinical and electrographic data were abstracted. All echocardiograms were in‐ terpreted by a single echocardiographer blinded to treatment strategy. Patients were… More >

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1029

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ARTICLE

Shihai Wang, Jun’an Pan, Bei Xiao, Yongjiang Tang, Jianjun Lan, Xuezhong Zheng, Chao Yang, Dawen Xu, Jiyu Zhang
Congenital Heart Disease, Vol.14, No.6, pp. 939-944, 2019, DOI:10.1111/chd.12844
Abstract Objectives: We sought to evaluate the safety and efficacy in improving cardiac function and functional capacity with device closure of large atrial septal defects (ASD) in senior adults.
Background: Atrial septal defect accounts for about 10% of all congenital heart dis‐ ease. It still remains unclear whether large ASD closure in senior people should be performed or not. Hence we aim to prospectively assess the safety and clinical status of senior patients after transcatheter closure in large ASD.
Patients and interventions: This was a prospective study of all patients aged over 50 years who underwent device closure of a secundum… More >

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1347

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1028

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ARTICLE

Anna‐Luisa Häcker^1,2, Renate Oberhoffer^1,2, Alfred Hager¹, Peter Ewert¹, Jan Müller^1,2
Congenital Heart Disease, Vol.14, No.6, pp. 945-951, 2019, DOI:10.1111/chd.12845
Abstract Aims: Age‐related cardiovascular diseases are a relevant risk in the aging population of adults with congenital heart diseases (ACHD). Risk factors such as the metabolic syndrome (MetS) impact the risk of increased carotid intima‐media thickness (cIMT) and thereby long‐term cardiovascular diseases. The aim of the study was to assess MetS in ACHD and outline a possible association to cIMT.
Methods and Results: In total, 512 ACHD (43.0 ± 9.6 years, 48.9% female) were screened for MetS by the standards of the International Diabetes Federation, and their cIMT by ultrasound from January 2017 to June 2019. MetS was prevalent in 72… More >

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1008

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ARTICLE

Jim T. Vehmeijer¹, Zeliha Koyak¹, A. Suzanne Vink¹, Werner Budts^2,3, Louise Harris⁴, Candice K. Silversides⁴, Erwin N. Oechslin⁴, Aeilko H. Zwinderman⁵, Barbara J.M. Mulder^1,6, Joris R. de Groot¹
Congenital Heart Disease, Vol.14, No.6, pp. 952-957, 2019, DOI:10.1111/chd.12847
Abstract Objective: Adult congenital heart disease (ACHD) patients are at risk of sudden cardiac death (SCD). However, methods for risk stratification are not yet well‐ defined. The T_peak‐T_end (TpTe) interval, a measure of dispersion of ventricular repolari‐ zation, is a risk factor for SCD in non‐ACHD patients. We aim to evaluate whether TpTe can be used in risk stratification for SCD in ACHD patients.
Design: From an international multicenter cohort of 25 790 ACHD patients, we iden‐ tified all SCD cases. Cases were matched to controls by age, gender, congenital de‐ fect, and (surgical) intervention.
Outcome Measures: TpTe was measured on… More >

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1113

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ARTICLE

Ari Cedars¹, Luke Burchill², S. Lucy Roche³, Jonathan Menachem⁴, Kelly Axsom⁵, Kristen Tecson⁶
Congenital Heart Disease, Vol.14, No.6, pp. 958-962, 2019, DOI:10.1111/chd.12851
Abstract Background: There are no published data on post‐transplant outcomes in durable ven‐ tricular assist device (VAD)‐supported adult congenital heart disease (ACHD) patients.
Methods: We compared post‐transplant outcomes in VAD‐supported vs non‐VAD‐ supported ACHD patients using the Scientific Registry of Transplant Recipients.
Results: At 1 year, there was no difference in post‐transplant mortality between VAD‐supported (12 patients) and non‐VAD‐supported (671 patients) ACHD patients.
Conclusions: In appropriate ACHD patients, VAD use as a bridge to transplant is a reasonable strategy. More >

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994

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ARTICLE

Dana Y. Fuhrman^1,2, Lan Nguyen³, Morgan Hindes³, John A. Kellum²
Congenital Heart Disease, Vol.14, No.6, pp. 963-967, 2019, DOI:10.1111/chd.12862
Abstract Background: There are significant implications for kidney disease in young adults with congenital heart disease. Prior investigations have not focused on the use of urinary tubular biomarkers for the early identification of kidney disease in this growing patient group.
Objective: Determine if young adults with congenital heart disease have differences in the baseline concentration of urinary tubular biomarkers when compared to healthy young adults.
Design/Methods: In a pilot case control study, 30 patients from 18 to 35 years of age with congenital heart disease and a normal serum creatinine were recruited during a routine follow-up visit. In the same age… More >

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1049

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ARTICLE

Uri Pollak^1,2,3,4,*, Tatyana Ruderman^5,*, Sharon Borik‐Chiger^5,6, David Mishaly^5,7, Alain Serraf^5,7, Amir Vardi^5,8
Congenital Heart Disease, Vol.14, No.6, pp. 968-977, 2019, DOI:10.1111/chd.12825
Abstract Objective: The final common pathway of single ventricle patients is the Fontan procedure. Among the immediate postoperative complications is acute hepatic injury presented by marked elevation of liver enzymes (alanine transaminase [ALT] and aspartate transaminase [AST]). We aimed to determine the contribution of blood products transfusion to acute hepatic injury.
Design: Single center retrospective cohort study.
Setting: Pediatric Cardiac Intensive Care Unit at a tertiary medical center.
Patients: Ninety‐nine pediatric patients undergoing the Fontan procedure between January 2009 and December 2016.
Interventions: None.
Measurements and Main Results: Out of the four types of blood products, transfusion of platelets was found… More >

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1674

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1063

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ARTICLE

Alexander Lemmer¹, Lisa VanWagner^1,2, Zaira Gasanova³, Steve Helmke^4,5, Gregory T. Everson^4,5, Daniel Ganger¹
Congenital Heart Disease, Vol.14, No.6, pp. 978-986, 2019, DOI:10.1111/chd.12831
Abstract Background & Aims: Fontan surgery for single ventricle congenital heart disease leads to Fontan‐associated liver disease (FALD). Typical laboratory tests, imaging, and histopathology cannot predict clinical severity in FALD. HepQuant SHUNT is a proprietary serum test of hepatic function and physiology that has not yet been evaluated in FALD.
Methods: Fourteen adult FALD patients at a single urban tertiary care center who underwent a Fontan procedure in childhood received HepQuant SHUNT testing between September 2015 and April 2018. The HepQuant SHUNT disease severity index (DSI) assesses global liver function and physiology from systemic and portal hepatic filtration rates (HFRs, clearances… More >

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1059

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ARTICLE

Paolo D’Ambrosio^1,2, Derek Tran^1,2, Charlotte E. Verrall^3,4, Chantal Attard⁵, Maria Fiatarone Singh^6,7, Julian Ayer^3,4,8, Yves d’Udekem^5,9,10, Stephen Twigg^2,11, David S. Celermajer^1,2,12, Rachael Cordina^1,2,5
Congenital Heart Disease, Vol.14, No.6, pp. 987-995, 2019, DOI:10.1111/chd.12836
Abstract Objective and Patients: This study aimed to characterize bone mineral density abnormalities and pathophysiological associations in young adults living with a Fontan circulation.
Design: Participants underwent bone mineral density measurement using dual‐energy X‐ray absorptiometry and serum biochemical analysis, cardiopulmonary exercise and strength testing and transthoracic echocardiography.
Results: In our cohort (n = 28), 29% had osteopenic‐range bone mineral density and one patient was osteoporotic (average hip t score: −0.6 ± 1.1; spine t score: −0.6 ± 0.9). Four patients (14%) had z scores < −2.0. Parathyroid hormone levels were increased compared with laboratory median (6.1 ± 3.5 vs 4 pmol/L,… More >

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1482

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998

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ARTICLE

Simone Goa Diab¹, Kristin Godang², Lil‐Sofie Ording Müller³, Runar Almaas⁴, Charlotte de Lange³, Leif Brunvand¹, Kari Margrethe Hansen¹, Anne Grethe Myhre⁵, Gaute Døhlen¹, Erik Thaulow^1,6, Jens Bollerslev^2,6, Thomas Möller¹
Congenital Heart Disease, Vol.14, No.6, pp. 996-1004, 2019, DOI:10.1111/chd.12848
Abstract Objective: We investigated bone mineral density (BMD) at different ages after the Fontan completion, and we evaluated the relationship between BMD, vitamin D levels, and pertinent patient variables.
Methods: A cross‐sectional sample of 64 patients was examined with dual‐energy X‐ray absorptiometry (DXA) scans to determine BMD. Of these patients, 24 were also examined with BoneXpert software to determine bone mass density (BMX), expressed as the bone health index (BHI). Blood samples from all patients were analyzed. Patients were divided into three different age groups; A: 4‐9 years old (n = 22), B: 10‐15 years old (n = 21), and C:… More >

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1021

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ARTICLE

Tim Takken¹, Alyanne Evertse¹, Fleur de Waard¹, Mandy Spoorenburg¹, Martijn Kuijpers¹, Christian Schroer^2,3, Erik H. Hulzebos¹
Congenital Heart Disease, Vol.14, No.6, pp. 1005-1012, 2019, DOI:10.1111/chd.12850
Abstract Background: Traveling to high altitude has become more popular. High‐altitude exposure causes hypobaric hypoxia. Exposure to acute high altitude, during air travel or mountain stays, seems to be safe for most patients with congenital heart disorders (CHD). Still, current guidelines for CHD patients express concerns regarding safety of altitude exposure for patients with a Fontan circulation. Therefore, investigating hemodynamic and pulmonary responses of acute high‐altitude exposure (±2500 m) at rest and during maximal exercise in patients with Fontan circulation can provide clarity in this dispute and may contribute to improvement of clinical counseling.
Methods: Twenty‐one Fontan patients with 21 age‐matched… More >

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1030

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ARTICLE

Brooke T. Davey¹, Olga H. Toro‐Salazar¹, Naomi Gauthier^2,3, Anne Marie Valente², Robert W. Elder⁴, Fred M. Wu², Norman Berman³, Phyllis Pollack⁵, Ji Hyun Lee¹, Rahul H. Rathod²
Congenital Heart Disease, Vol.14, No.6, pp. 1013-1023, 2019, DOI:10.1111/chd.12854
Abstract Introduction: Surveillance and management guidelines for Fontan patients are lacking due to the paucity of evidence in the literature of screening efficacy on outcome measures.
Methods: The Fontan Working Group within the New England Congenital Cardiology Association designed an electronic survey to assess surveillance practices for pa‐ tients with Fontan procedures among New England congenital cardiologists and to explore variability in screening low‐risk vs high‐risk Fontan patients across regional programs.
Results: Fifty‐six cardiologists representing 12 regional programs responded to the survey, comprising ~40% of the total New England congenital cardiac physicians. The majority of desired testing and consultation was available… More >

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1037

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ARTICLE

Aura A. Sanchez^1,2, Sara K. Sexson Tejtel¹, Myriam E. Almeida‐Jones^1,3, Douglas K. Feagin¹, Carolyn A. Altman¹, Ricardo H. Pignatelli¹
Congenital Heart Disease, Vol.14, No.6, pp. 1024-1031, 2019, DOI:10.1111/chd.12787
Abstract Objective: Children with Kawasaki disease (KD) with persistent coronary artery aneurysms (CAAs) can develop chronic vasculopathy and subsequent myocardial ischemia. Early detection of this process is challenging. Myocardial deformation anal‐ ysis can detect early alterations in myocardial performance. We aim to determine whether there are differences in myocardial deformation between KD patients with and without CAAs.
Design: This is a cross‐sectional study of 123 echocardiograms performed on 103 children with KD. Myocardial deformation was measured with two‐dimensional speckle tracking (2DSTE). The echocardiograms were divided into groups according to the KD phase in which they were performed: acute, subacute, and convalescent/… More >

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1029

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ARTICLE

Varun Aggarwal^1,2, Kristen Sexson-Tejtal¹, Wilson Lam¹, Santiago O. Valdes¹, Caridad M. de la Uz¹, Jeffrey J. Kim¹, Christina Y. Miyake¹
Congenital Heart Disease, Vol.14, No.6, pp. 1032-1036, 2019, DOI:10.1111/chd.12864
Abstract Objective: Based on 2017 guidelines, participation in competitive sports with prior history of Kawasaki Disease (KD) requires those with coronary artery aneurysms (CAA) z score ≥ 5 to undergo evaluation for evidence of inducible ischemia or arrhythmias. The use of exercise stress testing (EST) to evaluate arrhythmias among KD patients has never been reported. This retrospective single-center case series study sought to describe the presence of inducible arrhythmias during EST in KD patients with or without CAA.
Methods: Single-center retrospective review of medical records of patients diagnosed with KD between 1989-2015 at Texas Children’s Hospital, Houston, Texas who underwent EST… More >

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1084

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ARTICLE

Rachel L. Hansen^1,2, Iman Naimi³, Hongyue Wang¹, Nader Atallah^3,4, Frank Smith^3,4, Craig Byrum^3,4, Daniel Kveselis^3,4, Glenn Leonard^1,5, Rajiv Devanagondi¹, Matthew Egan^3,4
Congenital Heart Disease, Vol.14, No.6, pp. 1037-1045, 2019, DOI:10.1111/chd.12788
Abstract Objective: Evaluate long‐term outcomes following balloon pulmonary valvuloplasty (BPV) for pulmonary stenosis (PS).
Background: Long‐term data following BPV is limited to small, single center studies.
Methods: BPV from April 12, 1985 to January 7, 2015 from three centers were included. Outcomes studied were ≥ moderate PI by echocardiogram and residual PS ≥ 40 mm Hg. Risk factors for ≥ moderate PI, residual PS, and repeat intervention were assessed by univariate and multivariate analysis.
Results: Among 254 patients, mean age at BPV was 3.8 years (range 1 day‐67 years), initial PS catheter gradient was 56 mm Hg (IQR 40‐70), 19% had… More >

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1213

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ARTICLE

Sok‐Leng Kang¹, Aimee Armstrong², Gregor Krings³, Lee Benson¹
Congenital Heart Disease, Vol.14, No.6, pp. 1046-1057, 2019, DOI:10.1111/chd.12838
Abstract Three‐dimensional rotational angiography (3D‐RA) enables volumetric imaging through rotation of the C‐arm of an angiographic system and real‐time 3D recon‐ struction during cardiac catheterization procedures. In the field of congenital heart disease (CHD), 3D‐RA has gained considerable traction, owing to its capability for en‐ hanced visualization of spatial relationships in complex cardiac morphologies and real time image guidance in an intricate interventional environment. This review provides an overview of the current applications, strengths, and limitations of 3D‐RA acquisi‐ tion in the management of CHD and potential future directions. In addition, issues of dosimetry, radiation exposure, and optimization strategies will be… More >

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1138

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ARTICLE

Arene Butto¹, Laura Mercer‐Rosa¹, Christopher Teng¹, Carrie Daymont², Jonathan Edelson¹, Jennifer Faerber¹, Erika Mejia¹, Chitra Ravishankar¹, Meryl S. Cohen¹
Congenital Heart Disease, Vol.14, No.6, pp. 1058-1065, 2019, DOI:10.1111/chd.12843
Abstract Objective: Children with single ventricle cardiac disease (SVCD) have poor growth in early life. Tube‐assisted feeding (TF) is used to improve weight gain, but its impact on long‐term growth remains unknown. We sought to compare the longitudinal growth of SVCD patients receiving TF after initial cardiac surgery with those fed entirely by mouth.
Design: We conducted a retrospective cohort study of SVCD patients who under‐ went initial surgical palliation between 1999 and 2009. We defined TF as the use of nasogastric, gastrostomy, or jejunostomy TF. We compared maximal attained growth z‐scores for each year of life between TF and non‐TF… More >

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1027

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ARTICLE

Sara Bobillo‐Perez^1,2, Joan Sanchez‐de‐Toledo^3,4, Susana Segura², Monica Girona‐Alarcon², Maria Mele⁵, Anna Sole‐Ribalta², Debora Cañizo Vazquez⁶, Iolanda Jordan^2,7, Francisco Jose Cambra^1,2
Congenital Heart Disease, Vol.14, No.6, pp. 1066-1077, 2019, DOI:10.1111/chd.12846
Abstract Objective: Three scores have been proposed to stratify the risk of mortality for each cardiac surgical procedure: The RACHS‐1, the Aristotle Basic Complexity (ABC), and the STS‐EACTS complexity scoring model. The aim was to compare the ability to predict mortality and morbidity of the three scores applied to a specific population.
Design: Retrospective, descriptive study.
Setting: Pediatric and neonatal intensive care units in a referral hospital.
Patients: Children under 18 years admitted to the intensive care unit after surgery.
Interventions: None.
Outcome measures: Demographic, clinical, and surgical data were assessed. Morbidity was considered as prolonged length of stay (LOS >… More >

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1058

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ARTICLE

Christine M. Riley¹, Christopher W. Mastropietro², Peter Sassalos³, Jason R. Buckley⁴, John M. Costello⁴, Ilias Iliopoulos⁵, Aimee Jennings⁶, Katherine Cashen⁷, Sukumar Suguna Narasimhulu⁸, Keshava M. N. Gowda⁹, Arthur J. Smerling¹⁰, Michael Wilhelm¹¹, Aditya Badheka¹², Adnan Bakar^13,14, Elizabeth A. S. Moser¹⁵, Venu Amula¹⁶
Congenital Heart Disease, Vol.14, No.6, pp. 1078-1086, 2019, DOI:10.1111/chd.12849
Abstract Background: Elevated pulmonary vascular resistance (PVR) is common following repair of truncus arteriosus. Inhaled nitric oxide (iNO) is an effective yet costly therapy that is frequently implemented postoperatively to manage elevated PVR.
Objectives: We aimed to describe practice patterns of iNO use in a multicenter cohort of patients who underwent repair of truncus arteriosus, a lesion in which recovery is often complicated by elevated PVR. We also sought to identify patient and center factors that were more commonly associated with the use of iNO in the postoperative period.
Design: Retrospective cohort study.
Setting: 15 tertiary care pediatric referral centers.
Patients:… More >

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1000

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ARTICLE

Masashi Nishida, Shingo Kubo, Yuma Morishita, Kosuke Nishikawa, Kazuyuki Ikeda, Toshiyuki Itoi, Hajime Hosoi
Congenital Heart Disease, Vol.14, No.6, pp. 1087-1093, 2019, DOI:10.1111/chd.12853
Abstract Objective: This study aims to investigate the changes in renal function and levels of urinary biomarkers before and after cardiac angiography in children with congenital heart disease (CHD).
Setting: Children with CHD are at a risk for kidney injury during contrast exposure in cardiac angiography.
Outcome Measures: We measured urinary protein, albumin, N‐acetyl‐β‐D‐glucosaminidase (NAG), β2‐microglobulin (BMG), and liver‐type fatty acid‐binding protein (L‐FABP) levels, as well as serum creatinine and cystatin C levels, before and after cardiac angiography in 33 children with CHD.
Results: No significant decrease was noted in either the creatinine‐based or cystatin C‐based estimated glomerular filtration rate at… More >

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1031

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ARTICLE

Natasa Karadzov Orlic¹, Amira Egic¹, Barbara Damnjanovic‐Pazin MD2 | Relja Lukic¹, Ivana Joksic³, Zeljko Mikovic¹
Congenital Heart Disease, Vol.14, No.6, pp. 1094-1101, 2019, DOI:10.1111/chd.12852
Abstract Objective: The objective of this study was to analyze if the addition of simple cardiac scan in cases with increased nuchal translucency (NT) and/or abnormal ductus venosus (DV) blood flow, and/or tricuspid regurgitation (TCR) can improve detection of congenital heart defects (CHD) in chromosomally normal fetuses without non‐ cardiac defects at 11‐13 + 6 gestational weeks in a population of singleton pregnancies.
Methods: During the 10 years period, all singleton pregnancies at 11‐13 + 6 weeks were routinely scanned for NT, DV blood flow and TCR assessment and, if a sin‐ gle of these parameters was abnormal, simple cardiac scan… More >

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1331

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999

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ARTICLE

Alessandro Sgrò¹, Thomas M. Drake², Pedro Lopez‐Ayala³, Kevin Phan⁴
Congenital Heart Disease, Vol.14, No.6, pp. 1102-1112, 2019, DOI:10.1111/chd.12855
Abstract Background: Left cardiac sympathetic denervation (LCSD) has been proposed as useful therapy for long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT), in addition to anti‐arrhythmic agents and implant‐ able cardioverter defibrillators. This study aimed to assess the current evidence for LCSD and compare the open vs the video‐assisted thoracoscopic surgery (VATS) approaches.
Methods: MEDLINE, Embase and Cochrane library databases were searched up to December 2018 for studies reporting the long‐term outcomes of LCSD in LQTS, CPVT patients. The incidence of cardiac events (CEs) before and after surgery, the change in QTc interval, and surgical complications were pooled to… More >

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1449

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994

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ARTICLE

Nadya Golfenshtein¹, Alexandra L. Hanlon², Janet A. Deatrick³, Barbara Medoff‐Cooper^3,4
Congenital Heart Disease, Vol.14, No.6, pp. 1113-1122, 2019, DOI:10.1111/chd.12858
Abstract Objective: Parents of infants with congenital heart disease (CHD) experience increased parenting stress levels, potentially interfering with parenting practices and bear adverse family outcomes. Condition severity has been linked to parenting stress. The current study aimed to explore parenting stress trajectories over infancy in parents of infants with complex CHD, and to compare them by post‐operative cardiac physiology.
Design: Data from a larger prospective cohort study was analyzed using longitudinal mixed‐effects regression modeling.
Setting: Cardiac intensive care unit and outpatient clinic of a 480‐bed children's hospital in the American North‐Atlantic region.
Participants: Parents of infants with complex CHD (n =… More >

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1048

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ARTICLE

Reinder Evertz¹, Charlotte A. Houck², Tim ten Cate¹, Anthonie L. Duijnhouwer¹, Rypko Beukema¹, Sjoerd Westra¹, Kevin Vernooy ^1,3, Natasja M. S. de Groot²
Congenital Heart Disease, Vol.14, No.6, pp. 1123-1129, 2019, DOI:10.1111/chd.12859
Abstract Background: Patients with an atrial septal defect (ASD) are at increased risk of de‐ veloping atrial fibrillation (AF). Currently percutaneous ASD closure is the preferred therapeutic strategy and although pulmonary vein isolation (PVI) for AF is feasible after ASD closure, the transseptal puncture can be technically challenging and prob‐ ably increases the perioperative risk. A staged approach, with PVI several months be‐ fore ASD closure, has been recommended for patients already scheduled for closure, but no data are available on combined procedures.
Purpose: This pilot study evaluates the feasibility of a combined procedure of PVI and ASD closure in patients… More >

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1055

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ARTICLE

Varun Aggarwal^1,2, Sebastian C. Tume³, Marco Rodriguez¹, Iki Adachi⁴, Antonio G. Cabrera¹, Hari Tunuguntla¹, Athar M. Qureshi¹
Congenital Heart Disease, Vol.14, No.6, pp. 1130-1137, 2019, DOI:10.1111/chd.12860
Abstract Objective: Predictors of right ventricle (RV) dysfunction after continuous‐flow left ventricular assist device (CF‐LVAD) implantation in children are not well described. We explored the association of preimplantation Pulmonary Artery Pulsatility index (PAPi) and other hemodynamic parameters as predictors of prolonged postoperative inotropes/pulmonary vasodilator use after CF‐LVAD implantation.
Design: Retrospective chart review.
Setting: Single tertiary care pediatric referral center.
Patients: Patients who underwent CF‐LVAD implantation from January 2012 to October 2017.
Interventions: Preimplantation invasive hemodynamic parameters were analyzed to evaluate the association with post‐CF‐LVAD need for prolonged (>72 hours) use of inotropes/pulmonary vasodilators.
Measurements and main results: Preimplantation cardiac catheterization data… More >

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1448

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1104

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ARTICLE

Margaret M. Samyn^1,2, Ke Yan¹, Conor Masterson³, Benjamin H. Goot^1,2, David Saudek^1,2, Julie Lavoie², Aaron Kinney², Mary Krolikowski¹, Kan Hor^4,5, Scott Cohen^1,2
Congenital Heart Disease, Vol.14, No.6, pp. 1138-1148, 2019, DOI:10.1111/chd.12861
Abstract Objective: Patients with Dextro-transposition of the great arteries status post atrial switch (dTGA s/p atrial switch) are “at-risk” for systemic right ventricular (RV) dysfunction. Due to complex RV geometry, echocardiography (Echo) does not allow accurate determination of ejection fraction (EF), but cardiac magnetic resonance imaging (CMR) allows quantitative right ventricular assessment. Measures of ventricular deformation may be precursors to global ventricular dysfunction. The primary aim of this study was to characterize imaging and clinical findings for adult patients with dTGA s/p atrial switch.
Design: This was a retrospective cohort study of patients with dTGA s/p atrial switch operation (February 1966… More >

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1094

Open Access

ARTICLE

Mary K. Olive¹, Charles D. Fraser², Shelby Kutty³, Emmett D. McKenzie⁴, James M. Hammel⁵, Rajesh Krishnamurthy⁶, Nicolas A. Dodd⁷, Shiraz A. Maskatia⁸
Congenital Heart Disease, Vol.14, No.6, pp. 1149-1156, 2019, DOI:10.1111/chd.12863
Abstract Introduction: The right ventricular infundibular sparing approach (RVIS) to the repair of tetralogy of Fallot (TOF) avoids a full-thickness ventricular incision, typically utilized in the transinfundibular (TI) method.
Methods: We performed a retrospective, age-matched cohort study of patients who underwent RVIS at Texas Children’s Hospital or TI at Children’s Hospital Medical Center in Nebraska and subsequently underwent cardiac magnetic resonance imaging (CMR). We compared right ventricular end-diastolic and systolic volumes indexed to body surface area (RVEDVi and RVESVi) and right ventricular ejection fraction (RVEF) as primary endpoints. Secondary endpoints were indexed left ventricular diastolic and systolic volume (LVEDVi and LVESVi),… More >

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1052

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ARTICLE

Nicholas Y. Tan¹, Christine H. Attenhofer Jost¹, Murray D. Polkinghorne¹, Emily R. Vargas², David O. Hodge², Joseph A. Dearani³, Samuel J. Asirvatham^1,4, Heidi M. Connolly¹, Christopher J. McLeod¹
Congenital Heart Disease, Vol.14, No.6, pp. 1157-1165, 2019, DOI:10.1111/chd.12841
Abstract Introduction: Mechanisms and risk factors for cerebrovascular accidents (CVAs) in Ebstein’s anomaly (EA) are not well understood; hence, we aimed to clarify these in a large cohort of EA patients.
Methods: Patients with a confirmed diagnosis of EA were retrospectively reviewed. Baseline characteristics were compared between patients with and without a prior history of CVA using logistic regression modeling. Cox regression analysis was used to identify predictors of CVA following initial evaluation. CVA incidence from birth and following tricuspid valve surgery were estimated using the Kaplan‐Meier method.
Results: Nine hundred sixty‐eight patients (median age 21.1 years, 41.5% male) were included,… More >

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996

Open Access

REVIEW

Munes Fares¹, Paul J. Critser², Maria J. Arruda¹, Carolyn M. Wilhelm¹, Mantosh S. Rattan³, Sean M. Lang^2,4, Tarek Alsaied^2,4
Congenital Heart Disease, Vol.14, No.6, pp. 1166-1175, 2019, DOI:10.1111/chd.12840
Abstract Pharmacologic stress cardiovascular magnetic resonance (PSCMR) is a wellestablished and reliable diagnostic tool for evaluation of coronary artery disease in the adult population. Stress imaging overall and PSCMR in particular is less utilized in the pediatric population with limited reported data. In this review, we highlight the potential use of PSCMR in specific pediatric cohorts with congenital and acquired heart disease, and we review the reported experience. A suggested protocol is presented in addition to two case examples of patients with Kawasaki disease where PSCMR aided decision making. More >

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992

Open Access

ARTICLE

Rohit S. Loomba¹, Saul Flores²
Congenital Heart Disease, Vol.14, No.6, pp. 1176-1184, 2019, DOI:10.1111/chd.12837
Abstract Objective: The main goal of this study is to examine the variation in vasoactive agent prescription patterns across a large cohort of patients. In addition, we sought to determine the association between the number of vasoactive agents used during admissions and characteristics of admissions utilizing varying numbers of vasoactive agents.
Methods: This was a multi-institutional, cross-sectional study of the pediatric health information system database of patients who underwent congenital heart surgery and received vasoactive agents from 2004 to 2015. The international classification of disease-9 (ICD-9) codes were used to select admissions to those only pertaining to cardiac patients. The vasoactive… More >

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958

Open Access

ARTICLE

Andrew S. Kim¹, Colleen M. Witzenburg², Mark Conaway³, Jeffrey E. Vergales¹, Jeffrey W. Holmes^2,4, Thomas J. L’Ecuyer¹, Peter N. Dean¹
Congenital Heart Disease, Vol.14, No.6, pp. 1185-1192, 2019, DOI:10.1111/chd.12834
Abstract Background: Children with hypoplastic left heart syndrome (HLHS) have risk for mortality and/or transplantation. Previous studies have associated right ventricular (RV) indices in a single echocardiogram with survival, but none have related serial measurements to outcomes. This study sought to determine whether the trajectory of RV indices in the first year of life was associated with transplant‐free survival to stage 3 palliation (S3P).
Methods: HLHS patients at a single center who underwent stage 1 palliation (S1P) between 2000 and 2015 were reviewed. Echocardiographic indices of RV size and function were obtained before and following S1P and stage 2 palliation (S2P).… More >

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980

Open Access

ARTICLE

Jeremy M. Steele^1,2, Rukmini Komarlu², Sarah Worley³, Tarek Alsaied¹, Christopher Statile¹, Francine G. Erenberg²
Congenital Heart Disease, Vol.14, No.6, pp. 1193-1198, 2019, DOI:10.1111/chd.12829
Abstract Objective: Deciding on a surgical pathway for neonates with ≥2 left heart obstructive lesions is complex. Predictors of the successful biventricular (2V) repair in these patients are poorly defined. The goal of our study was to identify patients who underwent the 2V repair and assess anatomic and echocardiographic predictors of success.
Design: Infants born between July 2015 and August 2017 with ≥2 left heart obstructive lesions with no prior interventions were identified (n = 19). Patients with aortic or mitral valve (MV) atresia and critical aortic stenosis were excluded. Initial echocardiograms were reviewed for aortic, MV, tricuspid valve annulus size,… More >

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971

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ARTICLE

Andrew D. Spearman¹, Steven J. Kindel¹, Ronald K. Woods², Salil Ginde^1,3
Congenital Heart Disease, Vol.14, No.6, pp. 1199-1206, 2019, DOI:10.1111/chd.12828
Abstract Background: Hypoxia is a common and sometimes severe morbidity of single ven‐ tricle congenital heart disease (CHD). Creation of an arteriovenous fistula (AVF) is occasionally performed for patients after superior or total cavopulmonary connec‐ tion (SCPC or TCPC) in an attempt to improve oxygen saturations. Despite previ‐ ous reports, AVF creation is a rare palliation with inadequately defined benefits and risks. We sought to determine changes in peripheral oxygen saturation (SpO₂) and risk of adverse event after AVF creation in children with single ventricle CHD at our institution.
Methods: We conducted a retrospective chart review of patients with a history… More >

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967

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ARTICLE

Line Marie Holst¹, Faridis Serrano², Lara Shekerdemian², Hanne Berg Ravn¹, Danielle Guffey³, Nancy S. Ghanayem², Sonia Monteiro⁴
Congenital Heart Disease, Vol.14, No.6, pp. 1207-1213, 2019, DOI:10.1111/chd.12827
Abstract Objective: To investigate the impact of feeding mode on neurodevelopmental outcomes in children with congenital heart defects.
Design: A retrospective cohort study of 208 children with congenital heart disease (CHD), who had surgery from 1 January 2013 until 31 December 2016 at Texas Children’s Hospital, Houston, TX, US.
Settings: University Hospital, Developmental Outcome Clinic.
Outcomes measures: Standardized cognitive scores were assessed with Capute Scales and motor development with Revised Gesell Developmental Schedules. We analyzed anthropometrics, mode of feeding, surgical complexity, syndrome, and gen‐ der as predictors of developmental outcomes at four time points: hospital discharge, and 6, 12, and 24… More >

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979

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CORRIGENDUM

Congenital Heart Disease, Vol.14, No.6, pp. 1214-1214, 2019, DOI:10.1111/chd.12775
Abstract This article has no abstract. More >

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864