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 >
Open Access
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 >
Open Access
ARTICLE
Bradley Johnson1,2, Matthew Buelow1,2, Michael Earing1,2, Scott Cohen1,2, Peter Bartz1,2, Salil Ginde1,2
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 >
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 >
Open Access
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 >
Open Access
ARTICLE
Benjamin Zielonka1, Yuli Y. Kim2,3, Gregory E. Supple2, Sara L. Partington2,3, Emily S. Ruckdeschel2,3, 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 >
Open Access
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 >
Open Access
ARTICLE
Anna‐Luisa Häcker1,2, Renate Oberhoffer1,2, Alfred Hager1, Peter Ewert1, Jan Müller1,2
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 >
Open Access
ARTICLE
Jim T. Vehmeijer1, Zeliha Koyak1, A. Suzanne Vink1, Werner Budts2,3, Louise Harris4, Candice K. Silversides4, Erwin N. Oechslin4, Aeilko H. Zwinderman5, Barbara J.M. Mulder1,6, 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 Tpeak‐Tend (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 >
Open Access
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 >
Open Access
ARTICLE
Dana Y. Fuhrman1,2, 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 >
Open Access
ARTICLE
Uri Pollak1,2,3,4,*, Tatyana Ruderman5,*, Sharon Borik‐Chiger5,6, David Mishaly5,7, Alain Serraf5,7, Amir Vardi5,8
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 >
Open Access
ARTICLE
Alexander Lemmer1, Lisa VanWagner1,2, Zaira Gasanova3, Steve Helmke4,5, Gregory T. Everson4,5, 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 >
Open Access
ARTICLE
Paolo D’Ambrosio1,2, Derek Tran1,2, Charlotte E. Verrall3,4, Chantal Attard5, Maria Fiatarone Singh6,7, Julian Ayer3,4,8, Yves d’Udekem5,9,10, Stephen Twigg2,11, David S. Celermajer1,2,12, Rachael Cordina1,2,5
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 >
Open Access
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 Thaulow1,6, Jens Bollerslev2,6, 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 >
Open Access
ARTICLE
Tim Takken1, Alyanne Evertse1, Fleur de Waard1, Mandy Spoorenburg1, Martijn Kuijpers1, Christian Schroer2,3, 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 >
Open Access
ARTICLE
Brooke T. Davey1, Olga H. Toro‐Salazar1, Naomi Gauthier2,3, 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 >
Open Access
ARTICLE
Aura A. Sanchez1,2, Sara K. Sexson Tejtel1, Myriam E. Almeida‐Jones1,3, 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 >
Open Access
ARTICLE
Varun Aggarwal1,2, 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 >
Open Access
ARTICLE
Rachel L. Hansen1,2, Iman Naimi3, Hongyue Wang1, Nader Atallah3,4, Frank Smith3,4, Craig Byrum3,4, Daniel Kveselis3,4, Glenn Leonard1,5, Rajiv Devanagondi1, Matthew Egan3,4
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 >
Open Access
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 >
Open Access
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 >
Open Access
ARTICLE
Sara Bobillo‐Perez1,2, Joan Sanchez‐de‐Toledo3,4, Susana Segura2, Monica Girona‐Alarcon2, Maria Mele5, Anna Sole‐Ribalta2, Debora Cañizo Vazquez6, Iolanda Jordan2,7, Francisco Jose Cambra1,2
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 >
Open Access
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 Bakar13,14, 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 >
Open Access
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 >
Open Access
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 >
Open Access
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 >
Open Access
ARTICLE
Nadya Golfenshtein1, Alexandra L. Hanlon2, Janet A. Deatrick3, Barbara Medoff‐Cooper3,4
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 >
Open Access
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 >
Open Access
ARTICLE
Varun Aggarwal1,2, 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 >
Open Access
ARTICLE
Margaret M. Samyn1,2, Ke Yan1, Conor Masterson3, Benjamin H. Goot1,2, David Saudek1,2, Julie Lavoie2, Aaron Kinney2, Mary Krolikowski1, Kan Hor4,5, Scott Cohen1,2
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 >
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 >
Open Access
ARTICLE
Nicholas Y. Tan1, Christine H. Attenhofer Jost1, Murray D. Polkinghorne1, Emily R. Vargas2, David O. Hodge2, Joseph A. Dearani3, Samuel J. Asirvatham1,4, 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 >
Open Access
REVIEW
Munes Fares1, Paul J. Critser2, Maria J. Arruda1, Carolyn M. Wilhelm1, Mantosh S. Rattan3, Sean M. Lang2,4, Tarek Alsaied2,4
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 >
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 >
Open Access
ARTICLE
Andrew S. Kim1, Colleen M. Witzenburg2, Mark Conaway3, Jeffrey E. Vergales1, Jeffrey W. Holmes2,4, 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 >
Open Access
ARTICLE
Jeremy M. Steele1,2, 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 >
Open Access
ARTICLE
Andrew D. Spearman1, Steven J. Kindel1, Ronald K. Woods2, Salil Ginde1,3
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 >
Open Access
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 >
Open Access
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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