Open Access
EDITORIAL
Congenital Heart Disease, Vol.13, No.5, pp. 645-645, 2018, DOI:10.1111/chd.12689
Abstract This article has no abstract. More >
Open Access
ARTICLE
Rohit S. Loomba1, Seth B. Gray2, Saul Flores3
Congenital Heart Disease, Vol.13, No.5, pp. 646-654, 2018, DOI:10.1111/chd.12662
Abstract Introduction: Ketamine is a drug often used for procedural sedation or as adjunct
agent for general sedation in children with congenital heart disease. In the clinical
realm, there is often confusion regarding the effects of ketamine on hemodynamics,
particularly pulmonary vascular resistance and systemic vascular resistance. We per‐
formed a meta‐analysis of studies investigating the effects of ketamine on
hemodynamics.
Methods: A systematic review was conducted to identify studies characterizing the
hemodynamic effects of ketamine in children with congenital heart disease. Studies
were assessed for quality and those of satisfactory quality with pre‐ and postketa‐
mine hemodynamics for each patient… More >
Open Access
ARTICLE
Juan Lizandro Rodríguez‐Hernández1, Fayna Rodríguez‐González2, Marta Riaño‐Ruiz3, Efrén Martínez‐Quintana1,4
Congenital Heart Disease, Vol.13, No.5, pp. 655-662, 2018, DOI:10.1111/chd.12620
Abstract Introduction: Hyperuricemia has been associated with cardiovascular risk factors but
it remains controversial if uric acid is an independent predictor of cardiac mortality.
Methods: A total of 503 CHD patients (457 nonhypoxemic and 46 hypoxemic) and
772 control patients fulfilled inclusion criteria. Demographic, clinical, and analytical
data [serum uric acid and 24h urine uric acid levels, N‐terminal pro‐B‐type natriuretic
peptide (NT‐pro‐BNP), and C‐reactive‐protein (CRP) concentrations] were studied.
Survivals curves to determine cardiac death and arterial thrombosis in CHD patients
were also examined.
Results: Noncyanotic and cyanotic CHD patients had significant higher serum uric
acid concentration (5.2 ± 1.5 vs 4.9 ± 1.3mg/dL, P = .007 and… More >
Open Access
ARTICLE
Anita T. Cote1,2, Martin Hosking3, Christine Voss1,3, Derek G. Human3, George G. S. Sandor1,3, Kevin C. Harris1,3
Congenital Heart Disease, Vol.13, No.5, pp. 663-670, 2018, DOI:10.1111/chd.12629
Abstract Objective: Pediatric heart transplant recipients are at risk of posttransplant coronary
artery disease known as cardiac allograft vasculopathy (CAV), and also may develop
diastolic dysfunction. As CAV begins with a process of progressive intimal thickening,
these occult diffuse changes may be detected using optical coherence tomography
(OCT). We hypothesized that the development of CAV, as identified via OCT, may be
a mechanism of declining ventricular function. Accordingly, the purpose of this study
was to assess coronary artery intimal thickening and LV strain in children who have
undergone heart transplantation.
Methods: In 17 children, we analyzed OCT images for coronary intima… More >
Open Access
ARTICLE
Katharina Niedermayr1, Gerhard Pölzl2, Sabine Scholl‐Bürgi1, Christine Fauth3, Ulrich Schweigmann1, Edda Haberlandt1, Ursula Albrecht1, Manuela Zlamy1, Wolfgang Sperl4, Johannes A. Mayr4, Daniela Karall1
Congenital Heart Disease, Vol.13, No.5, pp. 671-677, 2018, DOI:10.1111/chd.12634
Abstract Objective: In general, a mitochondrial disorder is diagnosed on the basis of symptom
combinations and confirmed by genetic findings. However, patients carrying the
m.3243A>G mutation in the mitochondrial tRNA leucine 1 (MT‐TL1) do not always
meet all the proposed criteria for the most frequently encountered mitochondrial
syndrome “MELAS,” an acronym for Mitochondrial Encephalomyopathy, Lactic
Acidosis, and at least one Stroke‐like episode. We here present various phenotypic
characteristics of the mitochondrial mutation m.3243A>G with particular focus on
cardiac manifestations.
Methods and Results: We followed nine patients (1 month to 68 years old; median
42 years; four female and five male) from… More >
Open Access
ARTICLE
Ketina Arslani1*, Lukas Notz1*, Marzena Zurek1, Matthias Greutmann2, Markus Schwerzmann3, Judith Bouchardy4, Reto Engel5, Christine Attenhofer Jost6, Daniel Tobler1
Congenital Heart Disease, Vol.13, No.5, pp. 678-684, 2018, DOI:10.1111/chd.12627
Abstract Background: In adults with congenital heart disease (CHD) and atrial arrhythmias, recommendations for thromboprophylaxis are vague and evidence is lacking. We aimed to identify factors that
influence decision-making in daily practice.
Methods: From the Swiss Adult Congenital HEart disease Registry (SACHER) we identified 241
patients with either atrial fibrillation (Afib) or atrial flutter/intraatrial reentrant tachycardia (Aflut/
IART). The mode of anticoagulation was reviewed. Logistic regression models were used to assess
factors that were associated with oral anticoagulation therapy.
Results: Compared with patients with Aflut/IART, patients with Afib were older (51 ± 16.1 vs
37 ± 16 years, P < .001)… More >
Open Access
ARTICLE
Paul C. Helm1, Ulrike M. M. Bauer1,2, Hashim Abdul‐Khaliq2,3, Helmut Baumgartner1,4, Hans‐Heiner Kramer5, Christian Schlensak2,7, Thomas Pickardt1, Anne‐Karin Kahlert5,6*, Marc‐Phillip Hitz5*
Congenital Heart Disease, Vol.13, No.5, pp. 685-689, 2018, DOI:10.1111/chd.12630
Abstract Background: Congenital heart disease (CHD) affects up to 1% of live births the etiol‐
ogy remains relatively poorly understood. Thus, cardiac research is needed to under‐
stand the underlying pathomechanisms ofthe disease.About51 000CHDpatients are
registered in the German National Register for Congenital Heart Defects (NRCHD).
Patients and relatives were interviewed online about their willingness to support ge‐
netic heart research in order to donate a biological sample.
Methods: Study participants were recruited via the database of the NRCHD. Seven
thousand nine hundred eighty‐nine patients were invited to participate in the study.
Participants have been asked to rate three questions on… More >
Open Access
ARTICLE
Juan Jesús Sánchez‐Barriga
Congenital Heart Disease, Vol.13, No.5, pp. 690-699, 2018, DOI:10.1111/chd.12631
Abstract Background: Congenital heart disease (CHD) represents a global health problem. In
Mexico, in children <1 year of age it is the second cause of mortality. The aim was to
determine mortality trends from CHD and the great vessels in children and adults
nationwide, by state and socioeconomic region.
Methods: Records of mortality associated to CHD and the great vessels for
2000‐2015 were obtained from the National Institute of Statistics and Geography.
This information is collected from death certificates issued nationwide. International
Classification of Diseases, 10th revision, codes corresponding to the basic cause of
death from CHD and the great vessels.… More >
Open Access
ARTICLE
Cheryl L. Brosig1,2, Laurel Bear1,2, Sydney Allen1, Pippa Simpson1, Liyun Zhang1, Michele Frommelt1,2, Kathleen A. Mussatto2
Congenital Heart Disease, Vol.13, No.5, pp. 700-705, 2018, DOI:10.1111/chd.12632
Abstract Background and Objectives: Children with congenital heart disease (CHD) are at risk
for neurodevelopmental (ND) delays. The purpose of this study is to compare the ND
testing results of children with CHD at 2 and 4 years of age and determine if rates of
ND delays change over time.
Methods: Children with CHD completed the Bayley Scales of Infant Development‐III
(BSID‐III) at 2 years of age, and standardized neuropsychological measures at 4 years.
Scores were compared with test norms and were classified as: average (within one
SD of test mean); at risk (1‐2 SDs from the test mean); and delayed… More >
Open Access
ARTICLE
Jennifer P. Lundine1,2, Robert Dempster3,4, Kirby Carpenito5, Holly Miller‐Tate5, Wendelin Burdo‐Hartman4,6, Elizabeth Halpin2, Omar Khalid4,5
Congenital Heart Disease, Vol.13, No.5, pp. 706-712, 2018, DOI:10.1111/chd.12636
Abstract Background: Swallowing dysfunction is a known complication for infants with complex congenital heart disease (CHD), but few studies have examined swallowing outcomes following the hybrid procedure for stage 1 palliation in children with single
ventricle physiology.
Objectives: (1) Identify the incidence of aspiration in all infants with single ventricle
physiology who underwent the hybrid procedure and (2) Compare results of clinical
bedside and instrumental swallowing evaluations to examine the predictive value of
a less invasive swallowing assessment for this population of high‐risk infants.
Methods: This was a retrospective cohort chart review study. All patients with single‐ventricle physiology who underwent the… More >
Open Access
ARTICLE
Adam B. Christopher1, Abraham Apfel2, Tao Sun2, Jackie Kreutzer1, David S. Ezon3
Congenital Heart Disease, Vol.13, No.5, pp. 713-720, 2018, DOI:10.1111/chd.12637
Abstract Objective: The most accurate noninvasive parameter to predict whether a patient
with aortic coarctation will meet interventional criteria at catheterization remains
elusive. We aim to determine the best independent echocardiographic predictors of
a coarctation peak‐to‐peak pressure gradient ≥20 mm Hg at catheterization, the ac‐
cepted threshold for intervention.
Design: Retrospective query of our catheterization database from 1/2007 to 7/2016
for the diagnostic code of aortic coarctation was performed. Multiple echocardio‐
graphic measurements and blood pressure gradients prior to cardiac catheterization
were collected. Univariate correlation of variables with the continuous catheteriza‐
tion peak were calculated using Spearman’s rho. Univariate association with… More >
Open Access
ARTICLE
Jill M. Steiner1, James N. Kirkpatrick1, Susan R. Heckbert2, James Sibley3, James A. Fausto3, Ruth A. Engelberg3, J. Randall Curtis3
Congenital Heart Disease, Vol.13, No.5, pp. 721-727, 2018, DOI:10.1111/chd.12638
Abstract Objective: Overall health care resource utilization by adults with congenital heart
disease has increased dramatically in the past two decades, yet little is known about
utilization patterns at the end of life. The objective of this study is to better under‐
stand the patterns and influences on end‐of‐life care intensity for adults with con‐
genital heart disease.
Methods: We identified a sample of adults with congenital heart disease (n = 65),
cancer (n = 10 784), or heart failure (n = 3809) who died between January 2010 and
December 2015, cared for in one multi‐hospital health care system. We used… More >
Open Access
ARTICLE
Ashley Brunmeier1,2, Michael P Reis3, Michael G. Earing1,2, Laura Umfleet 4, Salil Ginde1,2, Peter J. Bartz1,2, Scott Cohen1,2
Congenital Heart Disease, Vol.13, No.5, pp. 728-733, 2018, DOI:10.1111/chd.12646
Abstract Objective: Children with congenital heart disease (CHD) and adults with acquired
heart disease are at an increased risk of neurocognitive impairment. The objective of
this study was to determine the prevalence of self‐reported neurocognitive impair‐
ment and its risk factors in the adult congenital heart disease (ACHD) population.
Design: The Wisconsin Adult Congenital Heart Disease Program recently began
screening ACHD patients to identify those with significant self‐perceived neurocog‐
nitive impairments. Screening consists of using a validated neuro‐oncology screening
instrument that has been modified for the ACHD population. Patients who answer
this survey in a predetermined fashion consistent with significant self‐perceived… More >
Open Access
ARTICLE
Brian Robichaud1, Garick Hill2, Scott Cohen1,3, Ronald Woods4, Michael Earing1,3, Peter Frommelt1, Salil Ginde1,3
Congenital Heart Disease, Vol.13, No.5, pp. 734-739, 2018, DOI:10.1111/chd.12639
Abstract Background: Pulmonary valve replacement (PVR) is a common operation in patients
with congenital heart disease (CHD). As survival with CHD improves, infective endo‐
carditis (IE) is a growing complication after PVR. The aim of this study was to assess
the incidence, risk factors, and clinical outcomes of IE after surgical PVR in patients
with CHD at our institution.
Methods: Retrospective analysis of all cases of surgical PVR performed at Children’s
Hospital of Wisconsin between 1975 and 2016 was performed. All cases of IE after
PVR were identified and clinical and imaging data were obtained by review of medical
records.
Results:… More >
Open Access
ARTICLE
Michelle Keir1, Barbara Bailey1, Angela Lee1, Adrienne H. Kovacs1,2, S. Lucy Roche1,3
Congenital Heart Disease, Vol.13, No.5, pp. 740-747, 2018, DOI:10.1111/chd.12647
Abstract Background: With access to surgical care, >90% of today’s infants with congenital
heart disease (CHD) will reach adulthood. During childhood, survivors accrue a
wealth of health care experience and develop strategies for navigating life with a
chronic disease.
Methods: Seeking to learn from this individualized process, we invited adults with complex CHD to participate in narrative analysis—an established qualitative research
method for studying how individuals derive meaning from their personal stories. Audio
recordings of 2‐4 hour free‐form interviews were transcribed and iteratively analyzed
to identify common themes and detect similarities or differences in language, viewpoint and interpretation. Recruitment continued until… More >
Open Access
ARTICLE
Heather Y. Sun1, James A. Proudfoot2, Rachel T. McCandless1
Congenital Heart Disease, Vol.13, No.5, pp. 748-756, 2018, DOI:10.1111/chd.12648
Abstract Background: Fetal echocardiography can accurately diagnose critical congenital heart disease
prenatally, but relies on referrals from abnormalities identified on routine obstetrical ultrasounds. Critical congenital heart disease that is frequently missed due to inadequate outflow
tract imaging includes anomalies such as truncus arteriosus, double outlet right ventricle,
transposition of the great arteries, tetralogy of Fallot, pulmonary stenosis, and aortic stenosis.
Objective: This study evaluated the prenatal detection rate of critical outflow tract anomalies
in a single urban pediatric hospital before and after “AIUM Practice Guideline for the
Performance of Obstetric Ultrasound Examinations,” which incorporated outflow tract
imaging.
Design: Infants with outflow… More >
Open Access
ARTICLE
Janet M. Simsic, Christina Phelps, Kristin Kirchner, Kirby‐Rose Carpenito, Robin Allen, Holly Miller‐Tate, Karen Texter, Mark Galantowicz
Congenital Heart Disease, Vol.13, No.5, pp. 757-763, 2018, DOI:10.1111/chd.12649
Abstract Objective: Interstage readmissions are common in infants with single ventricle congenital
heart disease undergoing staged surgical palliation. We retrospectively examined readmissions
during the interstage period.
Design: Retrospective analysis.
Setting: The Heart Center at Nationwide Children’s Hospital, Columbus, Ohio.
Patients: Newborns undergoing hybrid stage 1 palliation from January 2012 to December 2016
who survived to hospital discharge and were followed at our institution.
Interventions: All patients underwent hybrid stage 1 palliation.
Outcome Measures: Outcomes included (1) reason for interstage readmission; (2) feeding modality during interstage period; (3) major interstage adverse events; and (4) interstage
mortality.
Results: Study group comprised 57 patients.… More >
Open Access
ARTICLE
Jessica L. Schachter1, Manisha Patel2, Samuel R. Horton3, A. Mike Devane4, Alex Ewing5, Gary A. Abrams6
Congenital Heart Disease, Vol.13, No.5, pp. 764-770, 2018, DOI:10.1111/chd.12650
Abstract Objective: As the population of patients with Fontan circulation surviving into adult‐
hood increases, hepatic cirrhosis has grown to be a significant cause of morbidity and
mortality. Early detection of advanced hepatic fibrosis is imperative for proper inter‐
vention and consideration for heart or combined heart/liver transplantation.
Noninvasive biomarkers and elastography have been evaluated for their diagnostic
utility with variable results in the Fontan population.
Design: The cohort included 14 patients age 26.4 SD 7.5 who underwent Fontan
surgery. All patients were evaluated with FibroSURE, shear wave elastography (SWE),
hepatic duplex sonography, and liver biopsy. Liver fibrosis on biopsy was… More >
Open Access
ARTICLE
Michael E. Fenster1‡, John S. Hokanson2
Congenital Heart Disease, Vol.13, No.5, pp. 771-775, 2018, DOI:10.1111/chd.12651
Abstract Objective: To determine the clinical findings and management implications of
echocardiograms performed on infants with murmurs in the nursery.
Design: Retrospective cohort study conducted from January 2008 through
December 2015. Patients in the study were followed by chart review for up to
5 years. In addition, a survey of nursery providers was conducted in February 2016.
Setting: A single community hospital associated with a university.
Patients: All 26 573 infants who received care in the normal newborn nursery were
eligible for inclusion in the study. Infants with echocardiograms were analyzed. The
survey was sent by e‐mail to all 135 physicians… More >
Open Access
ARTICLE
Sudheer R. Gorla1, Nataley K. Jhingoeri1, Abhishek Chakraborty1, Kishore R. Raja1, Ashish Garg1, Satinder Sandhu1, Eliot R. Rosenkranz2, Sethuraman Swaminathan1
Congenital Heart Disease, Vol.13, No.5, pp. 776-781, 2018, DOI:10.1111/chd.12652
Abstract Introduction: The Fontan operation is the final stage of single ventricle palliation in
patients with complex congenital heart disease. Fenestration in the Fontan conduit,
providing an atrial level right to left shunt, has been shown to reduce early postoperative morbidity. However, there is limited data on the long‐term fate of this fenestration.
The aim of this study is to define the rate of spontaneous closure of the fenestration in
the Fontan conduit and factors predictive of the fate of the fenestration.
Methods: This was a retrospective study reviewing the medical records of the patients who underwent fenestrated Fontan operation at… More >
Open Access
ARTICLE
Varun Aggarwal, Venkatachalam Mulukutla, Athar M. Qureshi, Henri Justino
Congenital Heart Disease, Vol.13, No.5, pp. 782-787, 2018, DOI:10.1111/chd.12653
Abstract Background: Congenital coronary artery fistula is a rare coronary anomaly. Most
commonly, such fistulae drain into the right side of the heart or the pulmonary artery.
Children with coronary artery fistulae are generally asymptomatic, although they
may have left ventricular enlargement in the setting of a moderate sized left to right
shunt. Symptoms of congestive heart failure or ischemia are very rare in neonatal
period, and suggest the presence of a very large shunt and/or coronary steal.
Methods: Single center retrospective review of transcatheter intervention on coro‐
nary artery fistulae presenting with symptoms in the neonatal period from January
2000… More >
Open Access
ARTICLE
Ashley E. Neal1, Elizabeth Lehto1, Karen Hughes Miller2, Erin Davis3, Craig Ziegler2
Congenital Heart Disease, Vol.13, No.5, pp. 788-793, 2018, DOI:10.1111/chd.12626
Abstract Objective: The 2016 American Board of Pediatrics (ABP) content outline is comprehensive, including
more than 50 cardiology-specific objectives within eight content areas. This study complements the
quantitative analysis of a Kentucky-wide survey of trainees, pediatricians, and pediatric cardiologists
asking them to identify “most important” cardiology content by analyzing their open-ended comments about “what should be added” and “why?” within these eight categories.
Design, Methods, Outcome Measures: This cross-sectional study used an original, online survey
instrument based on the 2016 ABP cardiology-specific objectives. We began an initial analysis of
the qualitative data using Pandit’s version of Glaser and Strauss Grounded theory… More >
Open Access
ARTICLE
Joseph J. Knadler1, Daniel J. Penny1, Tyler H. Harris2, Gary D. Webb3, Antonio G. Cabrera1,4, William B. Kyle1
Congenital Heart Disease, Vol.13, No.5, pp. 794-798, 2018, DOI:10.1111/chd.12669
Abstract Objective: The growing body of medical literature in pediatric cardiology has made it
increasingly difficult for individual providers to stay abreast of the most current,
meaningful articles to help guide practice. Crowdsourcing represents a collaborative
process of obtaining information from a large group of individuals, typically from an
online or web‐based community, and could serve a potential mechanism to pool indi‐
vidual efforts to combat this issue. This study aimed to utilize crowdsourcing as a
novel way to generate a list of the most relevant, current publications in congenital
heart disease, utilizing input from an international group of professionals in… More >
Open Access
REVIEW
Xin Li MMed*, Chengxin Zhang*, Di Dai MMed, Haiyuan Liu, Shenglin Ge
Congenital Heart Disease, Vol.13, No.5, pp. 799-807, 2018, DOI:10.1111/chd.12674
Abstract Objective: We conducted a meta‐analysis to evaluate the effects of prophylactic
perioperative dexmedetomidine administration on postoperative junctional ectopic
tachycardia (JET) and acute kidney injury (AKI) in pediatric patients having under‐
gone cardiac surgery.
Design: This systematic review was registered with PROSPERO (CRD42017083880).
Databases including PubMed, Cochrane Central Register of Controlled Trials, and
Web of Science were searched for randomized controlled trials (RCTs) and observa‐
tional cohort studies from its inception to March 2018. Two reviewers independently
screened literature, extracted data, and assessed the quality of included studies using
the Jadad scale and Newcastle‐Ottawa score. Meta‐analysis was then conducted by
RevMan… More >
Open Access
ARTICLE
Shankar Baskar, Seth B. Gray, Erica L. Del Grippo, Onyekachukwu Osakwe, Adam W. Powell, Justin T. Tretter
Congenital Heart Disease, Vol.13, No.5, pp. 808-810, 2018, DOI:10.1111/chd.12675
Abstract Cardiology fellows‐in‐training, both in adult and pediatric hospitals, need structured
education in regards to congenital heart disease (CHD) nomenclature. With improved
survival of patients with CHD, it is not uncommon for these patients to seek care in
multiple adult and pediatric hospitals. A deep understanding of CHD nomenclature
would aid in providing accurate medical and surgical care for these patients. In this
forum, we share our experience with such structured education and also comment
on recent advances in morphologic imaging that would aid in understanding the
nomenclature. More >
Open Access
ARTICLE
Anoymous
Congenital Heart Disease, Vol.13, No.5, pp. 811-876, 2018, DOI:10.1111/chd.12693
Abstract This article has no abstract. More >
Open Access
EDITORIAL
Christopher S. Snyder
Congenital Heart Disease, Vol.13, No.5, pp. 877-878, 2018, DOI:10.1111/chd.12697
Abstract This article has no abstract. More >
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