Open Access
REVIEW
Robert W. Loar, Cory V. Noel, Hari Tunuguntla, John L. Colquitt, Ricardo H. Pignatelli
Congenital Heart Disease, Vol.13, No.1, pp. 5-15, 2018, DOI:10.1111/chd.12564
Abstract Chemotherapy-induced cardiotoxicity in adults and children is a topic with a growing interest in
the cardiology literature. The ability to detect cardiac dysfunction in a timely manner is essential in
order to begin adequate treatment and prevent further deterioration. This article aims to provide a
review on the myocardial injury process, chemotherapeutic agents that lead to cardiotoxicity, the
definition of cardiotoxicity, and the methods of timely detection and treatment. More >
Open Access
REVIEW
Uri Pollak1,2,3, David Mishaly3,4, Gili Kenet3,5, Amir Vardi1,3
Congenital Heart Disease, Vol.13, No.1, pp. 16-25, 2018, DOI:10.1111/chd.12557
Abstract Heparin-induced thrombocytopenia (HIT) is a life-threatening complication of heparin therapy. The
risk for HIT correlates with the cumulative dosage of heparin exposure. In Fontan patients,
recurrent systemic anticoagulation, traditionally with heparin, is used to alleviate the thrombotic
complications that may occur postoperatively when the venous pressure rises and the systemic
venous flow into the pulmonary arteries becomes sluggish, putting them at increased risk. As a
pressure gradient-dependent circulation, elevation in systemic venous pressure, most often by
venous thrombosis, contributes to circuit failure. Therefore, when HIT complicates patients after
the Fontan procedure, it is associated with a high thrombotic morbidity and… More >
Open Access
ARTICLE
Philip Wackel1,2, Bryan Cannon1,2, Joseph Dearani3, Kristen Sessions1,2, Kimberly Holst3, Jonathan Johnson1,2, Frank Cetta1,2
Congenital Heart Disease, Vol.13, No.1, pp. 26-30, 2018, DOI:10.1111/chd.12566
Abstract Background: The increased incidence of preoperative and postoperative arrhythmia in Ebstein
anomaly (EA) prompted some clinicians to perform an electrophysiology study (EPS) in all patients
prior to surgery for EA. The cone repair (CR) is the current surgical option of choice for most young
patients with EA but the effect of the CR on arrhythmia is not well established.
Objectives: To assess the burden of arrhythmia in young patients after CR and to assess the utility
of selective preoperative EPS.
Materials and Methods: A retrospective review of all patients <21 years of age with EA who had
a CR at… More >
Open Access
ARTICLE
Daniel E. Ehrmann1, Matthew Mulvahill2, Shaunda Harendt1,3, Jessica Church1, Amy Stimmler1, Piyagarnt Vichayavilas1,4, Sanja Batz1,5, Jennifer Rodgers1,5, Michael DiMaria1, James Jaggers1, Cindy Barrett1, Jon Kaufman1
Congenital Heart Disease, Vol.13, No.1, pp. 31-37, 2018, DOI: 10.1111/chd.12550
Abstract Background: Feeding practices after neonatal and congenital heart surgery are complicated and
variable, which may be associated with prolonged hospitalization length of stay (LOS). Systematic
assessment of feeding skills after cardiac surgery may earlier identify those likely to have
protracted feeding difficulties, which may promote standardization of care.
Methods: Neonates and infants ≤3 months old admitted for their first cardiac surgery were retrospectively identified during a 1-year period at a single center. A systematic feeding readiness
assessment (FRA) was utilized to score infant feeding skills. FRA scores were assigned immediately
prior to surgery and 1, 2, and 3 weeks after… More >
Open Access
ARTICLE
William N. Evans1,2, Ruben J. Acherman1,2, Michael L. Ciccolo1,3, Sergio A. Carrillo1,2, Alvaro Galindo1,2, Abraham Rothman1,2, Gary A. Mayman1,2, Elizabeth A. Adams1,2, Leigh C. Reardon1,4, Brody J. Winn5, Noel S. Yumiaco5, Lesley Shimuizu1, Yoko Inanaga1, Rowena J. Deleon1, Humberto Restrepo1,2
Congenital Heart Disease, Vol.13, No.1, pp. 38-45, 2018, DOI:10.1111/chd.12558
Abstract Objective: We hypothesized that clinic-based, hepatic-ultrasound, elastography measurements,
either alone or in combination with other noninvasive variables, might correlate with liver-biopsy
fibrosis scores in patients post-Fontan.
Methods: Between March 2012 and February 2017, we identified patients post-Fontan that
underwent elective cardiac catheterization and simultaneous transvenous hepatic biopsy. From
this group, we selected patients that met inclusion criteria for liver-ultrasound, shear-wave elastography. Utilizing the results of elastography, laboratory testing, and time post-Fontan, we
constructed a composite Fontan hepatic index as a sum of elastography measurements in kilopascals, model for end-stage liver disease excluding INR scores, and the square root of the… More >
Open Access
ARTICLE
Susan F. Saleeb1, Sarah R. McLaughlin2, Dionne A. Graham2, Kevin G. Friedman1, David R. Fulton1
Congenital Heart Disease, Vol.13, No.1, pp. 46-51, 2018, DOI:10.1111/chd.12539
Abstract Objective: Using a Standardized Clinical Assessment and Management Plan (SCAMP) for pediatric
patients presenting to clinic with chest pain, we evaluated the cost impact associated with
implementation of the care algorithm. Prior to introduction of the SCAMP, we analyzed charges
for 406 patients with chest pain, seen in 2009, and predicted 21% reduction of overall charges
had the SCAMP methodology been used. The SCAMP recommended an echocardiogram for
history, examination, or ECG findings suggestive of a cardiac etiology for chest pain.
Design: Resource utilization was reviewed for 1517 patients (7-21 years) enrolled in the SCAMP
from July 2010 to April… More >
Open Access
ARTICLE
Carissa M. Baker-Smith1, Karina Carlson2, Jose Ettedgui3, Takeshi Tsuda4, K. Anitha Jayakumar5, Matthew Park6, Nikola Tede7, Karen Uzark8, Craig Fleishman9, David Connuck10, Maggie Likes11, Daniel J. Penny12
Congenital Heart Disease, Vol.13, No.1, pp. 52-58, 2018, DOI:10.1111/chd.12540
Abstract Objective: To develop quality metrics (QMs) for the ambulatory care of patients with transposition of the great arteries following arterial switch operation (TGA/ASO).
Design: Under the auspices of the American College of Cardiology Adult Congenital and Pediatric
Cardiology (ACPC) Steering committee, the TGA/ASO team generated candidate QMs related to
TGA/ASO ambulatory care. Candidate QMs were submitted to the ACPC Steering Committee and
were reviewed for validity and feasibility using individual expert panel member scoring according
to the RAND-UCLA methodology. QMs were then made available for review by the entire ACC
ACPC during an “open comment period.” Final approval of each… More >
Open Access
ARTICLE
Fred H. Rodriguez III1,2,3,4, Georges Ephrem1,2, Jennifer F. Gerardin1,2, Cheryl Raskind-Hood5, Carol Hogue5, Wendy Book1,2
Congenital Heart Disease, Vol.13, No.1, pp. 59-64, 2018, DOI:10.1111/chd.12563
Abstract Objective: Although the ICD-9-CM code 745.5 is widely used to indicate the presence of a secundum atrial septal defect (ASD), it is also used for patent foramen ovale (PFO) which is a normal
variant and for “rule-out” congenital heart disease (CHD). The ICD-10-CM code Q21.1 perpetuates
this issue. The objective of this study was to assess whether code 745.5 in isolation or in combination with unspecified CHD codes 746.9 or 746.89 miscodes for CHD, and if true CHD positives
decrease with age.
Design: Echocardiograms of patients with an ICD-9-CM code of 745.5 in isolation or in combination with unspecified CHD… More >
Open Access
ARTICLE
Jill M. Steiner1, James N. Kirkpatrick1, Susan R. Heckbert2, Asma Habib1, James Sibley3, William Lober3, J. Randall Curtis3
Congenital Heart Disease, Vol.13, No.1, pp. 65-71, 2018, DOI:10.1111/chd.12524
Abstract Introduction: There is relatively sparse literature on the use of administrative datasets for research
in patients with adult congenital heart disease (ACHD). The goal of this analysis is to examine the
accuracy of administrative data for identifying patients with ACHD who died.
Methods: A list of the International Classification of Diseases codes representing ACHD of moderate- or great-complexity was created. A search for these codes in the electronic health record of
adults who received care in 2010–2016 was performed, and used state death records to identify
patients who died during this period. Manual record review was completed to evaluate performance… More >
Open Access
ARTICLE
Astrida Kaugars1, Clarissa Shields2, Cheryl Brosig3
Congenital Heart Disease, Vol.13, No.1, pp. 72-78, 2018, DOI:10.1111/chd.12547
Abstract Objective: The study examined parent stress and health-related quality of life (HRQOL) among
families of children with congenital heart disease (CHD) referred for psychological services.
Methods: Parents of 54 children (85% boys) aged 3 to 13 (Mage = 7.48, SD = 2.38) completed
measures to assess parenting stress (Parenting Stress Index – Short Form; Pediatric Inventory for
Parents) and the PedsQL Family Impact Module. Medical information was retrieved from medical
record review.
Results: Half of parents of children with single ventricle anatomy had clinically significant levels of
parenting stress. Parents of children with single ventricle anatomy reported more frequent illnessrelated… More >
Open Access
ARTICLE
Yumi Shiina1, Tomoaki Murakami2, Noriko Matsumoto3, Daisuke Okamura4, Yuta Takahashi4, Yosuke Nishihata1, Nobuyuki Komiyama1, Koichiro Niwa1
Congenital Heart Disease, Vol.13, No.1, pp. 79-84, 2018, DOI:10.1111/chd.12555
Abstract Objectives: To assess body composition and relationships among body composition, appetiterelated hormones, adipocytokines, and heart failure (HF) in adult patients with congenital heart disease (CHD).
Patients: This prospective study enrolled 46 consecutive adult patients with CHD and 12 agematched healthy controls. The patients and control subjects were divided into four groups: 13
patients with Fontan circulation (group A), 16 patients with cyanosis (group B), 17 patients who
previously underwent biventricular repair (group C), and 12 age-matched healthy controls.
Design: Body composition was measured using InBody730, and levels of appetite-related hormones (ghrelin and leptin) and adipocytokines (leptin, interleukin-6, and tumor necrosis… More >
Open Access
ARTICLE
Sumeet S. Vaikunth1,2, Roberta G. Williams3, Merujan Y. Uzunyan4, Han Tun5, Cheryl Barton3, Philip M. Chang5
Congenital Heart Disease, Vol.13, No.1, pp. 85-91, 2018, DOI:10.1111/chd.12549
Abstract Objective: Transition from pediatric to adult care is a critical time for patients with congenital heart
disease. Lapses in care can lead to poor outcomes, including increased mortality. Formal transition
clinics have been implemented to improve success of transferring care from pediatric to adult
providers; however, data regarding outcomes remain limited. We sought to evaluate outcomes of
transfer within a dedicated transition clinic for young adult patients with congenital heart disease.
Design, Setting, and Patients: We performed a retrospective analysis of all 73 patients seen in a
dedicated young adult congenital heart disease transition clinic from January 2012 to December… More >
Open Access
ARTICLE
Rohit S. Loomba1,2, Andrew N. Pelech1,2
Congenital Heart Disease, Vol.13, No.1, pp. 92-97, 2018, DOI:10.1111/chd.12510
Abstract Background: Pulmonary atresia with intact ventricular septum is a unique congenital malformation
of the heart in which patients may undergo single-, two- or 1½- ventricle repair. Size of the tricuspid valve annulus, morphology of the right ventricle and presence of ventriculocoronary
connections can all impact the selection of appropriate palliative strategy. We developed the aortic
perfusion score, a novel scoring system based on anterograde coronary perfusion with the aim of
being able to identify patients at risk for death or transplant.
Methods: A retrospective study was conducted. Patients were included if an initial catheterization
was done prior to any intervention.… More >
Open Access
ARTICLE
Ashley R. Kroeger1, Jacqueline Morrison2, Andrew H. Smith1
Congenital Heart Disease, Vol.13, No.1, pp. 98-104, 2018, DOI:10.1111/chd.12525
Abstract Objective: Unplanned readmission to the pediatric cardiac intensive care unit (CICU) is associated
with significant morbidity and mortality. The Pediatric Early Warning Score (PEWS) predicts ward
patients at risk for decompensation but has not been previously reported to identify at-risk
patients with cardiac disease prior to ward transfer. This study aimed to determine whether PEWS
prior to transfer may serve as a predictor of unplanned readmission to the CICU.
Design: All patients discharged from a tertiary children’s hospital CICU from September 2012
through August 2015 were included for analysis. PEWS assessment was performed following
transfer to the cardiac ward, and… More >
Open Access
ARTICLE
Alberto Cresti1, Raffaele Giordano2, Martin Koestenberger3, Isabella Spadoni4, Marco Scalese5, Ugo Limbruno1, Susanna Falorini6, Stefania Stefanelli1, Andrea Picchi1, Francesco De Sensi1, Angela Malandrino7, Massimiliano Cantinotti4,5
Congenital Heart Disease, Vol.13, No.1, pp. 105-112, 2018, DOI:10.1111/chd.12528
Abstract Background: Despite ventricular septal defects (VSDs) are the most common congenital heart
diseases (CHDs) in the neonatal period, their incidence and natural history are still debated and
their follow-up and management strategies remain controversial. Our aim was to evaluate the incidence and natural history of isolated VSDs.
Methods: From January 1996 to December 2015 all neonates with a CHD suspicion were
referred to the Cardiological Department of Grosseto Misericordia Hospital. Only newborns with
confirmed isolated VSD were enrolled in this study and followed for 6 years.
Results: Our 343 newborns with an isolated VSD (incidence of 10.45/1000/births) account for
64%… More >
Open Access
ARTICLE
Felicit e Kamdem1,2, Danielle Kedy Koum2,3, Ba Hamadou1,4, Melanie Yemdji1, Henry Luma1,4, Marie Solange Doualla1,4, Diomède Noukeu5, Esther Barla5, Christophe Akazong5, Anastase Dzudie1,4, Henry Ngote1, Yves Monkam1, Sidiki Mouliom1, Samuel Kingue4,6
Congenital Heart Disease, Vol.13, No.1, pp. 113-117, 2018, DOI:10.1111/chd.12529
Abstract Introduction: Cardiovascular diseases in pediatric pathologies have emerged in the recent years in
sub-Saharan Africa (SSA), with congenital heart diseases (CHDs) being the most frequent. Unfortunately, their diagnosis is usually delayed, thereby increasing childhood morbidity and mortality.
Objectives: Describe the clinical, echocardiographic, and therapeutic aspects of CHDs of children
at Douala General Hospital.
Methods: We carried out a cross-sectional descriptive study over a 10-year period, from January
2006 to December 2015. Files and reports of cardiac ultrasounds of patients aged ≤ 15 years
were reviewed.
Results: We reviewed the medical records of 1616 children, of which 370 (22.9%) had CHD.… More >
Open Access
ARTICLE
Randa M. Matter1, Iman A. Ragab1, Alaa M. Roushdy2, Ahmed G. Ahmed1, Hanan H. Aly1, Eman A. Ismail3
Congenital Heart Disease, Vol.13, No.1, pp. 118-123, 2018, DOI:10.1111/chd.12530
Abstract Objectives: Congenital heart defects are common noninfectious causes of mortality in children.
Bleeding and thrombosis are both limiting factors in the management of such patients. We
assessed the frequency of thrombocytopenia in pediatric patients with congenital cyanotic heart
disease (CCHD) and evaluated determinants of platelet count including immature platelet fraction
(IPF) and their role in the pathogenesis of thrombocytopenia.
Methods: Forty-six children and adolescents with CCHD during pre-catheter visits were studied;
median age was 20.5 months. Complete blood count including IPF as a marker of platelet production and reticulated hemoglobin content (RET-He) as a marker of red cell production and… More >
Open Access
ARTICLE
Jason F. Goldberg1, Craig L. Jensen2, Rajesh Krishnamurthy3, Nidhy P. Varghese4, Henri Justino1
Congenital Heart Disease, Vol.13, No.1, pp. 124-130, 2018, DOI:10.1111/chd.12537
Abstract Objective: We describe the long-term follow-up of a child with recurrent hemoptysis due to
severe pulmonary vein stenosis decompressing via collaterals to esophageal varices.
Design: Case report
Setting: Tertiary children’s hospital
Patient: Single child through ages 2- to 11-year old
Interventions: The child underwent cutting balloon angioplasty, bare metal stenting, and implantation of a PTFE-covered stent, all of which failed rapidly. Only after placement of a paclitaxel drug
eluting stent did he have prolonged relief from hemoptysis and long-term patency of the treated
vein. The stents were serially dilated to keep up with somatic growth of the child, eventually culminating… More >
Open Access
ARTICLE
Byung W. Yoo1, Jung O. Kim2, Lucy Y. Eun2, Jae Y. Choi2, Dong S. Kim3
Congenital Heart Disease, Vol.13, No.1, pp. 131-139, 2018, DOI:10.1111/chd.12541
Abstract Objective: The purpose of this study was to evaluate the changes in right ventricle (RV) and left
ventricle (LV) function after transcatheter atrial septal defect (ASD) closure and to assess the influence of the age and the amount of shunt.
Design: Retrospective study
Patients: Fifty-three adult patients who underwent transcatheter closure were enrolled, then
divided into subgroups according to the age (< 40 years vs ≥ 40 years), and the amount of shunt
flow (QpQs < 2.5 vs QpQs ≥ 2.5).
Outcome Measures: Two-dimensional tissue Doppler imaging was performed in a four-chamber
view at the basal ventricular septum (VS) and… More >
Open Access
ARTICLE
Wendy F. Li1, Heidi Pollard2, Mohsen Karimi3, Jeremy D. Asnes1, William E. Hellenbrand1, Veronika Shabanova4, Constance G. Weismann1,5
Congenital Heart Disease, Vol.13, No.1, pp. 140-146, 2018, DOI:10.1111/chd.12544
Abstract Objective: Trans-catheter (TC) pulmonary valve replacement (PVR) has become common practice
for patients with right ventricular outflow tract obstruction (RVOTO) and/or pulmonic insufficiency
(PI). Our aim was to compare PVR and right ventricular (RV) function of patients who received TC
vs surgical PVR.
Design: Retrospective review of echocardiograms obtained at three time points: before, immediately after PVR, and most recent.
Patients: Sixty-two patients (median age 19 years, median follow-up 25 months) following TC
(N = 32) or surgical (N = 30) PVR at Yale-New Haven Hospital were included.
Outcome Measures: Pulmonary valve and right ventricular function before, immediately after, and… More >
Open Access
ARTICLE
Ashley E. Neal1, Elizabeth Lehto1, Karen Hughes Miller2, Craig Ziegler2, Erin Davis2
Congenital Heart Disease, Vol.13, No.1, pp. 147-153, 2018, DOI:10.1111/chd.12559
Abstract Objective: Although pediatrician-reported relevance of Canadian cardiology-specific objectives
has been studied, similar data are not available for the 2016 American Board of Pediatrics (ABP)
cardiology-specific objectives. This study asked Kentucky trainees, pediatricians, and pediatric cardiologists to identify “most important” content within these objectives.
Design, Methods, Outcome Measures: This cross-sectional study used an original, online survey
instrument based on the 2016 ABP cardiology-specific objectives. We collected quantitative data
(numerical indications of importance) and qualitative data (open-ended replies regarding missing
content and difficulty in teaching and learning). Respondents indicated the top two choices of
most important items within eight content areas. Descriptive… More >
Open Access
ARTICLE
Joshua A. Daily1,2, Elijah Bolin1,2, Brian K. Eble1,2
Congenital Heart Disease, Vol.13, No.1, pp. 154-156, 2018, DOI:10.1111/chd.12561
Abstract Pediatric cardiologists teach complicated concepts to a diverse group of learners that include medical students, nurses, residents, fellows, patients, and parents. Unfortunately, much of what is
taught is not retained. In order to increase the likelihood of long-term retention, a cardiologist
should teach with both meaning and sense. The authors provide a review of these concepts and
give specific examples of how to teach in ways that both make sense and are meaningful to a cardiologist’s leaners. More >
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