Open Access
EDITORIAL
Douglas S. Moodie,
Congenital Heart Disease, Vol.13, No.4, pp. 491-491, 2018, DOI:10.1111/chd.12656
Abstract This article has no abstract. More >
Open Access
ARTICLE
Jonathan N. Menachem1, Edo Y. Birati2, Payman Zamani2, Anjali T. Owens2, Pavan Atluri3, Christian A. Bermudez3, David Drajpuch2, Stephanie Fuller4, Yuli Y. Kim2, Christopher E. Mascio4, Vikram Palanivel2, J. Eduardo Rame2, Joyce Wald2, Michael A. Acker3, Jeremy A. Mazurek2
Congenital Heart Disease, Vol.13, No.4, pp. 492-498, 2018, DOI:10.1111/chd.12606
Abstract Background: Heart failure (HF) is the leading cause of death in adults with congenital heart disease
(ACHD). Identification of disease progression and timing of referral for advanced therapies is often
delayed. However, increased awareness and understanding of ACHD and improvements in the
approach to treatment have led to improved outcomes. Pulmonary hypertension (PH) is a common
barrier to HT. In ACHD, the approach to PH and HT is quite complicated, given the anatomic heterogeneity and lower prevalence and experience. However, in some cases, PH is a result of
elevated systemic filling pressures and low output.
Methods: We describe the approach… More >
Open Access
ARTICLE
Shahnawaz M. Amdani1, Muhammad Umair M. Mian2, Ron L. Thomas3, Robert D. Ross4
Congenital Heart Disease, Vol.13, No.4, pp. 499-505, 2018, DOI:10.1111/chd.12601
Abstract Aim: To evaluate predictors of morbidity and mortality in pediatric patients with pulmonary hypertension (PH), laboratory and echocardiographic measures of PH were analyzed.
Methods: A retrospective review of all infants and children< 2 years of age with PH from January
2011 to August 2016 was conducted. Correlations were determined using Spearman’s rank correlation coefficients. Differences in characteristics between survivors and nonsurvivors were
analyzed and Kaplan-Meier survival curves were generated.
Results: Of 56 patients, the majority were extremely premature; of African American ethnicity;
and had bronchopulmonary dysplasia. Patients who died were more likely to have underlying congenital heart disease; have a… More >
Open Access
ARTICLE
Timur Mese1, Baris Guven2, Murat Muhtar Yilmazer1, Cem Karadeniz3, Rahmi Ozdemir1, Onder Doksoz1
Congenital Heart Disease, Vol.13, No.4, pp. 506-511, 2018, DOI:10.1111/chd.12616
Abstract Background: Mean platelet volume (MPV), platecrit, and platelet distribution width (PDW) are
markers of platelet activation. Previous studies have found that platelet activation occurs in
patients with pulmonary arterial hypertension. Platelet indices including MPV, PDW, and platecrit
have not been studied in children with congenital heart disease associated pulmonary arterial
hypertension (APAH-CHD) who survived and those who died.
Objective: The objective of this study to investigate the value of platelet indices with clinical and
hemodynamic indicators predicting the disease severity and survival in children with APAH-CHD.
Methods: This was a nested case-control study. MPV, platecrit, and PDW levels measured in… More >
Open Access
ARTICLE
Natasha L. Lopez, Charitha Gowda, Carl H. Backes, Deipanjan Nandi, Holly Miller-Tate, Samantha Fichtner, Robin Allen, Jamie Stewart, Clifford L. Cua
Congenital Heart Disease, Vol.13, No.4, pp. 512-518, 2018, DOI:10.1111/chd.12602
Abstract Introduction: Neonates with hypoplastic left heart syndrome (HLHS) are at increased risk for
necrotizing enterocolitis (NEC). Initial hospital outcomes are well described, but minimal midterm
data exist. Goal of this study was to compare outcomes of HLHS infants with NEC (HLHS-NEC) to
HLHS without NEC (HLHS-nNEC) during the interstage period.
Methods: Data were reviewed from 55 centers using the NPC-QIC database. Case-control study
with one HLHS-NEC matched to HLHS-nNEC neonates in a 1:3 ratio based on institutional site,
type of surgical repair, and gestational age ±1 week was performed. Baseline demographics as well
as outcome data were recorded. The t… More >
Open Access
ARTICLE
Parthak Prodhan1, Xinyu Tang2, Jeffrey Gossett2, Brandon Beam3, Janet Simsic4, Nancy Ghanayem5, Nahed O. ElHassan6
Congenital Heart Disease, Vol.13, No.4, pp. 519-527, 2018, DOI:10.1111/chd.12610
Abstract Objective: Different feeding strategies have been suggested to improve growth and survival of
infants with hypoplastic left heart syndrome following stage 1 palliation. The study objective was
to assess hospital mortality following stage 1 palliation among infants with hypoplastic left heart
syndrome who had two feeding modalities, gastrostomy tube vs no gastrostomy tube.
Design: Retrospective study design.
Setting: Multicenter pediatric heath information system database.
Patient: About 4287 patients with hypoplastic left heart syndrome who underwent stage 1 Norwood procedure from 2004 through 2013. Infants who had gastrostomy tube with or without
fundoplication procedure were identified and their clinical characteristics were… More >
Open Access
ARTICLE
Elizabeth C. Caris1, Nicole Dempster2, Gil Wernovsky3, Yongjie Miao2, Melissa Moore-Clingenpeel2, Trent Neely2, Rachel Fonseca2, Holly Miller-Tate2, Robin Allen2, Samantha Fichtner2, Jamie Stewart2, Clifford L. Cua2
Congenital Heart Disease, Vol.13, No.4, pp. 528-532, 2018, DOI:10.1111/chd.12619
Abstract Objectives: Siblings of children with chronic medical conditions endorse a lower quality of life compared to age-matched peers. Caregiver and sibling-self report of adjustment are often discordant.
Congenital heart disease significantly affects family life. To date, there have been no studies addressing the functioning of siblings of children with hypoplastic left heart syndrome, one of the most
severe forms of congenital heart disease. The goal of this study was to assess the impact of hypoplastic left heart syndrome on sibling’s quality of life as well as the caregiver’s perception of this effect.
Study Design: Cross-sectional study using a web-based survey… More >
Open Access
ARTICLE
Doris P. Yimgang1, John D. Sorkin2, Charles F. Evans3, Danielle S. Abraham1, Geoffrey L. Rosenthal1,4
Congenital Heart Disease, Vol.13, No.4, pp. 533-540, 2018, DOI:10.1111/chd.12622
Abstract Introduction: Angiotensin converting enzyme inhibitors are commonly prescribed medications
after the Norwood procedure. There are little data that can be used to determine if angiotensin
converting enzyme inhibitors improve interstage outcomes in children with single ventricle defects.
The objective of this study was to investigate the relationship between angiotensin converting
enzyme inhibitors and interstage failure among infants born with hypoplastic left heart syndrome.
Methods: We conducted a retrospective cohort study using data from the National Pediatric Cardiology Quality Improvement Collaborative database (collected between 2008 and 2015). We
used logistic regression models to assess the exposure-outcome associations and propensity score
matching… More >
Open Access
ARTICLE
Justin Georgekutty1, Amir Kazerouninia2, YunFei Wang3, Peter R. Ermis4, Dhaval R. Parekh4, Wayne J. Franklin4, Wilson W. Lam4
Congenital Heart Disease, Vol.13, No.4, pp. 541-547, 2018, DOI:10.1111/chd.12603
Abstract Objective: Adult Fontan patients are at increased risk for thrombosis and thromboembolic complications leading to increased morbidity and mortality. Most are prescribed antiplatelet or
anticoagulant therapy for thromboprophylaxis; novel oral anticoagulants (NOACs) are uncommonly
used given lack of data on their use in this population and generalized concerns regarding Fontan
patients’ abnormal coagulation. We report the largest single-center experience with the use of
NOACs for treatment and prophylaxis of thrombosis and thromboembolism in adult Fontan
patients.
Results: A retrospective chart review identified 21 patients (11 female, 10 male), median age 33
years (18-50) at first initiation, who were prescribed a… More >
Open Access
ARTICLE
Albert C. Hergenroeder1, Douglas S. Moodie2, Daniel J. Penny2, Constance M. Wiemann1, Blanca Sanchez-Fournier1, Lauren K. Moore2, Jane Head3
Congenital Heart Disease, Vol.13, No.4, pp. 548-553, 2018, DOI:10.1111/chd.12604
Abstract Objective: To describe changes in functional status between the last pediatric and first adult
congenital heart disease (CHD) clinic visits in patients with moderate to severe CHD after
implementing a healthcare transition (HCT) planning program.
Design: Quasi-experimental design. Patients were followed prospectively following the implementation of the intervention; Control patients transitioned from the Pediatric CHD Clinic into Adult
CHD Clinic before the intervention.
Setting: Texas Children’s Hospital (TCH).
Patients: Sixteen to 25-year-olds, cognitively normal, English speaking patients with moderate to
severe CHD who transitioned from the Pediatric to the Adult CHD clinic.
Interventions: An EMR-based transition planning tool (TPT) was… More >
Open Access
ARTICLE
Nancy McClung1,2, Jill Glidewell1, Sherry L. Farr1
Congenital Heart Disease, Vol.13, No.4, pp. 554-562, 2018, DOI:10.1111/chd.12605
Abstract Objective: To examine the financial burdens and mental health needs of families of children with
special healthcare needs (CSHCN) with congenital heart disease (CHD).
Methods: Data from the 2009–2010 National Survey of Children with Special Health Care Needs
(NS-CSHCN) were used to examine parent-reported financial burdens (out-of-pocket expenses,
financial problems, employment impact, caregiving hours) and family members’ need for mental
health services in families of CSHCN with CHD. Multivariable logistic regression was used to compare financial burdens and family members’ need for mental health services among CSHCN with
and without CHD. Among CSHCN with CHD, multivariable logistic regression, stratified by… More >
Open Access
ARTICLE
Kevin C. Harris1, Christine Voss1, Kathryn Rankin2, Basmina Aminzadah2, Ross Gardner1, Andrew S. Mackie2
Congenital Heart Disease, Vol.13, No.4, pp. 563-570, 2018, DOI:10.1111/chd.12612
Abstract Objective: Individuals with congenital heart disease (CHD) may be at higher risk of acquired cardiovascular disease than the general population due to their underlying physiology and/or surgical
sequelae. We sought to assess the prevalence of cardiovascular disease risk factors in youth and
adults with CHD.
Methods: We assessed cardiovascular health as per the Cardiovascular Health in Ambulatory Care
Research Team (CANHEART) health index in patients with CHD aged 15+ years who attended
cardiology outpatient clinics. Participants self-reported smoking behavior, fruit and vegetable consumption, physical activity, and whether they had diabetes and hypertension. Individual health
indices were categorized into ideal/not ideal,… More >
Open Access
ARTICLE
Michele M. Pasierb, Josiah M. Penalver, Margaret M. Vernon, Bhawna Arya
Congenital Heart Disease, Vol.13, No.4, pp. 571-577, 2018, DOI:10.1111/chd.12611
Abstract Background: Accurate prenatal diagnosis of congenital heart disease (CHD) allows for appropriate
delivery and postnatal management. Geographic constraints limit access to fetal cardiology subspecialists. In our approach, general pediatric cardiologists are first line in regional prenatal cardiac
screening. We aim to demonstrate the utility of this approach in diagnosing CHD requiring cardiac
interventions within 30 days of life.
Methods: This is a retrospective review of fetal echocardiograms performed at Seattle Children’s
Hospital regional cardiology sites (SCH-RC) from December 2008 to December 2015. Referrals to
Seattle Children’s Hospital Prenatal Program (SCH-PNP) were evaluated for referral timing, indication, diagnostic accuracy, and postnatal… More >
Open Access
ARTICLE
Natasha G. Boyes1, Michael K. Stickland2, Stephanie Fusnik1, Elizabeth Hogeweide1, Josie T.J. Fries1, Mark J. Haykowsky3, Chantelle L. Baril1, Shonah Runalls1, Ashok Kakadekar4, Scott Pharis4, Charissa Pockett4, Timothy J. Bradley4, Kristi D. Wright5, Marta Erlandson1, Corey R. Tomczak1
Congenital Heart Disease, Vol.13, No.4, pp. 578-583, 2018, DOI:10.1111/chd.12614
Abstract Children with congenital heart disease are at risk for developing increased arterial stiffness and this
may be modulated by physical activity.
Objective: To compare arterial stiffness in high- and low-physically active children with congenital
heart disease and healthy age- and sex-matched controls.
Patients: Seventeen children with congenital heart disease (12 ± 2 years; females = 9), grouped by
low- and high-physical activity levels from accelerometry step count values, and 20 matched controls (11 ± 3 years; females = 9) were studied.
Outcome Measures: Carotid-radial pulse wave velocity was assessed with applanation tonometry
to determine arterial stiffness. Body composition and 6-min… More >
Open Access
ARTICLE
Hieu Lan Nguyen1,2, Quang Tan Phan3,4, Linh Huynh Dinh2, Hieu Ba Tran2, Hoyoun Won4, Julian Johny Thottian4,5, Dung Doan Duc1, Truong Nguyen Quang3, Sang Wook Kim4
Congenital Heart Disease, Vol.13, No.4, pp. 584-593, 2018, DOI:10.1111/chd.12613
Abstract Objective: To evaluate the efficacy and safety of Nit-Occlud Le VSD Coil versus Duct Occluders ^
for percutaneous closure of perimembranous ventricular septal defect (pmVSD).
Introduction: VSD closure using conventional pmVSD occluders has been largely abandoned
because of an unacceptable high rate of complete heart block (CHB). The advantages of Duct
Occluders and VSD Coil are supposed to reduce the drawbacks of previous devices, especially
CHB complications.
Method: Patients underwent percutaneous pmVSD closure were divided into Coil group (using
VSD Coil, n = 71) and DO group (using Duct Occluders, n = 315). Patient demographics, clinical
presentations, echocardiography measurements, procedure… More >
Open Access
ARTICLE
Martin Christmann1,2, Alexandra Wipf1,2, Hitendu Dave2,3, Daniel Quandt1,2, Oliver Niesse1,2, Markus Deisenberg2,4, Martin Hersberger2,5, Oliver Kretschmar1,2, Walter Knirsch1,2
Congenital Heart Disease, Vol.13, No.4, pp. 594-601, 2018, DOI:10.1111/chd.12615
Abstract Objective: To find risk factors for a complicated early postoperative course after arterial switch
operation (ASO) in neonates with d-transposition of the great arteries (dTGA). In addition to anatomical and surgical parameters, the predictive value of early postoperative troponin T (TnT) values
in correlation to the early postoperative course after ASO is analyzed.
Methods: Seventy-nine neonates (57 (72%) male) with simple dTGA treated by ASO between
2009 and 2016 were included in the analysis. A complicated early postoperative course (30 days)
was defined by one of the following criteria: (A) moderate to severe cardiac dysfunction without
rhythm disturbances, (B) rhythm… More >
Open Access
ARTICLE
Christopher R. Broda1, Hamsini Sriraman2, Devanshi Wadhwa2, YunFei Wang1, Hari Tunuguntla1, Ayse Akcan-Arikan3,4, Peter R. Ermis1, Jack F. Price1
Congenital Heart Disease, Vol.13, No.4, pp. 602-607, 2018, DOI:10.1111/chd.12617
Abstract Purpose: Elevated central venous pressure (CVP) has deleterious effects on several organ systems
in patients with Fontan circulation. However, the relationship between CVP and estimated glomerular filtration rate (eGFR) has not been assessed in patients with Fontan circulation.
Methods: Patients with Fontan circulation whose hemodynamics were assessed by catheterization
between 1987 and 2015 and had a serum creatinine measured within 72 hours prior to the procedure were included for analysis. Patients with primary kidney disease were excluded. Renal
function was calculated by “bedside Schwartz” equation in children (< 18 years) and Modification
of Diet in Renal Disease equation in adults.… More >
Open Access
ARTICLE
Mi Kyoung Song1, Gi Beom Kim1, Eun Jung Bae1, Young Ah Lee1, Hyun-Young Kim2, Seung-Kee Min3, Jung Hee Kim4, Jae-Kyung Won5
Congenital Heart Disease, Vol.13, No.4, pp. 608-616, 2018, DOI:10.1111/chd.12625
Abstract Objective: Pheochromocytoma and paraganglioma (extra-adrenal pheochromocytoma) are rare
neuroendocrine tumors that arise from the neuroendocrine cells. Chronic hypoxia is known as a
possible cause, and a strong link between cyanotic congenital heart disease and these tumors has
been reported. However, reports of phechromocytoma/paraganglioma in Fontan patients were
scarce. We herein report seven cases of phechromocytoma/paraganglioma after Fontan operation
at a single tertiary center.
Methods: We retrospectively reviewed medical records and imaging studies who diagnosed as
phechromocytoma/paraganglioma after Fontan operation in Seoul National University Children’s
Hospital.
Results: Seven patients were identified during follow-up after Fontan operation, and the prevalence was 2.5%… More >
Open Access
ARTICLE
Alex J. Thompson1, Joseph A. Dearani2, Jonathan N. Johnson1,3, Hartzell V. Schaff2, Eric C. Towe1, Jared Palfreeman1, Philip L. Wackel1, Frank Cetta1,3
Congenital Heart Disease, Vol.13, No.4, pp. 617-623, 2018, DOI:10.1111/chd.12618
Abstract Background: The transapical approach has been utilized in adult HCM patients with either midventricular obstruction or a small LV cavity; however, there are little data on its use in children.
Methods: We retrospectively reviewed all patients (age <21 years) with HCM who underwent a
transapical myectomy from January 2002 to December 2016. Indication for surgery was midventricular obstruction in 19/23 (83%) and small LV cavity in 4 (17%). Preoperative symptoms
included: dyspnea (96%), chest pain (65%), presyncope (61%), and syncope (35%). The mean age at
the time of operation was 14 ± 4.0 years (range, 4–20).
Results: Overall, 23 patients… More >
Open Access
ARTICLE
William N. Evans1,2, Ruben J. Acherman1,2, Dean Berthoty3, Gary A. Mayman1,2, Michael L. Ciccolo1,4, Sergio A. Carrillo1,4, Humberto Restrepo1,2
Congenital Heart Disease, Vol.13, No.4, pp. 624-627, 2018, DOI:10.1111/chd.12623
Abstract Patients: We reviewed all patients evaluated at our center with situs solitus, levocardia, and a right
aortic arch that were born between January 2000 and January 2018. Results: From our databases,
we identified 204 patients. We excluded patients with a double aortic arch from analysis. Of the
204 patients, 103 (50%) were male. Of the 204 patients, 95 (47%) had an isolated right aortic arch.
Of the 95 with an isolated right aortic arch, 4 (4%) had chromosome 22q11.2 deletion syndrome,
and 89 (94%) had a vascular ring. Of the 204 patients, 109 (53%) had a surgical intracardiac malformation. Of… More >
Open Access
ARTICLE
Margaret M. Fuchs1, Heidi M. Connolly1, Sameh M. Said2, Alexander C. Egbe1
Congenital Heart Disease, Vol.13, No.4, pp. 628-632, 2018, DOI:10.1111/chd.12624
Abstract Objective: To describe outcomes in patients with cor triatriatum sinister (CTS).
Design: Retrospective review of patients with CTS followed at Mayo Clinic Rochester from 1990
to 2016. Clinical notes, operative reports, and baseline imaging studies were reviewed including
echocardiogram, magnetic resonance imaging, computed tomography, and cardiac catheterization.
Results: Fifty-seven patients (median age 34 years; men 32 (56%)) were enrolled. Definitive or suspected CTS diagnosis was made by transthoracic echocardiogram in 41 (72%) patients, and
additional multimodality imaging was required in 39 (68%) patients. Of these 57 patients, initial
diagnosis was made in adulthood in 35 (61%) patients, and 33 of… More >
Open Access
ARTICLE
Sherry L. Farr1, Karrie F. Downing1,2, Tiffany Riehle-Colarusso1, Ginnie Abarbanell1,3
Congenital Heart Disease, Vol.13, No.4, pp. 633-639, 2018, DOI:10.1111/chd.12621
Abstract Objective: To examine how cognitive and motor limitations in children with heart disease are
associated with education and participation in extracurricular activities.
Design: Using 2009–2010 parent-reported data from the National Survey of Children with Special
Health Care Needs (NS-CSHCN), we examined prevalence of five functional limitations (learning/
concentration, communication, self-care, gross motor skills, and fine motor skills) by diagnosed
heart disease status using chi-square tests and multivariable logistic regression. Among CSHCN
with heart disease, we examined the associations between severity of each functional limitation
and missing ≥11 days of school in the past year, receiving special education services, and interference… More >
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