Open Access
EDITORIAL
George F. Van Hare
Congenital Heart Disease, Vol.14, No.3, pp. 321-323, 2019, DOI:10.1111/chd.12760
Abstract This article has no abstract. More >
Open Access
ARTICLE
Fei Zhang1, Yifeng Yang2, Qin Wu2, Wancun Jin2, Haisong Bu2, Sijie Wu2, Tianli Zhao2, Shijun Hu2
Congenital Heart Disease, Vol.14, No.3, pp. 324-330, 2019, DOI:10.1111/chd.12753
Abstract Objective: To evaluate safety and effectiveness of intraoperative device closure for
secundum atrial septal defect (ASD) not referred to percutaneous closure.
Design and Patients: From April 2010 to December 2018, 231 secundum ASD children (≤14 years) directly recommended to surgical repair were enrolled in this study.
These patients were divided into two groups according to the parents’ choice based
on surgeons’ recommendation. Follow-up evaluations were adopted at 2 weeks,
3 months, 6 months, and 12 months after the procedure and yearly thereafter. In
Group A, 127 patients underwent an initial attempt at device closure. In Group B,
104 patients underwent… More >
Open Access
REVIEW
Ginnie Abarbanell1, Naomi K. Tepper2, Sherry L. Farr3
Congenital Heart Disease, Vol.14, No.3, pp. 331-340, 2019, DOI:10.1111/chd.12752
Abstract Objective: Women with congenital heart disease (CHD) are at increased risk of preg‐
nancy complications and need information on safe, effective contraceptive methods
to avoid unintended pregnancy. This systematic review examines evidence regarding
safety of contraceptive use among women with CHD.
Methods: The PubMed database was searched for any peer‐reviewed articles pub‐
lished through April 2018 that included safety outcomes associated with reversible
contraceptive methods among women with CHD.
Results: Five articles met inclusion criteria: three studies comparing contraceptive
users to nonusers and two noncomparative studies. Sample sizes ranged from 65 to
505 women with CHD. Two studies found a… More >
Open Access
ARTICLE
Christopher J. Petit1, Athar M. Qureshi2, Andrew C. Glatz3, Courtney E. McCracken1, Michael Kelleman1, George T. Nicholson4, Jeffery J. Meadows5, Shabana Shahanavaz6, Jeffrey D. Zampi7, Mark A. Law8, Joelle A. Pettus1, Bryan H. Goldstein9
Congenital Heart Disease, Vol.14, No.3, pp. 341-349, 2019, DOI:10.1111/chd.12737
Abstract Clinical research in the treatment of patients with congenital heart disease (CHD) is limited by the wide variety of CHD manifestations and therapeutic options as well as the generally low incidence of CHD. The availability of comprehensive, contemporary outcomes studies is therefore limited. This inadequacy may result in a lack of data‐driven medical decision making. In 2013, clinician scientists at two centers begana researchcollaboration,the Congenital Catheterization Research Collaborative (CCRC). Over time, the CCRC has grown to include nine cardiac centers from across
the United States, with a common data coordinating center. The CCRC seeks to generate high‐quality, contemporary, statistically robust,… More >
Open Access
ARTICLE
Soham Dasgupta1, Ishaan Dave2, Courtney E. McCracken2, Larry Mohl3, Ritu Sachdeva1, William Border1
Congenital Heart Disease, Vol.14, No.3, pp. 350-355, 2019, DOI:10.1111/chd.12723
Abstract Background: Physicians are exposed to workplace factors that may result in acute or
chronic stress resulting in burnout. This may impact the productivity and result in suboptimal patient care practices.
Methods: We surveyed pediatric cardiology attending physicians at our institution to
assess their perception of burnout and work-life balance using the Maslach Burnout
Inventory and the Areas of Work-Life Survey.
Results: Forty-five out of the 50 pediatric cardiology attendings responded to the survey.
They were divided into 4 groups: Interventional/Electrophysiology [n = 3], Cardiac Intensive
Care/Inpatient [n = 8], Non-Invasive Imaging [n = 6], and Outpatient [n = 28]. The… More >
Open Access
ARTICLE
Darcy N. Marckini1, Bennett P. Samuel1, Jessica L. Parker2, Stephen C. Cook1,3
Congenital Heart Disease, Vol.14, No.3, pp. 356-361, 2019, DOI:10.1111/chd.12745
Abstract Background: Physician burnout has many undesirable consequences, including nega‐
tive impact on patient care delivery and physician career satisfaction. Electronic
health records (EHRs) may exacerbate burnout by increasing physician workload.
Objective: To determine burnout in adult congenital heart disease (ACHD) specialists
by assessing stress associated with EHRs.
Design: Electronic survey study of ACHD providers.
Setting: Canada and United States.
Participants: Three hundred eighty‐three ACHD specialists listed on the Adult
Congenital Heart Association directory between February and April 2017.
Outcome Measures: Burnout was measured using the Maslach Burnout Inventory
(MBI) to understand factors contributing to work life and EHR satisfaction. Chi‐… More >
Open Access
ARTICLE
Maayke A. Sluman1,2, Silke Apers3,4, Judith K. Sluiter1,*, Karen Nieuwenhuijsen1, Philip Moons4,5, Koen Luyckx6,7, Adrienne H. Kovacs8,9, Corina Thomet10, Werner Budts11, Junko Enomoto12, Hsiao‐Ling Yang13, Jamie L. Jackson14, Paul Khairy15, Stephen C. Cook16, Raghavan Subramanyan17, Luis Alday18, Katrine Eriksen19, Mikael Dellborg20,21, Malin Berghammer5,22, Eva Mattsson23, Andrew S. Mackie24, Samuel Menahem25, Maryanne Caruana26, Kathy Gosney27, Alexandra Soufi28, Susan M. Fernandes29, Kamila S. White30, Edward Callus31, Shelby Kutty32, Berto J. Bouma33, Barbara J.M. Mulder33
Congenital Heart Disease, Vol.14, No.3, pp. 362-371, 2019, DOI:10.1111/chd.12747
Abstract Background: Conflicting results have been reported regarding employment status
and work ability in adults with congenital heart disease (CHD). Since this is an impor‐
tant determinant for quality of life, we assessed this in a large international adult CHD
cohort.
Methods: Data from 4028 adults with CHD (53% women) from 15 different countries
were collected by a uniform survey in the cross‐sectional APPROACH International
Study. Predictors for employment and work limitations were studied using general
linear mixed models.
Results: Median age was 32 years (IQR 25‐42) and 94% of patients had at least a high
school degree. Overall employment rate… More >
Open Access
ARTICLE
Sebastian Udholm, Christian Rex, Filip Eckerström, Mine Onat, Camilla Nyboe, Vibeke E. Hjortdal
Congenital Heart Disease, Vol.14, No.3, pp. 372-379, 2019, DOI:10.1111/chd.12740
Abstract Objective: Adult patients with small, unrepaired atrial septal defects have an in‐
creased risk of pneumonia, atrial fibrillation, and stroke. Furthermore, they have
higher late mortality than the background population. The functional capacity is un‐
known in these patients. Therefore, our objective was to determine exercise capacity
in adult patients diagnosed with an unrepaired atrial septal defect compared to
healthy controls.
Design: A cross‐sectional study.
Patients: Adult patients with small, unrepaired atrial septal defects, aged 18‐65, di‐
agnosed between 1953 and 2011.
Interventions: Cardiopulmonary exercise test was performed using an incremental
bicycle test and gas exchange was measured using breath‐by‐breath… More >
Open Access
ARTICLE
Emily M. Engelhardt1, Andrew T. Trout2,3, Rachel M. Sheridan4, Gruschen R. Veldtman5, Jonathan R. Dillman2,3
Congenital Heart Disease, Vol.14, No.3, pp. 380-388, 2019, DOI:10.1111/chd.12730
Abstract Purpose: Patients who have undergone Fontan palliation of single ventricle physiol‐
ogy congenital heart disease are prone to developing focal liver lesions. In our experi‐
ence, the variety of lesions occurring in this population is greater than that described
in the literature. The purpose of this study was to describe the breadth of biopsy‐
proven liver lesions in patients post–Fontan palliation of single ventricle physiology
cared for at our institution.
Methods: We retrospectively identified patients who had previously undergone the
Fontan operation and had a focal liver lesion biopsied between January 2000 and
June 2018. Medical records were reviewed for… More >
Open Access
ARTICLE
Anoosh Esmaeili, Kachina Behnke‐Hall, Roland Schrewe, Dietmar Schranz
Congenital Heart Disease, Vol.14, No.3, pp. 389-395, 2019, DOI:10.1111/chd.12731
Abstract Aim: The purpose of this study is to describe the special aspects of perimembranous
ventricular septal defects (pmVSD) closure by utilizing Amplatzer Duct Occluder II
(ADO II) devices with a rational request for bigger ADO‐II sizes, based on our experi‐
ence in transcatheter device closure of pmVSD.
Methods and Results: At our institution, placement of an ADO II device was used in
15 patients with pmVSD; the patients’ age ranged between 6 months and 20 years.
The indications for closure were CHF (n = 4), hemodynamically significant shunt
(n = 7), tricuspid regurgitation (n = 3), and high risk for… More >
Open Access
ARTICLE
Xing Chen, Wu Zhou, Qinghua Hu, Lingjin Huang
Congenital Heart Disease, Vol.14, No.3, pp. 396-402, 2019, DOI:10.1111/chd.12733
Abstract Objective: This study explores the role of the Notch3‐HES5 signal pathway in mono‐
crotaline‐induced pulmonary hypertension (PH) using rat models.
Method: Sprague Dawley rats (n = 45) were randomly grouped into normal group,
control group, and model group. Rats in the model group were used to establish the
PH rat model. Four weeks after model establishment, right catheterization was used
to measure the mean pulmonary arterial pressure (mPAP) and right ventricular sys‐
tolic pressure (RVSP) to analyze hemodynamic changes. The severity of PH was as‐
sessed by the right ventricular hypertrophy index (RVHI) and percentage of media
thickness (MT%). The… More >
Open Access
ARTICLE
Jill M. Steiner1, Karen Stout1, Laurie Soine1, James N. Kirkpatrick1, J. Randall Curtis2
Congenital Heart Disease, Vol.14, No.3, pp. 403-409, 2019, DOI:10.1111/chd.12735
Abstract Background: Patients with adult congenital heart disease (ACHD) report that ad‐
vance care planning (ACP) is important, and that they want information about prog‐
nosis. However, recognizing importance and being willing to participate are different
constructs, and how and when to begin ACP and palliative care discussions remains
ill‐defined.
Methods: We conducted a cross‐sectional survey of 150 consecutive outpatients to
assess willingness to participate in ACP, with whom, and important barriers and facili‐
tators to these discussions.
Results: The majority of participants (69%) reported being willing to participate in
ACP; 79% to have a meeting to discuss goals and care… More >
Open Access
ARTICLE
Karen E. Schultz1, George K. Lui1,2, Doff B. McElhinney1, Jin Long3, Vidhya Balasubramanian3, Charlotte Sakarovitch3, Susan M. Fernandes1,2, Anne M. Dubin1, Ian S. Rogers1,2, Anitra W. Romfh1,2, Kara S. Motonaga1, Mohan N. Viswanathan2, Scott R. Ceresnak1
Congenital Heart Disease, Vol.14, No.3, pp. 410-418, 2019, DOI:10.1111/chd.12736
Abstract Background: Arrhythmias are a leading cause of death in adults with congenital heart
disease (ACHD). While 24‐48‐hour monitors are often used to assess arrhythmia
burden, extended continuous ambulatory rhythm monitors (ECAM) can record 2
weeks of data. The utility of this device and the arrhythmia burden identified beyond
48‐hour monitoring have not been evaluated in the ACHD population. Additionally,
the impact of ECAM has not been studied to determine management
recommendations.
Objective: To address the preliminary question, we hypothesized that clinically sig‐
nificant arrhythmias would be detected on ECAM beyond 48 hours and this would
lead to clinical management changes.
… More >
Open Access
ARTICLE
Amanda Hoerst1, Adnan Bakar2, Steven C. Cassidy3, Martha Clabby4, Erica Del Grippo5, Margaret Graupe1, Ashraf S. Harahsheh6, Anthony M. Hlavacek7, Stephen A. Hart3, Alaina K. Kipps8, Nicolas L. Madsen1, Dora D. O’Neil9, Sonali S. Patel10, Courtney M. Strohacker11, Ronn E. Tanel12
Congenital Heart Disease, Vol.14, No.3, pp. 419-426, 2019, DOI:10.1111/chd.12739
Abstract Background: The Pediatric Acute Care Cardiology Collaborative (PAC3) was estab‐
lished in 2014 to improve the quality, value, and experience of hospital‐based cardiac
acute care outside of the intensive care unit. An initial PAC3 project was a compre‐
hensive survey to understand unit structure, practices, and resource utilization
across the collaborative. This report aims to describe the previously unknown degree
of practice variation across member institutions.
Methods: A 126‐stem question survey was developed with a total of 412 possible
response fields across nine domains including demographics, staffing, available re‐
sources and therapies, and standard care practices. Five supplemental questions ad‐… More >
Open Access
ARTICLE
Jan Hinnerk Hansen1, Lydia Kissner1, Jana Logoteta1, Olaf Jung1, Peter Dütschke2, Tim Attmann3, Jens Scheewe3, Hans‐Heiner Kramer1,4
Congenital Heart Disease, Vol.14, No.3, pp. 427-437, 2019, DOI:10.1111/chd.12741
Abstract Objectives: Neonates and infants undergoing surgery for congenital heart disease
are at risk for developmental impairment. Hypoxic‐ischemic brain injury might be one
contributing factor. We aimed to investigate the perioperative release of the astro‐
cyte protein S100B and its relation to cerebral oxygenation.
Methods: Serum S100B was measured before and 0, 12, 24, and 48 hours after sur‐
gery. Cerebral oxygen saturation was derived by near‐infrared spectroscopy. S100B
reference values based on preoperative samples; concentrations above the 75th per‐
centile were defined as elevated. Patients with elevated S100B at 24 or 48 hours
were compared to cases with S100B in… More >
Open Access
Jeannine M. Hoch1, Oluwatosin Fatusin2, Gayane Yenokyan3, W. Reid Thompson2, Maureen A. Lefton‐Greif4
Congenital Heart Disease, Vol.14, No.3, pp. 438-445, 2019, DOI:10.1111/chd.12742
Abstract Background: Tube feedings are often needed to achieve the growth and nutrition goals
associated with decreased morbidity and mortality in patients with single ventricle anat‐
omy. Variability in feeding method through the interstage period has been previously
described, however, comparable information following stage 2 palliation is lacking.
Objectives: To identify types of feeding methods following stage 2 palliation and
their influence on length of stay.
Design: Secondary analysis of the National Pediatric Cardiology Quality Improvement
Collaborative registry was performed on 932 patients. Demographic data, medical
characteristics, postoperative complications, type of feeding method, and length of
stay for stage 2 palliation… More >
Open Access
ARTICLE
Benjamin S. Frank1, Tracy T. Urban2, Karlise Lewis2, Suhong Tong3, Courtney Cassidy4, Max B. Mitchell5, Christopher S. Nichols6, Jesse A. Davidson1
Congenital Heart Disease, Vol.14, No.3, pp. 446-453, 2019, DOI:10.1111/chd.12744
Abstract Objective: Patients undergoing surgical repair of aortic coarctation have a 50% risk
of pathologic left ventricular remodeling (increased left ventricular mass or relative
wall thickness). Endothelin 1, ST2, galectin 3, norepinephrine and B‐natriuretic pep‐
tide are biomarkers that have been associated with pathologic LV change in adult
populations but their predictive value following pediatric coarctation repair are not
known.
Hypothesis: Biomarker levels at coarctation repair will predict persistent left ven‐
tricular remodeling at 1‐year follow up.
Design: Prospective, cohort study of 27 patients’ age 2 days‐12 years with coarcta‐
tion of the aorta undergoing surgical repair. Echocardiograms were performed pre‐… More >
Open Access
ARTICLE
Thomas G. Wilson1,2, Yves d’Udekem1,2,3, David S. Winlaw4,5, Rachael L. Cordina5,6, Julian Ayer4,5, Thomas L. Gentles7, Robert G. Weintraub1,2,8, Leeanne E. Grigg9, Michael Cheung1,8, Timothy M. Cain10, Padma Rao10, Charlotte Verrall4, Karin Du Plessis1, Kathryn Rice7, Ajay J. Iyengar1,3
Congenital Heart Disease, Vol.14, No.3, pp. 454-463, 2019, DOI:10.1111/chd.12746
Abstract Background: Patients with a Fontan circulation are at risk of renal dysfunction. We
analyzed cross‐sectional data in pediatric and adult Fontan patients in order to assess
the accuracy of commonly used serum creatinine‐based methods in estimating glo‐
merular filtration rate (GFR).
Methods: A total of 124 Fontan patients (58 children, 66 adults) were enrolled across
three study centers. Measurement of GFR (mGFR) using in vivo 99mTc‐DTPA clear‐
ance was performed. Various serum creatinine‐based equations were used to calcu‐
late estimated GFR (eGFR).
Results: Mean mGFR was 108 ± 28 mL/min/1.73 m2
in children and 92 ± 20 mL/
min/1.73 m2… More >
Open Access
ARTICLE
Mehmet Salih Bilal1, Arda Özyüksel1,2, Mustafa Kemal Avşar1, Şener Demiroluk3, Osman Küçükosmanoğlu4, Yalım Yalçın5
Congenital Heart Disease, Vol.14, No.3, pp. 464-469, 2019, DOI:10.1111/chd.12749
Abstract Objective: Management of the patients with transposition of the great arteries and
intact ventricular septum may be challenging beyond the newborn period. Herein, we
would like to present our alternative strategy for training the left ventricle in these
patients.
Methods: Six patients with transposition of the great arteries and intact ventricular
septum were evaluated in our clinic. Two of them were palliated with Glenn procedure and pulmonary banding as a definitive treatment strategy at other centers. Four
patients were operated on and a bidirectional cavopulmonary anastomosis in combination with pulmonary artery banding was performed (stage‐1: palliation and ventricular training) in… More >
Open Access
ARTICLE
Yuli Y. Kim1,2, Leah A. Goldberg2, Katherine Awh2, Tanmay Bhamare1,2, David Drajpuch2, Adi Hirshberg3, Sara L. Partington1,2, Rachel Rogers4, Emily Ruckdeschel1,2, Lynda Tobin1, Morgan Venuti2, Lisa D. Levine3
Congenital Heart Disease, Vol.14, No.3, pp. 470-478, 2019, DOI:10.1111/chd.12750
Abstract Objective: To assess performance of risk stratification schemes in predicting adverse
cardiac outcomes in pregnant women with congenital heart disease (CHD) and to
compare these schemes to clinical factors alone.
Design: Single‐center retrospective study.
Setting: Tertiary care academic hospital.
Patients: Women ≥18 years with International Classification of Diseases, Ninth
Revision, Clinical Modification codes indicating CHD who delivered between 1998
and 2014. CARPREG I and ZAHARA risk scores and modified World Health
Organization (WHO) criteria were applied to each woman.
Outcome Measures: The primary outcome was defined by ≥1 of the following: arrhyth‐
mia, heart failure/pulmonary edema, transient ischemic attack, stroke,… More >
Open Access
ARTICLE
Mahmoud Zaqout1,2, Kristof Vandekerckhove1, Nathalie Michels2, Laurent Demulier3, Thierry Bove4, Katrien François4, Julie De Backer3, Stefaan De Henauw2, Daniel De Wolf1
Congenital Heart Disease, Vol.14, No.3, pp. 479-486, 2019, DOI:10.1111/chd.12751
Abstract Objective: To investigate the status of body mass index (BMI) in adult people with
congenital heart disease (ACHD).
Methods: Five hundred thirty‐nine adults with CHD (53.8% men) were seen in the
outpatient clinic from 2013 to 2015 and compared to a reference population
(n = 1737). The severity of CHD was categorized as mild, moderate, and severe ac‐
cording to standard guidelines. Patients were categorized based on BMI as under‐
weight (<18.5), overweight (25‐30), or obese (>30). Echocardiography and magnetic
resonance imaging were used to measure ventricular function while exercise capac‐
ity was estimated via cardiopulmonary exercise test.
Results: Adults… More >
Open Access
ARTICLE
Christopher R. Broda
Congenital Heart Disease, Vol.14, No.3, pp. 487-490, 2019, DOI:10.1111/chd.12748
Abstract Heart failure is an emerging issue with important implications in adult patients with
congenital heart disease. Practitioners with expertise in both adult congenital heart
disease and heart failure are needed to manage this growing and often complex pop‐
ulation. In the United States, the optimal training pathway to enable practitioners to
best care for these patients is ill‐defined. This article explores possibilities and issues
that interested trainees may encounter during their training experience. More >
Open Access
ARTICLE
Alexander C. Egbe, Sindhura Ananthaneni, Raja Jadav, Srikanth Kothapalli, Charanjit S. Rihal, Muhammad Masood, Mounika Angirekula, Maria Najam, Numra Bajwa, Karim Tarek, Jessey Matthew, Heidi M. Connolly
Congenital Heart Disease, Vol.14, No.3, pp. 491-497, 2019, DOI:10.1111/chd.12782
Abstract Background: There are limited data about outcomes of coronary artery disease
(CAD) in adults with repaired tetralogy of Fallot (TOF). The purpose of this study was
to describe the prevalence and treatment of CAD in adults with TOF, and the impact
of CAD on long‐term survival.
Methods: Retrospective review of MACHD database for adults with repaired TOF
who underwent aortic root/selective coronary angiogram, 1990‐2017. Patients were
categorized into three groups: (1) No CAD defined as normal coronary angiogram; (2)
Mild CAD defined as ≤50% stenosis in all vessels; and, (3) Significant CAD defined
as >50% stenosis in any vessel.
Results:… More >