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 >

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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 >

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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 >

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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 >

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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 >

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ARTICLE

Darcy N. Marckini¹, Bennett P. Samuel¹, Jessica L. Parker², Stephen C. Cook^1,3
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 >

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ARTICLE

Maayke A. Sluman^1,2, Silke Apers^3,4, Judith K. Sluiter^1,*, Karen Nieuwenhuijsen¹, Philip Moons^4,5, Koen Luyckx^6,7, Adrienne H. Kovacs^8,9, Corina Thomet¹⁰, Werner Budts¹¹, Junko Enomoto¹², Hsiao‐Ling Yang¹³, Jamie L. Jackson¹⁴, Paul Khairy¹⁵, Stephen C. Cook¹⁶, Raghavan Subramanyan¹⁷, Luis Alday¹⁸, Katrine Eriksen¹⁹, Mikael Dellborg^20,21, Malin Berghammer^5,22, 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 >

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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 >

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706

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ARTICLE

Emily M. Engelhardt¹, Andrew T. Trout^2,3, Rachel M. Sheridan⁴, Gruschen R. Veldtman⁵, Jonathan R. Dillman^2,3
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 >

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701

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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 >

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747

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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 >

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777

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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 >

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712

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ARTICLE

Karen E. Schultz¹, George K. Lui^1,2, Doff B. McElhinney¹, Jin Long³, Vidhya Balasubramanian³, Charlotte Sakarovitch³, Susan M. Fernandes^1,2, Anne M. Dubin¹, Ian S. Rogers^1,2, Anitra W. Romfh^1,2, 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 >

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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 >

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741

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ARTICLE

Jan Hinnerk Hansen¹, Lydia Kissner¹, Jana Logoteta¹, Olaf Jung¹, Peter Dütschke², Tim Attmann³, Jens Scheewe³, Hans‐Heiner Kramer^1,4
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 >

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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 >

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687

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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 >

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725

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ARTICLE

Thomas G. Wilson^1,2, Yves d’Udekem^1,2,3, David S. Winlaw^4,5, Rachael L. Cordina^5,6, Julian Ayer^4,5, Thomas L. Gentles⁷, Robert G. Weintraub^1,2,8, Leeanne E. Grigg⁹, Michael Cheung^1,8, Timothy M. Cain¹⁰, Padma Rao¹⁰, Charlotte Verrall⁴, Karin Du Plessis¹, Kathryn Rice⁷, Ajay J. Iyengar^1,3
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 >

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ARTICLE

Mehmet Salih Bilal¹, Arda Özyüksel^1,2, 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 >

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ARTICLE

Yuli Y. Kim^1,2, Leah A. Goldberg², Katherine Awh², Tanmay Bhamare^1,2, David Drajpuch², Adi Hirshberg³, Sara L. Partington^1,2, Rachel Rogers⁴, Emily Ruckdeschel^1,2, 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 >

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ARTICLE

Mahmoud Zaqout^1,2, 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 >

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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 >

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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 >

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