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 >

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 >

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 >

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 >

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 >

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 >

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 >

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 >

Jamie L. Jackson¹, Jennifer Morack², Millie Harris², Jennifer DeSalvo³, Curt J. Daniels⁴, Deena J. Chisolm²

Congenital Heart Disease, Vol.14, No.2, pp. 305-310, 2019, DOI:10.1111/chd.12732

Abstract Objective: The current study aims to identify the rates of lapses in care and loss to follow‐up before age one through age five for white and nonwhite congenital heart disease (CHD) survivors. Nonwhite CHD survivors were hypothesized to experience an earlier lapse in care and be lost to follow‐up than whites.
Design: Patients were from a large pediatric hospital and had (1) at least one outpa‐ tient cardiology clinic visit or cardiac surgery visit before the age of one and (2) a di‐ agnosis of moderate or complex structural CHD. Cardiology outpatient utilization rates were tracked from before age one… More >

Charlotte A. Houck^1,2, Reinder Evertz³, Christophe P. Teuwen¹, Jolien W. Roos‐Hesselink¹, Janneke A. E. Kammeraad⁴, Anthonie L. Duijnhouwer³, Natasja M. S. de Groot³, Ad J. J. C. Bogers²

Congenital Heart Disease, Vol.14, No.2, pp. 280-287, 2019, DOI:10.1111/chd.12724

Abstract Objective: Outcomes after surgical repair of complete atrioventricular septal defect (cAVSD) have improved. With advancing age, the risk of development of dysrhythmias may increase. The aims of this study were to (1) examine development of sinus node dysfunction (SND), atrial and ventricular tachyarrhythmias, and (2) study progression of atrioventricular conduction abnormalities in young adult patients with repaired cAVSD.
Study design: In this retrospective multicenter study, 74 patients (68% female) with a cAVSD repaired in childhood were included. Patients’ medical files were evaluated for occurrence of SND, atrioventricular conduction block (AVB), atrial and ventricular tachyarrhythmias.
Results: Median age at repair was… More >