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 >

Dana Y. Fuhrman^1,2, Lan Nguyen³, Morgan Hindes³, John A. Kellum²

Congenital Heart Disease, Vol.14, No.6, pp. 963-967, 2019, DOI:10.1111/chd.12862

Abstract Background: There are significant implications for kidney disease in young adults with congenital heart disease. Prior investigations have not focused on the use of urinary tubular biomarkers for the early identification of kidney disease in this growing patient group.
Objective: Determine if young adults with congenital heart disease have differences in the baseline concentration of urinary tubular biomarkers when compared to healthy young adults.
Design/Methods: In a pilot case control study, 30 patients from 18 to 35 years of age with congenital heart disease and a normal serum creatinine were recruited during a routine follow-up visit. In the same age… More >

Nadya Golfenshtein¹, Alexandra L. Hanlon², Janet A. Deatrick³, Barbara Medoff‐Cooper^3,4

Congenital Heart Disease, Vol.14, No.6, pp. 1113-1122, 2019, DOI:10.1111/chd.12858

Abstract Objective: Parents of infants with congenital heart disease (CHD) experience increased parenting stress levels, potentially interfering with parenting practices and bear adverse family outcomes. Condition severity has been linked to parenting stress. The current study aimed to explore parenting stress trajectories over infancy in parents of infants with complex CHD, and to compare them by post‐operative cardiac physiology.
Design: Data from a larger prospective cohort study was analyzed using longitudinal mixed‐effects regression modeling.
Setting: Cardiac intensive care unit and outpatient clinic of a 480‐bed children's hospital in the American North‐Atlantic region.
Participants: Parents of infants with complex CHD (n =… More >

Michael A. Brock, John‐Anthony Coppola, Jana Reid, Diego Moguillansky

Congenital Heart Disease, Vol.14, No.6, pp. 924-930, 2019, DOI:10.1111/chd.12857

Abstract Objective: The primary aim of our work is to determine the incidence of atrial fibrillation following cardiac surgery in adults with congenital heart disease. Secondary aims include identifying risk factors predictive of developing early postoperative atrial fibrillation and morbidities associated with early postoperative atrial fibrillation.
Design: Retrospective analysis.
Setting: Single center, quaternary care children’s hospital.
Patients: This review included patients at least 18 years of age with known congenital heart disease who underwent cardiac surgery requiring a median sternotomy at our congenital heart center from January 1, 2012 to December 31, 2016.
Interventions: None.
Outcome Measures: The primary outcome was… More >

Christopher R. Broda, Katherine B. Salciccioli, Keila N. Lopez, Peter R. Ermis, Douglas S. Moodie, Heather A. Dickerson

Congenital Heart Disease, Vol.14, No.6, pp. 885-894, 2019, DOI:10.1111/chd.12856

Abstract Background: Heterotaxy syndrome (HS) is a condition in which the thoracoabdominal organs demonstrate an abnormal lateral arrangement and is often associated with con‐ genital heart disease (CHD). Little is known about the adult HS population with CHD.
Objective: To describe the outcomes and sociodemographics of the adult CHD popu‐ lation with HS.
Methods: Records of patients 18 years of age or older with diagnoses of both CHD and HS at Texas Children's Hospital from 1964 to 2018 were reviewed.
Results: Sixty‐two patients metinclusion criteria.Median agewas 22.7 [IQR19.6‐30.0] years; 26 (42%) were female; and 13 (21%) of patients had a gap… More >

Brooke T. Davey¹, Olga H. Toro‐Salazar¹, Naomi Gauthier^2,3, Anne Marie Valente², Robert W. Elder⁴, Fred M. Wu², Norman Berman³, Phyllis Pollack⁵, Ji Hyun Lee¹, Rahul H. Rathod²

Congenital Heart Disease, Vol.14, No.6, pp. 1013-1023, 2019, DOI:10.1111/chd.12854

Abstract Introduction: Surveillance and management guidelines for Fontan patients are lacking due to the paucity of evidence in the literature of screening efficacy on outcome measures.
Methods: The Fontan Working Group within the New England Congenital Cardiology Association designed an electronic survey to assess surveillance practices for pa‐ tients with Fontan procedures among New England congenital cardiologists and to explore variability in screening low‐risk vs high‐risk Fontan patients across regional programs.
Results: Fifty‐six cardiologists representing 12 regional programs responded to the survey, comprising ~40% of the total New England congenital cardiac physicians. The majority of desired testing and consultation was available… More >

Masashi Nishida, Shingo Kubo, Yuma Morishita, Kosuke Nishikawa, Kazuyuki Ikeda, Toshiyuki Itoi, Hajime Hosoi

Congenital Heart Disease, Vol.14, No.6, pp. 1087-1093, 2019, DOI:10.1111/chd.12853

Abstract Objective: This study aims to investigate the changes in renal function and levels of urinary biomarkers before and after cardiac angiography in children with congenital heart disease (CHD).
Setting: Children with CHD are at a risk for kidney injury during contrast exposure in cardiac angiography.
Outcome Measures: We measured urinary protein, albumin, N‐acetyl‐β‐D‐glucosaminidase (NAG), β2‐microglobulin (BMG), and liver‐type fatty acid‐binding protein (L‐FABP) levels, as well as serum creatinine and cystatin C levels, before and after cardiac angiography in 33 children with CHD.
Results: No significant decrease was noted in either the creatinine‐based or cystatin C‐based estimated glomerular filtration rate at… More >

Ari Cedars¹, Luke Burchill², S. Lucy Roche³, Jonathan Menachem⁴, Kelly Axsom⁵, Kristen Tecson⁶

Congenital Heart Disease, Vol.14, No.6, pp. 958-962, 2019, DOI:10.1111/chd.12851

Abstract Background: There are no published data on post‐transplant outcomes in durable ven‐ tricular assist device (VAD)‐supported adult congenital heart disease (ACHD) patients.
Methods: We compared post‐transplant outcomes in VAD‐supported vs non‐VAD‐ supported ACHD patients using the Scientific Registry of Transplant Recipients.
Results: At 1 year, there was no difference in post‐transplant mortality between VAD‐supported (12 patients) and non‐VAD‐supported (671 patients) ACHD patients.
Conclusions: In appropriate ACHD patients, VAD use as a bridge to transplant is a reasonable strategy. More >

Jim T. Vehmeijer¹, Zeliha Koyak¹, A. Suzanne Vink¹, Werner Budts^2,3, Louise Harris⁴, Candice K. Silversides⁴, Erwin N. Oechslin⁴, Aeilko H. Zwinderman⁵, Barbara J.M. Mulder^1,6, Joris R. de Groot¹

Congenital Heart Disease, Vol.14, No.6, pp. 952-957, 2019, DOI:10.1111/chd.12847

Abstract Objective: Adult congenital heart disease (ACHD) patients are at risk of sudden cardiac death (SCD). However, methods for risk stratification are not yet well‐ defined. The T_peak‐T_end (TpTe) interval, a measure of dispersion of ventricular repolari‐ zation, is a risk factor for SCD in non‐ACHD patients. We aim to evaluate whether TpTe can be used in risk stratification for SCD in ACHD patients.
Design: From an international multicenter cohort of 25 790 ACHD patients, we iden‐ tified all SCD cases. Cases were matched to controls by age, gender, congenital de‐ fect, and (surgical) intervention.
Outcome Measures: TpTe was measured on… More >

Sara Bobillo‐Perez^1,2, Joan Sanchez‐de‐Toledo^3,4, Susana Segura², Monica Girona‐Alarcon², Maria Mele⁵, Anna Sole‐Ribalta², Debora Cañizo Vazquez⁶, Iolanda Jordan^2,7, Francisco Jose Cambra^1,2

Congenital Heart Disease, Vol.14, No.6, pp. 1066-1077, 2019, DOI:10.1111/chd.12846

Abstract Objective: Three scores have been proposed to stratify the risk of mortality for each cardiac surgical procedure: The RACHS‐1, the Aristotle Basic Complexity (ABC), and the STS‐EACTS complexity scoring model. The aim was to compare the ability to predict mortality and morbidity of the three scores applied to a specific population.
Design: Retrospective, descriptive study.
Setting: Pediatric and neonatal intensive care units in a referral hospital.
Patients: Children under 18 years admitted to the intensive care unit after surgery.
Interventions: None.
Outcome measures: Demographic, clinical, and surgical data were assessed. Morbidity was considered as prolonged length of stay (LOS >… More >