Paolo D’Ambrosio^1,2, Derek Tran^1,2, Charlotte E. Verrall^3,4, Chantal Attard⁵, Maria Fiatarone Singh^6,7, Julian Ayer^3,4,8, Yves d’Udekem^5,9,10, Stephen Twigg^2,11, David S. Celermajer^1,2,12, Rachael Cordina^1,2,5

Congenital Heart Disease, Vol.14, No.6, pp. 987-995, 2019, DOI:10.1111/chd.12836

Abstract Objective and Patients: This study aimed to characterize bone mineral density abnormalities and pathophysiological associations in young adults living with a Fontan circulation.
Design: Participants underwent bone mineral density measurement using dual‐energy X‐ray absorptiometry and serum biochemical analysis, cardiopulmonary exercise and strength testing and transthoracic echocardiography.
Results: In our cohort (n = 28), 29% had osteopenic‐range bone mineral density and one patient was osteoporotic (average hip t score: −0.6 ± 1.1; spine t score: −0.6 ± 0.9). Four patients (14%) had z scores < −2.0. Parathyroid hormone levels were increased compared with laboratory median (6.1 ± 3.5 vs 4 pmol/L,… More >

Benjamin Zielonka¹, Yuli Y. Kim^2,3, Gregory E. Supple², Sara L. Partington^2,3, Emily S. Ruckdeschel^2,3, Francis E. Marchlinski², David S. Frankel²

Congenital Heart Disease, Vol.14, No.6, pp. 931-938, 2019, DOI:10.1111/chd.12833

Abstract Objective: Atrial arrhythmias and atrioventricular valve regurgitation (AVVR) are common causes of morbidity among adults with congenital heart disease (ACHD). The impact of rhythm control on AVVR in this population is unknown. We sought to determine whether a rhythm control strategy is associated with greater freedom from AV valve surgery than a rate control strategy.
Design: Patients evaluated by both ACHD and electrophysiology specialists at a single academic center were screened for atrial arrhythmias and at least moderate‐severe AVVR. Clinical and electrographic data were abstracted. All echocardiograms were in‐ terpreted by a single echocardiographer blinded to treatment strategy. Patients were… More >

Jeremy M. Steele^1,2, Rukmini Komarlu², Sarah Worley³, Tarek Alsaied¹, Christopher Statile¹, Francine G. Erenberg²

Congenital Heart Disease, Vol.14, No.6, pp. 1193-1198, 2019, DOI:10.1111/chd.12829

Abstract Objective: Deciding on a surgical pathway for neonates with ≥2 left heart obstructive lesions is complex. Predictors of the successful biventricular (2V) repair in these patients are poorly defined. The goal of our study was to identify patients who underwent the 2V repair and assess anatomic and echocardiographic predictors of success.
Design: Infants born between July 2015 and August 2017 with ≥2 left heart obstructive lesions with no prior interventions were identified (n = 19). Patients with aortic or mitral valve (MV) atresia and critical aortic stenosis were excluded. Initial echocardiograms were reviewed for aortic, MV, tricuspid valve annulus size,… More >

Line Marie Holst¹, Faridis Serrano², Lara Shekerdemian², Hanne Berg Ravn¹, Danielle Guffey³, Nancy S. Ghanayem², Sonia Monteiro⁴

Congenital Heart Disease, Vol.14, No.6, pp. 1207-1213, 2019, DOI:10.1111/chd.12827

Abstract Objective: To investigate the impact of feeding mode on neurodevelopmental outcomes in children with congenital heart defects.
Design: A retrospective cohort study of 208 children with congenital heart disease (CHD), who had surgery from 1 January 2013 until 31 December 2016 at Texas Children’s Hospital, Houston, TX, US.
Settings: University Hospital, Developmental Outcome Clinic.
Outcomes measures: Standardized cognitive scores were assessed with Capute Scales and motor development with Revised Gesell Developmental Schedules. We analyzed anthropometrics, mode of feeding, surgical complexity, syndrome, and gen‐ der as predictors of developmental outcomes at four time points: hospital discharge, and 6, 12, and 24… More >

M. Louise Morrison¹, Brian Grant¹, Brian A. McCrossan^1,2, Andrew J. Sands^1,2, Colum G. Owens², Mark S. Spence², Frank A. Casey¹, Brian G. Craig^1,2, Christopher J. Lockhart²

Congenital Heart Disease, Vol.14, No.5, pp. 846-853, 2019, DOI:10.1111/chd.12822

Abstract Objective: A significant body of patients who have undergone Mustard or Senning procedure require lifelong follow up. In this retrospective review, we examined the cohort of such patients currently attending our center.
Design: Patients who had undergone either Mustard or Senning procedure were identified. We retrospectively reviewed medical records, recorded demographic in‐ formation and data regarding the clinical state, NHYA class, cardiopulmonary exer‐ cise testing, NT‐proBNP measurement, and recent cardiac MRI findings.
Results: Forty‐six patients were identified, the mean age was 32.2 years (± 6.1 years), 67.4% were male. Thirty‐two patients (69.6%) had undergone a Senning procedure. The median length… More >

Katherine B. Salciccioli¹, Abiodun Oluyomi², Philip J. Lupo³, Peter R. Ermis¹, Keila N. Lopez¹

Congenital Heart Disease, Vol.14, No.5, pp. 752-759, 2019, DOI:10.1111/chd.12819

Abstract Background: Follow‐up at a regional adult congenital heart disease (ACHD) center is recommended for all ACHD patients at least once per the 2018 ACC/AHA guidelines. Other specialties have demonstrated poorer follow‐up and outcomes correlating with increased distance from health care providers, but driving time to regional ACHD centers has not been examined in the US population.
Objective: To identify and characterize potential disparities in access to ACHD care in the US based on drive time to ACHD centers and compounding sociodemographic factors.
Methods: Mid‐ to high‐volume ACHD centers with ≥500 outpatient ACHD visits and ≥20 ACHD surgeries annually were included… More >

Subeer K. Wadia¹, Gentian Lluri¹, Jamil A. Aboulhosn¹, Hillel Laks², Reshma M. Biniwale², Glen S. Van Arsdell², Daniel S. Levi³, Morris M. Salem³, Kevin M. Shannon^1,3, Jeremy P. Moore^1,3

Congenital Heart Disease, Vol.14, No.5, pp. 838-845, 2019, DOI:10.1111/chd.12818

Abstract Objective: We examined the atrial tachyarrhythmia (AT) burden among patients with congenital heart disease (CHD) following transcatheter (TC‐) or surgical (S‐) pulmo‐ nary valve replacement (PVR).
Design/Setting: This was a retrospective observational study of patients who under‐ went PVR from 2010 to 2016 at UCLA Medical Center.
Patients: Patients of all ages who had prior surgical repair for CHD were included. Patients with a history of congenitally corrected transposition of the great arteries, underwent a hybrid PVR procedure, or had permanent atrial fibrillation (AF) without a concomitant ablation were excluded.
Outcome Measures: The primary outcome was a time‐to‐event analysis of… More >

Gabrielle C. Geddes^1,2, Erin Syverson^1,2, Michael G. Earing^1,2

Congenital Heart Disease, Vol.14, No.5, pp. 832-837, 2019, DOI:10.1111/chd.12817

Abstract Objective: To describe the first 3 years of experience of having an inpatient “cardiogenetics” program which involves medical geneticist assessment of infants with major congenital heart disease (CHD) requiring surgical intervention in the first year of life.
Patients: Patients less than a year of age admitted to Children’s Hospital of Wisconsin’s Herma Heart Institute for surgical intervention for CHD seen by the cardiogenetics program. Patients with major trisomies (13, 18, and 21) were excluded.
Outcome Measures: Utilization and yield of genetic testing, and diagnostic rate were assessed as outcome measures and compared to a baseline time period and a genetic… More >

Anudeep K. Dodeja¹, Courtney Thomas¹, Curt J. Daniels^1,2, Naomi Kertesz^1,2, Anna Kamp^1,2

Congenital Heart Disease, Vol.14, No.5, pp. 745-751, 2019, DOI:10.1111/chd.12815

Abstract Background: Rhythm disorders are the leading cause of morbidity and mortality in adults with congenital heart disease (ACHD). Infrequent or asymptomatic arrhyth‐ mias may not be detected by routine monitoring. Implantable loop recorders (ILRs), such as the Reveal LINQ^TM, have been useful in long‐term monitoring for arrhythmias in adults with cryptogenic stroke.
Objective: We propose the Reveal LINQ^TM will detect arrhythmias, not documented by other monitoring modalities, resulting in change in management in ACHD patients.
Methods: This is a single center retrospective review of Reveal LINQ^TM use in ACHD patients from 2014‐2017. Medical records were reviewed to determine cardiac diag‐… More >

Natalie Bottega¹, Isabelle Malhamé², Liming Guo¹, Raluca Ionescu‐Ittu¹, Judith Therrien¹, Ariane Marelli¹

Congenital Heart Disease, Vol.14, No.5, pp. 735-744, 2019, DOI:10.1111/chd.12811

Abstract Background: The number of women with congenital heart disease (CHD) of reproductive age is increasing, yet a description of trends in pregnancy and delivery outcomes in this population is lacking.
Objective: To assess secular trends in pregnancy rates, delivery outcomes, and related health care utilization in the adult female CHD population in Quebec, Canada.
Methods: The Quebec CHD database was used to construct a cohort with all women with CHD aged 18‐45 years between 1992 and 2004. Pregnancy and delivery rates were determined yearly and compared to the general population. Secular trends in pregnancy and delivery rates were assessed with… More >