Congenital Heart Disease, Vol.14, No.6, pp. 1214-1214, 2019, DOI:10.1111/chd.12775

Abstract This article has no abstract. More >

Soham Dasgupta, Glen Iannucci, Chad Mao, Martha Clabby, Matthew E. Oster

Congenital Heart Disease, Vol.14, No.5, pp. 868-877, 2019, DOI:10.1111/chd.12835

Abstract Myocarditis has a variable clinical presentation and there is still debate regarding accurate diagnostic criteria. Adding to the controversy surrounding this diagnosis, there is no clear consensus for the treatment or ongoing follow‐up of patients with myocarditis. All of this makes the diagnosis and management of myocarditis a par‐ ticular challenge in the pediatric population. Furthermore, the literature with respect to this topic is dynamic and ever‐changing. In this review article, we aim to review and summarize the common clinical presentations of myocarditis, along with the latest recommendations for diagnostic criteria, treatment, and follow‐up of patients with myocarditis. More >

Kristina K. Rauser‐Foltz¹, Lois J. Starr², Anji T. Yetman¹

Congenital Heart Disease, Vol.14, No.5, pp. 864-867, 2019, DOI:10.1111/chd.12824

Abstract Objectives: This study assessed the frequency and utility of echocardiographic examination in patients with all forms of Ehlers‐Danlos syndrome and sought to iden‐ tify clinical variables associated with an abnormal echocardiogram.
Design/setting: This was a retrospective study of all patients carrying a diagnosis of Ehlers‐Danlos syndrome of any type who were evaluated by a pediatrician or pediatric subspecialist at a single tertiary medical center with an affiliated children’s hospital during the period January 2013 to December 2018.
Patients: Chart review was performed on all patients carrying a diagnosis of Ehlers‐ Danlos syndrome in the electronic medical record.
Outcome Measures: Data… More >

Jennifer K. Peterson¹, Shaun P. Setty^1,2, Jessica H. Knight³, Amanda S. Thomas⁴, James H. Moller⁵, Lazaros K. Kochilas^4,6

Congenital Heart Disease, Vol.14, No.5, pp. 854-863, 2019, DOI:10.1111/chd.12823

Abstract Objective: Patients with Trisomy 21 (T21) and single ventricle (SV) physiology present unique challenges compared to euploidic counterparts. This study reports postoperative and long‐term outcomes in patients with T21 and SV palliation.
Design: This retrospective cohort study from the Pediatric Cardiac Care Consortium (PCCC) included patients with T21 (<21 years old) that underwent surgical palliation for SV between 1982 and 2008 and control patients without known genetic anom‐ aly following Fontan palliation for similar diagnoses. Kaplan‐Meier survival plots were created based on death events obtained from the PCCC and by linkage with the National Death Index (NDI) and the Organ… 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 >

Ryan D. Byrne¹, Angela J. Weingarten², Daniel E. Clark², Shi Huang³, Roman E. Perri⁴, Andrew E. Scanga⁴, Jonathan N. Menachem², Larry W. Markham⁵, Benjamin P. Frischhertz²

Congenital Heart Disease, Vol.14, No.5, pp. 765-771, 2019, DOI:10.1111/chd.12820

Abstract Setting: Fontan‐associated liver disease universally affects adults with single ventricle heart disease. Chronic kidney disease is also highly prevalent in adult Fontan patients. In this study, we evaluate the relationship of Fontan hemodynamics invasively and noninvasively with extra‐cardiac dysfunction as measured by MELD and MELD‐XI.
Objective: We hypothesize that invasive and noninvasive measures of Fontan circuit congestion and ventricular dysfunction are associated with increased MELD and MELD‐XI scores.
Design: Single‐center data from adults with Fontan palliation who had ongoing care, including cardiac catheterization, were retrospectively collected. Hemodynamic data from cardiac catheterization and echocardiographic assessment of ventricular and atrioventricular valve function… 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 >

Daniel McLennan, Dunbar Ivy, Gareth J. Morgan

Congenital Heart Disease, Vol.14, No.5, pp. 819-831, 2019, DOI:10.1111/chd.12816

Abstract Aims: To evaluate in domestic pigs the histopathological processes after implanting the Occlutech Atrial Flow Regulator (AFR).
Methods and Results: Eleven pigs were chosen and had successful implantation of the AFR. Five pigs were sacrificed at 28 days, and 5 pigs at 90 days. One pig was sacrificed at day 3 after device embolization. Each pig had echocardiography performed at 3 weeks to check patency. Post mortem evaluation included Gross evaluation, radiographic evaluation, histology, and electron microscopy. Nine of the 10 devices implanted remained patent at time of autopsy with no thrombus and minimal inflammation. One device placed in the… More >