Taner Kasar¹, Pelin Ayyildiz¹, Gulhan Tunca Sahin¹, Erkut Ozturk¹, Selman Gokalp¹, Sertac Haydin², Alper Guzeltas¹, Yakup Ergul¹

Congenital Heart Disease, Vol.13, No.3, pp. 450-457, 2018, DOI:10.1111/chd.12595

Abstract Background: We aimed to evaluate rhythm abnormalities in cases of congenitally corrected transposition of the great arteries (ccTGA) and associated treatment strategies.
Patients and Methods: This retrospective cohort study included 65 pediatric patients with ccTGA who were admitted to the clinic between 2009 and 2017. The patients were divided into two groups, and surgical data, Holter electrocardiographic (ECG) recordings, ECG recordings, electrophysiological data, and device implantation data on the two groups were compared.
Results: Group I (n = 53, 82%) consisted of patients with significant associated lesions, and Group II (n = 12, 18%) consisted of those with… More >

Michael V. Di Maria¹, Lindsey Silverman², Adel K. Younoszai¹, Mariana L. Meyers³, Kelley E. Capocelli⁴, Michael R. Narkewicz⁵

Congenital Heart Disease, Vol.13, No.3, pp. 444-449, 2018, DOI:10.1111/chd.12594

Abstract Background: The current management paradigm for children with single ventricle heart disease involves a series of palliative surgeries, culminating in the Fontan operation. This physiology results in a high central venous pressure, and in the setting of single ventricle heart disease, results in hepatic injury and fibrosis over time. Fontan-associated liver disease (FALD) is universally present in this cohort, and the current gold standard for diagnosis remains biopsy. Noninvasive assessments of liver fibrosis, such as ultrasound with elastography or acoustic radiation force impulse (ARFI), has been utilized in this cohort. The effect of poor cardiovascular efficiency,… More >

M. Louise Morrison¹, Corrina McMahon², Riona Tully¹, Noelle Enright¹, Ricardo Pignatelli³, Jeffrey A. Towbin⁴, Colin J. McMahon¹

Congenital Heart Disease, Vol.13, No.3, pp. 440-443, 2018, DOI:10.1111/chd.12592

Abstract Objectives: Incidence of sickle cell disease (SCD) in Ireland has dramatically increased. Disease survival has also steadily improved however cardiovascular manifestations remain important causes of morbidity. These include reports of left ventricular hypertrabeculation (LVHT)/noncompaction. We sought to investigate the prevalence of LVHT among a large cohort of children with SCD.
Methods: We retrospectively reviewed the records of all patients with a diagnosis of SCD who had undergone surveillance echocardiography at Our Lady’s Children’s Hospital Crumlin (OLCHC) from 1998 to 2015. Demographics, hemoglobin phenotype and treatment information was recorded. LV systolic function, evidence of LVHT, and possible pulmonary… More >

Melissa S.W. Yamauchi^1,2, Michael D. Puchalski^1,2, Hsin Ti Weng¹, Nelangi M. Pinto^1,2, Susan P. Etheridge^1,2, Angela P. Presson¹, Lloyd Y. Tani^1,2, L. LuAnn Minich^1,2, Richard V. Williams^1,2

Congenital Heart Disease, Vol.13, No.3, pp. 432-439, 2018, DOI:10.1111/chd.12591

Abstract Background: Disease progression of an isolated bicuspid aortic valve (BAV) in children is poorly understood and adult management guidelines may not be applicable. Thus, we sought to evaluate disease progression of pediatric isolated BAV and its relationship to current management practices.
Methods: Children with a BAV and ≤mild aortic stenosis (AS) and/or aortic regurgitation (AR) at the time of initial evaluation were included in this retrospective cohort study (1/2005-12/ 2014). Outcomes included change in z-scores for aortic root and ascending aorta diameters, cardiac interventions, adverse outcomes, recommended follow-up interval, and frequency of cardiac imaging studies at each… More >

Adam S. Walpert¹, Ian D. Thomas², Merlin C. Lowe Jr¹, Michael D. Seckeler³

Congenital Heart Disease, Vol.13, No.3, pp. 428-431, 2018, DOI:10.1111/chd.12590

Abstract Objective: The aim of this study was to compare inpatient outcomes and costs for children with respiratory syncytial virus and congenital heart disease before and after the change in management guidelines for respiratory syncytial virus prophylaxis.
Design: Hospital discharge data from the Vizient (formerly University HealthSystem Consortium) were queried from October 2012 to June 2014 (Era 1) and July 2014 to April 2016 (Era 2) for patients aged <24 months with an any International Classification of Disease (ICD)-9 or ICD-10 code for congenital heart disease (745-747.49, Q20.0-Q26.4) and a primary or secondary admitting diagnosis of respiratory syncytial… More >

Omar Meziab, Dominic J. Abrams, Mark E. Alexander, Laura Bevilacqua, Vassilios Bezzerides, Doug Y. Mah, Edward P. Walsh, John K. Triedman

Congenital Heart Disease, Vol.13, No.3, pp. 419-427, 2018, DOI:10.1111/chd.12589

Abstract Objective: This study evaluates the ability of experienced pediatric electrophysiologists (EPs) to reliably classify incomplete right bundle branch block (IRBBB) and assesses its clinical utility as an isolated ECG finding in a group of healthy outpatient children without prior cardiac evaluation.
Design: We performed a retrospective analysis of all electrocardiographic and echocardiographic records at Boston Children’s Hospital between January 1, 2005, and December 31, 2014. Echocardiographic diagnoses were identified if registered between the date of the index electrocardiogram and the ensuing year. A selected subset of 473 ECGs was subsequently reanalyzed in a blinded manner by six… More >

Chin Siang Ong¹, Aravind Krishnan¹, Chen Yu Huang¹, Philip Spevak², Luca Vricella¹, Narutoshi Hibino¹, Juan R. Garcia², Lasya Gaur³

Congenital Heart Disease, Vol.13, No.3, pp. 357-361, 2018, DOI:10.1111/chd.12587

Abstract Objective: New platforms for patient imaging present opportunities for improved surgical planning in complex congenital heart disease (CHD). Virtual reality (VR) allows for interactive manipulation of high-resolution representations of patient-specific imaging data, as a supplement to traditional 2D visualizations and 3D printed heart models.
Design: We present the novel use of VR for the presurgical planning of cardiac surgery in two infants with complex CHD to demonstrate interactive real-time views of complex intra and extracardiac anatomy.
Results: The use of VR for cardiac presurgical planning is feasible using existing imaging data. The software was evaluated by both pediatric More >

Stephanie A. Goldstein, Martin J. LaPage, Brynn E. Dechert, Gerald A. Serwer, Sunkyung Yu, Ray E. Lowery, David J. Bradley

Congenital Heart Disease, Vol.13, No.3, pp. 413-418, 2018, DOI:10.1111/chd.12585

Abstract Objective: Inappropriate implantable cardioverter defibrillator (ICD) shocks in children and patients with congenital heart disease (CHD) remain a major complication of device therapy, occurring in as many as 50% of children with ICDs. New generation devices include algorithms designed to minimize inappropriate shocks. This study aimed to evaluate the effect of new generation ICDs on the incidence of inappropriate shocks in the pediatric and CHD population.
Design: Retrospective study of patients with CHD or under age 25 receiving ICDs between 2000 and 2015. New generation ICDs were defined as those with Medtronic “SmartShock” algorithms.
Results: Two hundred eight… More >

Rick D. Vavolizza^1,2, Pe’er Dar^3,4, Barrie Suskin^3,4, Robert M. Moore^4,5, Kenan W.D. Stern^1,4

Congenital Heart Disease, Vol.13, No.3, pp. 407-412, 2018, DOI:10.1111/chd.12584

Abstract Objective: Suboptimal cardiac imaging on obstetric ultrasound is a frequent referral indication for fetal echocardiography, even in the absence of typical risk factors for fetal cardiac disease. The clinical profile of patients and findings of examinations performed for such an indication are not well defined. Given the increased cost, time and resource utilization of fetal echocardiography, we sought to determine the clinical findings of such referrals.
Study Design: We performed a single-center review of such referrals from January 2010 to June 2016. Patients with commonly accepted indications for fetal echocardiography were excluded. Demographic variables and echocardiogram findings… More >

Christina E. Holbein¹, Nicholas D. Fogleman^1,2, Kevin Hommel¹, Silke Apers³, Jessica Rassart³, Philip Moons^3,4, Koen Luyckx³, Maayke A. Sluman⁵, Junko Enomoto⁶, Bengt Johansson⁷, Hsiao-Ling Yang⁸, Mikael Dellborg^4,9, Raghavan Subramanyan¹⁰, Jamie L. Jackson¹¹, Werner Budts^3,12, Adrienne H. Kovacs¹³, Stacey Morrison¹, Martha Tomlin¹, Kathy Gosney¹, Alexandra Soufi¹⁴, Katrine Eriksen¹⁵, Corina Thomet^3,16, Malin Berghammer^4,17, Luis Alday¹⁸, Edward Callus¹⁹, Susan M Fernandes²⁰, Maryanne Caruana²¹, Samuel Menahem²², Stephen C. Cook²³, Gwen R. Rempel²⁴, Kamila White²⁵, Paul Khairy²⁶, Shelby Kutty²⁷, Gruschen Veldtman¹

Congenital Heart Disease, Vol.13, No.3, pp. 392-400, 2018, DOI:10.1111/chd.12583

Abstract Objective: First, to compare QOL and illness perceptions between patients with a Fontan circulation and patients with anatomically simple defects (ie, atrial septal defects [ASD] or ventricular septal defects [VSD]). Second, to explore illness perceptions as a mediator of the association between congenital heart disease (CHD) diagnosis and QOL.
Design: Cross-sectional observational study.
Setting: Twenty-four cardiology centers from 15 countries across five continents.
Patients: Four hundred thirty-five adult patients with congenital heart disease (177 Fontan and 258 ASD/VSD) ages 18-83 years.
Outcome Measures: QOL and illness perceptions were assessed by the Satisfaction With Life Scale and the Brief Illness Perceptions… More >