Alka Anilkumar¹, D. M. Vasudevan², Mahesh Kappanayil³, K. R. Sundaram⁴, R. Krishna Kumar³, Sheela Nampoothiri⁵

Congenital Heart Disease, Vol.13, No.3, pp. 483-487, 2018, DOI:10.1111/chd.12600

Abstract Background and Objectives: The 22q11 deletion syndrome is associated with a wide spectrum of phenotypic features, hence clinical diagnosis is difficult. Individuals with this syndrome are found to have a risk of developing transfusion associated graft-versus-host reaction, if they are given nonirradiated blood. Our aim was to elucidate whether chromosome 22q11 deletion in children with syndromic conotruncal heart defects is associated with platelet abnormality.
Materials and Methods: The genetic analysis was performed by standard cytogenetic and Fluorescence in situ hybridization technique. The platelet parameters in 39 patients with chromosome 22q11 deletion were compared with 154 cases without deletion.
Results:… More >

Ginnie Abarbanell¹, William L. Border², Brian Schlosser², Gemma Morrow², Michael Kelleman², Ritu Sachdeva²

Congenital Heart Disease, Vol.13, No.3, pp. 476-482, 2018, DOI:10.1111/chd.12599

Abstract Objective: It is unclear whether neonates with interrupted aortic arch (IAA) and a smaller left ventricular outflow tract may have improved outcomes with a Yasui operation (ventricular outflow bypass procedure) over a primary complete repair. This study sought to identify preoperative echocardiographic parameters to differentiate which neonates may have improved outcomes with a primary vs Yasui operation.
Design: Patient demographics, cardiac surgery type, complications, need for reoperation and/or interventional catheterization, and date of last follow-up were collected on neonates who underwent a biventricular repair for IAA from 2003 to 2014. Preoperative echocardiograms were analyzed for: IAA type,… More >

Raymond P. Lorenzoni III¹, Jaeun Choi², Nadine F. Choueiter³, Iona M. Munjal¹, Chhavi Katyal⁴, Kenan W. D. Stern³

Congenital Heart Disease, Vol.13, No.3, pp. 470-475, 2018, DOI:10.1111/chd.12598

Abstract Objective: Kawasaki disease is the primary cause of acquired pediatric heart disease in developed nations. Timely diagnosis of Kawasaki disease incorporates transthoracic echocardiography for visualization of the coronary arteries. Sedation improves this visualization, but not without risks and resource utilization. To identify potential sedation criteria for suspected Kawasaki disease, we analyzed factors associated with diagnostically inadequate initial transthoracic echocardiography performed without sedation.
Design: This retrospective review of patients < 18 years old undergoing initial transthoracic echocardiography for the inpatient evaluation of suspected Kawasaki disease from 2009 to 2015 occurred at a medium-sized urban children’s hospital. The primary… More >

Shaji C. Menon¹, Ragheed Al-Dulaimi¹, Brian W. McCrindle², David J. Goldberg³, Ritu Sachdeva⁴, Bryan H. Goldstein⁵, Thomas Seery⁶, Karen C. Uzark⁷, Anjali Chelliah⁸, Ryan Butts⁹, Heather Henderson¹⁰, Tiffanie Johnson¹¹, Richard V. Williams¹

Congenital Heart Disease, Vol.13, No.3, pp. 463-469, 2018, DOI:10.1111/chd.12597

Abstract Introduction: We sought to evaluate the prevalence of delayed puberty and abnormal anthropometry and its association with quality of life (QoL) in young Fontan survivors.
Methods: This was a cross-sectional study at 11 Pediatric Heart Network centers. Demographic and clinical data, anthropomety, and Tanner stage were collected. Anthropometric measurements and pubertal stage were compared to US norms. QoL was assessed using Pediatric Quality of Life inventory (PedsQL). Mixed effects regression modeling adjusting for clustering by center was used to evaluate factors associated with abnormal anthropometry and delayed puberty and associations with QoL.
Results: Of the 299 subjects, 42%… More >

Gareth J. Morgan¹, Matteo Ciuffreda², Isabella Spadoni³, Joseph DeGiovanni⁴

Congenital Heart Disease, Vol.13, No.3, pp. 458-462, 2018, DOI:10.1111/chd.12596

Abstract Aim: To assess the acute results of the first human use of the Optimus covered stent in complex coarctation of the aorta.
Methods and Results: We successfully implanted the Optimus covered stent in eight cases in patients whose preprocedural anatomy looked challenging for currently available covered stents. Six of the patients had native coarctation with one recoarctation following surgical repair. There were no significant complications with reduction in the mean invasive gradient from 22 to 1 mm Hg. The length of stents used ranged from 33 to 57 mm with a median shortening after expansion of 13%. More >

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 >