Open Access

EDITORIAL

Hugh D. Allen
Congenital Heart Disease, Vol.13, No.3, pp. 347-348, 2018, DOI:10.1111/chd.12628
Abstract This article has no abstract. More >

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Hitesh Agrawal^1,2, Douglas Moodie¹, Athar M. Qureshi^1,2, Alisa A. Acosta³, Jose A. Hernandez⁴, Michael C. Braun³, Henri Justino^1,2
Congenital Heart Disease, Vol.13, No.3, pp. 349-356, 2018, DOI:10.1111/chd.12608
Abstract Background: Renovascular hypertension (RVH) can be caused by renal artery stenosis (RAS) and/ or middle aortic syndrome (MAS).
Methods: Patients who received surgical or transcatheter treatment for RVH between 1/1991 and 11/2017 were retrospectively reviewed using age = adjusted blood pressure ratio (BPR).
Results: Fifty-three patients diagnosed with RVH at a median age of 4.5 (0–18) years were included. Vascular involvement ranged from MAS with RAS (20), RAS only (32), and MAS only (1). The first intervention was transcatheter in 47 patients (transcatheter group: angioplasty = 41, stenting = 5, and thrombectomy = 1), and surgical in 6… More >

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972

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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 >

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1058

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Lindsay A. Edwards^1,2, Christine Bui^2,3, Antonio G. Cabrera^1,2, Jill Ann Jarrell^2,4,5
Congenital Heart Disease, Vol.13, No.3, pp. 362-368, 2018, DOI:10.1111/chd.12579
Abstract Objective: To improve outpatient advanced care planning (ACP) for adults with congenital/pediatric heart disease followed in a pediatric heart failure (HF) and transplant clinic through quality improvement (QI) methodology.
Design: A one-year QI project was completed. We conducted quarterly chart reviews and incorporated feedback from the providers to direct subsequent interventions.
Patients and Setting: Patients ≥18 years of age seen in the HF and Transplant Clinic for followup visit were included in analysis.
Interventions: Interventions focused on five main areas: identifying and training providers to have ACP discussions, standardizing the ACP discussion, standardizing ACP and advance directive (AD) documentation… More >

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934

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Andrew D. Spearman¹, Rohit S. Loomba², Michael Danduran¹, Joshua Kovach¹
Congenital Heart Disease, Vol.13, No.3, pp. 369-376, 2018, DOI:10.1111/chd.12577
Abstract Objective: Multiple studies demonstrate the association of intrauterine growth restriction (IUGR) with impaired aerobic fitness in adolescents and adults. To our knowledge, there are no studies including individuals with the history of both IUGR and congenital heart disease (CHD). Thus, we sought to evaluate the impact of IUGR on exercise capacity in adolescents with CHD.
Study Design: We conducted a retrospective chart review of patients <18 years of age who underwent cardiopulmonary exercise testing (CPET) between August 1, 2003 and July 1, 2016. Individuals with birth weight <10th percentile for gestational age were defined as IUGR. Patients… More >

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962

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Paul C. Helm¹, Sebastian Kempert², Marc-André Körten¹, Wiebke Lesch³, Katharina Specht³, Ulrike M. M. Bauer^1,3
Congenital Heart Disease, Vol.13, No.3, pp. 377-383, 2018, DOI:10.1111/chd.12581
Abstract Background: Children and adolescents with congenital heart disease (CHD) and their families require qualified combined medical and psychosocial information, care, and counseling. This study aimed to analyze CHD patients’ and parents’ perception of disease-specific knowledge, state of health, and impairments experienced in everyday life, as well as factors influencing these perceptions.
Materials and Methods: Analyses were based on a survey among patients/parents recruited via the German National Register for Congenital Heart Defects (NRCHD). The total sample (N = 818) was divided into four groups: “Children” (176 patients), “Adolescents” (142 patients), “Adults” (269 patients), and “Parents” (231 parents).… More >

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930

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Chien-Jung Lin¹, Eric Novak¹, Michael W. Rich¹, Joseph J. Billadello^1,2
Congenital Heart Disease, Vol.13, No.3, pp. 384-391, 2018, DOI:10.1111/chd.12582
Abstract Background: Adults with congenital heart disease (ACHD) have traditionally been viewed as an underinsured population. Whether this is true in the Affordable Care Act era is unknown. We determined insurance patterns in ACHD patients compared to the non-ACHD cardiology population in a contemporary cohort.
Methods: All cardiology outpatient visits between July 2016 and February 2017 to a large referral center in the United States were reviewed. The primary payer was categorized as health maintenance organization (HMO), preferred provider organization (PPO), Medicare, Medicaid, self-pay, or other. Diagnosis and lesion severity of ACHD were extracted from ICD-10 diagnostic codes… More >

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988

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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 >

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913

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Nelly Sabbaghian¹, Maria C. Digilio², Gillian M. Blue^3,4, Timothée Revil⁵, David S. Winlaw^3,4, William D. Foulkes^1,6
Congenital Heart Disease, Vol.13, No.3, pp. 401-406, 2018, DOI:10.1111/chd.12578
Abstract Objective: We previously identified a pathogenic germline DICER1 variant in a child with transposition of the great arteries who was a member of a family with DICER1 syndrome. In view of a report linking DICER1 knockout in murine cardiomyocytes to cardiac outflow defects, we investigated the involvement of DICER1 in transposition of the great arteries.
Design: We used Fluidigm access array followed by next generation sequencing to screen for variants in the coding exons, their exon/intron boundaries and the 30 untranslated region of DICER1 in patient DNA.
Cases: Germline DNA was collected from 129 patients with either sporadic or familial More >

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1013

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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 >

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979

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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 >

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919

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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 >

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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 >

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901

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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 >

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904

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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 >

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947

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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 >

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942

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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 >

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1213

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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 >

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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 >

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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 >

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924

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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 >

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908

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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 >

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