Open Access

EDITORIAL

Daniel J Penny
Congenital Heart Disease, Vol.12, No.3, pp. 241-241, 2017, DOI:10.1111/chd.12474
Abstract This article has no abstract. More >

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REVIEW

Ian K. Everitt¹, Jennifer F. Gerardin², Fred H. Rodriguez^2,3, Wendy M. Book²
Congenital Heart Disease, Vol.12, No.3, pp. 242-25, 2017, DOI:10.1111/chd.12463
Abstract The transition and transfer from pediatric to adult care is becoming increasingly important as improvements in the diagnosis and management of congenital heart disease allow patients to live longer. Transition is a complex and continuous process that requires careful planning. Inadequate transition has adverse effects on patients, their families and healthcare delivery systems. Currently, significant gaps exist in patient care as adolescents transfer to adult care and there are little data to drive the informed management of transition and transfer of care in adolescent congenital heart disease patients. Appropriate congenital heart disease care has been… More >

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REVIEW

Katherine Smith, Bernard Gros
Congenital Heart Disease, Vol.12, No.3, pp. 251-260, 2017, DOI:10.1111/chd.12465
Abstract A well-established association exists between acute aortic dissection and pregnancy, particularly in women with Marfan syndrome. However, there is debate regarding appropriate management guidelines. In particular, there are differing opinions regarding when prophylactic aortic root repair should be recommended as well as the efficacy of beta blockers in this clinical scenario. The current study evaluated 10 years of published literature (2005-2015) in the PubMed/Medline database. Fifty articles, describing 72 cases of women who presented with aortic dissection in the antepartum or postpartum period were identified. Comparisons on demographic variables and clinical outcomes between cases of… More >

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1087

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ARTICLE

Talha Niaz¹, Joseph T. Poterucha¹, Jonathan N. Johnson^1,2, Cecilia Craviari¹, Thomas Nienaber¹, Jared Palfreeman¹, Frank Cetta^1,2, Donald J. Hagler^1,2
Congenital Heart Disease, Vol.12, No.3, pp. 261-269, 2017, DOI:10.1111/chd.12429
Abstract Background: Bicuspid aortic valve (BAV) occurs both as an isolated cardiac lesion and in association with congenital heart defects (CHD). Their aim was to identify the incidence and morphology of BAV in patients with coexisting CHD and compare their disease progression to patients with isolated BAV.
Methods: The Mayo Clinic echocardiography database was retrospectively analyzed to identify pediatric and young adult patients (≤22 years) who were diagnosed with BAV from 1990 to 2015. The morphology of BAV was determined from the echocardiographic studies before any intervention.
Results: Overall, 1010 patients with BAV were identified, 619 (61%) with isolated… More >

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1050

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ARTICLE

Delaram Molkara, Jose A. Silva Sepulveda, Thomas Do, Christopher Davis, Gregory P. Goldstein, John W. Moore, Howaida G El-Said
Congenital Heart Disease, Vol.12, No.3, pp. 270-274, 2017, DOI:10.1111/chd.12432
Abstract Objective: Neonatal coronary thrombosis is a rarely reported disorder, with variable outcomes described. This study assessed the feasibility and safety of an institutional protocol using tissue plasminogen activator (tPA) administration for the treatment of neonatal coronary artery thrombi.
Methods: They reviewed the outcome of three neonates with clinical evidence of myocardial infarction secondary to coronary thrombosis. All three underwent the tPA treatment protocol.
Results: The three described cases presented at 5 hours, 15 hours, and 10 days of life. The patients identified underwent the tPA protocol at least once. There was clinical evidence of improvement in coronary flow, More >

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1104

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ARTICLE

Janet M. Simsic, Kirby-Rose Carpenito, Kristin Kirchner, Stephanie Peters, PNP-AC, Holly Miller-Tate, Brian Joy, Mark Galantowicz
Congenital Heart Disease, Vol.12, No.3, pp. 275-281, 2017, DOI:10.1111/chd.12435
Abstract Objective: Enteral feeding is associated with decreased infection rates, decreased mechanical ventilation, decreased hospital length of stay, and improved wound healing. Enteral feeding difficulties are common in congenital heart disease. Our objective was to develop experience-based newborn feeding guidelines for the initiation and advancement of enteral feeding in the cardiothoracic intensive care unit.
Design: This is a retrospective analysis of a quality improvement project.
Setting: This quality improvement project was performed in a cardiothoracic intensive care unit.
Patients: Newborns admitted to the cardiothoracic intensive care unit for cardiac surgery from January 2011 to May 2015 were retrospectively reviewed.
Intervention: Newborn… More >

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ARTICLE

Jeffrey H. Sacks^1,2, Michael Kelleman², Courtney McCracken², Michelle Glanville¹, Matthew Oster^1,2
Congenital Heart Disease, Vol.12, No.3, pp. 282-288, 2017, DOI:10.1111/chd.12436
Abstract Objective: Hospital readmissions are increasingly becoming a metric for quality in the current landscape of changing and cost effective medicine. However, no 30-d readmission rates have been established for pediatric cardiac medical patients in the United States. Thus, the objective was to determine 30 d readmission rates and risk factors associated with readmission for pediatric cardiac patients, hypothesizing that pediatric cardiac patients would have significantly higher readmission rates than their general pediatric counterparts.
Design: This was a retrospective cohort study.
Setting: The study took place at a large urban academic children’s hospital.
Patients: The 1124 included patients were discharged… More >

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923

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ARTICLE

Xinghua Gu¹, Qiuwang Zhang², Hourong Sun¹, Jianchun Fei¹, Xiquan Zhang¹, Michael J. Kutryk²
Congenital Heart Disease, Vol.12, No.3, pp. 289-293, 2017, DOI:10.1111/chd.12437
Abstract Objective: To present our experience in transcatheter closure of calcified patent ductus arteriosus (PDA) in older adult patients, which has rarely been reported.
Patients: From 2009 to 2014, a total of 16 patients (median age 58 years) with calcified PDA underwent transcatheter closure in our center. All patients were symptomatic with major symptoms being exertional dyspnea (in 12), palpitations (in 8), and fatigue (in 5). A continuous murmur was heard in all patients. The median ductus diameter was 4 mm (range 3–7 mm). The median Qp/Qs was 1.6 (range 1.4–2.9).
Interventions: Transcatheter closure was performed for all patients.… More >

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1033

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ARTICLE

Antonio R. Mott¹, Steven R. Neish², Melissa Challman³, Timothy F. Feltes⁴
Congenital Heart Disease, Vol.12, No.3, pp. 294-300, 2017, DOI:10.1111/chd.12438
Abstract Background: The treatment of children with cardiac disease is one of the most prevalent and costly pediatric inpatient conditions. The design of inpatient medical services for children admitted to and discharged from noncritical cardiology care units, however, is undefined. North American Pediatric Cardiology Programs were surveyed to define noncritical cardiac care unit models in current practice.
Method: An online survey that explored institutional and functional domains for noncritical cardiac care unit was crafted. All questions were multi-choice with comment boxes for further explanation. The survey was distributed by email four times over a 5-month period.
Results: Most programs… More >

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949

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ARTICLE

Maryanne Caruana¹, Victor Grech²
Congenital Heart Disease, Vol.12, No.3, pp. 301-308, 2017, DOI:10.1111/chd.12439
Abstract Objectives: To determine overall and reintervention-free survival for repaired Maltese tetralogy of Fallot patients and to investigate the potential impact of gender, age at repair, genetic syndromes, previous palliation, and type of repair on these outcomes.
Design: All 130 tetralogy of Fallot patients born before the end of 1997 included in the local database were extracted. Surgical repair type, age at repair and operative survival were analyzed among the 103/130 repaired patients. Kaplan–Meier survival analyses were performed on the 75 repair survivors with complete follow-up data (mean follow-up 26.37 ± 9.27 (range 9.95–51.21) years).
Results: Patients born after… More >

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1068

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ARTICLE

Paul Cooper¹, Morgan Hindes², Timothy M. Maul^3,4, Stephen C. Cook⁵
Congenital Heart Disease, Vol.12, No.3, pp. 309-314, 2017, DOI:10.1111/chd.12441
Abstract Objective: Medical residents are exposed to increasing numbers of adults with congenital heart disease (ACHD). While inadequate ACHD knowledge may lead to inappropriate practice, this educational deficit has not been investigated. Our aim was to analyze residents’ attitudes, perceived ability, and knowledge of ACHD medicine.
Design, Methods, Outcome Measures: A single center, multiprogram cross-sectional study was conducted in 2015 using an electronic survey to assess 472 medical residents’ perceived knowledge and self-assessed skills related to ACHD medicine. Demographic data obtained included age, gender, level of training and program type.
Results: The survey was completed by 25% of surveyed… More >

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987

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ARTICLE

Waldemar F. Carlo¹, James F. Cnota², Robert J. Dabal³, Jeffrey B. Anderson²
Congenital Heart Disease, Vol.12, No.3, pp. 315-321, 2017, DOI:10.1111/chd.12442
Abstract Objective: The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) was established in 2008 to improve outcomes of hypoplastic left heart syndrome (HLHS) during the interstage period. They evaluated changes in patient variables and practice variation between early and late eras.
Design: Data including demographic, operative, discharge, and follow-up variables from the first 100 patients (6/2008–1/2010) representing 18 centers were compared with the most recent 100 patients (1/2014–11/2014) from these same centers.
Results: Prenatal diagnosis increased from 69% to 82% (P = .05). There were no differences in gestational age or weight at Norwood. A composite of any preoperative… More >

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942

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ARTICLE

Stephanie M. Ford¹, Matthew T. McPheeters², Yves T. Wang³, Pei Ma¹³, Shi Gu³, James Strainic⁴, Christopher Snyder⁴, Andrew M. Rollins³, Michiko Watanabe², Michael W. Jenkins²
Congenital Heart Disease, Vol.12, No.3, pp. 322-331, 2017, DOI:10.1111/chd.12443
Abstract Background: The relationship between changes in endocardial cushion and resultant congenital heart diseases (CHD) has yet to be established. It has been shown that increased regurgitant flow early in embryonic heart development leads to endocardial cushion defects, but it remains unclear how abnormal endocardial cushions during the looping stages might affect the fully septated heart. The goal of this study was to reproducibly alter blood flow in vivo and then quantify the resultant effects on morphology of endocardial cushions in the looping heart and on CHDs in the septated heart.
Methods: Optical pacing was applied to create… More >

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ARTICLE

Rohit S. Loomba¹, Gabrielle Geddes¹, Amanda J. Shillingford², David A. Hehir²
Congenital Heart Disease, Vol.12, No.3, pp. 332-339, 2017, DOI:10.1111/chd.12448
Abstract Background: Splenic dysfunction is common in heterotaxy syndrome, and increases the risk of bacteremia and bacteremia related mortality. Despite the risks associated with bacteremia in this setting, best practice guidelines for management of infectious concerns are lacking. We conducted a survey of pediatric cardiologists to characterize practice regarding the diagnosis of splenic dysfunction, approach to antibiotic prophylaxis, and management of possible bacterial infection.
Methods: A 22-item web-based survey was distributed via email to pediatric cardiologists in North America.
Results: We received 230 responses from 63 centers, for a response rate of 22%. The majority (83%) always obtain abdominal… More >

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1067

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ARTICLE

Michael Perez¹, Tk Susheel Kumar², Deepthi Hoskoppal³, Yada Akkhawattanangkul⁴, Kimberly Allen⁵, Christopher J. Knott-Craig², Benjamin Rush Waller¹, Shyam Sathanandam¹
Congenital Heart Disease, Vol.12, No.3, pp. 340-349, 2017, DOI:10.1111/chd.12449
Abstract Objectives: The primary objective of this study was to demonstrate that pulmonary artery (PA) debanding via cardiac catheterization using balloon angioplasty is feasible and safe in swine. The secondary objectives were to determine the acute and long-term effects of this therapy.
Design: This is a chronic survival experimental study in newborn swine.
Background: PA bands are used in infants for transient palliation of congenital heart defects with excessive pulmonary blood flow. Although rare, if these defects should close spontaneously or become hemodynamically insignificant, a sternotomy and occasionally cardiopulmonary bypass may still be required for band removal. Alternatively, debanding… More >

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1032

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ARTICLE

Kenan W.D. Stern^1,2, Kimberlee Gauvreau^1,2, Sitaram Emani^3,4, Tal Geva^1,2
Congenital Heart Disease, Vol.12, No.3, pp. 350-356, 2017, DOI:10.1111/chd.12450
Abstract Objective: Stage 1 Norwood palliation is one of the highest risk procedures in congenital cardiac surgery. Patients with superior technical performance scores have more favorable outcomes. Intraoperative epicardial echocardiography may allow the surgeon to address residual lesions prior to leaving the operating room, resulting in improved technical performance. The ability of intraoperative epicardial echocardiography to visualize the relevant anatomy and its association with outcomes is not known.
Design: A standardized intraoperative epicardial echocardiography protocol was developed and performed at the conclusion of Stage 1 Norwood palliation. Data pertaining to visualization of relevant anatomy, and comparison of intraoperative… More >

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950

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ARTICLE

Bahareh Eslami^1,2
Congenital Heart Disease, Vol.12, No.3, pp. 357-363, 2017, DOI:10.1111/chd.12452
Abstract Objective: The aims of this study were to compare the level of posttraumatic stress disorder between adults with and without congenital heart disease, and to examine the correlates of posttraumatic stress disorder (e.g., sociodemographics).
Design: Cross-sectional.
Setting: Two university-affiliated heart hospitals in Tehran, Iran.
Patients: A sample of 347 adults with congenital heart disease aged 18–64 years (52% women), and 353 adults without congenital heart disease matched by sex and age (±2 years) was recruited. Outcome Measures: The PTSD Scale: Self-report version was used to assess the diagnosis and severity of posttraumatic stress disorder. Hierarchical multivariate logistic regression analyses… More >

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1124

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ARTICLE

Koert de Waal¹, Nilkant Phad¹, Nick Collins², Andrew Boyle²
Congenital Heart Disease, Vol.12, No.3, pp. 364-372, 2017, DOI:10.1111/chd.12454
Abstract Background: Sustained volume load due to a patent ductus arteriosus (PDA) leads to cardiac remodeling. Remodeling changes can become pathological and are associated with cardiovascular disease progression. Data on remodeling changes in preterm infants is not available.
Methods: Clinical and echocardiography data were collected in preterm infants <30 weeks gestation on postnatal day 3 and then every 7–14 days until closure of the ductus arteriosus. Images were analyzed using conventional techniques and speckle tracking. Remodeling changes of infants with prolonged (>14 days) exposure to a PDA were compared to control infants without a PDA.
Results: Thirty out of… More >

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938

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ARTICLE

Ritu Sachdeva¹, Pamela S. Douglas², Michael S. Kelleman¹, Courtney E. McCracken¹, Leo Lopez³, Kenan W.D. Stern⁴, Benjamin W. Eidem⁵, Oscar J. Benavidez⁶, Rory B. Weiner⁶, Elizabeth Welch³, Robert M. Campbell¹, Wyman W. Lai⁷
Congenital Heart Disease, Vol.12, No.3, pp. 373-381, 2017, DOI:10.1111/chd.12455
Abstract Objective: The objective of this study was to evaluate effectiveness of educational intervention (EI) in the Pediatric Appropriate Use of Echocardiography (PAUSE) study to improve appropriateness of transthoracic echocardiograms (TTEs) ordered in pediatric cardiology clinics.
Design: Data were prospectively collected after the publication of the Appropriate Use Criteria (AUC) document during 2 phases: the pre-EI phase (1/1/15 to 4/30/15) and the post-EI phase (7/ 1/15 to 10/30/15). Pre-EI, site-investigators (SI) determined AUC indications, by reviewing the clinic records. Post-EI, providers assigned indications prior to obtaining TTE.
Setting: Pediatric cardiology clinics at six centers.
Patients: Those ≤18 years old, receiving… More >

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1064

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ARTICLE

Katlyn Elizabeth McGrattan^1,2,3,4, Heather McGhee^2,3, Allan DeToma⁵, Elizabeth G. Hill⁵, Sinai C. Zyblewski⁶, Maureen Lefton-Greif^7,8,9, Lucinda Halstead^1,2, Scott M. Bradley¹⁰, Bonnie Martin-Harris^1,2,3,4
Congenital Heart Disease, Vol.12, No.3, pp. 382-388, 2017, DOI:10.1111/chd.12456
Abstract Background: Deficits in swallowing physiology are a leading morbidity for infants with functional single ventricles and systemic outflow tract obstruction following stage 1 palliation. Despite the high prevalence of this condition, the underlying deficits that cause this post-operative impairment remain poorly understood.
Objective: Identify the physiologic correlates of dysphagia in infants with functional single ventricles and systemic outflow tract obstruction following stage 1 palliative surgery.
Methods: Postoperative fiberoptic laryngoscopies and videofluoroscopic swallow studies (VFSS) were conducted sequentially on infants with functional single ventricles following stage 1 palliative surgery. Infants were dichotomized as having normal or impaired laryngeal function… More >

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1001