Home / Journals / CHD / Vol.12, No.3, 2017
  • Open Access

    EDITORIAL

    Speaking to children and their families about congenital heart disease: Ushering in a new era of healthcare literacy

    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 >

  • Open Access

    REVIEW

    Improving the quality of transition and transfer of care in young adults with congenital heart disease

    Ian K. Everitt1, Jennifer F. Gerardin2, Fred H. Rodriguez2,3, 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 shown to decrease mortality in… More >

  • Open Access

    REVIEW

    Pregnancy-related acute aortic dissection in Marfan syndrome: A review of the literature

    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 women with Marfan syndrome (n… More >

  • Open Access

    ARTICLE

    Incidence, morphology, and progression of bicuspid aortic valve in pediatric and young adult subjects with coexisting congenital heart defects

    Talha Niaz1, Joseph T. Poterucha1, Jonathan N. Johnson1,2, Cecilia Craviari1, Thomas Nienaber1, Jared Palfreeman1, Frank Cetta1,2, Donald J. Hagler1,2
    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 BAV and… More >

  • Open Access

    ARTICLE

    Tissue plasminogen activator for neonatal coronary thrombosis presenting with mitral valve regurgitation and impaired ventricular function

    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, as well… More >

  • Open Access

    ARTICLE

    Reducing variation in feeding newborns with congenital heart disease

    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 >

  • Open Access

    ARTICLE

    Pediatric cardiac readmissions: An opportunity for quality improvement?

    Jeffrey H. Sacks1,2, Michael Kelleman2, Courtney McCracken2, Michelle Glanville1, Matthew Oster1,2
    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 from… More >

  • Open Access

    ARTICLE

    Transcatheter closure of calcified patent ductus arteriosus in older adult patients: Immediate and 12-month follow-up results

    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. The size… More >

  • Open Access

    ARTICLE

    Defining pediatric inpatient cardiology care delivery models: A survey of pediatric cardiology programs in the USA and Canada

    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 (n =… More >

  • Open Access

    ARTICLE

    A first population-based long-term outcome study in adults with repaired tetralogy of Fallot in Malta

    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 1985 were… More >

  • Open Access

    ARTICLE

    Residents’ understanding of adult congenital heart disease

    Paul Cooper1, Morgan Hindes2, Timothy M. Maul3,4, 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 residents (N… More >

  • Open Access

    ARTICLE

    Practice trends over time in the care of infants with hypoplastic left heart syndrome: A report from the National Pediatric Cardiology Quality Improvement Collaborative

    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 risk factor… More >

  • Open Access

    ARTICLE

    Increased regurgitant flow causes endocardial cushion defects in an avian embryonic model of congenital heart disease

    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 regurgitant flow in… More >

  • Open Access

    ARTICLE

    Practice variability in management of infectious issues in heterotaxy: A survey of pediatric cardiologists

    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 ultrasound to… More >

  • Open Access

    ARTICLE

    Acute and long-term effects of endovascular debanding of pulmonary arteries in a swine model

    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 could be… More >

  • Open Access

    ARTICLE

    Utility of a standardized postcardiopulmonary bypass epicardial echocardiography protocol for stage I Norwood palliation

    Kenan W.D. Stern1,2, Kimberlee Gauvreau1,2, Sitaram Emani3,4, Tal Geva1,2
    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 echocardiogram findings with… More >

  • Open Access

    ARTICLE

    Correlates of posttraumatic stress disorder in adults with congenital heart disease

    Bahareh Eslami1,2
    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 were… More >

  • Open Access

    ARTICLE

    Cardiac remodeling in preterm infants with prolonged exposure to a patent ductus arteriosus

    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 189 infants… More >

  • Open Access

    ARTICLE

    Educational intervention for improving the appropriateness of transthoracic echocardiograms ordered by pediatric cardiologists

    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 initial… More >

  • Open Access

    ARTICLE

    Dysphagia in infants with single ventricle anatomy following stage 1 palliation: Physiologic correlates and response to treatment

    Katlyn Elizabeth McGrattan1,2,3,4, Heather McGhee2,3, Allan DeToma5, Elizabeth G. Hill5, Sinai C. Zyblewski6, Maureen Lefton-Greif7,8,9, Lucinda Halstead1,2, Scott M. Bradley10, Bonnie Martin-Harris1,2,3,4
    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 based on… More >

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