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  • 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… 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… 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… 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… 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… 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… 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… 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… 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… 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… More >

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