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  • Open Access


    The acute effects of 30 mg vs 60 mg of intravenous Fasudil on patients with congenital heart defects and severe pulmonary arterial hypertension

    Hongyun Ruan1, Yigang Zhang2, Ru Liu3, Xiangjun Yang1

    Congenital Heart Disease, Vol.14, No.4, pp. 645-650, 2019, DOI:10.1111/chd.12764

    Abstract Objective: The optimal dose of Fasudil is still controversial in congenital heart disease accompanied with severe pulmonary hypertension (CHD‐PAH). This study aimed to compare acute hemodynamic changes after different doses of Fasudil in 60 consecu‐ tive adult patients with CHD‐PAH.
    Design: Prospective randomized controlled trial.
    Setting: Tertiary cardiology center.
    Patients: Adult patients with CHD‐PAH.
    Interventions: Patients were randomized to Fasudil 30 or 60 mg.
    Outcome Measures: The hemodynamic parameters were measured at baseline and after 30 minutes of Fasudil through right cardiac catheterization. Blood gas results were obtained from the pulmonary artery, right ventricle, right atrium, superior and inferior vena… More >

  • Open Access


    Cognitive dysfunction is associated with abnormal responses in cerebral blood flow in patients with single ventricular physiology: Novel insights from transcranial Doppler ultrasound

    Rachel Wong1, Mohammed Al‐Omary2, David Baker2, Neil Spratt1,2, Andrew Boyle1,2, Natasha Baker Cert1, Peter Howe1, Nicholas Collins2

    Congenital Heart Disease, Vol.14, No.4, pp. 638-644, 2019, DOI:10.1111/chd.12763

    Abstract Objectives: Improvements in the management of complex congenital heart disease, including those with single ventricle physiology, have resulted in increased survival. As this population ages, the recognition of cognitive impairment is increasingly im‐ portant. At present, little is known about the potential mechanisms of cognitive dys‐ function. In this cross‐sectional study, we aimed to characterize the nature of abnormalities in cerebral blood flow and the relationship to cognitive deficits in adults with single ventricular physiology.
    Patients: Ten adults with single ventricular physiology (age 18‐40 years) and 12 age‐ and gender‐matched controls underwent transcranial Doppler ultrasound and ac‐ companying cognitive assessment.
    More >

  • Open Access


    Changing prevalence of severe congenital heart disease: Results from the National Register for Congenital Heart Defects in Germany

    Constanze Pfitzer1,2,3, Paul C. Helm4, Hannah Ferentzi1,5, Lisa-Maria Rosenthal1, Ulrike M. M. Bauer4,6, Felix Berger1,3,7, Katharina R. L. Schmitt1,3

    Congenital Heart Disease, Vol.12, No.6, pp. 787-793, 2017, DOI:10.1111/chd.12515

    Abstract Objective: To assess the prevalence of congenital heart disease (CHD) in Germany in relation to phenotypes, severity and gender.
    Design: Cross-sectional registry study.
    Setting: We analyzed data from patients with CHD born between 1996 and 2015.
    Patients: A total of 26 630 patients, registered with the NRCHD, were born between 1996 and 2015. 10 927 patients were excluded from the current analysis due to prior registration with the NRCHD under the German PAN Prevalence Study, which showed a potential bias in the inclusion of this patient population (proportion of mild cardiac lesions was comparatively high due to improved diagnostic capability… More >

  • Open Access


    Preparing adolescents with heart problems for transition to adult care, 2009–2010 National Survey of Children with Special Health Care Needs

    Karrie F. Downing1,2, Matthew E. Oster1,3, Sherry L. Farr1

    Congenital Heart Disease, Vol.12, No.4, pp. 497-506, 2017, DOI:10.1111/chd.12476

    Abstract Objective: A substantial percentage of children with congenital heart disease (CHD) fail to transfer to adult care, resulting in increased risk of morbidity and mortality. Transition planning discussions with a provider may increase rates of transfer, yet little is known about frequency and content of these discussions. We assessed prevalence and predictors of transition-related discussions between providers and parents of children with special healthcare needs (CSHCN) and heart problems, including CHD.
    Design: Using parent-reported data on 12- to 17-year-olds from the 2009–2010 National Survey of CSHCN, we calculated adjusted prevalence ratios (aPR) for associations between demographic factors and provider discussions… More >

  • Open Access


    Rates of autism and potential risk factors in children with congenital heart defects

    Jessica L. Bean Jaworski, Thomas Flynn, Nancy Burnham, Jesse L. Chittams, Therese Sammarco, Marsha Gerdes, Judy C. Bernbaum, Robert R. Clancy, Cynthia B. Solot, Elaine H. Zackai, Donna M. McDonald-McGinn, J. William Gaynor

    Congenital Heart Disease, Vol.12, No.4, pp. 421-429, 2017, DOI:10.1111/chd.12461

    Abstract Objective: Atypical development, behavioral difficulties, and academic underachievement are common morbidities in children with a history of congenital heart defects and impact quality of life. Language and social-cognitive deficits have been described, which are associated with autism spectrum disorders. The current study aimed to assess the rates of autism spectrum disorders in a large sample of children with a history of congenital heart defects and to assess medical, behavioral, and individual factors that may be associated with the risk of autism spectrum disorders.
    Design: Participants included 195 children with a history of congenital heart defects, who are followed in a… More >

  • Open Access


    Building a comprehensive team for the longitudinal care of single ventricle heart defects: Building blocks and initial results

    Karen Texter1,2, Jo Ann M. Davis1, Christina Phelps1,2, Sharon Cheatham1,2, John Cheatham1,2, Mark Galantowicz1,3, Timothy F. Feltes1,2

    Congenital Heart Disease, Vol.12, No.4, pp. 403-410, 2017, DOI:10.1111/chd.12459

    Abstract Introduction: With increasing survival of children with HLHS and other single ventricle lesions, the complexity of medical care for these patients is substantial. Establishing and adhering to best practice models may improve outcome, but requires careful coordination and monitoring.
    Methods: In 2013 our Heart Center began a process to build a comprehensive Single Ventricle Team designed to target these difficult issues.
    Results: Comprehensive Single Ventricle Team in 2014 was begun, to standardize care for children with single ventricle heart defects from diagnosis to adulthood within our institution. The team is a multidisciplinary group of providers committed to improving outcomes and… More >

  • Open Access


    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


    Readmissions after adult congenital heart surgery: Frequency and risk factors

    Yuli Y. Kim1, Wei He2, Thomas E. MacGillivray3, Oscar J. Benavidez2

    Congenital Heart Disease, Vol.12, No.2, pp. 159-165, 2017, DOI:10.1111/chd.12433

    Abstract Objective: Despite their clinical importance, 30-day readmission after adult congenital heart surgery has been understudied. They sought to determine the frequency of unplanned readmissions after adult congenital heart surgery and to identify any potential associated risk factors.
    Design: Retrospective cohort study using State Inpatient Databases for Washington, New York, Florida, and California from 2009 to 2011.
    Setting: Federal and nonfederal acute care hospitals.
    Patients: Admissions of patients age 18–49 years with International Classification of Diseases, Ninth Revision, Clinical Modification codes indicating adult congenital heart surgery.
    Outcome Measures: Readmission was defined as any nonelective hospitalization for a given patient ≤30 days… More >

  • Open Access


    Ability of noninvasive criteria to predict hemodynamically significant aortic obstruction in adults with coarctation of the aorta

    Marco Astengo1,2*, Caroline Berntsson3*, Åse A. Johnsson3,4, Peter Eriksson1,2, Mikael Dellborg1,2

    Congenital Heart Disease, Vol.12, No.2, pp. 174-180, 2017, DOI:10.1111/chd.12424

    Abstract Objective: Coarctation of the aorta (CoA) is a common condition. Adult patients with newly diagnosed CoA and patients with recurring or residual CoA require evaluation of the severity of aortic obstruction. Cardiac catheterization is considered the gold standard for the evaluation of hemodynamically significant CoA. The European Society of Cardiology (ESC) Guidelines for the management of grown-up congenital heart disease (GUCH) include noninvasive criteria for identifying significant CoA. Our aim was to investigate the ability of the Class I and Class IIa ESC recommendations to identify significant CoA at cardiac catheterization.
    Design: Sixty-six adult patients with native or recurrent CoA… More >

  • Open Access


    Utility of three‐dimensional models in resident education on simple and complex intracardiac congenital heart defects

    Shelby C. White1, Jennifer Sedler2, Trahern W. Jones3, Michael Seckeler1

    Congenital Heart Disease, Vol.13, No.6, pp. 1045-1049, 2018, DOI:10.1111/chd.12673

    Abstract Objective: Applications of three‐dimensional (3D) printed models in medicine in‐ clude preprocedure planning, patient education, and clinical training. Reproducing complex anatomy as a 3D printed model can be useful for understanding congenital heart defects (CHD). We hypothesized that using 3D printed models during didactic sessions with resident physicians will improve trainees’ understanding of CHD.
    Design and intervention: We performed a prospective, randomized educational in‐ tervention for teaching pediatric and pediatric/emergency medicine residents about simple (ventricular septal defect [VSD]) and moderately complex (tetralogy of Fallot [ToF]) CHD. Residents were divided into two groups: intervention and control. Each group completed a subjective… More >

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