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  • Open Access


    Acquired Coronary Artery Disease in Patients with Congenital Heart Disease: Issues in Diagnosis and Management

    Sotiria C. Apostolopoulou1,*, Stella Brili2, Eftihia Sbarouni3, Dimitris Tousoulis2, Konstantinos Toutouzas2

    Congenital Heart Disease, Vol.15, No.5, pp. 369-375, 2020, DOI:10.32604/CHD.2020.012092

    Abstract Objective: Acquired coronary artery disease, initially thought to rarely affect survivors of congenital heart disease, is increasingly recognized in this population, as these patients grow in age and numbers in the recent era. This study reports our experience with coronary artery disease in adults with congenital heart disease and discusses treatment issues and the existing literature. Methods: Retrospective review of all charts of adults with congenital heart disease and acquired coronary artery disease was performed. Patients’ clinical characteristics, diagnosis, risk factors, noninvasive and invasive imaging and management data were recorded. Results: Coronary artery disease was diagnosed at 35–70 of age… More >

  • Open Access


    Immunomodulatory miRNAs as Potential Biomarkers for the Postoperative Course Following Surgery for the Repair of Congenital Heart Defects in Children

    Or Bercovich1, Tal Tirosh-Wagner2, Lior Goldberg1, Amir Vardi3, David Mishali4, Gideon Paret1,#, Yael Nevo-Caspi1,*,#

    Congenital Heart Disease, Vol.15, No.4, pp. 239-249, 2020, DOI:10.32604/CHD.2020.011576

    Abstract Objective: To test the hypothesis that circulating miRNAs-146a, -146b, -155, and -21 reflect the inflammatory state of children following heart surgery, and that they may, therefore, correlate with postoperative parameters. We aimed to quantify miRNAs in blood samples from pediatric patients before and 6, 12, and 24 hours after surgery and to evaluate correlations between the miRNA levels and the postoperative course. Setting: PICU. Patients: Forty-two pediatric patients with CHD who underwent cardiac surgery at Safra Children’s Hospital between 2012–2016. Interventions: none. Outcome Measures: The primary outcomes were the postoperative cardiac complications and the secondary outcomes were the length of… More >

  • Open Access


    Use of Implantable Cardioverter-Defibrillators in Congenital Heart Disease and Pediatric Patients: Results from the German National Registry for Congenital Heart Defects

    Coralie Katharina Dicks1, Gerhard-Paul Diller1, Kristina Wasmer2, Paul C. Helm3,4, Ulrike M. M. Bauer3,4, Helmut Baumgartner1, Stefan Orwat1,#, Alicia Jeanette Fischer1,#,*

    Congenital Heart Disease, Vol.15, No.2, pp. 117-125, 2020, DOI:10.32604/CHD.2020.011520

    Abstract Background: Sudden cardiac death is a leading cause of death in patients with congenital heart disease (CHD). Risk stratification for implantable cardioverter defibrillators (ICD) remains difficult due to limited data about use and outcome of device therapy in CHD patients in larger community-based cohorts. Methods and results: Out of a dataset with more than 50,000 patients registered at the German National Register for Congenital Heart Defects, 109 patients (median age 35.5; IQR 23.75–46.00), 68 (62%) male) with an ICD were identified and were retrospectively analyzed. Although the number of implantations increased steadily throughout the investigated time interval from 2001 to… More >

  • Open Access


    Long‐term follow‐up of adult patients with congenital heart disease and an implantable cardioverter defibrillator

    Madalena Coutinho Cruz1, André Viveiros Monteiro1, Guilherme Portugal1, Sérgio Laranjo2, Ana Lousinha1, Bruno Valente1, Paulo Osório1, Pedro Silva Cunha1, Lídia de Sousa1, José Alberto Oliveira1, Ana Agapito1, Mário Martins Oliveira1, Fátima Pinto2, Rui Cruz Ferreira1

    Congenital Heart Disease, Vol.14, No.4, pp. 525-533, 2019, DOI:10.1111/chd.12767

    Abstract Objective: Sudden cardiac death is common in the adult congenital heart disease (ACHD) population. Knowledge and experience about the use of implantable cardio‐ verter defibrillators (ICD) in ACHD patients is very limited. We aimed to characterize a cohort of patients with ACHD and ICDs.
    Design: Thirty consecutive ACHD patients submitted to an ICD implantation in a single tertiary center were evaluated. Data on baseline clinical features, heart defect, indication for ICD, type of device, appropriate therapies, ICD‐related complication, and mortality during follow‐up were collected.
    Results: Of the 30 patients, 56.7% received appropriate therapies due to ventricular tachycardia (VT) or ventricular… More >

  • 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


    Contemporary outcomes and mortality risks of Ebstein anomaly: A single-center experience in Thailand

    Varisara Pornprasertchai, Chodchanok Vijarnsorn, Supaluck Kanjanauthai, Paweena Chungsomprasong, Prakul Chanthong, Kritvikrom Durongpisitkul, Jarupim Soongswang

    Congenital Heart Disease, Vol.14, No.4, pp. 619-627, 2019, DOI:10.1111/chd.12759

    Abstract Background: The increasing number of patients with Ebstein anomaly (EA) surviving into adulthood implies improvements in the treatments for the complex lesion. We revisited the clinical outcomes of patients with EA to demonstrate their “real world” survival.
    Objectives: To identify the survival and predictors of mortality in patients with EA who underwent medical or surgical management in the present era.
    Methods: All patients who had EA with atrioventricular concordance between 1994 and 2016 were retrospectively reviewed. Baseline characteristics, initial echocardio‐ graphic findings, treatments, and outcomes were explored. The survival analysis was performed at the end of 2017. A multivariate analysis… 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 >

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