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  • Open Access

    CASE REPORT

    2q37.3 Deletion with Complex Heart Defects Suggesting Interruption of Early Ventricular Looping

    Sanam Safi1,2, Takato Yamasaki1,3, David J. Glidden4, Stephen P. Sanders1,5, Chrystalle Katte Carreon1,6,*

    Congenital Heart Disease, Vol.17, No.2, pp. 141-146, 2022, DOI:10.32604/chd.2022.019743 - 26 January 2022

    Abstract A maternally inherited 828 kb microdeletion of 2q37.3 manifested in a 3-week-old premature boy as left juxtaposition of the atrial appendages associated with tricuspid atresia, double outlet infundibulum, subvalvar pulmonary atresia, large secundum atrial septal defect, and right aortic arch with mirror-image branching, consistent with developmental arrest early in heart looping. To the best of our knowledge, no previous 2q37 deletion syndrome has been reported with such a severe cardiac dysmorphology. Hence, this case adds to the cardiac phenotypes identified in 2q37 deletion syndrome. More >

  • Open Access

    ARTICLE

    Characteristics of In-Hospital Patients with Congenital Heart Disease Requiring Rapid Response System Activations: A Japanese Database Study

    Taiki Haga1,*, Tomoyuki Masuyama2, Yoshiro Hayashi3, Takahiro Atsumi4, Kenzo Ishii5, Shinsuke Fujiwara6

    Congenital Heart Disease, Vol.17, No.1, pp. 31-43, 2022, DOI:10.32604/CHD.2022.017407 - 26 October 2021

    Abstract Objectives: This study aimed to study the characteristics of in-hospital deterioration in patients with congenital heart disease who required rapid response system activation and identify risk factors associated with 1-month mortality. Methods: We retrospectively analysed data from a Japanese rapid response system registry with 35 participating hospitals. We included consecutive patients with congenital heart disease who required rapid response system activation between January 2014 and March 2018. Logistic regression analyses were performed to examine the associations between 1-month mortality and other patient-specific variables. Results: Among 9,607 patients for whom the rapid response system was activated, only 82… More >

  • Open Access

    ARTICLE

    Epicardial Versus Endocardial Pacemakers in the Pediatric Population: A Comparative Inquiry

    Mohammadrafie Khorgami1, Ali Sadeghpour Tabaei2,*, Elio Caruso3,*, Silvia Farruggio3, Negar Omidi4, Maryam Moradian1, Behzad Mohammadpour Ahranjani5, Zahra Khajali6 and Rahele Zamani1

    Congenital Heart Disease, Vol.16, No.6, pp. 573-584, 2021, DOI:10.32604/CHD.2021.016271 - 08 July 2021

    Abstract Background: Most children in need of cardiac pacemakers remain dependent on the function of the permanent from childhood to adulthood. We sought to evaluate and compare the function between epicardial and endocardial pacemakers in pediatric groups with different conditions. Methods: Between 2012 and 2018, this single-canter study evaluated 44 pediatric patients with indications for epicardial or endocardial pacemakers. Results: The 2 groups, at a median age of 5 (0.1–16) years, were compared concerning the characteristics of the leads used (n = 80: bipolar, unipolar, steroid-eluting, and non–steroid-eluting), survival data, and complications. The reason for pacemaker implantation was… More >

  • Open Access

    ARTICLE

    Higher Child-Reported Internalizing and Parent-Reported Externalizing Behaviors were Associated with Decreased Quality of Life among Pediatric Cardiac Patients Independent of Diagnosis: A Cross-Sectional Mixed-Methods Assessment

    Jacqueline S. Lee1,2, Angelica Blais1,2, Julia Jackson1, Bhavika J. Patel1, Lillian Lai4, Gary Goldfield1,3, Renee Sananes5, Patricia E. Longmuir1,2,3,*

    Congenital Heart Disease, Vol.16, No.3, pp. 255-267, 2021, DOI:10.32604/CHD.2021.014628 - 02 March 2021

    Abstract Background: Pediatric cardiology patients often experience decreased quality of life (QoL) and higher rates of mental illness, particularly with severe disease, but the relationship between them and comparisons across diagnostic groups are limited. This mixed-methods cross-sectional study assessed the association between QoL anxiety and behavior problems among children with structural heart disease, arrhythmia, or other cardiac diagnoses. Methods: Children (6–14 years, n = 76, 50% female) and their parents completed measures of QoL (PedsQL), behavior (BASC-2, subset of 19 children) and anxiety (MASC-2, children 8+ years). Pearson correlations/regression models examined associations between QoL, behavior and anxiety,… More >

  • Open Access

    ARTICLE

    Lesion-based Patterns of Morbidity and Mortality in Hospitalized Adolescents with Congenital Heart Disease

    Aparna Kulkarni1,*, Richard Neugebauer2, Shelby Kutty3

    Congenital Heart Disease, Vol.16, No.3, pp. 299-307, 2021, DOI:10.32604/CHD.2021.014495 - 02 March 2021

    Abstract Objective: The objective of this analysis is to describe the characteristics and morbidity during hospitalizations among adolescents with congenital heart disease (AdoCHD) from the Pediatric Health Information System (PHIS) database. Methods: The PHIS database was queried for all AdoCHD admissions aged 12–18 years (1/1/2004–12/31/2013). Major forms of CHD were identified by their International Classification of Diseases, ninth revision codes, further verified based on their secondary diagnosis and/or procedure codes. Patient characteristics, diagnoses, procedures and vital status were assessed. Results: In total, there were 4,267 adolescents admitted to 42 Children’s Hospitals, 58.3% were males, 24.6% single ventricle (SV)… More >

  • Open Access

    ARTICLE

    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 - 23 September 2020

    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… More >

  • Open Access

    ARTICLE

    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 - 07 September 2020

    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 More >

  • Open Access

    ARTICLE

    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 - 23 June 2020

    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… More >

  • Open Access

    ARTICLE

    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 cava,… More >

  • Open Access

    ARTICLE

    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‐… More >

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