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  • Online-Ressource  (4)
  • Cambridge University Press (CUP)  (4)
  • 2020-2024  (4)
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  • Online-Ressource  (4)
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  • Cambridge University Press (CUP)  (4)
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  • 2020-2024  (4)
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  • 1
    Online-Ressource
    Online-Ressource
    Coronary artery dilation associated with bicuspid and unicuspid aortic valve disease in children: a series of 17 patients
    Cambridge University Press (CUP) ; 2023
    In:  Cardiology in the Young
    Details
    In: Cardiology in the Young, Cambridge University Press (CUP)
    Kurzfassung: Coronary artery dilation associated with bicuspid/unicuspid aortic valves is described in adults with limited data in children. We aimed to describe the clinical course of children with bicuspid/unicuspid aortic valves and coronary dilation including coronary Z-score changes over time, association of coronary changes with aortic valve anatomy/function, and complications. Materials and methods: Institutional databases were searched for children ≤18 years with both bicuspid/unicuspid aortic valves and coronary dilation (1/2006-6/2021). Kawasaki disease and isolated supra-/subvalvar aortic stenosis were excluded. Statistics were descriptive with associations measured by Fisher’s exact test and overlapping 83.7% confidence intervals. Results: Of 17 children, bicuspid/unicuspid aortic valve was diagnosed at birth in 14 (82%). Median age at coronary dilation diagnosis was 6.4 years (range: 0-17.0). Aortic stenosis was present in 14 (82%) [2 (14%) moderate, 8 (57%) severe]; 10 (59%) had aortic regurgitation; 8 (47%) had aortic dilation. The right coronary was dilated in 15 (88%), left main in 6 (35%), and left anterior descending in 1 (6%) with no relationship between leaflet fusion pattern or severity of aortic regurgitation/stenosis on coronary Z-score. Follow-up evaluations were available for 11 (mean 9.3 years, range 1.1–14.8) with coronary Z-scores increasing in 9/11 (82%). Aspirin was used in 10 (59%). There were no deaths or coronary artery thrombosis. Discussion: In children with bicuspid/unicuspid aortic valves and coronary dilation, the right coronary artery was most frequently involved. Coronary dilation was observed in early childhood and frequently progressed. Antiplatelet medication use was inconsistent, but no child died nor developed thrombosis.
    Materialart: Online-Ressource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S104795112300077X
    Sprache: Englisch
    Verlag: Cambridge University Press (CUP)
    Publikationsdatum: 2023
    ZDB Id: 2060876-7
    Standort Signatur Einschränkungen Verfügbarkeit
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    Online-Ressource
  • 2
    Online-Ressource
    Online-Ressource
    Challenges and lessons learned from the Pediatric Heart Network Normal Echocardiogram Database study
    Cambridge University Press (CUP) ; 2020
    In:  Cardiology in the Young Vol. 30, No. 4 ( 2020-04), p. 456-461
    Details
    In: Cardiology in the Young, Cambridge University Press (CUP), Vol. 30, No. 4 ( 2020-04), p. 456-461
    Kurzfassung: The Pediatric Heart Network Normal Echocardiogram Database Study had unanticipated challenges. We sought to describe these challenges and lessons learned to improve the design of future studies. Methods: Challenges were divided into three categories: enrolment, echocardiographic imaging, and protocol violations. Memoranda, Core Lab reports, and adjudication logs were reviewed. A centre-level questionnaire provided information regarding local processes for data collection. Descriptive statistics were used, and chi-square tests determined differences in imaging quality. Results: For the 19 participating centres, challenges with enrolment included variations in Institutional Review Board definitions of “retrospective” eligibility, overestimation of non-White participants, centre categorisation of Hispanic participants that differed from National Institutes of Health definitions, and exclusion of potential participants due to missing demographic data. Institutional Review Board amendments resolved many of these challenges. There was an unanticipated burden imposed on centres due to high numbers of echocardiograms that were reviewed but failed to meet submission criteria. Additionally, image transfer software malfunctions delayed Core Lab image review and feedback. Between the early and late study periods, the proportion of unacceptable echocardiograms submitted to the Core Lab decreased (14 versus 7%, p 〈 0.01). Most protocol violations were from eligibility violations and inadvertent protected health information disclosure (overall 2.5%). Adjudication committee reviews led to protocol changes. Conclusions: Numerous challenges encountered during the Normal Echocardiogram Database Study prolonged study enrolment. The retrospective design and flaws in image transfer software were key impediments to study completion and should be considered when designing future studies collecting echocardiographic images as a primary outcome.
    Materialart: Online-Ressource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S1047951120000438
    Sprache: Englisch
    Verlag: Cambridge University Press (CUP)
    Publikationsdatum: 2020
    ZDB Id: 2060876-7
    Standort Signatur Einschränkungen Verfügbarkeit
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    Online-Ressource
  • 3
    Online-Ressource
    Online-Ressource
    The impact of the American Heart Association guidelines on patients treated for incomplete Kawasaki disease
    Cambridge University Press (CUP) ; 2022
    In:  Cardiology in the Young Vol. 32, No. 7 ( 2022-07), p. 1066-1070
    Details
    In: Cardiology in the Young, Cambridge University Press (CUP), Vol. 32, No. 7 ( 2022-07), p. 1066-1070
    Kurzfassung: To compare patients treated for incomplete Kawasaki disease whose practitioners followed versus did not follow American Heart Association criteria and to evaluate the association of cardiology consultation with adherence to these guidelines. Study design: Single centre retrospective cohort study of patients 〈 18 years old who received ≥1 dose of intravenous immunoglobulin for Kawasaki disease between 01/2006 and 01/2018. We collected demographics, clinical and laboratory data, coronary artery abnormalities, and cardiology consultation status. Patients treated for incomplete Kawasaki disease were divided into two groups based on adherence versus nonadherence to American Heart Association guidelines and compared by Wilcoxon rank sum test and chi-squared or Fisher’s exact test. Results: Of the 357 patients treated for Kawasaki disease, 109 (31%) were classified as incomplete Kawasaki disease. The American Heart Association algorithm for identifying patients with incomplete Kawasaki disease was followed in 81/109 (74%). Coronary artery abnormalities were present in 46/109 (42%) of the patients who were treated for incomplete Kawasaki disease. Cardiology consultation was more frequent in those fulfilling American Heart Association criteria for the diagnosis of incomplete Kawasaki disease versus those who did not fulfill criteria (76% versus 48%, p = 0.005). Conclusions: Over 25% of patients treated for incomplete Kawasaki disease did not meet American Heart Association guidelines. Guidelines were more frequently followed when the paediatric cardiology team was consulted. Consulting physicians with experience and expertise in the evaluation and management of incomplete KD should be strongly considered in the care of these patients.
    Materialart: Online-Ressource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S1047951121003632
    Sprache: Englisch
    Verlag: Cambridge University Press (CUP)
    Publikationsdatum: 2022
    ZDB Id: 2060876-7
    Standort Signatur Einschränkungen Verfügbarkeit
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    Online-Ressource
  • 4
    Online-Ressource
    Online-Ressource
    Variation in provider compliance with sports restriction guidelines in children with an isolated bicuspid aortic valve
    Cambridge University Press (CUP) ; 2023
    In:  Cardiology in the Young Vol. 33, No. 10 ( 2023-10), p. 1813-1818
    Details
    In: Cardiology in the Young, Cambridge University Press (CUP), Vol. 33, No. 10 ( 2023-10), p. 1813-1818
    Kurzfassung: Published guidelines for sports restriction for children with a bicuspid aortic valve remain controversial. We sought to describe practice variation and factors influencing sports restrictions in these children. Methods: This retrospective single-centre study included children (7–18 years old) with an isolated bicuspid aortic valve at baseline from 1 January, 2005 to 31 December, 2014. Sports restrictions, factors potentially influencing decision-making, and outcomes were collected. Descriptive statistics and multivariable mixed-effects logistic regression models were performed with providers and patients as random effects. Provider variation was estimated using intraclass correlation coefficients. Odds ratios, 95% confidence intervals, and p-values were reported from the models. Results: In 565 encounters (253 children; 34 providers), 41% recommended no sports restrictions, 40% recommended high-static and high-dynamic restrictions, and 19% had no documented recommendations. Based on published guidelines, 22% of children were inappropriately restricted while 30% were not appropriately restricted. The paediatric cardiology provider contributed to 37% of observed practice variation (p 〈 0.001). Sports restriction was associated with older age, males, greater ascending aorta z-score, and shorter follow-up interval. There were no aortic dissections or deaths and one cardiac intervention. Conclusion: Physicians frequently fail to document sports restrictions for children with a bicuspid aortic valve, and documented recommendations often conflict with published guidelines. Despite this, no adverse outcomes occurred. Providers accounted for a significant proportion of the variation in sports restrictions. Further research to provide evidence-based guidelines may improve provider compliance with activity recommendations in this population.
    Materialart: Online-Ressource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S1047951122003110
    Sprache: Englisch
    Verlag: Cambridge University Press (CUP)
    Publikationsdatum: 2023
    ZDB Id: 2060876-7
    Standort Signatur Einschränkungen Verfügbarkeit
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    Online-Ressource
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