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  • Online Resource  (12)
  • Cambridge University Press (CUP)  (12)
  • 1
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    Online Resource
    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)
    Abstract: 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.
    Type of Medium: Online Resource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S104795112300077X
    Language: English
    Publisher: Cambridge University Press (CUP)
    Publication Date: 2023
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  • 2
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    The Pediatric Heart Network Scholar Award programme: a unique mentored award embedded within a multicentre network
    Cambridge University Press (CUP) ; 2018
    In:  Cardiology in the Young Vol. 28, No. 6 ( 2018-06), p. 854-861
    Details
    In: Cardiology in the Young, Cambridge University Press (CUP), Vol. 28, No. 6 ( 2018-06), p. 854-861
    Abstract: The Pediatric Heart Network designed a career development award to train the next generation of clinician scientists in paediatric-cardiology-related research, a historically underfunded area. We sought to identify the strengths/weaknesses of the programme and describe the scholars’ academic achievements and the network’s return on investment. Methods Survey questions designed to evaluate the programme were sent to applicants – 13 funded and 19 unfunded applicants – and 20 mentors and/or principal investigators. Response distributions were calculated. χ 2 tests of association assessed differences in ratings of the application/selection processes among funded scholars, unfunded applicants, and mentors/principal investigators. Scholars reported post-funding academic achievements. Results Survey response rates were 88% for applicants and 100% for mentor/principal investigators. Clarity and fairness of the review were rated as “clear/fair” or “very clear/very fair” by 98% of respondents, but the responses varied among funded scholars, unfunded applicants, and mentors/principal investigators (clarity χ 2 =10.85, p=0.03; fairness χ 2 =16.97, p=0.002). Nearly half of the unfunded applicants rated feedback as “not useful” (47%). “Expanding their collaborative network” and “increasing publication potential” were the highest-rated benefits for scholars. Mentors/principal investigators found the programme “very” valuable for the scholars (100%) and the network (75%). The 13 scholars were first/senior authors for 97 abstracts and 109 manuscripts, served on 22 Pediatric Heart Network committees, and were awarded $9,673,660 in subsequent extramural funding for a return of ~$10 for every scholar dollar spent. Conclusions Overall, patient satisfaction with the Scholar Award was high and scholars met many academic markers of success. Despite this, programme challenges were identified and improvement strategies were developed.
    Type of Medium: Online Resource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S1047951118000483
    Language: English
    Publisher: Cambridge University Press (CUP)
    Publication Date: 2018
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  • 3
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    Evidence for lack of myocardial injury in children with acute rheumatic carditis
    Cambridge University Press (CUP) ; 2002
    In:  Cardiology in the Young Vol. 12, No. 6 ( 2002-12), p. 519-523
    Details
    In: Cardiology in the Young, Cambridge University Press (CUP), Vol. 12, No. 6 ( 2002-12), p. 519-523
    Abstract: Despite pathologic evidence of myocardial inflammation, the significance of myocarditis in children with acute rheumatic carditis remains controversial. Elevations in cardiac troponin I have been demonstrated in other forms of myocarditis. The purpose of our study was to determine if levels of cardiac troponin I are elevated, suggesting myocardial injury, in patients with acute rheumatic carditis. We identified all those patients with acute rheumatic fever, presenting between July 1998 and December 2000, who had clinical evidence of carditis, such as a new murmur of mitral or aortic regurgitation, and who had an echocardiogram, measurements of levels of cardiac troponin I, erythrocyte sedimentation rate, and/or C-reactive protein performed at the time of presentation. Their charts were reviewed for demographic and clinical data. Echocardiograms were reviewed for severity of aortic and mitral regurgitation, and measurements made of left ventricular ejection fraction, fractional shortening, and end-diastolic dimension. We found 16 patients with acute rheumatic carditis, ranging in age from 2.0 to 16.1 years, with just over one-third having symptoms of congestive heart failure. All patients had evidence of acute inflammation. There was a significant relationship between symptoms and severity of mitral regurgitation. No patient had elevated levels of cardiac troponin I level. The fact that levels of cardiac troponin I are not elevated in the serum of children with acute rheumatic carditis suggests that there is minimal myocytic necrosis in this setting. This supports the concept that acute valvar regurgitation is the major hemodynamic abnormality in these patients.
    Type of Medium: Online Resource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S104795110200094X
    Language: English
    Publisher: Cambridge University Press (CUP)
    Publication Date: 2002
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  • 4
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    Variation in care for children undergoing the Fontan operation for hypoplastic left heart syndrome
    Cambridge University Press (CUP) ; 2019
    In:  Cardiology in the Young Vol. 29, No. 12 ( 2019-12), p. 1510-1516
    Details
    In: Cardiology in the Young, Cambridge University Press (CUP), Vol. 29, No. 12 ( 2019-12), p. 1510-1516
    Abstract: The Single Ventricle Reconstruction Trial randomised neonates with hypoplastic left heart syndrome to a shunt strategy but otherwise retained standard of care. We aimed to describe centre-level practice variation at Fontan completion. Methods: Centre-level data are reported as median or median frequency across all centres and range of medians or frequencies across centres. Classification and regression tree analysis assessed the association of centre-level factors with length of stay and percentage of patients with prolonged pleural effusion ( 〉 7 days). Results: The median Fontan age (14 centres, 320 patients) was 3.1 years (range from 1.7 to 3.9), and the weight-for-age z-score was −0.56 (−1.35 + 0.44). Extra-cardiac Fontans were performed in 79% (4–100%) of patients at the 13 centres performing this procedure; lateral tunnels were performed in 32% (3–100%) at the 11 centres performing it. Deep hypothermic circulatory arrest (nine centres) ranged from 6 to 100%. Major complications occurred in 17% (7–33%). The length of stay was 9.5 days (9–12); 15% (6–33%) had prolonged pleural effusion. Centres with fewer patients ( 〈 6%) with prolonged pleural effusion and fewer ( 〈 41%) complications had a shorter length of stay ( 〈 10 days; sensitivity 1.0; specificity 0.71; area under the curve 0.96). Avoiding deep hypothermic circulatory arrest and higher weight-for-age z-score were associated with a lower percentage of patients with prolonged effusions ( 〈 9.5%; sensitivity 1.0; specificity = 0.86; area under the curve 0.98). Conclusions: Fontan perioperative practices varied widely among study centres. Strategies to decrease the duration of pleural effusion and minimise complications may decrease the length of stay. Further research regarding deep hypothermic circulatory arrest is needed to understand its association with prolonged pleural effusion.
    Type of Medium: Online Resource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S1047951119002658
    Language: English
    Publisher: Cambridge University Press (CUP)
    Publication Date: 2019
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  • 5
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    Spectrum and influence of hypoplasia of the left heart in Neonatal aortic coarctation
    Cambridge University Press (CUP) ; 2000
    In:  Cardiology in the Young Vol. 10, No. 2 ( 2000-03), p. 90-97
    Details
    In: Cardiology in the Young, Cambridge University Press (CUP), Vol. 10, No. 2 ( 2000-03), p. 90-97
    Abstract: Obstruction of the left ventricular outflow tract may be associated with hypoplasia of the left heart, which importantly influences the options for treatment. Although the influence of the size of the left heart on the outcome for critical aortic stenosis has been described, less is known about the spectrum of such hypoplasia seen with neonatal aortic coarctation, and how this influences outcome. To determine, first, the spectrum and influence of hypoplasia of the left heart in neonatal coarctation, second, if the previously described critical values for adequacy of the left heart in neonates with critical aortic stenosis are applicable to neonates with coarctation, and, third, if any of the variables or associated abnormalities are risk factors for recoarctation, we studied 63 neonates who underwent repair of coarctation. From the initial echocar diogram, we measured multiple structures in the left heart, and calculated a score for adequacy as has been done for critical aortic stenosis. The sizes were compared to previously reported minimal values. We then analyzed the influence of the variables and the associated anomalies on outcome. There were no deaths. There was a broad spectrum of sizes that did not correlate with the need for re-intervention. The calculated score for adequacy would have predicted survival in only 56% of the patients, and 73% of the neonates had at least one parameter measured in the left heart below the previously reported minimal values. There is, therefore, a broad spectrum of sizes for the left heart in neonates with aortic coarctation that is not predictive of outcome. Minimal sizes, and the score for adequacy used for critical aortic stenosis, are not applicable to neonates with coarctation.
    Type of Medium: Online Resource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S1047951100006533
    Language: English
    Publisher: Cambridge University Press (CUP)
    Publication Date: 2000
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  • 6
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    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
    Abstract: 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.
    Type of Medium: Online Resource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S1047951120000438
    Language: English
    Publisher: Cambridge University Press (CUP)
    Publication Date: 2020
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  • 7
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    An unusual variant of anomalous systemic venous return
    Cambridge University Press (CUP) ; 2003
    In:  Cardiology in the Young Vol. 13, No. 6 ( 2003-12), p. 563-564
    Details
    In: Cardiology in the Young, Cambridge University Press (CUP), Vol. 13, No. 6 ( 2003-12), p. 563-564
    Type of Medium: Online Resource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S1047951103001173
    Language: English
    Publisher: Cambridge University Press (CUP)
    Publication Date: 2003
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  • 8
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    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
    Abstract: 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.
    Type of Medium: Online Resource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S1047951121003632
    Language: English
    Publisher: Cambridge University Press (CUP)
    Publication Date: 2022
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  • 9
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    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
    Abstract: 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.
    Type of Medium: Online Resource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S1047951122003110
    Language: English
    Publisher: Cambridge University Press (CUP)
    Publication Date: 2023
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  • 10
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    Variation in care for infants undergoing the Stage II palliation for hypoplastic left heart syndrome
    Cambridge University Press (CUP) ; 2018
    In:  Cardiology in the Young Vol. 28, No. 10 ( 2018-10), p. 1109-1115
    Details
    In: Cardiology in the Young, Cambridge University Press (CUP), Vol. 28, No. 10 ( 2018-10), p. 1109-1115
    Abstract: The Single Ventricle Reconstruction trial randomised neonates with hypoplastic left heart syndrome to a systemic-to-pulmonary-artery shunt strategy. Patients received care according to usual institutional practice. We analysed practice variation at the Stage II surgery to attempt to identify areas for decreased variation and process control improvement. Methods Prospectively collected data were available in the Single Ventricle Reconstruction public-use database. Practice variation across 14 centres was described for 397 patients who underwent Stage II surgery. Data are centre-level specific and reported as interquartile ranges across all centres, unless otherwise specified. Results Preoperative Stage II median age and weight across centres were 5.4 months (interquartile range 4.9–5.7) and 5.7 kg (5.5–6.1), with 70% performed electively. Most patients had pre-Stage-II cardiac catheterisation (98.5–100%). Digoxin was used by 11/14 centres in 25% of patients (23–31%), and 81% had some oral feeds (68–84%). The majority of the centres (86%) performed a bidirectional Glenn versus hemi-Fontan. Median cardiopulmonary bypass time was 96 minutes (75–113). In aggregate, 26% of patients had deep hypothermic circulatory arrest 〉 10 minutes. In 13/14 centres using deep hypothermic circulatory arrest, 12.5% of patients exceeded 10 minutes (8–32%). Seven centres extubated 5% of patients (2–40) in the operating room. Postoperatively, ICU length of stay was 4.8 days (4.0–5.3) and total length of stay was 7.5 days (6–10). Conclusions In the Single Ventricle Reconstruction Trial, practice varied widely among centres for nearly all perioperative factors surrounding Stage II. Further analysis may facilitate establishing best practices by identifying the impact of practice variation.
    Type of Medium: Online Resource
    ISSN: 1047-9511 , 1467-1107
    URL: Article
    DOI: 10.1017/S1047951118000999
    Language: English
    Publisher: Cambridge University Press (CUP)
    Publication Date: 2018
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