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Diffuse left ventricular interstitial fibrosis is associated with sub-clinical myocardial dysfunction in Alström Syndrome: an observational study

Overview of attention for article published in Orphanet Journal of Rare Diseases, June 2015
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Title
Diffuse left ventricular interstitial fibrosis is associated with sub-clinical myocardial dysfunction in Alström Syndrome: an observational study
Published in
Orphanet Journal of Rare Diseases, June 2015
DOI 10.1186/s13023-015-0292-z
Pubmed ID
Authors

Nicola C. Edwards, William E. Moody, Mengshi Yuan, Adrian T. Warfield, Robert Cramb, Richard B. Paisey, Tarekegn Geberhiwot, Richard P. Steeds

Abstract

Alström syndrome is a rare inherited ciliopathy with progressive multisystem involvement. Dilated cardiomyopathy is common in infancy and recurs or presents de novo in adults with high rates of premature cardiovascular death. Although Alström syndrome is characterised by fibrosis in solid organs such as the liver, the pathogenesis of related cardiomyopathy are not clear. To date it is not known whether diffuse interstitial myocardial fibrosis is present before the onset of heart failure symptoms or changes in conventional parameters of left ventricular function. In this observational study, 26 patients with Alström syndrome (mean age 27 ± 9 years, 65% male, 24 h ABPM 130 ± 14 / 77 ± 9 mmHg) without symptomatic cardiovascular disease were recruited from a single centre and compared to matched healthy controls. All subjects underwent cardiac MRI (1.5 T) to assess ventricular function, diffuse interstitial myocardial fibrosis by measurement of extracellular volume on T1-mapping (MOLLI) and coarse replacement fibrosis using standard late gadolinium enhancement imaging. Global extracellular volume was increased in Alström syndrome with wider variation compared to controls (0.30 ± 0.05 vs. 0.25 ± 0.01, p < 0.05). Left ventricular long axis function and global longitudinal strain were impaired in Alström syndrome without change in ejection fraction, ventricular size or atrial stress (NT-proBNP) (p < 0.05). Global extracellular volume was associated with reduced peak systolic longitudinal strain (r = -0.73, p < 0.01) and strain rate (r = -0.57, p < 0.01), increased QTc interval (r = 0.49, p < 0.05) and serum triglycerides (r = 0.66, p < 0.01). Nine (35%) patients had diffuse mid-wall late gadolinium enhancement in a non-coronary artery distribution. Diffuse interstitial myocardial fibrosis is common in Alström syndrome and is associated with impaired left ventricular systolic function. Serial studies are required to determine whether global extracellular volume may be an independent imaging biomarker of vulnerability to dilated cardiomyopathy and heart failure.

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Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 43 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 43 100%

Demographic breakdown

Readers by professional status Count As %
Student > Ph. D. Student 8 19%
Student > Master 7 16%
Researcher 6 14%
Student > Bachelor 4 9%
Student > Postgraduate 2 5%
Other 5 12%
Unknown 11 26%
Readers by discipline Count As %
Medicine and Dentistry 24 56%
Psychology 2 5%
Agricultural and Biological Sciences 1 2%
Veterinary Science and Veterinary Medicine 1 2%
Biochemistry, Genetics and Molecular Biology 1 2%
Other 1 2%
Unknown 13 30%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 1. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 24 June 2015.
All research outputs
#20,281,599
of 22,815,414 outputs
Outputs from Orphanet Journal of Rare Diseases
#2,459
of 2,615 outputs
Outputs of similar age
#220,012
of 264,050 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#33
of 35 outputs
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So far Altmetric has tracked 2,615 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 7.5. This one is in the 1st percentile – i.e., 1% of its peers scored the same or lower than it.
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