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Re-evaluation of hypoplastic left heart syndrome from a developmental and morphological perspective

Overview of attention for article published in Orphanet Journal of Rare Diseases, August 2017
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  • Above-average Attention Score compared to outputs of the same age (63rd percentile)
  • Above-average Attention Score compared to outputs of the same age and source (56th percentile)

Mentioned by

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6 tweeters

Citations

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16 Dimensions

Readers on

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26 Mendeley
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Title
Re-evaluation of hypoplastic left heart syndrome from a developmental and morphological perspective
Published in
Orphanet Journal of Rare Diseases, August 2017
DOI 10.1186/s13023-017-0683-4
Pubmed ID
Authors

A. Crucean, A. Alqahtani, D. J. Barron, W. J. Brawn, R. V. Richardson, J. O’Sullivan, R. H. Anderson, D. J. Henderson, B. Chaudhry

Abstract

Hypoplastic left heart syndrome (HLHS) covers a spectrum of rare congenital anomalies characterised by a non-apex forming left ventricle and stenosis/atresia of the mitral and aortic valves. Despite many studies, the causes of HLHS remain unclear and there are conflicting views regarding the role of flow, valvar or myocardial abnormalities in its pathogenesis, all of which were proposed prior to the description of the second heart field. Our aim was to re-evaluate the patterns of malformation in HLHS in relation to recognised cardiac progenitor populations, with a view to providing aetiologically useful sub-groupings for genomic studies. We examined 78 hearts previously classified as HLHS, with subtypes based on valve patency, and re-categorised them based on their objective ventricular phenotype. Three distinct subgroups could be identified: slit-like left ventricle (24%); miniaturised left ventricle (6%); and thickened left ventricle with endocardial fibroelastosis (EFE; 70%). Slit-like ventricles were always found in combination with aortic atresia and mitral atresia. Miniaturised left ventricles all had normally formed, though smaller aortic and mitral valves. The remaining group were found to have a range of aortic valve malformations associated with thickened left ventricular walls despite being described as either atresia or stenosis. The degree of myocardial thickening was not correlated to the degree of valvar stenosis. Lineage tracing in mice to investigate the progenitor populations that form the parts of the heart disrupted by HLHS showed that whereas Nkx2-5-Cre labelled myocardial and endothelial cells within the left and right ventricles, Mef2c-AHF-Cre, which labels second heart field-derived cells only, was largely restricted to the endocardium and myocardium of the right ventricle. However, like Nkx2-5-Cre, Mef2c-AHF-Cre lineage cells made a significant contribution to the aortic and mitral valves. In contrast, Wnt1-Cre made a major contribution only to the aortic valve. This suggests that discrete cardiac progenitors might be responsible for the patterns of defects observed in the distinct ventricular sub-groups. Only the slit-like ventricle grouping was found to map to the current nomenclature: the combination of mitral atresia with aortic atresia. It appears that slit-like and miniature ventricles also form discrete sub-groups. Thus, reclassification of HLHS into subgroups based on ventricular phenotype, might be useful in genetic and developmental studies in investigating the aetiology of this severe malformation syndrome.

Twitter Demographics

The data shown below were collected from the profiles of 6 tweeters who shared this research output. Click here to find out more about how the information was compiled.

Mendeley readers

The data shown below were compiled from readership statistics for 26 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 26 100%

Demographic breakdown

Readers by professional status Count As %
Student > Bachelor 4 15%
Professor > Associate Professor 3 12%
Student > Postgraduate 3 12%
Student > Ph. D. Student 2 8%
Professor 2 8%
Other 5 19%
Unknown 7 27%
Readers by discipline Count As %
Medicine and Dentistry 8 31%
Agricultural and Biological Sciences 4 15%
Biochemistry, Genetics and Molecular Biology 3 12%
Neuroscience 1 4%
Engineering 1 4%
Other 0 0%
Unknown 9 35%

Attention Score in Context

This research output has an Altmetric Attention Score of 4. 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 15 May 2018.
All research outputs
#4,984,292
of 15,921,538 outputs
Outputs from Orphanet Journal of Rare Diseases
#687
of 1,696 outputs
Outputs of similar age
#117,467
of 323,439 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#49
of 115 outputs
Altmetric has tracked 15,921,538 research outputs across all sources so far. This one has received more attention than most of these and is in the 68th percentile.
So far Altmetric has tracked 1,696 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 7.3. This one has gotten more attention than average, scoring higher than 58% of its peers.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 323,439 tracked outputs that were published within six weeks on either side of this one in any source. This one has gotten more attention than average, scoring higher than 63% of its contemporaries.
We're also able to compare this research output to 115 others from the same source and published within six weeks on either side of this one. This one has gotten more attention than average, scoring higher than 56% of its contemporaries.