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Congenital long QT syndrome

Overview of attention for article published in Orphanet Journal of Rare Diseases, July 2008
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About this Attention Score

  • In the top 5% of all research outputs scored by Altmetric
  • High Attention Score compared to outputs of the same age (94th percentile)
  • High Attention Score compared to outputs of the same age and source (99th percentile)

Mentioned by

news
2 news outlets
blogs
1 blog
twitter
6 tweeters
wikipedia
1 Wikipedia page

Citations

dimensions_citation
176 Dimensions

Readers on

mendeley
219 Mendeley
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Title
Congenital long QT syndrome
Published in
Orphanet Journal of Rare Diseases, July 2008
DOI 10.1186/1750-1172-3-18
Pubmed ID
Authors

Lia Crotti, Giuseppe Celano, Federica Dagradi, Peter J Schwartz

Abstract

Congenital long QT syndrome (LQTS) is a hereditary cardiac disease characterized by a prolongation of the QT interval at basal ECG and by a high risk of life-threatening arrhythmias. Disease prevalence is estimated at close to 1 in 2,500 live births. The two cardinal manifestations of LQTS are syncopal episodes, that may lead to cardiac arrest and sudden cardiac death, and electrocardiographic abnormalities, including prolongation of the QT interval and T wave abnormalities. The genetic basis of the disease was identified in the mid-nineties and all the LQTS genes identified so far encode cardiac ion channel subunits or proteins involved in modulating ionic currents. Mutations in these genes (KCNQ1, KCNH2, KCNE1, KCNE2, CACNA1c, CAV3, SCN5A, SCN4B) cause the disease by prolonging the duration of the action potential. The most prevalent LQTS variant (LQT1) is caused by mutations in the KCNQ1 gene, with approximately half of the genotyped patients carrying KCNQ1 mutations. Given the characteristic features of LQTS, the typical cases present no diagnostic difficulties for physicians aware of the disease. However, borderline cases are more complex and require the evaluation of various electrocardiographic, clinical, and familial findings, as proposed in specific diagnostic criteria. Additionally, molecular screening is now part of the diagnostic process. Treatment should always begin with beta-blockers, unless there are valid contraindications. If the patient has one more syncope despite a full dose beta-blockade, left cardiac sympathetic denervation (LCSD) should be performed without hesitation and implantable cardioverter defibrillator (ICD) therapy should be considered with the final decision being based on the individual patient characteristics (age, sex, clinical history, genetic subgroup including mutation-specific features in some cases, presence of ECG signs - including 24-hour Holter recordings - indicating high electrical instability). The prognosis of the disease is usually good in patients that are correctly diagnosed and treated. However, there are a few exceptions: patients with Timothy syndrome, patients with Jervell Lange-Nielsen syndrome carrying KCNQ1 mutations and LQT3 patients with 2:1 atrio-ventricular block and very early occurrence of cardiac arrhythmias.

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 219 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
United States 2 <1%
Bulgaria 1 <1%
France 1 <1%
Italy 1 <1%
Korea, Republic of 1 <1%
Netherlands 1 <1%
Unknown 212 97%

Demographic breakdown

Readers by professional status Count As %
Researcher 38 17%
Student > Bachelor 35 16%
Student > Master 29 13%
Student > Ph. D. Student 27 12%
Other 20 9%
Other 49 22%
Unknown 21 10%
Readers by discipline Count As %
Medicine and Dentistry 112 51%
Agricultural and Biological Sciences 30 14%
Biochemistry, Genetics and Molecular Biology 15 7%
Pharmacology, Toxicology and Pharmaceutical Science 7 3%
Engineering 6 3%
Other 26 12%
Unknown 23 11%

Attention Score in Context

This research output has an Altmetric Attention Score of 30. 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 23 June 2021.
All research outputs
#892,015
of 18,925,022 outputs
Outputs from Orphanet Journal of Rare Diseases
#81
of 2,029 outputs
Outputs of similar age
#10,493
of 204,409 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#2
of 125 outputs
Altmetric has tracked 18,925,022 research outputs across all sources so far. Compared to these this one has done particularly well and is in the 95th percentile: it's in the top 5% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 2,029 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 7.5. This one has done particularly well, scoring higher than 96% 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 204,409 tracked outputs that were published within six weeks on either side of this one in any source. This one has done particularly well, scoring higher than 94% of its contemporaries.
We're also able to compare this research output to 125 others from the same source and published within six weeks on either side of this one. This one has done particularly well, scoring higher than 99% of its contemporaries.