Congenital short-QT syndrome

Abstract Congenital Short QT Syndrome is a cardiac channelopathy first described in 2000, with total number of published patients reaching 234 in 144 families and reportedly a male predominance. Most cases are from Western Europe and Japan. The total number of published patients is remarkably similar to the number of LQTS patients published within a similar time frame, but among LQTS patients female predominance has been reported. This difference in dominant sex category in the two syndromes becomes apparent after puberty, when earlier studies in healthy people have shown significantly shorter QT in males than in females, and later studies relating the QT shortening to testosterone. In the absence of definite sex-specific diagnostic criteria for QT duration, it has been suggested, however, that these differences may induce a bias, favoring LQTS diagnosis among adult females and SQTS diagnosis among adult males. Looking at only genotype-positive patients within the two syndromes there is no significant sex preference.

• Publication year

  2020

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  Unknown venue

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• Fields of study

  Medicine

• Identifiers
  DOI 10.1016/b978-0-12-817728-0.00023-1
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  Open on Semantic Scholar

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  Semantic Scholar

• No claims are published for this paper.

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  2000cited by this paper
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  1998cited by this paper
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