Key result
Short QT syndrome is a rare autosomal dominant channelopathy characterized by a strikingly short QT interval (<320 milliseconds) and an increased risk for sudden cardiac death.
Population
Infants, children, or young adults with hereditary short QT syndrome
Design
Review
Authors
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Raises awareness for screening in unexplained sudden death; leaves open optimal management strategies pending higher-level evidence.
Short QT syndrome is a recently recognized channelopathy with a high risk of sudden cardiac death, manageable with ICDs and potentially QT-prolonging drugs like quinidine and disopyramide.
Patel et al. (2009) conducted a review in Short QT Syndrome. Short QT syndrome is a rare autosomal dominant channelopathy characterized by a strikingly short QT interval (<320 milliseconds) and an increased risk for sudden cardiac death.
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