What are the HRS/EHRA/APHRS diagnostic criteria for long QT syndrome (LQTS)?

Updated: Nov 29, 2017
  • Author: Ali A Sovari, MD, FACP, FACC; Chief Editor: Mikhael F El-Chami, MD  more...
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Answer

2013 HRS/EHRA/APHRS recommendations

In its 2013 expert consensus statement on inherited primary arrhythmia syndromes, the Heart Rhythm Society/European Heart Rhythm Association/Asia Pacific Heart Rhythm Society (HRS/EHRA/APHRS) recommended a diagnosis of long QT syndrome (LQTS) when any of the following criteria is met [36] :

  • LQTS risk score of at least 3.5 in the absence of a secondary cause for QT prolongation 
  • Presence of confirmed LQTS gene mutation
  • QTc of at least 500 ms in repeated 12-lead electrocardiograms (ECGs) and in the absence of a secondary cause for QT prolongation.

LQTS can also be diagnosed in the presence of a QTc between 480 and 499 ms in repeated 12-lead ECGs in a patient with unexplained syncope in the absence of a secondary cause for QT prolongation and in the absence of a pathogenic mutation.


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