Diagnostic accuracy of screening electrocardiograms in long QT syndrome I

Misha D. Miller, Co Burn J Porter, Michael John Ackerman

Research output: Contribution to journalArticle

37 Citations (Scopus)

Abstract

Objective. Inherited long QT syndrome (LQTS) may present with syncope, seizures, and/or sudden death as a result of ventricular tachyarrhythmias. Identification of family members who are at risk because they harbor the genetic substrate for LQTS is critical. Presently, such identification relies on the 12-lead electrocardiogram (ECG). The purpose of this study was to evaluate the efficacy of the automated ECG as a screening tool for LQTS. Method. Molecular testing of a proband and 22 additional family members for the KVLQT1 mutation and symptomatic status facilitated the classification of each family member into the following patient groups:non-carriers (13), asymptomatic carriers (5), and symptomatic carriers (5). Each individual had a standard 12-lead ECG from which the computer and manual (lead II) corrected QT interval were determined. In addition, we determined the accuracy of the computer ECG diagnostic interpretation for each patient group. Results. With the use of a corrected QT interval of ≥460 ms as a diagnostic cutoff, the positive and negative predictive values for identifying at-risk individuals were 100%. Despite this, the computer-generated ECG diagnostic interpretation erroneously classified 6 of 23 family members. Moreover, half of the family members, proved to have the ion channel defect, received the diagnostic interpretation "normal ECG." Conclusion. Reliance on the computer-generated ECG diagnostic interpretation alone will fail to identify many at-risk family members. It is suggest`ed that all first-degree relatives of an identified LQTS proband have a 12-lead ECG that is reviewed independently by a physician who is familiar with LQTS in an effort to improve screening for this potentially lethal syndrome.

Original languageEnglish (US)
Pages (from-to)8-12
Number of pages5
JournalPediatrics
Volume108
Issue number1
DOIs
StatePublished - 2001

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Long QT Syndrome
Electrocardiography
Syncope
Sudden Death
Ion Channels
Tachycardia
Seizures
Physicians
Mutation
Lead

Keywords

  • Electrocardiogram
  • Long QT syndrome
  • QT interval
  • Sudden death

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Diagnostic accuracy of screening electrocardiograms in long QT syndrome I. / Miller, Misha D.; Porter, Co Burn J; Ackerman, Michael John.

In: Pediatrics, Vol. 108, No. 1, 2001, p. 8-12.

Research output: Contribution to journalArticle

Miller, Misha D. ; Porter, Co Burn J ; Ackerman, Michael John. / Diagnostic accuracy of screening electrocardiograms in long QT syndrome I. In: Pediatrics. 2001 ; Vol. 108, No. 1. pp. 8-12.
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