open access

Vol 66, No 12 (2008)
Other
Published online: 2009-01-07
Submitted: 2012-12-28
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Electrocardiogram of the month
QTc duration in the long QT syndrome type 2 – diagnostic dilemma

Agnieszka Zienciuk, Jakub Machejek, Celina Cybulska
Kardiol Pol 2008;66(12):1333-1337.

open access

Vol 66, No 12 (2008)
Other
Published online: 2009-01-07
Submitted: 2012-12-28

Abstract

We present a case of a 43-year old woman with a long QT syndrome (LQTS). Despite her family history of several sudden deaths in close relatives the patient was misdiagnosed, being treated many years for epilepsy. Finally, after another series of syncopal spells she was diagnosed with LQTS. Although QTc interval was normal on surface ECG, the presence of notched T-waves and QT prolongation up to 543 ms on Holter recording were helpful in establishing the correct diagnosis, which was later confirmed by genotyping revealing G601S mutation in the HERG gene. The patient refused an ICD and despite beta-blocker treatment died suddenly several months later.

Abstract

We present a case of a 43-year old woman with a long QT syndrome (LQTS). Despite her family history of several sudden deaths in close relatives the patient was misdiagnosed, being treated many years for epilepsy. Finally, after another series of syncopal spells she was diagnosed with LQTS. Although QTc interval was normal on surface ECG, the presence of notched T-waves and QT prolongation up to 543 ms on Holter recording were helpful in establishing the correct diagnosis, which was later confirmed by genotyping revealing G601S mutation in the HERG gene. The patient refused an ICD and despite beta-blocker treatment died suddenly several months later.
Get Citation

Keywords

long QT syndrome (LQTS); LQTS type 2; notched T-wave; HERG G601S; QTc length

About this article
Title

Electrocardiogram of the month
QTc duration in the long QT syndrome type 2 – diagnostic dilemma

Journal

Kardiologia Polska (Polish Heart Journal)

Issue

Vol 66, No 12 (2008)

Pages

1333-1337

Published online

2009-01-07

Bibliographic record

Kardiol Pol 2008;66(12):1333-1337.

Keywords

long QT syndrome (LQTS)
LQTS type 2
notched T-wave
HERG G601S
QTc length

Authors

Agnieszka Zienciuk
Jakub Machejek
Celina Cybulska

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