Inadvertent treatment with a pure IKr blocker in LQT2 syndrome

AUTHORS

Mohsen Hosseinkhani1 1 , *

1 Iran

How to Cite: Hosseinkhani1 M . Inadvertent treatment with a pure IKr blocker in LQT2 syndrome, Multidiscip Cardio Annal. 2010 ; 3(5):e8757.

ARTICLE INFORMATION

Multidisciplinary Cardiovascular Annals: 3 (5); e8757
Published Online: September 14, 2016
Article Type: Case Report
Received: September 14, 2016
Accepted: April 14, 2010

Crossmark

CHEKING

READ FULL TEXT
Abstract

Long QT syndrome (LQTS) results from
structural abnormalities in the potassium
channels of the heart, which predispose
affected persons to an accelerated
heart rhythm (arrhythmia). Nifekalant,
a new class III antiarrhythmic agent
developed in US, blocks selectively a
rapidly-activating component of the
delayed rectifier potassium channel
(IKr) in cardiac myocytes, and causes
dose-dependent increase in artrial
and ventricular refractory periods and
repolarization. We report a case of
congenital long QT syndrome (LQTS)
with recurrent ventricular fibrillation
inadvertently treated with intravenous
nifekalant. Treatment neither modified
the rate-corrected QT interval nor
induced torsades de points. Subsequent
genotyping of the patient revealed a
missense mutation in the extracellular
loop between S5 and the pore region of
HERG (K595E). Since HERG encodes
the IKr channel, LQT2 patient may be
more tolerant of pure IKr blockers than
other LQTS genotypes.

Keywords

Long QT syndrome, torsades de points, Nifekalant, genotype, missense mutation

© 0, Multidisciplinary Cardiovascular Annals. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (http://creativecommons.org/licenses/by-nc/4.0/) which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited.

Fulltext

Full Text Available is PDF

References

  • 1.

    Full Text Available is PDF

  • COMMENTS

    LEAVE A COMMENT HERE: