Targeted therapy for short QT syndrome

Abstract

Identified in 2000, short QT syndrome is an electrical disease of the heart characterised as a channelopathy. At first considered extremely rare, families with this disease have been found in Brazil, Finland, Germany, Spain, the Netherlands, France, Turkey, Italy and the US. The focus of the paper is to present a current review of short QT syndrome, as well as providing an overview upon the potential molecular target-based strategies for management of this very deadly disease. Abnormalities in three different potassium channels have been recognised as the cause of the disease and targets for therapy will be discussed for each potassium channel individually. In addition to pharmacological strategies, gene therapy with transfer of genes coding for specific ion channel subunits or regulatory proteins are discussed.

Keywords::

• channelopathy
• potassium channel mutation
• short QT syndrome (SQTS)
• sudden cardiac death (SCD)

Acknowledgments

A Jahangir is supported by grants from the American Heart Association, National Institute on ageing and the Mayo Clinic Robert and Arlene Kogod Programme on ageing.