Abstract
Long QT syndrome type 3 (LQT3) is caused by mutations of the SCN5A gene encoding the α-subunit of the human cardiac sodium channel. Specific ST-T wave patterns, triggers, and risk for cardiac events are associated with this LQTS variant. Bench studies have gathered enough knowledge to devise gene-specific therapies to specifically counteract the effects of the mutations. In this article, the authors delineate the LQT3 pathophysiology and epidemiology. They also discuss the clinical management with a focus on the appropriate use of gene-specific therapy.
Original language | English |
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Pages (from-to) | 705-713 |
Number of pages | 9 |
Journal | Cardiac Electrophysiology Clinics |
Volume | 6 |
Issue number | 4 |
DOIs | |
Publication status | Published - Dec 1 2014 |
Keywords
- Electrophysiology
- Genetics
- Ion channel
- Long QT syndrome
- Pharmacology
- Sodium
ASJC Scopus subject areas
- Cardiology and Cardiovascular Medicine
- Physiology (medical)