August 1, 1997Pacing and Clinical Electrophysiology
Molecular Biology of the Long QT Syndrome: Impact on Management
Why the study?
Do gene-specific therapies such as mexiletine and increased heart rate shorten the QT interval in specific Long QT Syndrome genotypes?
Population
Patients with Long QT Syndrome and isolated guinea pig ventricular myocytes
Comparison
Gene-specific therapies including sodium channel… vs LQT2 patients vs LQT3 patients vs healthy controls
Design
Review
Authors
Discussion
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Implication
Supports differential mexiletine response by LQTS genotype; hypothesis-generating for gene-specific therapy trials.
Structured PICO
Do gene-specific therapies such as mexiletine and increased heart rate shorten the QT interval in specific Long QT Syndrome genotypes?
P
Population
Patients with Long QT Syndrome (specifically LQT2 and LQT3 variants) and isolated guinea pig ventricular myocytes
I
Intervention
Gene-specific therapies including sodium channel blockade (mexiletine), rapid cardiac pacing, and increased extracellular potassium
C
Comparator
LQT2 patients vs LQT3 patients vs healthy controls
O
Outcome
QT interval shorteningsurrogate
Understanding the molecular biology of Long QT Syndrome allows for the development of gene-specific therapies, such as mexiletine for LQT3 and potassium supplementation for LQT2, to supplement standard antiadrenergic therapy.
Cite This Study
Priori et al. (1997) studied this question.