In vivo Effects of Mutant HERG K+ Channel Inhibition by Disopyramide in Patients with a Short QT‐1 Syndrome: A Pilot Study
Key result
Oral disopyramide increased the QTc interval from 329 and 315 ms off drugs to 358 and 333 ms in two patients with short QT-1 syndrome, and increased ventricular effective refractory period by 40 ms.
Why the study?
Does oral disopyramide prolong the QT interval and ventricular effective refractory periods in patients with short QT-1 syndrome?
Population
2 female patients with short QT-1 syndrome caused by a N588K mutation in HERG
Comparison
Oral disopyramide vs Off drugs and oral quinidine
Design
Case_series
Authors
Discussion
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Implication
Caution advised before clinical use in short QT-1; leaves open efficacy and safety in larger trials.
Study Design
Type
Case Report (n=2)
Structured PICO
Does oral disopyramide prolong the QT interval and ventricular effective refractory periods in patients with short QT-1 syndrome?
P
Population
2 female patients with short QT-1 syndrome caused by a N588K-HERG mutation.
I
Intervention
Oral disopyramide
C
Comparator
Off drugs and oral quinidine
O
Outcome
QT interval (QTc)surrogate
Oral disopyramide may be a suitable alternative to quinidine for prolonging the QT interval and ventricular effective refractory periods in patients with short QT-1 syndrome.
Limitations
- Small sample size (n=2)
- Preliminary observations
Cite This Study
Schimpf et al. (2007) conducted a case report in Short QT-1 syndrome (n=2). Oral disopyramide vs. Off drugs and oral quinidine was evaluated on QT interval (QTc). Oral disopyramide increased the QTc interval from 329 and 315 ms off drugs to 358 and 333 ms in two patients with short QT-1 syndrome, and increased ventricular effective refractory period by 40 ms.