We experienced a 72-year-old woman carrying G38S, major KCNE1 polymorphism [KCNE1(G38S)] with remarkable QT prolongation (corrected QT interval 568 ms) and recurrent torsades de pointes under conditions of hypokalemia and hypomagnesemia.
The QT-RR slope in her 24-hour Holter electrocardiogram was steep and this enhanced bradycardia-dependent QT prolongation was similar to that in LQTS.
Patients with KCNE1(G38S) could have similar potential risk of ventricular arrhythmia as with long QT syndrome and analysis of QT-RR relationship could also evaluate the latent arrhythmogenicity of KCNE1(G38S).