Long-term (subacute) potassium treatment in congenital HERG-related long QT syndrome (LQTS2)

Congenital long QT syndrome (LQTS) is subdivided according to the underlying gene defect. In LQTS2, an aberrant HERG gene that encodes the potassium channel IKr leads to insufficient IKr activity and delayed repolarization, causing ECG abnormalities and torsades de pointes (TdP). Increasing serum potassium levels by potassium infusion normalizes the ECG in LQTS2 because IKr activity varies with serum potassium levels.

In an LQTS2 patient who presented with TdP, we attempted to achieve a long-term (subacute) elevation of serum potassium by increased potassium intake and potassium-sparing drugs. However, due to renal potassium homeostasis, it was impossible to achieve a long-lasting rise of serum potassium above 4.0 mmol/L.

Although raising serum potassium reverses the ECG abnormalities in LQTS2, a long-lasting rise of serum potassium is only partially achievable because in the presence of normal renal function, potassium homeostasis limits the amount of serum potassium increase.