Oral adrenergic agents produced Ventricular fibrillation and QT
prolongation in an elderly patient carrying an RYR2 variant.
Mutant cardiac ryanodine receptor channels (RyR2) are “leaky,” and
spontaneous Ca2+ release through these channels causes delayed
afterdepolarizations that can deteriorate into ventricular fibrillation
(VF). RYR2 is a causative gene of type 1 catecholaminergic polymorphic
ventricular tachycardia (CPVT). Some patients carrying RYR2 mutations in
CPVT exhibit QT prolongation and are initially diagnosed with long QT
syndrome. However, none have been reported to cause drug-induced VF in
patients with RYR2 variants. We describe the first case of an elderly
woman with drug-induced QT prolongation and VF who carried a novel
RYR2variant but no other mutations related to long QT syndrome.