50 Years of Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) - Time to Explore the Dark Side of the Moon

Despite significant progress in understanding catecholaminergic polymorphic ventricular tachycardia (CPVT), there are still multiple uncertainties and gaps in our knowledge. Like the dark side of the moon, we cannot see them directly. Unfortunately, clinicians must make diagnostic and therapeutic decisions without solid evidence. Instead of summarising the current state of science and reiterating the guidelines, we review difficulties in understanding the disease mechanism, diagnosis and therapy. Highlighting these truths helps to avoid misconceptions, think clearly about our patients and direct future research efforts. It has become clear that CPVT encompasses more than just uniformly expressed ryanodine receptor mutations leading to bidirectional ventricular tachycardia, rather it is a disease caused by different genetic mutations, overlapping with other entities and possibly affecting not only the heart. Treatment in addition to beta blockers is often necessary: flecainide and left cardiac sympathetic denervation are therapies that come before consideration of defibrillator implantation and new treatment options are on the horizon.