What is Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)?
Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is a rare inherited arrhythmia syndrome in which the heart is structurally normal but specific triggers — particularly physical exertion or emotional stress — can provoke dangerous ventricular arrhythmias. These can cause palpitations, blackouts, or sudden cardiac arrest.
CPVT is caused by mutations affecting calcium regulation within heart muscle cells, most commonly in the RYR2 gene. It typically presents in childhood or adolescence and can cause sudden cardiac arrest in young people who appear otherwise completely healthy. A standard resting ECG may be normal; an exercise stress test often reveals the characteristic bidirectional ventricular tachycardia.
Treatment includes beta-blockers to reduce the risk of arrhythmias triggered by adrenaline, strict avoidance of competitive and high-intensity exercise, and in many cases ICD implantation. Some patients are also treated with flecainide.
Because CPVT is inherited, family screening is essential following a diagnosis. First-degree relatives should be assessed with an exercise stress test as well as a resting ECG and echocardiogram, as the resting ECG may appear entirely normal even in affected individuals.
Category: Inherited Conditions