What is CPVT? Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited cardiac condition that causes sudden rhythm disturbances, called arrhythmias, in otherwise healthy children. These abnormal rhythms most often occur at times of high adrenaline levels, such as during exercise or strong emotion.

If CPVT is not recognized and treated, an episode of ventricular tachycardia may cause the heart to stop beating (cardiac arrest), leading to sudden death. Researchers suspect that CPVT may be a significant cause of sudden death in children and young adults without recognized heart abnormalities.

Most people with CPVT need treatment with medicine. Some people need an implantable cardioverter defibrillator. Others may need a procedure called an ablation. Other members of your family may need to be checked and watched for CPVT.

CPVT occurs in children and adolescents and causes syncope and sudden cardiac death at a young age, in the absence of structural heart disease. The resting ECG, including the QTc interval, is normal. The mortality of CPVT is extremely high, reaching 31% by the age of 30 years when untreated.