What is Catecholaminergic polymorphic ventricular tachycardia?

an inherited genetic disorder that predisposes those affected to potentially life-threatening abnormal heart rhythms or arrhythmias. The arrhythmias seen in CPVT typically occur during exercise or at times of emotional stress, and classically take the form of bidirectional ventricular tachycardia or ventricular fibrillation

How is Catecholaminergic polymorphic ventricular tachycardia inherited?

autosomal dominant fashion and has a prevalence of around 1:10,000

What defect causes Catecholaminergic polymorphic ventricular tachycardia?

the most common cause is a defect in the ryanodine receptor (RYR2) which is found in the myocardial sarcoplasmic reticulum

What are the features of Catecholaminergic polymorphic ventricular tachycardia?

• exercise or emotion induced polymorphic ventricular tachycardia resulting in syncope
• sudden cardiac death
• symptoms generally develop before the age of 20 years

What is the management of catecholaminergic polymorphic ventricular tachycardia?

• beta-blockers
• implantable cardioverter-defibrillator