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Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited cardiac condition that causes sudden rhythm disturbances, called “arrhythmias,” in otherwise healthy children. The arrhythmias typically occur during exercise or emotional stress and can lead to a sensation of a rapid heartbeat (palpitations), sudden loss of consciousness (syncope), cardiac arrest or sudden cardiac death.
CPVT is a rare disorder estimated to be about one in 10,000 people. Left untreated, CPVT may cause the heart to stop beating.
The fundamental problem with CPVT is an excessive amount of calcium in the heart due to a leak in the heart’s calcium channels, called ryanodine receptors. The extra calcium affects the heart’s electrical system, causing ventricular ectopic beats and ventricular tachycardia.
These abnormal rhythms predominantly occur at times of high adrenaline levels (such as during exercise or strong emotion). An adrenaline rush stimulates the heart’s beta-receptors, which then cause the ryanodine receptors to leak more calcium.
The Inherited Cardiac Arrhythmia Program at Boston Children’s specializes in family-based services for children with CPVT. The Family Arrhythmia Clinic meets with and assesses entire families all at once, usually in one day. Our specialists use clinical and genetic testing to identify if any family member is at risk of developing the condition.
We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”