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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 first line treatment for CPVT is beta-blockers, which block the beta-receptors and prevent adrenaline from asserting its effect on the heart. Ventricular arrhythmias typically start at heart rates of 110-120 beats per minute. Beta-blockers also limit the maximal heart rate during exercise. Reports of large numbers of patients with CPVT suggest that nadolol is the optimal beta-blocker, due to its broad effect against diferente beta-receptors and long duration of action.
A newer addition to the medical therapy for CPVT is flecainide. The predominant effect appears to be by affecting sodium and calcium exchange within the cardiac cell. This can be very effective in some patients, but not all.
The effect of a medication can be assessed with exercise testing or Holter monitoring to determine the degree of ventricular ectopy and arrhythmias. Implantable loop recorders placed just under the skin, on the chest wall, constantly record every heartbeat and transmit any abnormal rhythms to the Boston Children’s Heart Center electronically. These small devices, which can be implanted in 10-15 minutes, are increasingly used for CPVT patients.
A more invasive alternative is a procedure called left cardiac sympathetic denervation (or sympathectomy). In this 45 minute surgical procedure, a surgeon divides the nerves going into the heart (called the sympathetic chain) using a thoracoscopic keyhole approach through the chest wall. The sympathetic nerves supply adrenaline to the heart. Therefore, interrupting this chain can prevent adrenaline’s stimulation of the heart, which causes arrhythmias. A recent multi-center, international study (which the Heart Center was a part of) showed this can be a very effective therapy in preventing arrhythmias.
For patients considered very high risk or continue to have symptoms of ventricular arrhythmias despite other therapies, implantable cardioverter-defibrillators can be used. These detect ventricular arrhythmias and treat them by administering a shock to restore normal cardiac rhythm. However, due to numerous side effects — especially in patients with CPVT — these are used only in carefully selected cases.
Like many inherited heart disorders, the management of CPVT requires psychological input and counseling to help patients understand their condition and the different treatment strategies. This is a key component to the care of patients with CPVT within our clinic.
Treatment of CPVT requires an individualized approach by a specialist team.
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