Table 5. [Treatment of Manifestations in Individuals...]. - GeneReviews®

Manifestation/ Concern	Treatment	Considerations/Other
Adrenergic- dependent triggered activity	Beta-blocker therapy: nadolol (1-2.5 mg/kg/day in a single dose or divided into 2 doses per day) or propranolol (2-4 mg/kg/day divided into 3-4 doses per day)	Nadolol is reported to be more effective than selective beta blockers [Leren et al 2016]. However, selective beta blockers can be used in persons w/asthma or other respiratory conditions. Chronic treatment w/full-dose beta-blocking agents prevents recurrence of syncope in majority of affected persons. Dose needs to be individualized w/exercise stress testing & efficacy periodically retested (see Surveillance).
Arrhythmia control	Flecainide (100-300 mg/day)	Can be given along w/beta blockers to persons who have syncope recurrence or complex arrhythmias during exercise Beta blockers & flecainide together are also indicated for affected persons who have experienced a previous aborted sudden death, to ↓ probability of ICD shocks. The antiarrhythmic effects of flecainide appear to be independent of specific CPVT genetic subtype. ¹
Cardiac arrest, recurrent syncope, or polymorphic/ bidirectional VT despite optimal therapy	Implantable cardioverter defibrillator	Whenever possible, pharmacologic therapy should be maintained/optimized even in persons w/an ICD to ↓ probability of ICD firing.
Left cardiac sympathetic denervation	May be considered in those w/several appropriate ICD shocks while on beta blocker & flecainide & in those who are intolerant of or w/contraindication to beta-blocker therapy. Side effects incl palpebral ptosis, ↑ of left hemidiaphragm, & lack of sweating from left arm & face. Recurrence of cardiac events may occur even w/LCSD, so it cannot be considered an alternative to ICD.

Manifestation/
Concern

Treatment

Considerations/Other

Adrenergic-
dependent
triggered
activity

Beta-blocker therapy: nadolol (1-2.5 mg/kg/day in a single dose or divided into 2 doses per day) or propranolol (2-4 mg/kg/day divided into 3-4 doses per day)

Nadolol is reported to be more effective than selective beta blockers [Leren et al 2016]. However, selective beta blockers can be used in persons w/asthma or other respiratory conditions.
Chronic treatment w/full-dose beta-blocking agents prevents recurrence of syncope in majority of affected persons.
Dose needs to be individualized w/exercise stress testing & efficacy periodically retested (see Surveillance).

Arrhythmia
control

Flecainide (100-300 mg/day)

Can be given along w/beta blockers to persons who have syncope recurrence or complex arrhythmias during exercise
Beta blockers & flecainide together are also indicated for affected persons who have experienced a previous aborted sudden death, to ↓ probability of ICD shocks.
The antiarrhythmic effects of flecainide appear to be independent of specific CPVT genetic subtype. ¹

Cardiac arrest,
recurrent syncope,
or polymorphic/
bidirectional VT
despite optimal
therapy

Implantable cardioverter defibrillator

Whenever possible, pharmacologic therapy should be maintained/optimized even in persons w/an ICD to ↓ probability of ICD firing.

Left cardiac sympathetic denervation

May be considered in those w/several appropriate ICD shocks while on beta blocker & flecainide & in those who are intolerant of or w/contraindication to beta-blocker therapy.
Side effects incl palpebral ptosis, ↑ of left hemidiaphragm, & lack of sweating from left arm & face.
Recurrence of cardiac events may occur even w/LCSD, so it cannot be considered an alternative to ICD.

From: Catecholaminergic Polymorphic Ventricular Tachycardia

GeneReviews^® [Internet].

Adam MP, Feldman J, Mirzaa GM, et al., editors.

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