Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is a familial cardiac calcium ion channelopathy that can result in sudden death in athletes.
Inheritance: autosomal dominant or recessive
Onset: pediatric
Clinical manifestations:
(1) seizures
(2) palpitations
(3) sudden cardiac arrest
Triggers:
(1) strenuous exercise
(2) stress
(3) competitive sports
High risk:
(1) onset during childhood
(2) absence of beta blockers
(3) proband status
(4) history of aborted cardiac arrest before diagnosis
The resting ECG is typically normal.
ECG during episodes may show:
(1) polymorphic PVCs
(2) nonsustained ventricular tachycardia (NSVT)
(3) polymorphic ventricular tachycardia (PMVT)
(4) bidirectional ventricular tachycardia
Therapy:
(1) beta-blockers (nadolol or propranolol)
(2) flecainide
(3) verapamil plus beta blockers
(4) ICD
(5) left cardiac sympathetic denervation (LCSD)
|
Status
|
ACC/AHA
|
European ESC
|
|
symptomatic
|
not recommended (may engage in class IA sports)
|
should not engage
|
|
asymptomatic
|
participate with precautions
|
not applicable
|
