CATECHOLAMINERGIC
POLYMORPHIC VENTRICULAR TACHYCARDIA (CPVT)
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a
disorder triggered by exercise or stress in which the heart's pumping
chambers go into wild, uncontrolled rhythms that do not pump blood
effectively. In contrast to long QT syndrome, another inherited heart
rhythm condition, CPVT cannot be diagnosed through a resting
electrocardiogram (ECG). The first indication of CPVT often is
exercise-induced fainting or sudden death. For this reason, CPVT is
known as the ‘silent assassin’.
CPVT is
characterized by episodic syncope occurring during exercise or acute
emotion in individuals without structural cardiac abnormalities. The
underlying cause of these episodes is the onset of fast ventricular
tachycardia (bidirectional or polymorphic). Spontaneous recovery occurs
when these arrhythmias self-terminate. In other instances, ventricular
tachycardia may degenerate into ventricular fibrillation and cause
sudden death if cardiopulmonary resuscitation is not readily available. The mean age of onset of CPVT is between seven and nine years; however,
onset as late as the fourth decade of life has been reported.
Additional
Information
Sudden
Arrithymia Death Syndrome
www.sadsuk.org/cpvt
Cardiac
Inherited Disease Registry
www.cidg.org/webcontent/Portals/1/RegistryEnrolement/CPVT%20Info%20Sheet.pdf
