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.
Sudden Arrithymia Death Syndrome www.sadsuk.org/cpvt
Cardiac Inherited Disease Registry www.cidg.org/webcontent/Portals/1/RegistryEnrolement/CPVT%20Info%20Sheet.pdf