Treatment
Treatments for CPVT aim to prevent lethal abnormal heart rhythms from occurring, and to rapidly restore a normal rhythm if they do occur. As the arrhythmias in CPVT generally occur at times when the heart is exposed to high levels of adrenaline or other similar chemical messengers (catecholamines), many treatments for CPVT aim to lower the levels of catecholamines the heart is exposed to or block their effects on the heart.The first-line treatment for those with CPVT involves lifestyle advice. This includes avoiding competitive sports, very strenuous exercise and highly stressful environments, as high levels of adrenaline can occur in these settings, which can provoke arrhythmias. Medication
Several medications can be useful for those with CPVT. The mainstays of treatment are beta blockers, which block the effects of adrenaline and other catecholamines on the heart, reducing the chance of abnormal heart rhythms developing. Of all the beta blockers, nadolol has been proven to be the most effective for treating CPVT. This drug lowers the heart rate to a greater extent than other beta blockers and only needs to be taken once daily, reducing the risk of missed doses. Nadolol may be difficult to obtain and is not available in all countries, and an alternative beta blocker suitable for use in CPVT may be propranolol, which however has a more complex dosing regimen.
The bidirectional ventricular tachycardia associated with this condition was described in 1975. The term "Catecholaminergic Polymorphic Ventricular Tachycardia" was first used in 1978. In 1999, the first genetic mutation causing CPVT to be identified was localised to chromosome 1q42-q43, which was found to be a variant in the RYR2 gene in 2001. Ongoing research aims to identify better treatments for CPVT, to increase understanding of the mechanisms of arrhythmia, and to identify other genes causing the condition. References


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