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Individuals with CPVT may have symptoms from abnormal heart rhythms (arrhythmias), including palpitations, episodic lightheadedness, or fainting episodes. Individuals may also report a history of seizure, though these are sometimes misdiagnosed episodes of arrhythmias. The arrhythmias in this condition almost exclusively occur during activities associated with high amounts of adrenaline (heavy exertion, sports, or emotional stress). 

There is a large amount of variability in the risk of arrhythmias among individuals with CPVT, even within the same family. Evaluation of the whole family is important since the condition is often passed on to the next generation of a family but may not cause symptoms initially.

Exercise testing is very important in diagnosis, since the resting EKG is normal with CPVT. 

Medication to blunt the effects of adrenaline is critically important in CPVT. Additional medication options have now also proven to be effective, making CPVT a highly treatable condition. An important part of evaluation is determining the personalized risk level for each individual with CPVT and providing guidance in decisions for implantable defibrillators (ICDs) that prevent sudden death. ICDs have specific disadvantages in CPVT that must be considered, and the decision for ICD implantation should be reserved for only a minority of individuals with CPVT who remain at high risk despite being on the right medication.

Learn more about Inherited Cardiomyopathies and Arrhythmias