What's catecholaminergic polymorphic ventricular tachycardia?
Catecholaminergic polymorphic ventricular tachycardia is a rare but potentially lethal inherited arrhythmia syndrome, characterized by bidirectional or polymorphic ventricular tachycardia provoked by exercise or emotion.
| Also indexed as | ORPHA:3286, MONDO:0017990 |
|---|---|
| Features mapped | 9 |
| Treatments mapped | 2 |
| Published sources | 9 |
| Last reviewed | 2026-08-04 |
Signs and symptoms
Sudden cardiac death
Sudden cardiac death remains a risk in this condition even for people already on medical therapy. That residual risk is why treatment is often intensified beyond a single drug.
Cardiac arrest
The heart can stop pumping without warning. Illness is most severe in younger people, who typically first come to attention with cardiac arrest or with fainting caused by an abnormal rhythm.
Polymorphic ventricular tachycardia
Polymorphic ventricular tachycardia is a fast, unstable rhythm of the heart's main pumping chambers in which each beat takes a different shape. In this condition it is brought on by exercise or by strong emotion.
Supraventricular tachycardia
Fast rhythms starting in the heart's upper chambers also occur. In a cohort of 206 people with this condition, 17, or 8.3 percent, had one, including atrial fibrillation, atrial flutter, atrial tachycardia and supraventricular tachycardia.
Syncope
Fainting after exercise or after becoming upset is the way this condition most often announces itself. Fainting and sudden death following exertion or agitation are the main clinical signs.
Bidirectional ventricular tachycardia
Bidirectional ventricular tachycardia is a fast rhythm in which beats alternate direction from one to the next. It is the signature tracing of this condition, and it appears when exercise or emotion raises adrenaline. It can lead to repeated fainting and to sudden death.
Structurally normal heart
The heart itself is built normally. Imaging shows no damage or malformation; the problem is electrical, and the abnormal rhythms are driven by adrenaline.
Unremarkable resting electrocardiogram
A resting electrocardiogram is usually normal, and so is a Holter monitor. The abnormal rhythm shows up only when the heart is stressed, which is why exercise stress testing is what reveals bidirectional ventricular tachycardia.
Onset in childhood
Symptoms usually begin in childhood. The median age at which the condition first declares itself is about ten years.
Treatment and management
What the research describes, not a recommendation. Treatment decisions belong with your clinician.
This covers treatments that appear in the published research mapped here. Investigational and experimental therapies are not included, so their absence is a boundary of this map, not a sign they do not exist.
flecainide
Flecainide is added to beta-blocker treatment. Adding it raises the share of people whose arrhythmia is controlled from 60 to 70 percent on a beta-blocker alone to 80 to 90 percent, and it works by acting directly on the RyR2 calcium-release channel.
Used to help with: Catecholaminergic polymorphic ventricular tachycardia.
“…the addition of flecainide to beta-blocker therapy was associated with a significant reduction in arrhythmic burden and improvement in exercise-related parameters during long-term…”
left cardiac sympathetic denervation
Left cardiac sympathetic denervation is surgery that interrupts the nerves carrying adrenaline signals to the heart. Combined with a beta-blocker and flecainide it significantly reduced breakthrough cardiac events, though it did not prevent aborted cardiac arrest or sudden death.
Used to help with: Catecholaminergic polymorphic ventricular tachycardia.
“Triple therapy with beta-blockers, flecainide, and LCSD significantly reduced BCEs (p = 0.044), but not ACA/SCD (p =…”
How to read the evidence labels
Where this comes from
This guide is built from 9 published source(s). Every claim above links back to one of them. Click any source ID to read the original on PubMed.
Take it further
Printed, source-linked documents built from this condition's graph — ready to bring to an appointment or attach to a coverage request. Every claim carries its published source, the same as this guide.