---
title: Catecholaminergic polymorphic ventricular tachycardia
description: What catecholaminergic polymorphic ventricular tachycardia (CPVT) is, why a dangerous arrhythmia starts during exertion or emotional stress, why the resting ECG is usually normal, how the condition is detected, which treatment approaches clinical guidelines describe and when urgent help is needed.
type: disease
preset: bidirectional-ventricular-tachycardia
aliases:
  - CPVT
  - catecholaminergic polymorphic VT
  - catecholamine-dependent polymorphic ventricular tachycardia
sources:
  - tier: guideline
    org: minzdrav-rf
    title: Желудочковые нарушения ритма сердца. Внезапная сердечная смерть
    year: 2025
    doi: 10.15829/1560-4071-2025-6670
    url: https://cr.minzdrav.gov.ru/view-cr/569_2
  - tier: reference
    org: msd-manuals
    title: Catecholaminergic Polymorphic Ventricular Tachycardia
    year: 2026
    url: https://www.msdmanuals.com/professional/cardiovascular-disorders/arrhythmogenic-cardiac-disorders/catecholaminergic-polymorphic-ventricular-tachycardia
  - tier: guideline
    org: nice
    title: Transient loss of consciousness ('blackouts') in over 16s (CG109)
    year: 2023
    url: https://www.nice.org.uk/guidance/cg109
sources_checked: 2026-10-13
review: null
---

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited channelopathy: under
the effect of adrenaline and other stress hormones, a dangerous
[ventricular tachycardia](../ventricular-tachycardia/) starts.<Cite n={[1, 2]} /> An attack is
usually triggered by physical exertion or strong emotion.<Cite n={2} /> Between attacks the heart
looks healthy: the resting ECG is usually normal and there are no signs of structural heart
disease.<Cite n={[1, 2]} /> The arrhythmia can cause fainting and cardiac arrest, and CPVT is a
common cause of sudden death in young people.<Cite n={2} /> Its estimated prevalence is about 1 in
10,000 people.<Cite n={[1, 2]} />

<Diagram name="catecholaminergic-polymorphic-ventricular-tachycardia">
  <p slot="normal">
    At rest. The diagram shows the impulse taking its usual route from the sinus node through the AV
    node to the ventricles, with a regular rhythm; more in the article on the
    [conduction system](../../anatomy/conduction-system/). Below is a ventricular cell close up:
    calcium is kept in its internal store (the sarcoplasmic reticulum).<Cite n={2} />
  </p>
  <p slot="disease">
    Exertion or emotion. In CPVT, attacks are usually set off by stress hormones released during
    physical or emotional stress.<Cite n={2} /> In response, the store in the cell releases too much
    calcium, the cell becomes overloaded with it and produces extra electrical impulses.<Cite
    n={2} /> The impulses start at different points in the ventricles, and bidirectional or
    polymorphic ventricular tachycardia begins, which can turn into ventricular
    fibrillation.<Cite n={[1, 2]} /> The structure of the heart does not change.<Cite n={2} />
  </p>
</Diagram>

## Why it happens

<Patient>

CPVT is caused by changes (mutations) in the genes for proteins that control calcium inside heart
cells.<Cite n={2} /> The more common form is dominant, with a mutation in the gene for the ryanodine
receptor (RYR2); a rarer form is recessive, with a mutation in the gene for calsequestrin
(CASQ2).<Cite n={[1, 2]} /> In some people no gene change can be found.<Cite n={2} /> Men and women
are affected equally often.<Cite n={2} />

An attack is triggered by physical or emotional stress: stress hormones increase the release of
calcium in heart cells, and extra impulses appear in the ventricles.<Cite n={2} />

</Patient>

<Student>

The Russian Ministry of Health guideline describes two genetic types of CPVT: a dominant type caused
by mutations in the gene for the cardiac ryanodine receptor (RyR2), and a rare recessive type caused
by a mutation in the gene for cardiac calsequestrin (CASQ2).<Cite n={1} />

The mutations affect intracellular calcium regulation, particularly diastolic calcium release from
the sarcoplasmic reticulum through the ryanodine receptor. Adrenergic stimulation increases this
release; the resulting myocyte calcium overload causes delayed afterdepolarisations and a
propensity to atrial and/or ventricular tachyarrhythmias.<Cite n={2} />

</Student>

<HeartModel highlight={['left-ventricle', 'right-ventricle']} />

## Symptoms

<Patient>

Some people have no symptoms and are found only when their family is screened.<Cite n={2} /> When
symptoms occur, they are palpitations, fainting or cardiac arrest, usually during physical exertion
or strong emotion.<Cite n={2} />

Doctors think of CPVT and of [long QT syndrome](../long-qt-syndrome/) when a person, especially a
young person without heart disease, loses consciousness at the peak of physical or emotional
stress.<Cite n={1} /> Fainting during exercise is probably caused by an arrhythmia, while fainting
shortly after stopping exercise is more likely a [vasovagal faint](../vasovagal-syncope/).<Cite
n={3} />

</Patient>

<Student>

CPVT shows a marked variety of rhythm disturbances: from bradycardia, a short PR interval, AV
dissociation and supraventricular tachycardias to multifocal and polymorphic tachyarrhythmias; as a
result, the causes of syncope are often misinterpreted and the diagnosis is delayed.<Cite n={1} />

The most characteristic arrhythmia is bidirectional VT: the frontal QRS axis alternates from beat to
beat.<Cite n={[1, 2]} /> Any polymorphic VT or ventricular fibrillation may also occur in CPVT.<Cite
n={2} /> Independent predictors of arrhythmic events are diagnosis in childhood, no β-blocker
therapy and persistent complex arrhythmias.<Cite n={1} />

</Student>

## How it is diagnosed

<Patient>

The resting [ECG](../../diagnostic/ecg/) is usually normal and there are no signs of structural
heart disease, so tests at rest may find nothing.<Cite n={2} /> The key test is an exercise test:
the ECG is recorded during physical exercise. The diagnosis is confirmed if exercise brings on
polymorphic, especially bidirectional, ventricular tachycardia that reproduces the person's
symptoms.<Cite n={[1, 2]} />

Genetic testing is recommended for everyone with CPVT and for people in whom it is very likely.<Cite
n={1} /> If a mutation is found, all close relatives are tested for it, even if they have no
symptoms.<Cite n={1} />

If a close family member died suddenly and the post-mortem found no clear cause, relatives are
advised to be examined by a cardiologist.<Cite n={1} /> In about half of such cases an inherited
condition is found, most often a channelopathy: long QT syndrome, [Brugada
syndrome](../brugada-syndrome/) or CPVT.<Cite n={1} />

</Patient>

<Student>

An exercise stress test is recommended in adult patients to clarify the diagnosis and prognosis in
exercise-induced ventricular tachyarrhythmias or when they are suspected, including CPVT (ESC
IB).<Cite n={1} /> Invasive electrophysiological study is not recommended for sudden cardiac death
risk stratification in CPVT.<Cite n={1} />

Molecular genetic testing of RYR2, CASQ2, CALM1-3, TRDN, TECRL and KCNJ2 is recommended for all
patients with CPVT and for patients whose clinical presentation is very likely due to it (ESC
IC).<Cite n={1} />

</Student>

## How it is treated

<Patient>

The mainstay of treatment is beta-blockers plus lifestyle changes with limited physical
exertion.<Cite n={1} /> The Russian Ministry of Health guideline recommends beta-blockers for
everyone with confirmed CPVT, and also for relatives found to carry the mutation, even if they have
no symptoms.<Cite n={1} /> Beta-blocker treatment is also recommended during pregnancy and after
childbirth.<Cite n={1} />

The Russian Ministry of Health guideline advises everyone with CPVT to avoid competitive sports,
strength training and stressful situations.<Cite n={1} /> Competitive sport means amateur or
professional sport practised regularly with participation in organised competitions.<Cite n={1} />

If fainting or tachycardia comes back despite beta-blockers, the doctor may add another
antiarrhythmic medicine.<Cite n={[1, 2]} /> People who have survived cardiac arrest, and people
whose fainting or dangerous tachycardia keeps coming back despite treatment, are advised to have an
implantable cardioverter-defibrillator (ICD), together with medicines rather than instead of
them.<Cite n={1} /> ICDs have a particular problem in CPVT: a shock can be painful, increase the
nervous system's drive on the heart and set off new attacks, creating a vicious circle; this is why
the device is programmed in a special way.<Cite n={[1, 2]} />

Another option is an operation called left cardiac sympathetic denervation. It is considered if
fainting, tachycardia or ICD shocks keep coming back despite medicines, or if the medicines are not
tolerated or not suitable.<Cite n={1} /> The doctor decides which treatment is needed; do not change
or stop medicines on your own.

</Patient>

<Student>

First-line therapy for CPVT is non-selective β-blockers without intrinsic sympathomimetic activity
and lifestyle modification with exercise restriction.<Cite n={1} /> β-blockers are recommended for
all patients with diagnosed CPVT (ESC IC) and for mutation-carrying relatives even without symptoms
(ESC IIaC).<Cite n={1} /> If syncope, polymorphic or bidirectional VT or persistent exercise-induced
ventricular ectopy recurs on β-blockers, adding an antiarrhythmic drug is recommended (ESC
IIaC).<Cite n={1} />

An ICD in addition to β-blockers (with or without class I and III antiarrhythmic drugs) is
recommended for patients who survived cardiac arrest and for those with recurrent syncope or
episodes of polymorphic/bidirectional VT despite optimal medical therapy (ESC IC).<Cite n={1} /> ICD
shocks can be painful, raise sympathetic tone and provoke recurrent arrhythmia episodes, up to
electrical storm.<Cite n={[1, 2]} />

Left cardiac sympathetic denervation should be considered for recurrent syncope,
polymorphic/bidirectional VT or several appropriate ICD shocks on β-blockers (alone or with
antiarrhythmic drugs), and when these drugs are not tolerated or contraindicated (ESC IIbC).<Cite
n={1} /> MSD mentions catheter ablation of the polymorphic premature ventricular beats that trigger
VT or VF for refractory cases.<Cite n={2} />

</Student>

## When to call an ambulance

**Call your local emergency number (112 in the EU, 911 in the US and Canada, 999 in the UK)**
if:<Cite n={1} />

- the person has lost consciousness, especially during physical exertion or strong
  emotion;<Cite n={[1, 3]} />
- palpitations come with dizziness, weakness or feeling faint;<Cite n={1} />
- the person is unconscious and not breathing: this may be cardiac arrest. Start cardiopulmonary
  resuscitation (CPR) at once and use an automated external defibrillator (AED) if one is
  nearby.<Cite n={1} /> The Russian Ministry of Health guideline advises placing such
  defibrillators in accessible places in gyms and sports arenas.<Cite n={1} />

Even if the person has come round, NICE (for people over 16) treats the following as red flags: a
blackout during exertion, and a family history of sudden cardiac death in people younger than 40 or
an inherited cardiac condition. Such a person should be referred urgently for cardiovascular assessment, with the referral reviewed and prioritised by an appropriate specialist within 24 hours.<Cite n={3} /> While the person waits for a
specialist assessment, NICE advises the doctor to explain what to do if another blackout happens
and, if appropriate, how to modify their activity, for example by avoiding physical exertion.<Cite n={3} />

<Quiz />