the use of beta blockers. There are minimal data available to guide the long-term management of genotype-positive, phenotype-negative patients who are diagnosed solely based upon genetic screening. Expert opinion differs slightly on the long-term role of beta blocker therapy, based upon the age at diagnosis: https://www.uptodate.com/contents/catecholaminergic-polymorphic-ventricular-tachycardia/print 12/23 7/6/23, 3:39 PM Catecholaminergic polymorphic ventricular tachycardia - UpToDate Expert opinion supports the use of beta blockers in patients without clinical manifestations who are diagnosed in childhood based upon genetic analysis [38]. The usefulness and/or efficacy of beta blockers is less well established in patients without clinical evidence of arrhythmias who are diagnosed in adulthood based upon genetic analysis [38]. SOCIETY GUIDELINE LINKS Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Inherited arrhythmia syndromes" and "Society guideline links: Ventricular arrhythmias" and "Society guideline links: Cardiac implantable electronic devices".) SUMMARY AND RECOMMENDATIONS Definition Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a genetic disorder that presents as familial as well as sporadic cases. Genetics Pathogenic variants in the ryanodine receptor and calsequestrin 2 protein are found in 70 percent of people with CPVT. Other pathogenic variants can cause CPVT, but they are less common. (See 'Genetics' above.) Clinical presentation and risk factors CPVT occurs in the absence of structural heart disease and typically presents in adolescence. The clinical presentation of CPVT is variable; some patients are asymptomatic and identified as part of family screening. Symptomatic patients typically present with syncope or cardiac arrest due to ventricular tachycardia (VT) or ventricular fibrillation (VF) precipitated by emotional or physical stress. (See 'Clinical presentation' above.) Risk factors for sudden death include documented VF, a family history of sudden death, and onset of symptoms in childhood. (See 'Epidemiology' above.) Diagnosis CPVT is diagnosed by an exercise stress test that shows increased frequency of nonsustained or sustained VT during exercise or recovery. In patients with a suspicious clinical presentation or pedigree, appropriate genetic testing can provide additional diagnostic support. (See 'Diagnostic testing and diagnosis' above.) https://www.uptodate.com/contents/catecholaminergic-polymorphic-ventricular-tachycardia/print 13/23 7/6/23, 3:39 PM Catecholaminergic polymorphic ventricular tachycardia - UpToDate Acute management The acute management of patients with CPVT (or suspected CPVT) includes: Patients with polymorphic VT who are hemodynamically unstable or become pulseless require prompt treatment with electrical cardioversion/defibrillation. (See "Overview of the acute management of tachyarrhythmias", section on 'Wide QRS complex tachyarrhythmias'.) Propranolol is used for acute suppression of recurrent polymorphic VT. Propranolol is given as 40 mg oral doses (or appropriate weight-based dosing in children) every six hours for the first 48 hours, with additional intravenous doses as needed for recurrent breakthrough ventricular arrhythmias. (See "Electrical storm and incessant ventricular tachycardia", section on 'Initial management' and 'Beta blockers' above.) Subsequent management Management of CPVT is summarized in the algorithm ( algorithm 1). All patients - For all patients with CPVT (high and low risk), we recommend initiation of beta blocker therapy (Grade 1B). We suggest the use of long-acting nonselective beta blockers (such as nadolol) rather than short-acting or beta-1 selective beta blockers (Grade 2B). (See 'Beta blockers' above.) We advise that patients avoid strenuous exercise; however, some patients may reasonably choose to continue to participate. Appropriate cautionary measures include an emergency action plan with an automated external defibrillator immediately available. (See 'Avoidance of strenuous exercise' above and "Athletes: Overview of sudden cardiac death risk and sport participation", section on 'Inherited arrhythmia syndromes'.) Monitoring for recurrent ventricular arrythmias is done by regular device checks, exercise testing, and 24-hour Holter monitoring to confirm suppression of exercise- induced heart rate. High-risk patients are survivors of cardiac arrest (SCA), syncope, or sustained VT or VF. Others are low risk unless they have recurrent arrhythmia despite treatment with beta blockers. High-risk patients require an implantable cardioverter-defibrillator (ICD) in addition to beta blocker therapy. ICDs should not be used alone without pharmacologic therapy. https://www.uptodate.com/contents/catecholaminergic-polymorphic-ventricular-tachycardia/print 14/23 7/6/23, 3:39 PM Catecholaminergic polymorphic ventricular tachycardia - UpToDate (See 'Implantable cardioverter-defibrillators' above and "Secondary prevention of sudden cardiac death in heart failure and cardiomyopathy".) For patients with continued arrythmia despite beta blocker therapy, we suggest the addition of flecainide (Grade 2C). (See 'Flecainide' above.) Therapy for refractory arrythmia or spontaneous cardiac arrest Additional treatment options for patients with refractory arrhythmias include verapamil and left sympathetic denervation. Side effects from sympathetic denervation are common, although overall patient satisfaction following surgery is high. (See 'Sympathetic denervation' above.) Genotype-positive, phenotype-negative patients For patients without clinical manifestations who are diagnosed in childhood based upon genetic analysis, we suggest the use of beta blockers (Grade 2C). (See 'Management of genotype positive, phenotype negative patients' above.) Use of UpToDate is subject to the Terms of Use. REFERENCES 1. Nguyen PT, Scheinman MM, Seger J. Polymorphous ventricular tachycardia: clinical characterization, therapy, and the QT interval. Circulation 1986; 74:340. 2. Wren C, Rowland E, Burn J, Campbell RW. Familial ventricular tachycardia: a report of four families. Br Heart J 1990; 63:169. 3. 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Topic 916 Version 38.0 https://www.uptodate.com/contents/catecholaminergic-polymorphic-ventricular-tachycardia/print 20/23 7/6/23, 3:39 PM Catecholaminergic polymorphic ventricular tachycardia - UpToDate GRAPHICS Catecholaminergic polymorphic ventricular tachycardia and nonsustained ventricular tachycardia Ambulatory ECG recordings from a 28-year-old man with exercise-related palpitations. The figure shows two episodes of exercise-related (note sinus tachycardia prior to VT onset) polymorphic VT (arrows). The first episode of VT begins with a VPD on the downstroke of the T wave. The second, briefer episode appears to begin with a later-onset VPD. This patient's arrhythmias were suppressed in response to exercise restriction and nadolol. Genetic testing did not reveal a recognized RYR2 or CASQ2 mutation. Note that the QT interval appears normal for rate. ECG: electrocardiogram; VT: ventricular tachycardia; VPD: ventricular premature depolarization. Courtesy of Alfred Buxton, MD. Graphic 133240 Version 1.0 https://www.uptodate.com/contents/catecholaminergic-polymorphic-ventricular-tachycardia/print 21/23 7/6/23, 3:39 PM Catecholaminergic polymorphic ventricular tachycardia - UpToDate Algorithm for the long- term management of catecholaminergic polymorphic ventricular tachycardia CPVT: catecholaminergic polymorphic ventricular tachycardia; ICD: implantable cardioverter-defibrillator; AV: atrioventricular. We counsel patients with CPVT to avoid competitive sports and strenuous exercise. Long-acting, nonselective beta blockers (such as nadolol) are preferred over short-acting or B1 selective agents. Flecainide is contraindicated in patients with known structural heart disease (eg, coronary artery disease) o patients with high grade AV block or bifascicular block. Patients who have a high burden of arrhythmias or are intolerant of beta blockers should generally underg sympathetic denervation. Graphic 118659 Version 2.0 https://www.uptodate.com/contents/catecholaminergic-polymorphic-ventricular-tachycardia/print 22/23 7/6/23, 3:39 PM Catecholaminergic polymorphic ventricular tachycardia - UpToDate Contributor Disclosures Alfred Buxton, MD No relevant financial relationship(s) with ineligible companies to disclose. Peter J Zimetbaum, MD Consultant/Advisory Boards: Abbott Medical [Lead extraction]. All of the relevant financial relationships listed have been mitigated. Nisha Parikh, MD, MPH No relevant financial relationship(s) with ineligible companies to disclose. Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are addressed by vetting through a multi-level review process, and through requirements for references to be provided to support the content. Appropriately referenced content is required of all authors and must conform to UpToDate standards of evidence. Conflict of interest policy https://www.uptodate.com/contents/catecholaminergic-polymorphic-ventricular-tachycardia/print 23/23