Glikson M, Nielsen JC, Kronborg MB, et al; ESC Scientific Document Group. 2021 ESC Guidelines on cardiac pacing and cardiac resynchronization therapy. Eur Heart J. 2021 Sep 14;42(35):3427-3520. doi: 10.1093/eurheartj/ehab364. Erratum in: Eur Heart J. 2022 May 1;43(17):1651. PMID: 34455430.
Cronin EM, Bogun FM, Maury P, et al; ESC Scientific Document Group. 2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias. Europace. 2019 Aug 1;21(8):1143-1144. doi: 10.1093/europace/euz132. Erratum in: Europace. 2019 Aug 1;21(8):1144. Erratum in: J Arrhythm. 2020 Jan 12;36(1):214. Erratum in: Europace. 2020 Mar 1;22(3):505. PMID: 31075787; PMCID: PMC7967791.
Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation. 2018 Sep 25;138(13):e272-e391. doi: 10.1161/CIR.0000000000000549. Erratum in: Circulation. 2018 Sep 25;138(13):e419-e420. PMID: 29084731.
Bennett M, Parkash R, Nery P, et al. Canadian Cardiovascular Society/Canadian Heart Rhythm Society 2016 Implantable Cardioverter-Defibrillator Guidelines. Can J Cardiol. 2017 Feb;33(2):174-188. doi: 10.1016/j.cjca.2016.09.009. Epub 2016 Oct 6. PMID: 28034580.
Priori SG, Wilde AA, Horie M, et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes: document endorsed by HRS, EHRA, and APHRS in May 2013 and by ACCF, AHA, PACES, and AEPC in June 2013. Heart Rhythm. 2013 Dec;10(12):1932-63. doi: 10.1016/j.hrthm.2013.05.014. Epub 2013 Aug 30. Review. PubMed PMID: 24011539.
Definition and EtiologyTop
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare genetic disorder characterized by symptomatic ventricular tachycardia (VT) associated with adrenergic activation in patients with no structural heart disease.
More than 60% of patients have an episode of syncope or cardiac arrest before the age of 20 years. Life-threatening arrhythmias are recurrent. In addition to malignant VT, patients may have other types of arrhythmia, such as atrial fibrillation.
The key symptoms include syncope caused by fast polymorphic VT, cardiac arrest, or sudden cardiac death, most commonly triggered by exercise or emotions.
Diagnosis is based on identifying the arrhythmia typical for CPVT: a fast, polymorphic, frequently bidirectional VT (beat-to-beat changes in the QRS polarity from negative to positive or vice versa), which is most easily triggered by an exercise test.
Classification of antiarrhythmic drugs: see Table 3.4-1.
Antiarrhythmic agents: see Table 3.4-2.
Avoidance of exercise, treatment with beta-blockers. Placement of an implantable cardioverter-defibrillator (ICD) is indicated in patients with a history of cardiac arrest as well as in those with syncope or VT despite beta-blocker treatment.
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