Background
Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is an inherited arrhythmia
syndrome, characterised by polymorphic ventricular tachycardia induced by adrenergic
stress. CPVT can be caused by mutations the cardiac ryanodine receptor gene (RYR2) or mutations in the cardiac calsequestrin gene CASQ2. Structural heart disease is usually absent and the baseline ECG is usually normal.
Patients with CPVT often present with exercise- or emotion induced syncope, the first
presentation can also be sudden cardiac death.
Management
Besides removal of triggers treatment with beta blockers is currently a class I indication
in clinically diagnosed patients. Beta blockage should be titrated up to an effective
level. The addition of flecainide seems to be a promising approach in patients where
arrhythmias are not completely suppressed by beta blockers. A cardioverter-defibrillator
(ICD) or left cervical sympathetic denervation might be considered under special circumstances.
Genetic counselling is recommended and all first degree relatives should be properly
evaluated.
Keywords
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Heart, Lung and CirculationAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Clinical and molecular characterization of patients with catecholaminergic polymorphic ventricular tachycardia.Circulation. 2002; 106: 69-74
- Catecholaminergic polymorphic ventricular tachycardia in children. A 7-year follow-up of 21 patients.Circulation. 1995; 91: 1512-1519
- Familial polymorphic ventricular arrhythmias: a quarter century of successful medical treatment based on serial exercise-pharmacologic testing.J Am Coll Cardiol. 1999; 34: 2015-2022
- Ryanodine receptor and calsequestrin in arrhythmogenesis: what we have learnt from genetic diseases and transgenic mice.J Mol Cell Cardiol. 2009; 46: 149-159
- Recommendations for physical activity and recreational sports participation for young patients with genetic cardiovascular diseases.Circulation. 2004; 109: 2807-2816
- Incidence and risk factors of arrhythmic events in catecholaminergic polymorphic ventricular tachycardia.Circulation. 2009; 119: 2426-2434
- ACC/AHA/ESC 2006 Guidelines 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 and the European Society of Cardiology Committee for Practice Guidelines (writing committee to develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death): developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society.Circulation. 2006; 114: e385-e484
- Calcium channel blockers and beta-blockers versus beta-blockers alone for preventing exercise-induced arrhythmias in catecholaminergic polymorphic ventricular tachycardia.Heart Rhythm. 2007; 4: 1149-1154
- Calcium channel antagonism reduces exercise-induced ventricular arrhythmias in catecholaminergic polymorphic ventricular tachycardia patients with RyR2 mutations.J Cardiovasc Electrophysiol. 2005; 16: 162-166
- Flecainide prevents catecholaminergic polymorphic ventricular tachycardia in mice and humans.Nat Med. 2009; 15: 380-383
- Flecainide therapy reduces exercise-induced ventricular arrhythmias in patients with catecholaminergic polymorphic ventricular tachycardia.J Am Coll Cardiol. 2011; 57: 2244-2254
- Sudden death in a young man with catecholaminergic polymorphic ventricular tachycardia and paroxysmal atrial fibrillation.J Cardiovasc Electrophysiol. 2008; 19: 1319-1321
- Sudden cardiac death despite an implantable cardioverter-defibrillator in a young female with catecholaminergic ventricular tachycardia.Heart Rhythm. 2006; 3: 1486-1489
- Left cardiac sympathetic denervation for the treatment of long QT syndrome and catecholaminergic polymorphic ventricular tachycardia using video-assisted thoracic surgery.Heart Rhythm. 2009; 6: 752-759
- Video-assisted thoracoscopic cardiac denervation: a potential novel therapeutic option for children with intractable ventricular arrhythmias.Ann Thorac Surg. 2008; 86: 1620-1625
- Left cardiac sympathetic denervation for catecholaminergic polymorphic ventricular tachycardia.New Engl J Med. 2008; 358: 2024-2029
- Clinical phenotype and functional characterization of CASQ2 mutations associated with catecholaminergic polymorphic ventricular tachycardia.Circulation. 2006; 114: 1012-1019
- High prevalence of exercise-induced arrhythmias in catecholaminergic polymorphic ventricular tachycardia mutation-positive family members diagnosed by cascade genetic screening.Europace. 2010; 12: 417-423
Article info
Publication history
Accepted:
October 28,
2011
Received in revised form:
October 25,
2011
Received:
August 1,
2011
Identification
Copyright
© 2011 Published by Elsevier Inc. All rights reserved.
