32 Asymptomatic family members All first degree relatives should be evaluated with ECG Holter monitoring and exercise stress testing Echocardiography might be useful in cases where CPVT is not yet ID: 189149
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CSANZ Guidelines for the diagnosis and management of Catecholaminergic Polymorphic Ventricular Tachycardia Page 4 3.2 Asymptomatic family members All first degree relatives should be evaluated with ECG, Holter monitoring and exercise stress testing. Echocardiography might be useful in cases where CPVT is not yet proven in the family or overlap with other conditions might be suspected. Genetic analysis might identify silent carriers of CPVT related mutations. The mean penetrance of RYR2 mutations is over 80%. Recent studies suggest that it is indicated to treat even completely symptom free carriers with beta blockers. As a consequence cascade genetic testing should be considered in conjunction with genetic counselling.4. Further Information 4.1 Flowchart CSANZ Guidelines for the diagnosis and management of Catecholaminergic Polymorphic Ventricular Tachycardia Page 5 Useful Websites for patients and family www.cidg.org (Cardiac Inherited Disease Group New Zealand) www.sads.org/sads-australia 4.2. References Priori SG, Napolitano C, Memmi M, Colombi B, Drago F, Gasparini M, DeSimone L, Coltorti F, Bloise R, Keegan R, Cruz Filho FE, Vignati G, Benatar A, DeLogu A: Clinical and molecular characterization of patients with catecholaminergic polymorphic ventricular tachycardia. Circulation 2002; 106:69-74 Leenhardt A, Lucet V, Denjoy I, Grau F, Ngoc DD, Coumel P: Catecholaminergic polymorphic ventricular tachycardia in children. A 7-year follow-up of 21 patients. Circulation 1995; 91:1512-1519 Fisher JD, Krikler D, Hallidie-Smith KA: 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 Liu N, Rizzi N, Boveri L, Priori SG: Ryanodine receptor and calsequestrin in arrhythmogenesis: what we have learnt from genetic diseases and transgenic mice. J Mol Cell Cardiol 2009; 46:149-159 Maron BJ, Chaitman BR, Ackerman MJ, Bayés de Luna A, Corrado D, Crosson JE, Deal BJ, Driscoll DJ, Estes NAM, Araújo CGS, Liang DH, Mitten MJ, Myerburg RJ, Pelliccia A, Thompson PD, Towbin JA, Van Camp SP, Unknown: Recommendations for physical activity and recreational sports participation for young patients with genetic cardiovascular diseases. Circulation 2004; 109:2807-16 Hayashi M, Denjoy I, Extramiana F, Maltret A, Buisson NR, Lupoglazoff J, Klug D, Hayashi M, Takatsuki S, Villain E, Kamblock J, Messali A, Guicheney P, Lunardi J, Leenhardt A: Incidence and risk factors of arrhythmic events in catecholaminergic polymorphic ventricular tachycardia. Circulation 2009; 119:2426-34 Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M, Gregoratos G, Klein G, Moss AJ, Myerburg RJ, Priori SG, Quinones MA, Roden DM, Silka MJ, Tracy C, Smith SC, Jacobs AK, Adams CD, Antman EM, Anderson JL, Hunt SA, Halperin JL, Nishimura R, Ornato JP, Page RL, Riegel B, Blanc J, Budaj A, Dean V, Deckers JW, Despres C, Dickstein K, Lekakis J, McGregor K, Metra M, Morais J, Osterspey A, Tamargo JL, Zamorano JL, Unknown: 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:e385-484 Rosso R, Kalman JM, Rogowski O, Diamant S, Birger A, Biner S, Belhassen B, Viskin S: Calcium channel blockers and beta-blockers versus beta-blockers alone for preventing exercise-induced arrhythmias in catecholaminergic polymorphic ventricular tachycardia. Heart Rhythm 2007; 4:1149-54 Swan H, Laitinen P, Kontula K, Toivonen L: Calcium channel antagonism reduces exercise-induced ventricular arrhythmias in catecholaminergic polymorphic ventricular tachycardia patients with RyR2 mutations. J Cardiovasc Electrophysiol 2005; 16:162-166 Watanabe H, Chopra N, Laver D, Hwang HS, Davies SS, Roach DE, Duff HJ, Roden DM, Wilde AA, Knollmann BC: Flecainide prevents catecholaminergic polymorphic ventricular tachycardia in mice and humans. Nat Med 2009; 15:380-383