Catecholaminergic polymorphic ventricular tachycardia surgery

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Catecholaminergic polymorphic ventricular tachycardia Microchapters


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Differentiating Catecholaminergic polymorphic ventricular tachycardia from other Diseases

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mounika Reddy Vadiyala, M.B.B.S.[2]


Surgery is not the first-line treatment option for patients with catecholaminergic polymorphic ventricular tachycardia. Sympathectomy is usually reserved for patients who experience recurrent symptoms and/or implantable cardioverter-defibrillator (ICD) shocks despite optimal medical therapy or in those who are intolerant or have contraindications to beta blockers



Catheter ablation

  • The onset of CPVT may be initiated from purkinje cells and successful catheter ablation has been reported.[8]
  • Catheter ablation of the bidirectional VPCs that trigger VF has been reported and this procedure could become an adjunctive therapy in patients with refractory CPVT.[9]
  • Further evidence and experiences are required for its recommendation.

2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death (DO NOT EDIT)[10]

Class I
"1.In patients with catecholaminergic polymorphic ventricular tachycardia, a beta blocker is recommended.(Level of Evidence: B)"
"2.In patients with catecholaminergic polymorphic ventricular tachycardia and recurrent sustained VT or syncope, while receiving adequate or maximally tolerated beta blocker, treatment intensification with either combination medication therapy (e.g., beta blocker, flecainide), left cardiac sympathetic denervation, and/or an ICD is recommended (Level of Evidence: B)"
Class IIa
"1.In patients with catecholaminergic polymorphic ventricular tachycardia and with clinical VT or exceptional syncope, genetic counseling and genetic testing are reasonable (Level of Evidence: B)"


  1. 1.0 1.1 Priori, Silvia G.; Blomström-Lundqvist, Carina; Mazzanti, Andrea; Blom, Nico; Borggrefe, Martin; Camm, John; Elliott, Perry Mark; Fitzsimons, Donna; Hatala, Robert; Hindricks, Gerhard; Kirchhof, Paulus; Kjeldsen, Keld; Kuck, Karl-Heinz; Hernandez-Madrid, Antonio; Nikolaou, Nikolaos; Norekvål, Tone M.; Spaulding, Christian; Van Veldhuisen, Dirk J. (2015). "2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death". European Heart Journal. 36 (41): 2793–2867. doi:10.1093/eurheartj/ehv316. ISSN 0195-668X.
  2. Schneider, Heike E.; Steinmetz, Michael; Krause, Ulrich; Kriebel, Thomas; Ruschewski, Wolfgang; Paul, Thomas (2012). "Left cardiac sympathetic denervation for the management of life-threatening ventricular tachyarrhythmias in young patients with catecholaminergic polymorphic ventricular tachycardia and long QT syndrome". Clinical Research in Cardiology. 102 (1): 33–42. doi:10.1007/s00392-012-0492-7. ISSN 1861-0684.
  3. Scott, P.A. (October 2008). "Successful treatment of catecholaminergic polymorphic ventricular tachycardia with bilateral thoracoscopic sympathectomy". Heart Rhythm. 5 (10): 1461–1463. doi:10.1016/j.hrthm.2008.07.007. PMID 18760972. Unknown parameter |coauthors= ignored (help)
  4. Collura, Christopher A.; Johnson, Jonathan N.; Moir, Christopher; Ackerman, Michael J. (2009). "Left cardiac sympathetic denervation for the treatment of long QT syndrome and catecholaminergic polymorphic ventricular tachycardia using video-assisted thoracic surgery". Heart Rhythm. 6 (6): 752–759. doi:10.1016/j.hrthm.2009.03.024. ISSN 1547-5271.
  5. De Ferrari, Gaetano M.; Dusi, Veronica; Spazzolini, Carla; Bos, J. Martijn; Abrams, Dominic J.; Berul, Charles I.; Crotti, Lia; Davis, Andrew M.; Eldar, Michael; Kharlap, Maria; Khoury, Asaad; Krahn, Andrew D.; Leenhardt, Antoine; Moir, Christopher R.; Odero, Attilio; Olde Nordkamp, Louise; Paul, Thomas; Rosés i Noguer, Ferran; Shkolnikova, Maria; Till, Jan; Wilde, Arthur A.M.; Ackerman, Michael J.; Schwartz, Peter J. (2015). "Clinical Management of Catecholaminergic Polymorphic Ventricular Tachycardia". Circulation. 131 (25): 2185–2193. doi:10.1161/CIRCULATIONAHA.115.015731. ISSN 0009-7322.
  6. Wilde, Arthur A.M.; Bhuiyan, Zahurul A.; Crotti, Lia; Facchini, Mario; De Ferrari, Gaetano M.; Paul, Thomas; Ferrandi, Chiara; Koolbergen, Dave R.; Odero, Attilio; Schwartz, Peter J. (2008). "Left Cardiac Sympathetic Denervation for Catecholaminergic Polymorphic Ventricular Tachycardia". New England Journal of Medicine. 358 (19): 2024–2029. doi:10.1056/NEJMoa0708006. ISSN 0028-4793.
  7. Waddell-Smith, Kathryn E.; Ertresvaag, Kjetil N.; Li, Jian; Chaudhuri, Krish; Crawford, Jackie R.; Hamill, James K.; Haydock, David; Skinner, Jonathan R. (2015). "Physical and Psychological Consequences of Left Cardiac Sympathetic Denervation in Long-QT Syndrome and Catecholaminergic Polymorphic Ventricular Tachycardia". Circulation: Arrhythmia and Electrophysiology. 8 (5): 1151–1158. doi:10.1161/CIRCEP.115.003159. ISSN 1941-3149.
  8. Priori, Silvia G.; Wilde, Arthur A.; Horie, Minoru; Cho, Yongkeun; Behr, Elijah R.; Berul, Charles; Blom, Nico; Brugada, Josep; Chiang, Chern-En; Huikuri, Heikki; Kannankeril, Prince; Krahn, Andrew; Leenhardt, Antoine; Moss, Arthur; Schwartz, Peter J.; Shimizu, Wataru; Tomaselli, Gordon; Tracy, Cynthia; Ackerman, Michael; Belhassen, Bernard; Estes, N. A. Mark; Fatkin, Diane; Kalman, Jonathan; Kaufman, Elizabeth; Kirchhof, Paulus; Schulze-Bahr, Eric; Wolpert, Christian; Vohra, Jitendra; Refaat, Marwan; Etheridge, Susan P.; Campbell, Robert M.; Martin, Edward T.; Quek, Swee Chye (2013). "Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes". EP Europace. 15 (10): 1389–1406. doi:10.1093/europace/eut272. ISSN 1532-2092.
  9. Kaneshiro, Takashi; Naruse, Yoshihisa; Nogami, Akihiko; Tada, Hiroshi; Yoshida, Kentaro; Sekiguchi, Yukio; Murakoshi, Nobuyuki; Kato, Yoshiaki; Horigome, Hitoshi; Kawamura, Mihoko; Horie, Minoru; Aonuma, Kazutaka (2012). "Successful Catheter Ablation of Bidirectional Ventricular Premature Contractions Triggering Ventricular Fibrillation in Catecholaminergic Polymorphic Ventricular Tachycardia With RyR2 Mutation". Circulation: Arrhythmia and Electrophysiology. 5 (1). doi:10.1161/CIRCEP.111.966549. ISSN 1941-3149. line feed character in |title= at position 179 (help)
  10. Al-Khatib, Sana M.; Stevenson, William G.; Ackerman, Michael J.; Bryant, William J.; Callans, David J.; Curtis, Anne B.; Deal, Barbara J.; Dickfeld, Timm; Field, Michael E.; Fonarow, Gregg C.; Gillis, Anne M.; Granger, Christopher B.; Hammill, Stephen C.; Hlatky, Mark A.; Joglar, José A.; Kay, G. Neal; Matlock, Daniel D.; Myerburg, Robert J.; Page, Richard L. (2018). "2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: Executive Summary". Journal of the American College of Cardiology. 72 (14): 1677–1749. doi:10.1016/j.jacc.2017.10.053. ISSN 0735-1097.

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