Catecholaminergic polymorphic ventricular tachycardia
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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]
Synonyms and keywords: CPVT, bidirectional tachycardia induced by catecholamines, catecholamine-induced polymorphic ventricular tachycardia, familial polymorphic ventricular tachycardia, FPVT, polymorphic ventricular tachycardia.
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Catecholaminergic polymorphic ventricular tachycardia from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnostic study of choice | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Exercise Stress Testing | Genetic Testing | X-Ray Findings | Echocardiography and Ultrasound | CT-Scan Findings | MRI Findings | Other Imaging Findings | Other Diagnostic Studies
Treatment
Medical Therapy | Implantable Cardioverter-Defibrillator | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies
- The therapeutic approach to CPVT includes changes in lifestyle, medical therapy, left ventricular sympathetic denervation, and the use of implantable cardioverter-defibrillators.
Sympathectomy
- Left cardiac sympathetic denervation, where a portion of the sympathetic chain is surgically or endoscopically resected, and bilateral thoracoscopic sympathectomy have reported to be useful therapeutic methods for suppressing ventricular arrhythmias in CPVT patients.[1][2][3][4]
- Indications:[1][5][6]
- Patients who experience recurrent symptoms and/or implantable cardioverter-defibrillator (ICD) shocks despite optimal medical therapy
- Patients who are intolerant or have contraindications to beta blockers
- Limitations:
- Complexity of the surgical procedure
- Requirement of a specialised surgical centre
- Complications, such as:[7]
- In spite of the side-effects and complications, the procedure was safe and satisfactory among the vast majority of patients.
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.
Prevention
- Limit or avoid competitive sports.
- Limit or avoid strenuous exercises.
- Limit exposure to stressful environments.
- The limits for allowed physical activity can be set on the basis of exercise stress testing done in the hospital.
- Holter monitor can be helpful in keeping the heart-rate within a safe range during physical activity.
- Follow-up visits with a cardiologist every six to twelve months to monitor the efficacy of therapy.
ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death (DO NOT EDIT) [10]
Class I |
"1. Beta blockers are indicated for patients who are clinically diagnosed with CPVT on the basis of the presence of spontaneous or documented stress-induced ventricular arrhythmias. (Level of Evidence: C)" |
"2. Implantation of an ICD with use of beta blockers is indicated for patients with CPVT who are survivors of cardiac arrest and who have reasonable expectation of survival with a good functional status for more than 1 y. (Level of Evidence: C)" |
Class IIa |
"1. Beta blockers can be effective in patients without clinical manifestations when the diagnosis of CPVT is established during childhood based on genetic analysis. (Level of Evidence: C)" |
"2. Implantation of an ICD with the use of beta blockers can be effective for affected patients with CPVT with syncope and/or documented sustained VT while receiving beta blockers and who have reasonable expectation of survival with a good functional status for more than 1 y. (Level of Evidence: C)" |
Class IIb |
"1. Beta blockers may be considered for patients with CPVT who were genetically diagnosed in adulthood and never manifested clinical symptoms of tachyarrhythmias. (Level of Evidence: C)" |
References
- ↑ 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.
- ↑ 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.
- ↑ 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
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ignored (help) - ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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
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at position 179 (help) - ↑ Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M; et al. (2006). "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. 114 (10): e385–484. doi:10.1161/CIRCULATIONAHA.106.178233. PMID 16935995.