Catecholaminergic polymorphic ventricular tachycardia: Difference between revisions

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==Diagnosis==
==Diagnosis==
[[Catecholaminergic polymorphic ventricular tachycardia diagnostic study of choice|Diagnostic study of choice]] | [[Catecholaminergic polymorphic ventricular tachycardia history and symptoms|History and Symptoms]] | [[Catecholaminergic polymorphic ventricular tachycardia physical examination|Physical Examination]] | [[Catecholaminergic polymorphic ventricular tachycardia laboratory findings|Laboratory Findings]] | [[Catecholaminergic polymorphic ventricular tachycardia electrocardiogram|Electrocardiogram]] | [[Catecholaminergic polymorphic ventricular tachycardia exercise stress testing|Exercise Stress Testing]] | [[Catecholaminergic polymorphic ventricular tachycardia genetic testing|Genetic Testing]] | [[Catecholaminergic polymorphic ventricular tachycardia x ray|X-Ray Findings]] | [[Catecholaminergic polymorphic ventricular tachycardia echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Catecholaminergic polymorphic ventricular tachycardia CT scan|CT-Scan Findings]] | [[Catecholaminergic polymorphic ventricular tachycardia MRI|MRI Findings]] | [[Catecholaminergic polymorphic ventricular tachycardia other imaging findings|Other Imaging Findings]] | [[Catecholaminergic polymorphic ventricular tachycardia other diagnostic studies|Other Diagnostic Studies]]
[[Catecholaminergic polymorphic ventricular tachycardia diagnostic study of choice|Diagnostic study of choice]] | [[Catecholaminergic polymorphic ventricular tachycardia history and symptoms|History and Symptoms]] | [[Catecholaminergic polymorphic ventricular tachycardia physical examination|Physical Examination]] | [[Catecholaminergic polymorphic ventricular tachycardia laboratory findings|Laboratory Findings]] | [[Catecholaminergic polymorphic ventricular tachycardia electrocardiogram|Electrocardiogram]] | [[Catecholaminergic polymorphic ventricular tachycardia exercise stress testing|Exercise Stress Testing]] | [[Catecholaminergic polymorphic ventricular tachycardia genetic testing|Genetic Testing]] | [[Catecholaminergic polymorphic ventricular tachycardia x ray|X-Ray Findings]] | [[Catecholaminergic polymorphic ventricular tachycardia echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Catecholaminergic polymorphic ventricular tachycardia CT scan|CT-Scan Findings]] | [[Catecholaminergic polymorphic ventricular tachycardia MRI|MRI Findings]] | [[Catecholaminergic polymorphic ventricular tachycardia other imaging findings|Other Imaging Findings]] | [[Catecholaminergic polymorphic ventricular tachycardia other diagnostic studies|Other Diagnostic Studies]]


==Treatment==
==Treatment==

Revision as of 14:20, 23 July 2020

Catecholaminergic polymorphic ventricular tachycardia Microchapters

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Overview

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Classification

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Differentiating Catecholaminergic polymorphic ventricular tachycardia from other Diseases

Epidemiology and Demographics

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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

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

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Medical Therapy

Implantable Cardioverter-Defibrillator

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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 | Interventions | 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.

Medical therapy

Medications to treat CPVT include beta blockers, flecainide and verapamil.

Beta-blockers

Verampil

Flecainide

Implantable cardioverter-defibrillator

Sympathectomy

Catheter ablation

  • The onset of CPVT may be initiated from purkinje cells and successful catheter ablation has been reported.[22]
  • 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.[23]
  • 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) [24]

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

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  2. Hayashi, Miyuki; Denjoy, Isabelle; Hayashi, Meiso; Extramiana, Fabrice; Maltret, Alice; Roux-Buisson, Nathalie; Lupoglazoff, Jean-Marc; Klug, Didier; Maury, Philippe; Messali, Anne; Guicheney, Pascale; Leenhardt, Antoine (2012). "The role of stress test for predicting genetic mutations and future cardiac events in asymptomatic relatives of catecholaminergic polymorphic ventricular tachycardia probands". EP Europace. 14 (9): 1344–1351. doi:10.1093/europace/eus031. ISSN 1532-2092.
  3. 3.0 3.1 Hayashi, Meiso; Denjoy, Isabelle; Extramiana, Fabrice; Maltret, Alice; Buisson, Nathalie Roux; Lupoglazoff, Jean-Marc; Klug, Didier; Hayashi, Miyuki; Takatsuki, Seiji; Villain, Elisabeth; Kamblock, Joël; Messali, Anne; Guicheney, Pascale; Lunardi, Joël; Leenhardt, Antoine (2009). "Incidence and Risk Factors of Arrhythmic Events in Catecholaminergic Polymorphic Ventricular Tachycardia". Circulation. 119 (18): 2426–2434. doi:10.1161/CIRCULATIONAHA.108.829267. ISSN 0009-7322.
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  9. Swan, Heikki; Laitinen, Paivi; Kontula, Kimmo; Toivonen, Lauri (2005). "Calcium Channel Antagonism Reduces Exercise-Induced Ventricular Arrhythmias in Catecholaminergic Polymorphic Ventricular Tachycardia Patients with RyR2 Mutations". Journal of Cardiovascular Electrophysiology. 16 (2): 162–166. doi:10.1046/j.1540-8167.2005.40516.x. ISSN 1045-3873.
  10. Rosso, Rafael; Kalman, Jonathan M.; Rogowski, Ori; Diamant, Shmuel; Birger, Amir; Biner, Simon; Belhassen, Bernard; Viskin, Sami (2007). "Calcium channel blockers and beta-blockers versus beta-blockers alone for preventing exercise-induced arrhythmias in catecholaminergic polymorphic ventricular tachycardia". Heart Rhythm. 4 (9): 1149–1154. doi:10.1016/j.hrthm.2007.05.017. ISSN 1547-5271.
  11. 11.0 11.1 Watanabe, Hiroshi; Chopra, Nagesh; Laver, Derek; Hwang, Hyun Seok; Davies, Sean S; Roach, Daniel E; Duff, Henry J; Roden, Dan M; Wilde, Arthur A M; Knollmann, Björn C (2009). "Flecainide prevents catecholaminergic polymorphic ventricular tachycardia in mice and humans". Nature Medicine. 15 (4): 380–383. doi:10.1038/nm.1942. ISSN 1078-8956.
  12. van der Werf, Christian; Kannankeril, Prince J.; Sacher, Frederic; Krahn, Andrew D.; Viskin, Sami; Leenhardt, Antoine; Shimizu, Wataru; Sumitomo, Naokata; Fish, Frank A.; Bhuiyan, Zahurul A.; Willems, Albert R.; van der Veen, Maurits J.; Watanabe, Hiroshi; Laborderie, Julien; Haïssaguerre, Michel; Knollmann, Björn C.; Wilde, Arthur A.M. (2011). "Flecainide Therapy Reduces Exercise-Induced Ventricular Arrhythmias in Patients With Catecholaminergic Polymorphic Ventricular Tachycardia". Journal of the American College of Cardiology. 57 (22): 2244–2254. doi:10.1016/j.jacc.2011.01.026. ISSN 0735-1097.
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  14. Roston, Thomas M.; Jones, Karolina; Hawkins, Nathaniel M.; Bos, J. Martijn; Schwartz, Peter J.; Perry, Frances; Ackerman, Michael J.; Laksman, Zachary W.M.; Kaul, Padma; Lieve, Krystien V.V.; Atallah, Joseph; Krahn, Andrew D.; Sanatani, Shubhayan (2018). "Implantable cardioverter-defibrillator use in catecholaminergic polymorphic ventricular tachycardia: A systematic review". Heart Rhythm. 15 (12): 1791–1799. doi:10.1016/j.hrthm.2018.06.046. ISSN 1547-5271.
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  16. 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.
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  18. 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.
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Electrocardiography