Catecholaminergic polymorphic ventricular tachycardia medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
There is no treatment for [disease name]; the mainstay of therapy is supportive care.
OR
Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].
OR
The majority of cases of [disease name] are self-limited and require only supportive care.
OR
[Disease name] is a medical emergency and requires prompt treatment.
OR
The mainstay of treatment for [disease name] is [therapy].
OR
The optimal therapy for [malignancy name] depends on the stage at diagnosis.
OR
[Therapy] is recommended among all patients who develop [disease name].
OR
Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
OR
Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
OR
Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
OR
Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
Medical Therapy
Pharmacologic medical therapies for CPVT include beta blockers, flecainide and verapamil.
Beta-blockers
- The first-line therapeutic option for patients with CPVT is exercise restriction combined with beta-blockers without intrinsic sympathomimetic activity.[1]
- Because of the adrenergic nature of arrhythmias in CPVT, non-selective beta-blockers, titrated at the maximum tolerated dose in the absence of contraindications (example, asthma) are considered the most effective pharmacological therapy.
- Indications:
- All patients with stress-induced ventricular arrhythmias.
- Silent carriers of a pathogenic mutation, even when they do not exhibit arrhythmias during exercise stress testing since cardiac arrest may occur in them.[2]
- Drugs used:
- Nadolol[3][4]
- Long-acting, non-selective beta-blocker.
- Preferred for prophylactic treatment of CPVT.
- It is considered the most clinically effective choice.
- Dosage: 1-2 mg/kg per day.
- Propranolol
- Long-acting, non-selective beta-blocker.
- It is also considered an effective medication when Nadolol is unavailable.
- Dosage: 3-5 mg/kg per day.
- Nadolol[3][4]
- Holter monitoring and exercise stress testing should be repeated periodically throughout beta blocker therapy, to ensure that the heart-rate is in control during exercise.
- Non-compliance and abrupt interruption of beta blockade may cause a rebound effect of catecholamines on the heart, leading to arrhythmic events while on therapy. Thus, it is important to educate and highlight to patients the need to be compliant with therapy.[5][6]
- Even with appropriate use, beta blockers cannot completely suppress the arrhythmias.
- Recurrent arrhythmias or persistence of complex arrhythmias at exercise stress test may occur in up to one-third of the CPVT patients, with the annual arrhythmic event rate ranging between 11% per year and 3% per year.[3][7][8]
Verampil
- Calcium channel blocker.
- Verapamil might be considered as adjunctive therapy for CPVT patients with ongoing ventricular arrhythmias despite therapy with beta blockers.[9][10]
- However, the long-term efficacy of verapamil is still controversial.
Flecainide
- Flecainide which is best known as a cardiac sodium channel blocker (a Class IC antiarrhythmic) is also found to inhibit the cardiac ryanodine receptor (RyR2. This dual-action makes it an effective medication for CPVT.[11]
- Indications:[11][12]
- Patients with persistent arrhythmias despite beta blocker therapy.
- Patients with an ICD who continue to have stress-induced ventricular arrhythmias despite beta-blocker therapy.
- Dosage: 100-300 mg/day (1.5-4.5 mg/kg/day).[6]
- Randomized clinical trials for the long-term efficacy of flecainide are still ongoing.[13]
Medical therapy
Medications to treat CPVT include beta blockers, flecainide and verapamil.
Beta-blockers
- The first-line therapeutic option for patients with CPVT is exercise restriction combined with beta-blockers without intrinsic sympathomimetic activity.[1]
- Because of the adrenergic nature of arrhythmias in CPVT, non-selective beta-blockers, titrated at the maximum tolerated dose in the absence of contraindications (example, asthma) are considered the most effective pharmacological therapy.
- Indications:
- All patients with stress-induced ventricular arrhythmias.
- Silent carriers of a pathogenic mutation, even when they do not exhibit arrhythmias during exercise stress testing since cardiac arrest may occur in them.[2]
- Drugs used:
- Nadolol[3][4]
- Long-acting, non-selective beta-blocker.
- Preferred for prophylactic treatment of CPVT.
- It is considered the most clinically effective choice.
- Dosage: 1-2 mg/kg per day.
- Propranolol
- Long-acting, non-selective beta-blocker.
- It is also considered an effective medication when Nadolol is unavailable.
- Dosage: 3-5 mg/kg per day.
- Nadolol[3][4]
- Holter monitoring and exercise stress testing should be repeated periodically throughout beta blocker therapy, to ensure that the heart-rate is in control during exercise.
- Non-compliance and abrupt interruption of beta blockade may cause a rebound effect of catecholamines on the heart, leading to arrhythmic events while on therapy. Thus, it is important to educate and highlight to patients the need to be compliant with therapy.[5][6]
- Even with appropriate use, beta blockers cannot completely suppress the arrhythmias.
- Recurrent arrhythmias or persistence of complex arrhythmias at exercise stress test may occur in up to one-third of the CPVT patients, with the annual arrhythmic event rate ranging between 11% per year and 3% per year.[3][7][8]
Verampil
- Calcium channel blocker.
- Verapamil might be considered as adjunctive therapy for CPVT patients with ongoing ventricular arrhythmias despite therapy with beta blockers.[9][10]
- However, the long-term efficacy of verapamil is still controversial.
Flecainide
- Flecainide which is best known as a cardiac sodium channel blocker (a Class IC antiarrhythmic) is also found to inhibit the cardiac ryanodine receptor (RyR2. This dual-action makes it an effective medication for CPVT.[11]
- Indications:[11][12]
- Patients with persistent arrhythmias despite beta blocker therapy.
- Patients with an ICD who continue to have stress-induced ventricular arrhythmias despite beta-blocker therapy.
- Dosage: 100-300 mg/day (1.5-4.5 mg/kg/day).[6]
- Randomized clinical trials for the long-term efficacy of flecainide are still ongoing.[14]
References
- ↑ 1.0 1.1 Sumitomo, Naokata (January 2003). "Catecholaminergic polymorphic ventricular tachycardia: electrocardiographic characteristics and optimal therapeutic strategies to prevent sudden death". Heart. 89 (1): 66–70. doi:10.1136/heart.89.1.66. PMC 1767500. PMID 12482795. Unknown parameter
|coauthors=ignored (help) - ↑ 2.0 2.1 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.
- ↑ 3.0 3.1 3.2 3.3 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.
- ↑ 4.0 4.1 Leren, Ida S.; Saberniak, Jørg; Majid, Eman; Haland, Trine F.; Edvardsen, Thor; Haugaa, Kristina H. (2016). "Nadolol decreases the incidence and severity of ventricular arrhythmias during exercise stress testing compared with β1-selective β-blockers in patients with catecholaminergic polymorphic ventricular tachycardia". Heart Rhythm. 13 (2): 433–440. doi:10.1016/j.hrthm.2015.09.029. ISSN 1547-5271.
- ↑ 5.0 5.1 Leenhardt, Antoine; Lucet, Vincent; Denjoy, Isabelle; Grau, Francis; Ngoc, Dien Do; Coumel, Philippe (1995). "Catecholaminergic Polymorphic Ventricular Tachycardia in Children". Circulation. 91 (5): 1512–1519. doi:10.1161/01.CIR.91.5.1512. ISSN 0009-7322.
- ↑ 6.0 6.1 6.2 6.3 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.
- ↑ 7.0 7.1 Cerrone, Marina; Colombi, Barbara; Santoro, Massimo; di Barletta, Marina Raffaele; Scelsi, Mario; Villani, Laura; Napolitano, Carlo; Priori, Silvia G (2005). "Bidirectional Ventricular Tachycardia and Fibrillation Elicited in a Knock-In Mouse Model Carrier of a Mutation in the Cardiac Ryanodine Receptor". Circulation Research. 96 (10). doi:10.1161/01.RES.0000169067.51055.72. ISSN 0009-7330.
- ↑ 8.0 8.1 Priori, Silvia G.; Napolitano, Carlo; Memmi, Mirella; Colombi, Barbara; Drago, Fabrizio; Gasparini, Maurizio; DeSimone, Luciano; Coltorti, Fernando; Bloise, Raffaella; Keegan, Roberto; Cruz Filho, Fernando E.S.; Vignati, Gabriele; Benatar, Abraham; DeLogu, Angelica (2002). "Clinical and Molecular Characterization of Patients With Catecholaminergic Polymorphic Ventricular Tachycardia". Circulation. 106 (1): 69–74. doi:10.1161/01.CIR.0000020013.73106.D8. ISSN 0009-7322.
- ↑ 9.0 9.1 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.
- ↑ 10.0 10.1 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.
- ↑ 11.0 11.1 11.2 11.3 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.
- ↑ 12.0 12.1 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.
- ↑ "Flecainide for Catecholaminergic Polymorphic Ventricular Tachycardia - Full Text View - ClinicalTrials.gov".
- ↑ "Flecainide for Catecholaminergic Polymorphic Ventricular Tachycardia - Full Text View - ClinicalTrials.gov".