Catecholaminergic polymorphic ventricular tachycardia: Difference between revisions

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__NOTOC__
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{{Catecholaminergic polymorphic ventricular tachycardia}}
{{Catecholaminergic polymorphic ventricular tachycardia}}
'''For patient information, click [[Catecholaminergic polymorphic ventricular tachycardia(patient information)|here]].'''<br>
{{CMG}}; {{AE}} {{MRV}}
{{CMG}}; {{AE}} {{MRV}}


{{SK}} CPVT, bidirectional tachycardia induced by catecholamines, catecholamine-induced polymorphic ventricular tachycardia, familial polymorphic ventricular tachycardia, FPVT, polymorphic ventricular tachycardia.
{{SK}} CPVT, catecholaminergic polymorphic VT, bidirectional ventricular tachycardia induced by catecholamines, bidirectional VT, catecholamine-induced polymorphic ventricular tachycardia, catecholamine induced polymorphic ventricular tachycardia, familial polymorphic ventricular tachycardia, FPVT, polymorphic ventricular tachycardia, polymorphic VT induced by catecholamines.  


==[[Catecholaminergic polymorphic ventricular tachycardia overview|Overview]]==
==[[Catecholaminergic polymorphic ventricular tachycardia overview|Overview]]==
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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 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]]
 
 
===Exercise Stress Testing===
*[[CPVT]] is a diagnosis based on reproducing [[ventricular arrhythmias]] during [[exercise stress testing]], [[syncope]] occurring during physical activity and acute emotion, and a history of exercise or emotion-related [[palpitations]] and [[dizziness]] with an absence of structural [[cardiac]] abnormalities.
*It has been observed that [[arrhythmias]] in [[CPVT]] often appear in a uniform and reproducible pattern that facilitates the recognition of affected patients.<ref name="LeenhardtLucet1995">{{cite journal|last1=Leenhardt|first1=Antoine|last2=Lucet|first2=Vincent|last3=Denjoy|first3=Isabelle|last4=Grau|first4=Francis|last5=Ngoc|first5=Dien Do|last6=Coumel|first6=Philippe|title=Catecholaminergic Polymorphic Ventricular Tachycardia in Children|journal=Circulation|volume=91|issue=5|year=1995|pages=1512–1519|issn=0009-7322|doi=10.1161/01.CIR.91.5.1512}}</ref>
*[[Exercise Stress Testing]] is the primary [[diagnostic]] test and the most helpful clinical tool in diagnosing [[CPVT]] as it can reproducibly evoke the typical [[ventricular tachycardia]] during acute [[adrenergic]] activation (e.g., exercise, acute emotion).
*It may also be useful in monitoring the response to [[beta-blocker]] therapy of affected individuals in reproducible conditions.
*During [[Exercise Stress Testing|exercise testing]], [[sinus rhythm]] accelerates and beyond a [[heart rate]] of 120-130 [[beats per minute]], isolated and often monomorphic [[Premature ventricular contraction|ventricular premature beats]] ([[Premature ventricular contraction|VPBs]]) typically occur first and then increase with [[heart rate]] to [[quadrigeminy]], [[trigeminy]], and [[Bigeminal rhythm|bigeminy]].
*Subsequently, the [[Premature ventricular contraction|VPBs]] become polymorphic, and as the exercise increase, they form bursts of non-sustained [[polymorphic ventricular tachycardia]] ([[VT]]).
*If the activity is stopped, the [[arrhythmia]] disappears in the reverse order without clinical symptoms.
*However, when the activity is continued, the [[arrhythmia]] persists and becomes more rapid, eventually assuming the appearance of [[polymorphic ventricular tachycardia]] ([[VT]]), which is very fast, [[fibrillation]]-like and leads to [[syncope]].
* Of note, in a subset of patients the [[ventricular arrhythmias]] already disappear with ongoing exercise.<ref name="FaggioniHwang2013">{{cite journal|last1=Faggioni|first1=Michela|last2=Hwang|first2=Hyun Seok|last3=van der Werf|first3=Christian|last4=Nederend|first4=Ineke|last5=Kannankeril|first5=Prince J.|last6=Wilde|first6=Arthur A.M.|last7=Knollmann|first7=Björn C.|title=Accelerated Sinus Rhythm Prevents Catecholaminergic Polymorphic Ventricular Tachycardia in Mice and in Patients|journal=Circulation Research|volume=112|issue=4|year=2013|pages=689–697|issn=0009-7330|doi=10.1161/CIRCRESAHA.111.300076}}</ref>
* Another type of [[Polymorphic ventricular tachycardia|polymorphic VT]] observed in [[CPVT]] patients is the bidirectional [[VT]], which is a peculiar form of [[polymorphic ventricular tachycardia|polymorphic VT]] characterized by 180° rotation of the [[QRS]] complex from beat to beat
*The occurrence of a bidirectional [[ventricular tachycardia]] ([[VT]]), which is the hallmark sign of [[CPVT]] is highly [[specificty|specific]] but not present in all patients.
*The bidirectional [[VT]] seen in [[CPVT]] are thought to originate from the [[His-Purkinje system]] from the alternating activation of the [[purkinje fibers]] of the two [[ventricles]].<ref name="CerroneNoujaim2007">{{cite journal|last1=Cerrone|first1=Marina|last2=Noujaim|first2=Sami F.|last3=Tolkacheva|first3=Elena G.|last4=Talkachou|first4=Arkadzi|last5=O’Connell|first5=Ryan|last6=Berenfeld|first6=Omer|last7=Anumonwo|first7=Justus|last8=Pandit|first8=Sandeep V.|last9=Vikstrom|first9=Karen|last10=Napolitano|first10=Carlo|last11=Priori|first11=Silvia G.|last12=Jalife|first12=José|title=Arrhythmogenic Mechanisms in a Mouse Model of Catecholaminergic Polymorphic Ventricular Tachycardia|journal=Circulation Research|volume=101|issue=10|year=2007|pages=1039–1048|issn=0009-7330|doi=10.1161/CIRCRESAHA.107.148064}}</ref><ref name="HerronMilstein2010">{{cite journal|last1=Herron|first1=Todd J.|last2=Milstein|first2=Michelle L.|last3=Anumonwo|first3=Justus|last4=Priori|first4=Silvia G.|last5=Jalife|first5=José|title=Purkinje cell calcium dysregulation is the cellular mechanism that underlies catecholaminergic polymorphic ventricular tachycardia|journal=Heart Rhythm|volume=7|issue=8|year=2010|pages=1122–1128|issn=15475271|doi=10.1016/j.hrthm.2010.06.010}}</ref><ref name="CerroneColombi2005">{{cite journal|last1=Cerrone|first1=Marina|last2=Colombi|first2=Barbara|last3=Santoro|first3=Massimo|last4=di Barletta|first4=Marina Raffaele|last5=Scelsi|first5=Mario|last6=Villani|first6=Laura|last7=Napolitano|first7=Carlo|last8=Priori|first8=Silvia G|title=Bidirectional Ventricular Tachycardia and Fibrillation Elicited in a Knock-In Mouse Model Carrier of a Mutation in the Cardiac Ryanodine Receptor|journal=Circulation Research|volume=96|issue=10|year=2005|issn=0009-7330|doi=10.1161/01.RES.0000169067.51055.72}}</ref>
 
<br>{{familytree/start}}
{{familytree | | | | | | | | | A01 | | | | | |A01=[[Exercise stress testing]]}}
{{familytree | | | | | | | | | |!| | | | | | | | }}
{{familytree | | | | | | | | | B01 | | | | | |B01=Increase in [[sinus rhythm]]}}
{{familytree | | | | | | | | | |!| | | | | | | | }}
{{familytree | | | | | | | | | C01 | | | | | |C01=Monomorphic [[premature ventricular contractions]] ([[PVC]]s)}}
{{familytree | | |,|-|-|-|-|-|-|^|-|-|-|-|-|-|.| }}
{{familytree | | D01 | | | | | | | | | | | |D02|D01=Polymorphic [[Premature ventricular contraction|PVC]] [[Bigeminy]]|D02=Bidirectional [[Premature ventricular contraction|PVC]] [[Bigeminy]]}}
{{familytree | | |!| | | | | | | | | | | | | |!| }}
{{familytree | | E01 | | | | | | | | | | | |E02|E01=Polymorphic [[VT]]|E02=Bidirectional [[VT]]}}
{{familytree/end}}
<br>
 
===Epinephrine Infusion===
*[[Epinephrine]] infusion is an alternative to establish the diagnosis [[CPVT]] in patients who cannot perform an [[Exercise stress testing|exercise stress test]].<ref name="PrioriWilde2013">{{cite journal|last1=Priori|first1=Silvia G.|last2=Wilde|first2=Arthur A.|last3=Horie|first3=Minoru|last4=Cho|first4=Yongkeun|last5=Behr|first5=Elijah R.|last6=Berul|first6=Charles|last7=Blom|first7=Nico|last8=Brugada|first8=Josep|last9=Chiang|first9=Chern-En|last10=Huikuri|first10=Heikki|last11=Kannankeril|first11=Prince|last12=Krahn|first12=Andrew|last13=Leenhardt|first13=Antoine|last14=Moss|first14=Arthur|last15=Schwartz|first15=Peter J.|last16=Shimizu|first16=Wataru|last17=Tomaselli|first17=Gordon|last18=Tracy|first18=Cynthia|last19=Ackerman|first19=Michael|last20=Belhassen|first20=Bernard|last21=Estes|first21=N. A. Mark|last22=Fatkin|first22=Diane|last23=Kalman|first23=Jonathan|last24=Kaufman|first24=Elizabeth|last25=Kirchhof|first25=Paulus|last26=Schulze-Bahr|first26=Eric|last27=Wolpert|first27=Christian|last28=Vohra|first28=Jitendra|last29=Refaat|first29=Marwan|last30=Etheridge|first30=Susan P.|last31=Campbell|first31=Robert M.|last32=Martin|first32=Edward T.|last33=Quek|first33=Swee Chye|title=Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes|journal=EP Europace|volume=15|issue=10|year=2013|pages=1389–1406|issn=1532-2092|doi=10.1093/europace/eut272}}</ref>
*In a study of 36 [[CPVT]] patients and 45 unaffected relatives, reported doses of [[epinephrine]] escalated from 0.05 mcg/kg/min to 0.1 mcg/kg/min to a maximum of 0.20 mcg/kg/min; and the  mean maximum [[heart rate]] was significantly lower than the maximum heart rate achieved during [[Exercise stress testing|exercise testing]].<ref name="MarjamaaHiippala2012">{{cite journal|last1=Marjamaa|first1=Annukka|last2=Hiippala|first2=Anita|last3=Arrhenius|first3=Bianca|last4=Lahtinen|first4=Annukka M.|last5=Kontula|first5=Kimmo|last6=Toivonen|first6=Lauri|last7=Happonen|first7=Juha-Matti|last8=Swan|first8=Heikki|title=Intravenous Epinephrine Infusion Test in Diagnosis of Catecholaminergic Polymorphic Ventricular Tachycardia|journal=Journal of Cardiovascular Electrophysiology|volume=23|issue=2|year=2012|pages=194–199|issn=10453873|doi=10.1111/j.1540-8167.2011.02188.x}}</ref>
*[[Epinephrine]] test appears to be highly [[specificity|specific]] (98%), but not as [[sensitivity|sensitive]] as the [[Exercise stress testing|exercise test]] for provoking [[arrhythmia]] in [[CPVT]] patients.<ref name="MarjamaaHiippala2012">{{cite journal|last1=Marjamaa|first1=Annukka|last2=Hiippala|first2=Anita|last3=Arrhenius|first3=Bianca|last4=Lahtinen|first4=Annukka M.|last5=Kontula|first5=Kimmo|last6=Toivonen|first6=Lauri|last7=Happonen|first7=Juha-Matti|last8=Swan|first8=Heikki|title=Intravenous Epinephrine Infusion Test in Diagnosis of Catecholaminergic Polymorphic Ventricular Tachycardia|journal=Journal of Cardiovascular Electrophysiology|volume=23|issue=2|year=2012|pages=194–199|issn=10453873|doi=10.1111/j.1540-8167.2011.02188.x}}</ref>
*Patients undergoing an [[epinephrine]] infusion should have continuous [[ECG]] monitoring.
 
===Holter monitoring===
*[[Exercise stress testing]] and [[epinephrine]] infusion should be complemented by 24-hours [[Holter monitor|Holter]] recordings.
*In individuals unable to perform an [[Exercise stress testing|exercise test]], especially infants and children or patients whose symptoms are more emotion-related rather than exercise-related, [[Holter monitor|Holter monitoring]] can be performed.
*[[Holter monitor|Holter monitoring]] is also useful to detect the presence of [[supraventricular arrhythmias]].
*[[Holter monitor|Holter monitoring]] is less [[sensitivity|sensitive]] than [[Exercise stress testing|exercise testing]].<ref name="SyGollob2011">{{cite journal|last1=Sy|first1=Raymond W.|last2=Gollob|first2=Michael H.|last3=Klein|first3=George J.|last4=Yee|first4=Raymond|last5=Skanes|first5=Allan C.|last6=Gula|first6=Lorne J.|last7=Leong-Sit|first7=Peter|last8=Gow|first8=Robert M.|last9=Green|first9=Martin S.|last10=Birnie|first10=David H.|last11=Krahn|first11=Andrew D.|title=Arrhythmia characterization and long-term outcomes in catecholaminergic polymorphic ventricular tachycardia|journal=Heart Rhythm|volume=8|issue=6|year=2011|pages=864–871|issn=15475271|doi=10.1016/j.hrthm.2011.01.048}}</ref>
 
===Imaging===
*There are no [[echocardiography|echocardiographic]], [[CT]] and [[MRI]] findings associated with [[CPVT]].
*However, [[cardiac imaging]] including [[MRI]] or [[CT]] helps in excluding structural abnormalities such as [[hypertrophic cardiomyopathy]], [[Coronary heart disease|coronary artery diseases]], and [[arrhythmogenic right ventricular dysplasia]], that may present similar to [[CPVT]].
 
===Genetic testing===
 
*[[Genetic testing]] helps in the confirmation of the diagnosis of [[CPVT]].
*[[Genetic screening]] allows the identification of [[mutations]] in up to 65% of patients with a clinical diagnosis of [[CPVT]].
*Identification of [[heterozygous]] pathogenic variants in [[Ryanodine receptor 2|RYR2]] or [[Calmouldin|CALM1]] or of [[allele|biallelic]] pathogenic variants in [[Calsequestrin|CASQ2]] or [[Triadin|TRDN]] can also establish the diagnosis of [[CPVT]].
*Recommendations for [[genetic testing]] are:<ref name="AckermanPriori2011">{{cite journal|last1=Ackerman|first1=M. J.|last2=Priori|first2=S. G.|last3=Willems|first3=S.|last4=Berul|first4=C.|last5=Brugada|first5=R.|last6=Calkins|first6=H.|last7=Camm|first7=A. J.|last8=Ellinor|first8=P. T.|last9=Gollob|first9=M.|last10=Hamilton|first10=R.|last11=Hershberger|first11=R. E.|last12=Judge|first12=D. P.|last13=Le Marec|first13=H.|last14=McKenna|first14=W. J.|last15=Schulze-Bahr|first15=E.|last16=Semsarian|first16=C.|last17=Towbin|first17=J. A.|last18=Watkins|first18=H.|last19=Wilde|first19=A.|last20=Wolpert|first20=C.|last21=Zipes|first21=D. P.|title=HRS/EHRA Expert Consensus Statement on the State of Genetic Testing for the Channelopathies and Cardiomyopathies: This document was developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA)|journal=Europace|volume=13|issue=8|year=2011|pages=1077–1109|issn=1099-5129|doi=10.1093/europace/eur245}}</ref><ref name="HofmanTan2013">{{cite journal|last1=Hofman|first1=Nynke|last2=Tan|first2=Hanno L.|last3=Alders|first3=Mariëlle|last4=Kolder|first4=Iris|last5=de Haij|first5=Simone|last6=Mannens|first6=Marcel M.A.M.|last7=Lombardi|first7=Maria Paola|last8=Lekanne dit Deprez|first8=Ronald H.|last9=van Langen|first9=Irene|last10=Wilde|first10=Arthur A.M.|title=Yield of Molecular and Clinical Testing for Arrhythmia Syndromes|journal=Circulation|volume=128|issue=14|year=2013|pages=1513–1521|issn=0009-7322|doi=10.1161/CIRCULATIONAHA.112.000091}}</ref>
*#Comprehensive or [[CPVT]]1 and [[CVPT]]2 ([[Ryanodine receptor 2|RYR2]] and [[Calsequestrin|CASQ2]]) targeted [[CPVT]] [[genetic testing]] is recommended for any patient in whom a clinical index of suspicion for [[CPVT]] has been established based on examination of the patient's clinical history, family history, and expressed [[electrocardiography|electrocardiographic]] [[phenotype]] during [[exercise stress testing]] or [[catecholamine]] infusion.
*#[[Mutation]]-specific [[genetic testing]] is recommended for family members and appropriate relatives following the identification of the [[CPVT]]-causative [[mutation]] in an [[index case]]. Those family members with identified [[mutations]] should be treated even in the absence of a positive [[exercise stress test]].<ref name="PrioriBlomström-Lundqvist2015">{{cite journal|last1=Priori|first1=Silvia G.|last2=Blomström-Lundqvist|first2=Carina|last3=Mazzanti|first3=Andrea|last4=Blom|first4=Nico|last5=Borggrefe|first5=Martin|last6=Camm|first6=John|last7=Elliott|first7=Perry Mark|last8=Fitzsimons|first8=Donna|last9=Hatala|first9=Robert|last10=Hindricks|first10=Gerhard|last11=Kirchhof|first11=Paulus|last12=Kjeldsen|first12=Keld|last13=Kuck|first13=Karl-Heinz|last14=Hernandez-Madrid|first14=Antonio|last15=Nikolaou|first15=Nikolaos|last16=Norekvål|first16=Tone M.|last17=Spaulding|first17=Christian|last18=Van Veldhuisen|first18=Dirk J.|title=2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death|journal=European Heart Journal|volume=36|issue=41|year=2015|pages=2793–2867|issn=0195-668X|doi=10.1093/eurheartj/ehv316}}</ref>


==Treatment==
==Treatment==
*The therapeutic approach to CPVT includes changes in lifestyle, medical therapy, left ventricular sympathetic denervation, and the use of implantable cardioverter-defibrillators.
[[Catecholaminergic polymorphic ventricular tachycardia medical therapy|Medical Therapy]] | [[Catecholaminergic polymorphic ventricular tachycardia implantable cardioverter-defibrillator|Implantable Cardioverter-Defibrillator]] | [[Catecholaminergic polymorphic ventricular tachycardia surgery|Surgery]] | [[Catecholaminergic polymorphic ventricular tachycardia primary prevention|Primary Prevention]] | [[Catecholaminergic polymorphic ventricular tachycardia secondary prevention|Secondary Prevention]] | [[Catecholaminergic polymorphic ventricular tachycardia cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Catecholaminergic polymorphic ventricular tachycardia future or investigational therapies|Future or Investigational Therapies]]
 
===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 [[beta-blockers|intrinsic sympathomimetic activity]].<ref name="nihon">{{cite journal|title=Catecholaminergic polymorphic ventricular tachycardia: electrocardiographic characteristics and optimal therapeutic strategies to prevent sudden death|journal=Heart|date=January 2003|first=Naokata|last=Sumitomo|coauthors=Harada K, Nagashima M, Yasuda T, Nakamura Y, Aragaki Y, Saito A, Kurosaki K, Jouo K, Koujiro M, Konishi S, Matsuoka S, Oono T, Hayakawa S, Miura M, Ushinohama H, Shibata T, Niimura I|volume=89|issue=1|pages=66–70|pmid=12482795 |format=|doi=10.1136/heart.89.1.66|pmc=1767500 }}</ref>
*Because of the [[adrenergic]] nature of [[arrhythmias]] in [[CPVT]], [[beta-blockers|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]].
**[[carrier|Silent carriers]] of a pathogenic [[mutation]], even when they do not exhibit [[arrhythmias]] during [[exercise stress testing]] since [[cardiac arrest]] may occur in them.<ref name="HayashiDenjoy2012">{{cite journal|last1=Hayashi|first1=Miyuki|last2=Denjoy|first2=Isabelle|last3=Hayashi|first3=Meiso|last4=Extramiana|first4=Fabrice|last5=Maltret|first5=Alice|last6=Roux-Buisson|first6=Nathalie|last7=Lupoglazoff|first7=Jean-Marc|last8=Klug|first8=Didier|last9=Maury|first9=Philippe|last10=Messali|first10=Anne|last11=Guicheney|first11=Pascale|last12=Leenhardt|first12=Antoine|title=The role of stress test for predicting genetic mutations and future cardiac events in asymptomatic relatives of catecholaminergic polymorphic ventricular tachycardia probands|journal=EP Europace|volume=14|issue=9|year=2012|pages=1344–1351|issn=1532-2092|doi=10.1093/europace/eus031}}</ref>
*Drugs used:
**[[Nadolol]]<ref name="HayashiDenjoy2009">{{cite journal|last1=Hayashi|first1=Meiso|last2=Denjoy|first2=Isabelle|last3=Extramiana|first3=Fabrice|last4=Maltret|first4=Alice|last5=Buisson|first5=Nathalie Roux|last6=Lupoglazoff|first6=Jean-Marc|last7=Klug|first7=Didier|last8=Hayashi|first8=Miyuki|last9=Takatsuki|first9=Seiji|last10=Villain|first10=Elisabeth|last11=Kamblock|first11=Joël|last12=Messali|first12=Anne|last13=Guicheney|first13=Pascale|last14=Lunardi|first14=Joël|last15=Leenhardt|first15=Antoine|title=Incidence and Risk Factors of Arrhythmic Events in Catecholaminergic Polymorphic Ventricular Tachycardia|journal=Circulation|volume=119|issue=18|year=2009|pages=2426–2434|issn=0009-7322|doi=10.1161/CIRCULATIONAHA.108.829267}}</ref><ref name="LerenSaberniak2016">{{cite journal|last1=Leren|first1=Ida S.|last2=Saberniak|first2=Jørg|last3=Majid|first3=Eman|last4=Haland|first4=Trine F.|last5=Edvardsen|first5=Thor|last6=Haugaa|first6=Kristina H.|title=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|journal=Heart Rhythm|volume=13|issue=2|year=2016|pages=433–440|issn=15475271|doi=10.1016/j.hrthm.2015.09.029}}</ref>
***Long-acting, [[beta-blockers|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, [[beta-blockers|non-selective beta-blocker]].
***It is also considered an effective medication when [[Nadolol]] is unavailable.
***Dosage: 3-5 mg/kg per day.
*[[Holter monitor|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 [[arrhythmias|arrhythmic events]] while on therapy. Thus, it is important to educate and highlight to patients the need to be [[compliance|compliant]] with therapy.<ref name="LeenhardtLucet1995">{{cite journal|last1=Leenhardt|first1=Antoine|last2=Lucet|first2=Vincent|last3=Denjoy|first3=Isabelle|last4=Grau|first4=Francis|last5=Ngoc|first5=Dien Do|last6=Coumel|first6=Philippe|title=Catecholaminergic Polymorphic Ventricular Tachycardia in Children|journal=Circulation|volume=91|issue=5|year=1995|pages=1512–1519|issn=0009-7322|doi=10.1161/01.CIR.91.5.1512}}</ref><ref name="PrioriBlomström-Lundqvist2015">{{cite journal|last1=Priori|first1=Silvia G.|last2=Blomström-Lundqvist|first2=Carina|last3=Mazzanti|first3=Andrea|last4=Blom|first4=Nico|last5=Borggrefe|first5=Martin|last6=Camm|first6=John|last7=Elliott|first7=Perry Mark|last8=Fitzsimons|first8=Donna|last9=Hatala|first9=Robert|last10=Hindricks|first10=Gerhard|last11=Kirchhof|first11=Paulus|last12=Kjeldsen|first12=Keld|last13=Kuck|first13=Karl-Heinz|last14=Hernandez-Madrid|first14=Antonio|last15=Nikolaou|first15=Nikolaos|last16=Norekvål|first16=Tone M.|last17=Spaulding|first17=Christian|last18=Van Veldhuisen|first18=Dirk J.|title=2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death|journal=European Heart Journal|volume=36|issue=41|year=2015|pages=2793–2867|issn=0195-668X|doi=10.1093/eurheartj/ehv316}}</ref>
*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 [[arrhythmias|arrhythmic]] event [[rate]] ranging between 11% per year and 3% per year.<ref name="HayashiDenjoy2009">{{cite journal|last1=Hayashi|first1=Meiso|last2=Denjoy|first2=Isabelle|last3=Extramiana|first3=Fabrice|last4=Maltret|first4=Alice|last5=Buisson|first5=Nathalie Roux|last6=Lupoglazoff|first6=Jean-Marc|last7=Klug|first7=Didier|last8=Hayashi|first8=Miyuki|last9=Takatsuki|first9=Seiji|last10=Villain|first10=Elisabeth|last11=Kamblock|first11=Joël|last12=Messali|first12=Anne|last13=Guicheney|first13=Pascale|last14=Lunardi|first14=Joël|last15=Leenhardt|first15=Antoine|title=Incidence and Risk Factors of Arrhythmic Events in Catecholaminergic Polymorphic Ventricular Tachycardia|journal=Circulation|volume=119|issue=18|year=2009|pages=2426–2434|issn=0009-7322|doi=10.1161/CIRCULATIONAHA.108.829267}}</ref><ref name="CerroneColombi2005">{{cite journal|last1=Cerrone|first1=Marina|last2=Colombi|first2=Barbara|last3=Santoro|first3=Massimo|last4=di Barletta|first4=Marina Raffaele|last5=Scelsi|first5=Mario|last6=Villani|first6=Laura|last7=Napolitano|first7=Carlo|last8=Priori|first8=Silvia G|title=Bidirectional Ventricular Tachycardia and Fibrillation Elicited in a Knock-In Mouse Model Carrier of a Mutation in the Cardiac Ryanodine Receptor|journal=Circulation Research|volume=96|issue=10|year=2005|issn=0009-7330|doi=10.1161/01.RES.0000169067.51055.72}}</ref><ref name="PrioriNapolitano2002">{{cite journal|last1=Priori|first1=Silvia G.|last2=Napolitano|first2=Carlo|last3=Memmi|first3=Mirella|last4=Colombi|first4=Barbara|last5=Drago|first5=Fabrizio|last6=Gasparini|first6=Maurizio|last7=DeSimone|first7=Luciano|last8=Coltorti|first8=Fernando|last9=Bloise|first9=Raffaella|last10=Keegan|first10=Roberto|last11=Cruz Filho|first11=Fernando E.S.|last12=Vignati|first12=Gabriele|last13=Benatar|first13=Abraham|last14=DeLogu|first14=Angelica|title=Clinical and Molecular Characterization of Patients With Catecholaminergic Polymorphic Ventricular Tachycardia|journal=Circulation|volume=106|issue=1|year=2002|pages=69–74|issn=0009-7322|doi=10.1161/01.CIR.0000020013.73106.D8}}</ref>
 
====Verampil====
*[[Calcium channel blocker]].
*[[Verapamil]] might be considered as adjunctive therapy for [[CPVT]] patients with ongoing [[ventricular arrhythmias]] despite therapy with [[beta blockers]].<ref name="SwanLaitinen2005">{{cite journal|last1=Swan|first1=Heikki|last2=Laitinen|first2=Paivi|last3=Kontula|first3=Kimmo|last4=Toivonen|first4=Lauri|title=Calcium Channel Antagonism Reduces Exercise-Induced Ventricular Arrhythmias in Catecholaminergic Polymorphic Ventricular Tachycardia Patients with RyR2 Mutations|journal=Journal of Cardiovascular Electrophysiology|volume=16|issue=2|year=2005|pages=162–166|issn=1045-3873|doi=10.1046/j.1540-8167.2005.40516.x}}</ref><ref name="RossoKalman2007">{{cite journal|last1=Rosso|first1=Rafael|last2=Kalman|first2=Jonathan M.|last3=Rogowski|first3=Ori|last4=Diamant|first4=Shmuel|last5=Birger|first5=Amir|last6=Biner|first6=Simon|last7=Belhassen|first7=Bernard|last8=Viskin|first8=Sami|title=Calcium channel blockers and beta-blockers versus beta-blockers alone for preventing exercise-induced arrhythmias in catecholaminergic polymorphic ventricular tachycardia|journal=Heart Rhythm|volume=4|issue=9|year=2007|pages=1149–1154|issn=15475271|doi=10.1016/j.hrthm.2007.05.017}}</ref>
*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 [[Ryanodine receptor 2|cardiac ryanodine receptor]] ([[Ryanodine receptor 2|RyR2]]. This dual-action makes it an effective medication for [[CPVT]].<ref name="WatanabeChopra2009">{{cite journal|last1=Watanabe|first1=Hiroshi|last2=Chopra|first2=Nagesh|last3=Laver|first3=Derek|last4=Hwang|first4=Hyun Seok|last5=Davies|first5=Sean S|last6=Roach|first6=Daniel E|last7=Duff|first7=Henry J|last8=Roden|first8=Dan M|last9=Wilde|first9=Arthur A M|last10=Knollmann|first10=Björn C|title=Flecainide prevents catecholaminergic polymorphic ventricular tachycardia in mice and humans|journal=Nature Medicine|volume=15|issue=4|year=2009|pages=380–383|issn=1078-8956|doi=10.1038/nm.1942}}</ref>
*Indications:<ref name="WatanabeChopra2009">{{cite journal|last1=Watanabe|first1=Hiroshi|last2=Chopra|first2=Nagesh|last3=Laver|first3=Derek|last4=Hwang|first4=Hyun Seok|last5=Davies|first5=Sean S|last6=Roach|first6=Daniel E|last7=Duff|first7=Henry J|last8=Roden|first8=Dan M|last9=Wilde|first9=Arthur A M|last10=Knollmann|first10=Björn C|title=Flecainide prevents catecholaminergic polymorphic ventricular tachycardia in mice and humans|journal=Nature Medicine|volume=15|issue=4|year=2009|pages=380–383|issn=1078-8956|doi=10.1038/nm.1942}}</ref><ref name="van der WerfKannankeril2011">{{cite journal|last1=van der Werf|first1=Christian|last2=Kannankeril|first2=Prince J.|last3=Sacher|first3=Frederic|last4=Krahn|first4=Andrew D.|last5=Viskin|first5=Sami|last6=Leenhardt|first6=Antoine|last7=Shimizu|first7=Wataru|last8=Sumitomo|first8=Naokata|last9=Fish|first9=Frank A.|last10=Bhuiyan|first10=Zahurul A.|last11=Willems|first11=Albert R.|last12=van der Veen|first12=Maurits J.|last13=Watanabe|first13=Hiroshi|last14=Laborderie|first14=Julien|last15=Haïssaguerre|first15=Michel|last16=Knollmann|first16=Björn C.|last17=Wilde|first17=Arthur A.M.|title=Flecainide Therapy Reduces Exercise-Induced Ventricular Arrhythmias in Patients With Catecholaminergic Polymorphic Ventricular Tachycardia|journal=Journal of the American College of Cardiology|volume=57|issue=22|year=2011|pages=2244–2254|issn=07351097|doi=10.1016/j.jacc.2011.01.026}}</ref>
**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).<ref name="PrioriBlomström-Lundqvist2015">{{cite journal|last1=Priori|first1=Silvia G.|last2=Blomström-Lundqvist|first2=Carina|last3=Mazzanti|first3=Andrea|last4=Blom|first4=Nico|last5=Borggrefe|first5=Martin|last6=Camm|first6=John|last7=Elliott|first7=Perry Mark|last8=Fitzsimons|first8=Donna|last9=Hatala|first9=Robert|last10=Hindricks|first10=Gerhard|last11=Kirchhof|first11=Paulus|last12=Kjeldsen|first12=Keld|last13=Kuck|first13=Karl-Heinz|last14=Hernandez-Madrid|first14=Antonio|last15=Nikolaou|first15=Nikolaos|last16=Norekvål|first16=Tone M.|last17=Spaulding|first17=Christian|last18=Van Veldhuisen|first18=Dirk J.|title=2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death|journal=European Heart Journal|volume=36|issue=41|year=2015|pages=2793–2867|issn=0195-668X|doi=10.1093/eurheartj/ehv316}}</ref>
*[[Randomized clinical trials]] for the long-term [[efficacy]] of [[flecainide]] are still ongoing.<ref>{{cite web |url=https://clinicaltrials.gov/ct2/show/NCT01117454 |title=Flecainide for Catecholaminergic Polymorphic Ventricular Tachycardia - Full Text View - ClinicalTrials.gov |format= |work= |accessdate=}}</ref>
 
===Implantable cardioverter-defibrillator===
*[[ICD]] should be used with pharmacologic therapy.<ref name="RostonJones2018">{{cite journal|last1=Roston|first1=Thomas M.|last2=Jones|first2=Karolina|last3=Hawkins|first3=Nathaniel M.|last4=Bos|first4=J. Martijn|last5=Schwartz|first5=Peter J.|last6=Perry|first6=Frances|last7=Ackerman|first7=Michael J.|last8=Laksman|first8=Zachary W.M.|last9=Kaul|first9=Padma|last10=Lieve|first10=Krystien V.V.|last11=Atallah|first11=Joseph|last12=Krahn|first12=Andrew D.|last13=Sanatani|first13=Shubhayan|title=Implantable cardioverter-defibrillator use in catecholaminergic polymorphic ventricular tachycardia: A systematic review|journal=Heart Rhythm|volume=15|issue=12|year=2018|pages=1791–1799|issn=15475271|doi=10.1016/j.hrthm.2018.06.046}}</ref>
*Indications:<ref name="PrioriBlomström-Lundqvist2015">{{cite journal|last1=Priori|first1=Silvia G.|last2=Blomström-Lundqvist|first2=Carina|last3=Mazzanti|first3=Andrea|last4=Blom|first4=Nico|last5=Borggrefe|first5=Martin|last6=Camm|first6=John|last7=Elliott|first7=Perry Mark|last8=Fitzsimons|first8=Donna|last9=Hatala|first9=Robert|last10=Hindricks|first10=Gerhard|last11=Kirchhof|first11=Paulus|last12=Kjeldsen|first12=Keld|last13=Kuck|first13=Karl-Heinz|last14=Hernandez-Madrid|first14=Antonio|last15=Nikolaou|first15=Nikolaos|last16=Norekvål|first16=Tone M.|last17=Spaulding|first17=Christian|last18=Van Veldhuisen|first18=Dirk J.|title=2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death|journal=European Heart Journal|volume=36|issue=41|year=2015|pages=2793–2867|issn=0195-668X|doi=10.1093/eurheartj/ehv316}}</ref>
**Patients who are at high risk of [[cardiac arrest]]
**Patients who have survived a [[sudden cardiac arrest]]
**Patients who have experienced [[syncope]] or sustained [[VT]] despite optimal medical therapy and left [[cardiac]] [[sympathetic]] [[denervation]]. [].
*[[Implantable cardioverter-defibrillator]] may have harmful pro-[[arrhythmia|arrhythmic]] effects in some patients, since painful shocks can increase [[catecholamine]] release and trigger further [[arrhythmias]] and triggering [[VT]] storm, leading to a malignant cycle of shocks that may even culminate in [[death]].
*To reduce the risk of inappropriate shocks, it is important to program [[ICD]] with long delays before shock delivery and high cut-off rates for heart rate recognition; and always administer [[beta blockers]] concurrently.<ref name="Al-KhatibStevenson2018">{{cite journal|last1=Al-Khatib|first1=Sana M.|last2=Stevenson|first2=William G.|last3=Ackerman|first3=Michael J.|last4=Bryant|first4=William J.|last5=Callans|first5=David J.|last6=Curtis|first6=Anne B.|last7=Deal|first7=Barbara J.|last8=Dickfeld|first8=Timm|last9=Field|first9=Michael E.|last10=Fonarow|first10=Gregg C.|last11=Gillis|first11=Anne M.|last12=Granger|first12=Christopher B.|last13=Hammill|first13=Stephen C.|last14=Hlatky|first14=Mark A.|last15=Joglar|first15=José A.|last16=Kay|first16=G. Neal|last17=Matlock|first17=Daniel D.|last18=Myerburg|first18=Robert J.|last19=Page|first19=Richard L.|title=2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death|journal=Journal of the American College of Cardiology|volume=72|issue=14|year=2018|pages=e91–e220|issn=07351097|doi=10.1016/j.jacc.2017.10.054}}</ref>
 
===Sympathectomy===
*Left [[cardiac]] [[sympathetic]] [[denervation]], where a portion of the [[sympathetic chain]] is [[surgical|surgically]] or [[endoscopic|endoscopically]] resected, and bilateral [[thoracoscopy|thoracoscopic]] [[sympathectomy]] have reported to be useful therapeutic methods for suppressing [[ventricular arrhythmias]] in [[CPVT]] patients.<ref name="PrioriBlomström-Lundqvist2015">{{cite journal|last1=Priori|first1=Silvia G.|last2=Blomström-Lundqvist|first2=Carina|last3=Mazzanti|first3=Andrea|last4=Blom|first4=Nico|last5=Borggrefe|first5=Martin|last6=Camm|first6=John|last7=Elliott|first7=Perry Mark|last8=Fitzsimons|first8=Donna|last9=Hatala|first9=Robert|last10=Hindricks|first10=Gerhard|last11=Kirchhof|first11=Paulus|last12=Kjeldsen|first12=Keld|last13=Kuck|first13=Karl-Heinz|last14=Hernandez-Madrid|first14=Antonio|last15=Nikolaou|first15=Nikolaos|last16=Norekvål|first16=Tone M.|last17=Spaulding|first17=Christian|last18=Van Veldhuisen|first18=Dirk J.|title=2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death|journal=European Heart Journal|volume=36|issue=41|year=2015|pages=2793–2867|issn=0195-668X|doi=10.1093/eurheartj/ehv316}}</ref><ref name="SchneiderSteinmetz2012">{{cite journal|last1=Schneider|first1=Heike E.|last2=Steinmetz|first2=Michael|last3=Krause|first3=Ulrich|last4=Kriebel|first4=Thomas|last5=Ruschewski|first5=Wolfgang|last6=Paul|first6=Thomas|title=Left cardiac sympathetic denervation for the management of life-threatening ventricular tachyarrhythmias in young patients with catecholaminergic polymorphic ventricular tachycardia and long QT syndrome|journal=Clinical Research in Cardiology|volume=102|issue=1|year=2012|pages=33–42|issn=1861-0684|doi=10.1007/s00392-012-0492-7}}</ref><ref>{{cite journal|title=Successful treatment of catecholaminergic polymorphic ventricular tachycardia with bilateral thoracoscopic sympathectomy|journal=Heart Rhythm|date=October 2008|first=P.A.|last=Scott|coauthors=A.J. Sandilands, G.E. Morris, J.M. Morgan |volume=5|issue=10|pages=1461–1463|pmid=18760972 |url=|format=|doi=10.1016/j.hrthm.2008.07.007 }}</ref><ref name="ColluraJohnson2009">{{cite journal|last1=Collura|first1=Christopher A.|last2=Johnson|first2=Jonathan N.|last3=Moir|first3=Christopher|last4=Ackerman|first4=Michael J.|title=Left cardiac sympathetic denervation for the treatment of long QT syndrome and catecholaminergic polymorphic ventricular tachycardia using video-assisted thoracic surgery|journal=Heart Rhythm|volume=6|issue=6|year=2009|pages=752–759|issn=15475271|doi=10.1016/j.hrthm.2009.03.024}}</ref>
*Indications:<ref name="PrioriBlomström-Lundqvist2015">{{cite journal|last1=Priori|first1=Silvia G.|last2=Blomström-Lundqvist|first2=Carina|last3=Mazzanti|first3=Andrea|last4=Blom|first4=Nico|last5=Borggrefe|first5=Martin|last6=Camm|first6=John|last7=Elliott|first7=Perry Mark|last8=Fitzsimons|first8=Donna|last9=Hatala|first9=Robert|last10=Hindricks|first10=Gerhard|last11=Kirchhof|first11=Paulus|last12=Kjeldsen|first12=Keld|last13=Kuck|first13=Karl-Heinz|last14=Hernandez-Madrid|first14=Antonio|last15=Nikolaou|first15=Nikolaos|last16=Norekvål|first16=Tone M.|last17=Spaulding|first17=Christian|last18=Van Veldhuisen|first18=Dirk J.|title=2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death|journal=European Heart Journal|volume=36|issue=41|year=2015|pages=2793–2867|issn=0195-668X|doi=10.1093/eurheartj/ehv316}}</ref><ref name="De FerrariDusi2015">{{cite journal|last1=De Ferrari|first1=Gaetano M.|last2=Dusi|first2=Veronica|last3=Spazzolini|first3=Carla|last4=Bos|first4=J. Martijn|last5=Abrams|first5=Dominic J.|last6=Berul|first6=Charles I.|last7=Crotti|first7=Lia|last8=Davis|first8=Andrew M.|last9=Eldar|first9=Michael|last10=Kharlap|first10=Maria|last11=Khoury|first11=Asaad|last12=Krahn|first12=Andrew D.|last13=Leenhardt|first13=Antoine|last14=Moir|first14=Christopher R.|last15=Odero|first15=Attilio|last16=Olde Nordkamp|first16=Louise|last17=Paul|first17=Thomas|last18=Rosés i Noguer|first18=Ferran|last19=Shkolnikova|first19=Maria|last20=Till|first20=Jan|last21=Wilde|first21=Arthur A.M.|last22=Ackerman|first22=Michael J.|last23=Schwartz|first23=Peter J.|title=Clinical Management of Catecholaminergic Polymorphic Ventricular Tachycardia|journal=Circulation|volume=131|issue=25|year=2015|pages=2185–2193|issn=0009-7322|doi=10.1161/CIRCULATIONAHA.115.015731}}</ref><ref name="WildeBhuiyan2008">{{cite journal|last1=Wilde|first1=Arthur A.M.|last2=Bhuiyan|first2=Zahurul A.|last3=Crotti|first3=Lia|last4=Facchini|first4=Mario|last5=De Ferrari|first5=Gaetano M.|last6=Paul|first6=Thomas|last7=Ferrandi|first7=Chiara|last8=Koolbergen|first8=Dave R.|last9=Odero|first9=Attilio|last10=Schwartz|first10=Peter J.|title=Left Cardiac Sympathetic Denervation for Catecholaminergic Polymorphic Ventricular Tachycardia|journal=New England Journal of Medicine|volume=358|issue=19|year=2008|pages=2024–2029|issn=0028-4793|doi=10.1056/NEJMoa0708006}}</ref>
**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:<ref name="Waddell-SmithErtresvaag2015">{{cite journal|last1=Waddell-Smith|first1=Kathryn E.|last2=Ertresvaag|first2=Kjetil N.|last3=Li|first3=Jian|last4=Chaudhuri|first4=Krish|last5=Crawford|first5=Jackie R.|last6=Hamill|first6=James K.|last7=Haydock|first7=David|last8=Skinner|first8=Jonathan R.|title=Physical and Psychological Consequences of Left Cardiac Sympathetic Denervation in Long-QT Syndrome and Catecholaminergic Polymorphic Ventricular Tachycardia|journal=Circulation: Arrhythmia and Electrophysiology|volume=8|issue=5|year=2015|pages=1151–1158|issn=1941-3149|doi=10.1161/CIRCEP.115.003159}}</ref>
***[[Horner's syndrome]]
***[[Pneumothorax]] 
*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.<ref name="PrioriWilde2013">{{cite journal|last1=Priori|first1=Silvia G.|last2=Wilde|first2=Arthur A.|last3=Horie|first3=Minoru|last4=Cho|first4=Yongkeun|last5=Behr|first5=Elijah R.|last6=Berul|first6=Charles|last7=Blom|first7=Nico|last8=Brugada|first8=Josep|last9=Chiang|first9=Chern-En|last10=Huikuri|first10=Heikki|last11=Kannankeril|first11=Prince|last12=Krahn|first12=Andrew|last13=Leenhardt|first13=Antoine|last14=Moss|first14=Arthur|last15=Schwartz|first15=Peter J.|last16=Shimizu|first16=Wataru|last17=Tomaselli|first17=Gordon|last18=Tracy|first18=Cynthia|last19=Ackerman|first19=Michael|last20=Belhassen|first20=Bernard|last21=Estes|first21=N. A. Mark|last22=Fatkin|first22=Diane|last23=Kalman|first23=Jonathan|last24=Kaufman|first24=Elizabeth|last25=Kirchhof|first25=Paulus|last26=Schulze-Bahr|first26=Eric|last27=Wolpert|first27=Christian|last28=Vohra|first28=Jitendra|last29=Refaat|first29=Marwan|last30=Etheridge|first30=Susan P.|last31=Campbell|first31=Robert M.|last32=Martin|first32=Edward T.|last33=Quek|first33=Swee Chye|title=Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes|journal=EP Europace|volume=15|issue=10|year=2013|pages=1389–1406|issn=1532-2092|doi=10.1093/europace/eut272}}</ref>
*[[Catheter ablation]] of the bidirectional [[VPC]]s that trigger [[VF]] has been reported and this procedure could become an adjunctive therapy in patients with refractory [[CPVT]].<ref name="KaneshiroNaruse2012">{{cite journal|last1=Kaneshiro|first1=Takashi|last2=Naruse|first2=Yoshihisa|last3=Nogami|first3=Akihiko|last4=Tada|first4=Hiroshi|last5=Yoshida|first5=Kentaro|last6=Sekiguchi|first6=Yukio|last7=Murakoshi|first7=Nobuyuki|last8=Kato|first8=Yoshiaki|last9=Horigome|first9=Hitoshi|last10=Kawamura|first10=Mihoko|last11=Horie|first11=Minoru|last12=Aonuma|first12=Kazutaka|title=
            Successful Catheter Ablation of Bidirectional Ventricular Premature Contractions Triggering Ventricular Fibrillation in Catecholaminergic Polymorphic Ventricular Tachycardia With
            RyR2
            Mutation
          |journal=Circulation: Arrhythmia and Electrophysiology|volume=5|issue=1|year=2012|issn=1941-3149|doi=10.1161/CIRCEP.111.966549}}</ref>
*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) <ref name="pmid16935995">{{cite journal| author=Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M et al.| title=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. | journal=Circulation | year= 2006 | volume= 114 | issue= 10 | pages= e385-484 | pmid=16935995 | doi=10.1161/CIRCULATIONAHA.106.178233 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16935995}}</ref> ==
 
{|class="wikitable"
|-
| colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class I]]
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''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]]. ''([[ACC AHA Guidelines Classification Scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''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. ''([[ACC AHA Guidelines Classification Scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|}


{|class="wikitable"
==Case Studies==
|-
[[Catecholaminergic polymorphic ventricular tachycardia case study one|Case #1]]
| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class IIa]]
|-
|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.''' [[Beta blockers]] can be effective in patients without clinical manifestations when the diagnosis of CPVT is established during childhood based on [[genetic analysis]]. ''([[ACC AHA Guidelines Classification Scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|-
|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''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. ''([[ACC AHA Guidelines Classification Scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|}


{|class="wikitable"
==Related Chapters==
|-
*[[Ventricular tachycardia]]
| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class IIb]]
*[[Ventricular fibrillation]]
|-
*[[Long QT syndrome]]
|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.''' [[Beta blockers]] may be considered for patients with CPVT who were genetically diagnosed in adulthood and never manifested clinical symptoms of [[tachyarrhythmias]]. ''([[ACC AHA Guidelines Classification Scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
*[[Polymorphic ventricular tachycardia]]
|}


==References==
{{reflist|2}}
[[Electrocardiography]]
[[Category:Cardiology]]
[[Category:Cardiology]]

Latest revision as of 10:12, 24 July 2020

Catecholaminergic polymorphic ventricular tachycardia Microchapters

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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, catecholaminergic polymorphic VT, bidirectional ventricular tachycardia induced by catecholamines, bidirectional VT, catecholamine-induced polymorphic ventricular tachycardia, catecholamine induced polymorphic ventricular tachycardia, familial polymorphic ventricular tachycardia, FPVT, polymorphic ventricular tachycardia, polymorphic VT induced by catecholamines.

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

Case Studies

Case #1

Related Chapters