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| __NOTOC__ | | __NOTOC__ |
| {{Catecholaminergic polymorphic ventricular tachycardia}} | | {{Catecholaminergic polymorphic ventricular tachycardia}} |
| | '''For patient information, click [[Catecholaminergic polymorphic ventricular tachycardia(patient information)|here]].'''<br> |
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| {{CMG}}; {{AE}} {{MRV}} | | {{CMG}}; {{AE}} {{MRV}} |
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| {{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. |
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| ==[[Catecholaminergic polymorphic ventricular tachycardia overview|Overview]]== | | ==[[Catecholaminergic polymorphic ventricular tachycardia overview|Overview]]== |
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| [[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]] | | [[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]] |
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| | ==Case Studies== |
| | [[Catecholaminergic polymorphic ventricular tachycardia case study one|Case #1]] |
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| *The therapeutic approach to CPVT includes changes in lifestyle, medical therapy, left ventricular sympathetic denervation, and the use of implantable cardioverter-defibrillators.
| | ==Related Chapters== |
| | | *[[Ventricular tachycardia]] |
| | | *[[Ventricular fibrillation]] |
| | | *[[Long QT syndrome]] |
| ===Sympathectomy=== | | *[[Polymorphic ventricular tachycardia]] |
| *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
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| **Patients who are intolerant or have contraindications to [[beta blockers]] | |
| *Limitations:
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| **Complexity of the surgical procedure
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| **Requirement of a specialised surgical centre
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| **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>
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| ***[[Horner's syndrome]]
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| ***[[Pneumothorax]] | |
| *In spite of the side-effects and complications, the procedure was safe and satisfactory among the vast majority of patients.
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| ===Catheter ablation===
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| *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>
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| *[[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=
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| Successful Catheter Ablation of Bidirectional Ventricular Premature Contractions Triggering Ventricular Fibrillation in Catecholaminergic Polymorphic Ventricular Tachycardia With
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| RyR2
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| Mutation
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| |journal=Circulation: Arrhythmia and Electrophysiology|volume=5|issue=1|year=2012|issn=1941-3149|doi=10.1161/CIRCEP.111.966549}}</ref>
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| *Further evidence and experiences are required for its recommendation.
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| ===Prevention===
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| *Limit or avoid competitive sports.
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| *Limit or avoid strenuous exercises.
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| *Limit exposure to stressful environments.
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| *The limits for allowed physical activity can be set on the basis of [[exercise stress testing]] done in the hospital.
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| *[[Holter monitor]] can be helpful in keeping the [[heart-rate]] within a safe range during physical activity.
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| *Follow-up visits with a [[cardiologist]] every six to twelve months to monitor the [[efficacy]] of therapy.
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| == 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> ==
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| | colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class I]]
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| | 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>
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| | 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>
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| {|class="wikitable"
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| | colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class IIa]]
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| |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>
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| |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>
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| {|class="wikitable"
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| | colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class IIb]]
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| |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>
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| ==References==
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| {{reflist|2}}
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| [[Electrocardiography]]
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| [[Category:Cardiology]] | | [[Category:Cardiology]] |