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Polymorphic ventricular tachycardia

MedGen UID:
138002
Concept ID:
C0344432
Disease or Syndrome
Synonyms: Polymorphic Ventricular Tachycardia; polymorphic ventricular tachycardia; Ventricular tachycardia, polymorphic; ventricular tachycardia, polymorphic
SNOMED CT: Ventricular tachycardia, polymorphic (251159007)
 
HPO: HP:0031677
Monarch Initiative: MONDO:0020575

Definition

A type of ventricular tachycardia that is characterized by variable QRS complexes within each lead (i.e., QRS complexes may be different from beat to beat). [from HPO]

Conditions with this feature

Catecholaminergic polymorphic ventricular tachycardia 2
MedGen UID:
393837
Concept ID:
C2677794
Disease or Syndrome
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is characterized by episodic syncope occurring during exercise or acute emotion. The underlying cause of these episodes is the onset of fast ventricular tachycardia (bidirectional or polymorphic). Spontaneous recovery may occur when these arrhythmias self-terminate. In other instances, ventricular tachycardia may degenerate into ventricular fibrillation and cause sudden death if cardiopulmonary resuscitation is not readily available. The mean onset of symptoms (usually a syncopal episode) is between age seven and 12 years; onset as late as the fourth decade of life has been reported. If untreated, CPVT is highly lethal, as approximately 30% of affected individuals experience at least one cardiac arrest and up to 80% have one or more syncopal spells. Sudden death may be the first manifestation of the disease.
Catecholaminergic polymorphic ventricular tachycardia 3
MedGen UID:
462813
Concept ID:
C3151463
Disease or Syndrome
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is characterized by episodic syncope occurring during exercise or acute emotion. The underlying cause of these episodes is the onset of fast ventricular tachycardia (bidirectional or polymorphic). Spontaneous recovery may occur when these arrhythmias self-terminate. In other instances, ventricular tachycardia may degenerate into ventricular fibrillation and cause sudden death if cardiopulmonary resuscitation is not readily available. The mean onset of symptoms (usually a syncopal episode) is between age seven and 12 years; onset as late as the fourth decade of life has been reported. If untreated, CPVT is highly lethal, as approximately 30% of affected individuals experience at least one cardiac arrest and up to 80% have one or more syncopal spells. Sudden death may be the first manifestation of the disease.
Catecholaminergic polymorphic ventricular tachycardia 5
MedGen UID:
815866
Concept ID:
C3809536
Disease or Syndrome
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is characterized by episodic syncope occurring during exercise or acute emotion. The underlying cause of these episodes is the onset of fast ventricular tachycardia (bidirectional or polymorphic). Spontaneous recovery may occur when these arrhythmias self-terminate. In other instances, ventricular tachycardia may degenerate into ventricular fibrillation and cause sudden death if cardiopulmonary resuscitation is not readily available. The mean onset of symptoms (usually a syncopal episode) is between age seven and 12 years; onset as late as the fourth decade of life has been reported. If untreated, CPVT is highly lethal, as approximately 30% of affected individuals experience at least one cardiac arrest and up to 80% have one or more syncopal spells. Sudden death may be the first manifestation of the disease.
Long QT syndrome 15
MedGen UID:
864132
Concept ID:
C4015695
Disease or Syndrome
Long QT syndrome (LQTS) is a cardiac electrophysiologic disorder, characterized by QT prolongation and T-wave abnormalities on the EKG that are associated with tachyarrhythmias, typically the ventricular tachycardia torsade de pointes (TdP). TdP is usually self-terminating, thus causing a syncopal event, the most common symptom in individuals with LQTS. Such cardiac events typically occur during exercise and emotional stress, less frequently during sleep, and usually without warning. In some instances, TdP degenerates to ventricular fibrillation and causes aborted cardiac arrest (if the individual is defibrillated) or sudden death. Approximately 50% of untreated individuals with a pathogenic variant in one of the genes associated with LQTS have symptoms, usually one to a few syncopal events. While cardiac events may occur from infancy through middle age, they are most common from the preteen years through the 20s. Some types of LQTS are associated with a phenotype extending beyond cardiac arrhythmia. In addition to the prolonged QT interval, associations include muscle weakness and facial dysmorphism in Andersen-Tawil syndrome (LQTS type 7); hand/foot, facial, and neurodevelopmental features in Timothy syndrome (LQTS type 8); and profound sensorineural hearing loss in Jervell and Lange-Nielson syndrome.

Professional guidelines

PubMed

Writing Committee Members, Shah MJ, Silka MJ, Silva JNA, Balaji S, Beach CM, Benjamin MN, Berul CI, Cannon B, Cecchin F, Cohen MI, Dalal AS, Dechert BE, Foster A, Gebauer R, Gonzalez Corcia MC, Kannankeril PJ, Karpawich PP, Kim JJ, Krishna MR, Kubuš P, LaPage MJ, Mah DY, Malloy-Walton L, Miyazaki A, Motonaga KS, Niu MC, Olen M, Paul T, Rosenthal E, Saarel EV, Silvetti MS, Stephenson EA, Tan RB, Triedman J, Bergen NHV, Wackel PL
Heart Rhythm 2021 Nov;18(11):1888-1924. Epub 2021 Jul 29 doi: 10.1016/j.hrthm.2021.07.038. PMID: 34363988
Stiles MK, Wilde AAM, Abrams DJ, Ackerman MJ, Albert CM, Behr ER, Chugh SS, Cornel MC, Gardner K, Ingles J, James CA, Jimmy Juang JM, Kääb S, Kaufman ES, Krahn AD, Lubitz SA, MacLeod H, Morillo CA, Nademanee K, Probst V, Saarel EV, Sacilotto L, Semsarian C, Sheppard MN, Shimizu W, Skinner JR, Tfelt-Hansen J, Wang DW
Heart Rhythm 2021 Jan;18(1):e1-e50. Epub 2020 Oct 19 doi: 10.1016/j.hrthm.2020.10.010. PMID: 33091602Free PMC Article
Kloesel B, Ackerman MJ, Sprung J, Narr BJ, Weingarten TN
Can J Anaesth 2011 Sep;58(9):824-36. Epub 2011 Jun 23 doi: 10.1007/s12630-011-9546-y. PMID: 21698509

Recent clinical studies

Etiology

Tfelt-Hansen J, Garcia R, Albert C, Merino J, Krahn A, Marijon E, Basso C, Wilde AAM, Haugaa KH
Europace 2023 Aug 25;25(8) doi: 10.1093/europace/euad203. PMID: 37622576Free PMC Article
Mazzanti A, Kukavica D, Trancuccio A, Memmi M, Bloise R, Gambelli P, Marino M, Ortíz-Genga M, Morini M, Monteforte N, Giordano U, Keegan R, Tomasi L, Anastasakis A, Davis AM, Shimizu W, Blom NA, Santiago DJ, Napolitano C, Monserrat L, Priori SG
JAMA Cardiol 2022 May 1;7(5):504-512. doi: 10.1001/jamacardio.2022.0219. PMID: 35353122Free PMC Article
Etchegoyen CV, Keller GA, Mrad S, Cheng S, Di Girolamo G
Curr Clin Pharmacol 2017;12(4):210-222. doi: 10.2174/1574884713666180223123947. PMID: 29473523
McKenna WJ, Maron BJ, Thiene G
Circ Res 2017 Sep 15;121(7):722-730. doi: 10.1161/CIRCRESAHA.117.309711. PMID: 28912179
Kloesel B, Ackerman MJ, Sprung J, Narr BJ, Weingarten TN
Can J Anaesth 2011 Sep;58(9):824-36. Epub 2011 Jun 23 doi: 10.1007/s12630-011-9546-y. PMID: 21698509

Diagnosis

Almarzuqi A, Kimber S, Quadros K, Senaratne J
Vasc Health Risk Manag 2022;18:397-406. Epub 2022 Jun 7 doi: 10.2147/VHRM.S274857. PMID: 35698640Free PMC Article
Mazzanti A, Kukavica D, Trancuccio A, Memmi M, Bloise R, Gambelli P, Marino M, Ortíz-Genga M, Morini M, Monteforte N, Giordano U, Keegan R, Tomasi L, Anastasakis A, Davis AM, Shimizu W, Blom NA, Santiago DJ, Napolitano C, Monserrat L, Priori SG
JAMA Cardiol 2022 May 1;7(5):504-512. doi: 10.1001/jamacardio.2022.0219. PMID: 35353122Free PMC Article
Viskin S, Chorin E, Viskin D, Hochstadt A, Schwartz AL, Rosso R
Circulation 2021 Sep 7;144(10):823-839. doi: 10.1161/CIRCULATIONAHA.121.055783. PMID: 34491774
McKenna WJ, Maron BJ, Thiene G
Circ Res 2017 Sep 15;121(7):722-730. doi: 10.1161/CIRCRESAHA.117.309711. PMID: 28912179
Lieve KV, van der Werf C, Wilde AA
Circ J 2016 May 25;80(6):1285-91. Epub 2016 May 13 doi: 10.1253/circj.CJ-16-0326. PMID: 27180891

Therapy

Almarzuqi A, Kimber S, Quadros K, Senaratne J
Vasc Health Risk Manag 2022;18:397-406. Epub 2022 Jun 7 doi: 10.2147/VHRM.S274857. PMID: 35698640Free PMC Article
Mazzanti A, Kukavica D, Trancuccio A, Memmi M, Bloise R, Gambelli P, Marino M, Ortíz-Genga M, Morini M, Monteforte N, Giordano U, Keegan R, Tomasi L, Anastasakis A, Davis AM, Shimizu W, Blom NA, Santiago DJ, Napolitano C, Monserrat L, Priori SG
JAMA Cardiol 2022 May 1;7(5):504-512. doi: 10.1001/jamacardio.2022.0219. PMID: 35353122Free PMC Article
Bezzerides VJ, Prondzynski M, Carrier L, Pu WT
Cardiovasc Res 2020 Jul 15;116(9):1635-1650. doi: 10.1093/cvr/cvaa107. PMID: 32321160Free PMC Article
Etchegoyen CV, Keller GA, Mrad S, Cheng S, Di Girolamo G
Curr Clin Pharmacol 2017;12(4):210-222. doi: 10.2174/1574884713666180223123947. PMID: 29473523
Kloesel B, Ackerman MJ, Sprung J, Narr BJ, Weingarten TN
Can J Anaesth 2011 Sep;58(9):824-36. Epub 2011 Jun 23 doi: 10.1007/s12630-011-9546-y. PMID: 21698509

Prognosis

Piccini JP, Russo AM, Sharma PS, Kron J, Tzou W, Sauer W, Park DS, Birgersdotter-Green U, Frankel DS, Healey JS, Hummel J, Koruth J, Linz D, Mittal S, Nair DG, Nattel S, Noseworthy PA, Steinberg BA, Trayanova NA, Wan EY, Wissner E, Zeitler EP, Wang PJ
Circ Arrhythm Electrophysiol 2022 Dec;15(12):e009911. Epub 2022 Nov 28 doi: 10.1161/CIRCEP.121.009911. PMID: 36441565
Stiles MK, Wilde AAM, Abrams DJ, Ackerman MJ, Albert CM, Behr ER, Chugh SS, Cornel MC, Gardner K, Ingles J, James CA, Jimmy Juang JM, Kääb S, Kaufman ES, Krahn AD, Lubitz SA, MacLeod H, Morillo CA, Nademanee K, Probst V, Saarel EV, Sacilotto L, Semsarian C, Sheppard MN, Shimizu W, Skinner JR, Tfelt-Hansen J, Wang DW
Heart Rhythm 2021 Jan;18(1):e1-e50. Epub 2020 Oct 19 doi: 10.1016/j.hrthm.2020.10.010. PMID: 33091602Free PMC Article
Pérez-Riera AR, Barbosa-Barros R, Samesina N, Pastore CA, Scanavacca M, Daminello-Raimundo R, de Abreu LC, Nikus K, Brugada P
Cardiol Rev 2021 Jul-Aug 01;29(4):165-177. doi: 10.1097/CRD.0000000000000326. PMID: 32947483
Manolis AS, Manolis AA, Manolis TA, Apostolopoulos EJ, Papatheou D, Melita H
Trends Cardiovasc Med 2020 Nov;30(8):451-460. Epub 2020 Aug 16 doi: 10.1016/j.tcm.2020.08.002. PMID: 32814095Free PMC Article
McKenna WJ, Maron BJ, Thiene G
Circ Res 2017 Sep 15;121(7):722-730. doi: 10.1161/CIRCRESAHA.117.309711. PMID: 28912179

Clinical prediction guides

Shen L, Liu S, Hu F, Zhang Z, Li J, Lai Z, Zheng L, Yao Y
J Am Heart Assoc 2023 Dec 19;12(24):e031768. Epub 2023 Dec 8 doi: 10.1161/JAHA.123.031768. PMID: 38063176Free PMC Article
Crotti L, Spazzolini C, Nyegaard M, Overgaard MT, Kotta MC, Dagradi F, Sala L, Aiba T, Ayers MD, Baban A, Barc J, Beach CM, Behr ER, Bos JM, Cerrone M, Covi P, Cuneo B, Denjoy I, Donner B, Elbert A, Eliasson H, Etheridge SP, Fukuyama M, Girolami F, Hamilton R, Horie M, Iascone M, Jiménez-Jaimez J, Jensen HK, Kannankeril PJ, Kaski JP, Makita N, Muñoz-Esparza C, Odland HH, Ohno S, Papagiannis J, Porretta AP, Prandstetter C, Probst V, Robyns T, Rosenthal E, Rosés-Noguer F, Sekarski N, Singh A, Spentzou G, Stute F, Tfelt-Hansen J, Till J, Tobert KE, Vinocur JM, Webster G, Wilde AAM, Wolf CM, Ackerman MJ, Schwartz PJ
Eur Heart J 2023 Sep 14;44(35):3357-3370. doi: 10.1093/eurheartj/ehad418. PMID: 37528649Free PMC Article
Piccini JP, Russo AM, Sharma PS, Kron J, Tzou W, Sauer W, Park DS, Birgersdotter-Green U, Frankel DS, Healey JS, Hummel J, Koruth J, Linz D, Mittal S, Nair DG, Nattel S, Noseworthy PA, Steinberg BA, Trayanova NA, Wan EY, Wissner E, Zeitler EP, Wang PJ
Circ Arrhythm Electrophysiol 2022 Dec;15(12):e009911. Epub 2022 Nov 28 doi: 10.1161/CIRCEP.121.009911. PMID: 36441565
Pérez-Riera AR, Barbosa-Barros R, Samesina N, Pastore CA, Scanavacca M, Daminello-Raimundo R, de Abreu LC, Nikus K, Brugada P
Cardiol Rev 2021 Jul-Aug 01;29(4):165-177. doi: 10.1097/CRD.0000000000000326. PMID: 32947483
Pérez-Riera AR, Barbosa-Barros R, de Rezende Barbosa MPC, Daminello-Raimundo R, de Lucca AA Jr, de Abreu LC
Ann Noninvasive Electrocardiol 2018 Jul;23(4):e12512. Epub 2017 Oct 19 doi: 10.1111/anec.12512. PMID: 29048771Free PMC Article

Recent systematic reviews

Medeiros P, Santos M, Arantes C, Pereira VH, Rocha S
Heart Lung 2023 Jul-Aug;60:1-7. Epub 2023 Feb 28 doi: 10.1016/j.hrtlng.2023.02.012. PMID: 36863123
Roston TM, Jones K, Hawkins NM, Bos JM, Schwartz PJ, Perry F, Ackerman MJ, Laksman ZWM, Kaul P, Lieve KVV, Atallah J, Krahn AD, Sanatani S
Heart Rhythm 2018 Dec;15(12):1791-1799. Epub 2018 Jul 3 doi: 10.1016/j.hrthm.2018.06.046. PMID: 30063211
Olde Nordkamp LR, Postema PG, Knops RE, van Dijk N, Limpens J, Wilde AA, de Groot JR
Heart Rhythm 2016 Feb;13(2):443-54. Epub 2015 Sep 15 doi: 10.1016/j.hrthm.2015.09.010. PMID: 26385533
Refaat MM, Hotait M, Tseng ZH
Ann Noninvasive Electrocardiol 2014 Jul;19(4):311-8. doi: 10.1111/anec.12191. PMID: 25040480Free PMC Article
Clausen H, Pflaumer A, Kamberi S, Davis A
Pacing Clin Electrophysiol 2013 Mar;36(3):391-401. Epub 2012 Dec 18 doi: 10.1111/pace.12050. PMID: 23252797

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