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Ventricular tachycardia, catecholaminergic polymorphic 6(CPVT6)

MedGen UID:
1712279
•Concept ID:
C5394069
•
Disease or Syndrome
Synonyms: CPVT6; VENTRICULAR TACHYCARDIA, CATECHOLAMINERGIC POLYMORPHIC 6; VENTRICULAR TACHYCARDIA, CATECHOLAMINERGIC POLYMORPHIC, 6
 
Monarch Initiative: MONDO:0800374
OMIM®: 114183; 618782

Professional guidelines

PubMed

Cardiogenetics: the role of genetic testing for inherited arrhythmia syndromes and sudden death.
Specterman MJ, Behr ER
Heart 2023 Feb 23;109(6):434-441. doi: 10.1136/heartjnl-2021-320015. PMID: 36167638
Psychosocial Implications of Living with Catecholaminergic Polymorphic Ventricular Tachycardia in Adulthood.
Richardson E, Spinks C, Davis A, Turner C, Atherton J, McGaughran J, Semsarian C, Ingles J
J Genet Couns 2018 Jun;27(3):549-557. Epub 2017 Sep 23 doi: 10.1007/s10897-017-0152-1. PMID: 28940060
A Focus on Pharmacological Management of Catecholaminergic Polymorphic Ventricular Tachycardia.
Claudio B, Alice M, Daniel S
Mini Rev Med Chem 2018;18(6):476-482. doi: 10.2174/1389557517666170707100923. PMID: 28685702

Recent clinical studies

Etiology

Outcomes of Patients With Catecholaminergic Polymorphic Ventricular Tachycardia Treated With β-Blockers.
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
Inherited cardiac arrhythmias.
Schwartz PJ, Ackerman MJ, Antzelevitch C, Bezzina CR, Borggrefe M, Cuneo BF, Wilde AAM
Nat Rev Dis Primers 2020 Jul 16;6(1):58. doi: 10.1038/s41572-020-0188-7. PMID: 32678103Free PMC Article
Catecholaminergic Polymorphic Ventricular Tachycardia.
Wall JJ, Iyer RV
Pediatr Emerg Care 2017 Jun;33(6):427-431. doi: 10.1097/PEC.0000000000001156. PMID: 28570361
Electrical storm in children.
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
Calsequestrin 2 and arrhythmias.
Faggioni M, Knollmann BC
Am J Physiol Heart Circ Physiol 2012 Mar 15;302(6):H1250-60. Epub 2011 Dec 23 doi: 10.1152/ajpheart.00779.2011. PMID: 22198169Free PMC Article

Diagnosis

Latent Causes of Sudden Cardiac Arrest.
Krahn AD, Tfelt-Hansen J, Tadros R, Steinberg C, Semsarian C, Han HC
JACC Clin Electrophysiol 2022 Jun;8(6):806-821. doi: 10.1016/j.jacep.2021.12.014. PMID: 35738861
Outcomes of Patients With Catecholaminergic Polymorphic Ventricular Tachycardia Treated With β-Blockers.
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
Clinical spectrum and long-term course of sustained ventricular tachycardia in pediatric patients: 10 years of experience.
Şengül FS, Kafalı HC, Güzeltaş A, Ergül Y
Anatol J Cardiol 2021 May;25(5):313-322. doi: 10.14744/AnatolJCardiol.2020.95759. PMID: 33960306Free PMC Article
Inherited cardiac arrhythmias.
Schwartz PJ, Ackerman MJ, Antzelevitch C, Bezzina CR, Borggrefe M, Cuneo BF, Wilde AAM
Nat Rev Dis Primers 2020 Jul 16;6(1):58. doi: 10.1038/s41572-020-0188-7. PMID: 32678103Free PMC Article
Catecholaminergic Polymorphic Ventricular Tachycardia.
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

Electrophysiological Characteristics and Ablation Outcomes in Patients With Catecholaminergic Polymorphic Ventricular Tachycardia.
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
Outcomes of Patients With Catecholaminergic Polymorphic Ventricular Tachycardia Treated With β-Blockers.
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
Prognostic impact of misdiagnosis of cardiac channelopathies as epilepsy.
Ramos-Maqueda J, Bermúdez-Jiménez F, Ruiz RM, Ramos MC, Lerma MM, Millán PS, López MÁ, Sánchez LT, Jiménez-Jáimez J
PLoS One 2020;15(4):e0231442. Epub 2020 Apr 16 doi: 10.1371/journal.pone.0231442. PMID: 32298319Free PMC Article
Catecholaminergic Polymorphic Ventricular Tachycardia.
Wall JJ, Iyer RV
Pediatr Emerg Care 2017 Jun;33(6):427-431. doi: 10.1097/PEC.0000000000001156. PMID: 28570361
Calsequestrin 2 and arrhythmias.
Faggioni M, Knollmann BC
Am J Physiol Heart Circ Physiol 2012 Mar 15;302(6):H1250-60. Epub 2011 Dec 23 doi: 10.1152/ajpheart.00779.2011. PMID: 22198169Free PMC Article

Prognosis

Cardiogenetics: the role of genetic testing for inherited arrhythmia syndromes and sudden death.
Specterman MJ, Behr ER
Heart 2023 Feb 23;109(6):434-441. doi: 10.1136/heartjnl-2021-320015. PMID: 36167638
Evaluation of age at symptom onset, proband status, and sex as predictors of disease severity in pediatric catecholaminergic polymorphic ventricular tachycardia.
Kallas D, Roston TM, Franciosi S, Brett L, Lieve KVV, Kwok SY, Kannankeril PJ, Krahn AD, LaPage MJ, Etheridge S, Hill A, Johnsrude C, Perry J, Knight L, Fischbach P, Balaji S, Tisma-Dupanovic S, Law I, Atallah J, Backhoff D, Kamp A, Kubus P, Kean A, Aziz PF, Kovach J, Lau Y, Kron J, Clur SA, Sarquella-Brugada G, Wilde AAM, Sanatani S
Heart Rhythm 2021 Nov;18(11):1825-1832. Epub 2021 Jul 29 doi: 10.1016/j.hrthm.2021.07.061. PMID: 34333088
Clinical spectrum and long-term course of sustained ventricular tachycardia in pediatric patients: 10 years of experience.
Şengül FS, Kafalı HC, Güzeltaş A, Ergül Y
Anatol J Cardiol 2021 May;25(5):313-322. doi: 10.14744/AnatolJCardiol.2020.95759. PMID: 33960306Free PMC Article
Ventricular Dysrhythmias During Long-Term Follow-Up in Patients With Inherited Cardiac Arrhythmia.
Sitorus GDS, Ragab AAY, Houck CA, Lanters EAH, Heida A, van Gastel VE, Muskens AJQM, de Groot NMS
Am J Cardiol 2019 Nov 1;124(9):1436-1441. Epub 2019 Aug 8 doi: 10.1016/j.amjcard.2019.07.050. PMID: 31481179
Arrhythmia characterization and long-term outcomes in catecholaminergic polymorphic ventricular tachycardia.
Sy RW, Gollob MH, Klein GJ, Yee R, Skanes AC, Gula LJ, Leong-Sit P, Gow RM, Green MS, Birnie DH, Krahn AD
Heart Rhythm 2011 Jun;8(6):864-71. Epub 2011 Feb 9 doi: 10.1016/j.hrthm.2011.01.048. PMID: 21315846

Clinical prediction guides

Electrophysiological Characteristics and Ablation Outcomes in Patients With Catecholaminergic Polymorphic Ventricular Tachycardia.
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
Cardiogenetics: the role of genetic testing for inherited arrhythmia syndromes and sudden death.
Specterman MJ, Behr ER
Heart 2023 Feb 23;109(6):434-441. doi: 10.1136/heartjnl-2021-320015. PMID: 36167638
Evaluation of age at symptom onset, proband status, and sex as predictors of disease severity in pediatric catecholaminergic polymorphic ventricular tachycardia.
Kallas D, Roston TM, Franciosi S, Brett L, Lieve KVV, Kwok SY, Kannankeril PJ, Krahn AD, LaPage MJ, Etheridge S, Hill A, Johnsrude C, Perry J, Knight L, Fischbach P, Balaji S, Tisma-Dupanovic S, Law I, Atallah J, Backhoff D, Kamp A, Kubus P, Kean A, Aziz PF, Kovach J, Lau Y, Kron J, Clur SA, Sarquella-Brugada G, Wilde AAM, Sanatani S
Heart Rhythm 2021 Nov;18(11):1825-1832. Epub 2021 Jul 29 doi: 10.1016/j.hrthm.2021.07.061. PMID: 34333088
Clinical spectrum and long-term course of sustained ventricular tachycardia in pediatric patients: 10 years of experience.
Şengül FS, Kafalı HC, Güzeltaş A, Ergül Y
Anatol J Cardiol 2021 May;25(5):313-322. doi: 10.14744/AnatolJCardiol.2020.95759. PMID: 33960306Free PMC Article
Ventricular Dysrhythmias During Long-Term Follow-Up in Patients With Inherited Cardiac Arrhythmia.
Sitorus GDS, Ragab AAY, Houck CA, Lanters EAH, Heida A, van Gastel VE, Muskens AJQM, de Groot NMS
Am J Cardiol 2019 Nov 1;124(9):1436-1441. Epub 2019 Aug 8 doi: 10.1016/j.amjcard.2019.07.050. PMID: 31481179

Recent systematic reviews

Life-threatening cardiac arrhythmia and sudden death during electronic gaming: An international case series and systematic review.
Lawley CM, Tester M, Sanatani S, Prendiville T, Beach CM, Vinocur JM, Horie M, Uhm JS, Khongphatthanayothin A, Ayers MD, Starling L, Yoshida Y, Shah MJ, Skinner JR, Turner C
Heart Rhythm 2022 Nov;19(11):1826-1833. Epub 2022 Oct 11 doi: 10.1016/j.hrthm.2022.08.003. PMID: 37850595
Implantable cardioverter-defibrillator use in catecholaminergic polymorphic ventricular tachycardia: A systematic review.
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
Implantable cardioverter-defibrillator harm in young patients with inherited arrhythmia syndromes: A systematic review and meta-analysis of inappropriate shocks and complications.
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
Implantable defibrillators versus medical therapy for cardiac channelopathies.
McNamara DA, Goldberger JJ, Berendsen MA, Huffman MD
Cochrane Database Syst Rev 2015 Oct 7;2015(10):CD011168. doi: 10.1002/14651858.CD011168.pub2. PMID: 26445202Free PMC Article
Electrical storm in children.
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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