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Ventrikuläre Arrhythmien bei Ionenkanalerkrankungen

Ventricular arrhythmias in ion channel diseases

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Zusammenfassung

Bei Patienten mit Ionenkanalerkrankungen treten in nahezu allen Fällen polymorphe ventrikuläre Tachykardien (VT), Torsade-de-Pointes-Tachykardien oder Kammerflimmern auf. Eine schnelle monomorphe VT ist eine absolute Rarität. Daher sollten implantierbare Kardioverter-Defibrillatoren (ICD) immer so eingestellt werden, dass sie erst bei hohen Herzfrequenzen therapieren, um unnötige Schocks oder Überstimulationen zu vermeiden. Während bei idiopathischem Kammerflimmern, wie z. B. bei DPP6-Mutation und der katecholaminergen polymorphen ventrikulären Tachykardie (CPVT), in Ruhe keinerlei EKG-Auffälligkeiten zu sehen sind, treten bei den anderen Ionenkanalerkrankungen bereits in Ruhe, wenn auch häufig nur transient, schon Alterationen auf. Diese sind für die Risikostratifizierung von nicht unerheblicher Bedeutung.

Abstract

In patients with ion channel disease the predominant arrhythmias are polymorphic ventricular tachycardias (VT), torsade de pointes tachycardia and ventricular fibrillation (VF). In only extremely rare cases is very rapid monomorphic ventricular tachycardia observed. This is why implantable cardioverter–defibrillators (ICDs) should always be programmed for treatment of VF only with high detection rates to avoid inappropriate discharges. In idiopathic VF and catecholaminergic polymorphic ventricular tachycardia (CPVT), no baseline electrocardiographic abnormalities can be detected, whereas in Brugada syndrome, long QT syndrome, early repolarisation syndrome and Anderson–Tawil syndrome alterations of the baseline ECG are very important to identify patients at risk.

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Literatur

  1. di Barletta MR, Viatchenko-Karpinski S, Nori A, Memmi M, Terentyev D, Turcato F, Valle G, Rizzi N, Napolitano C, Gyorke S, Volpe P, Priori SG (2006) Clinical phenotype and functional characterization of CASQ2 mutations associated with catecholaminergic polymorphic ventricular tachycardia. Circulation 114:1012–1019

    Article  PubMed  Google Scholar 

  2. Brugada J, Brugada R, Brugada P (2003) Determinants of sudden cardiac death in individuals with the electrocardiographic pattern of Brugada syndrome and no previous cardiac arrest. Circulation 108:3092–3096

    Article  PubMed  Google Scholar 

  3. Calo L, Giustetto C, Martino A, Sciarra L, Cerrato N, Marziali M, Rauzino J, Carlino G, De Ruvo E, Guerra F, Rebecchi M, Lanzillo C, Anselmino M, Castro A, Turreni F, Penco M, Volpe M, Capucci A, Gaita F (2016) A new electrocardiographic marker of sudden death in Brugada syndrome: the S‑wave in lead I. J Am Coll Cardiol 67(12):1427–1440

    Article  PubMed  Google Scholar 

  4. Cerrone M, Noujaim SF, Tokacheva EG, Talkachou A, O’Connell R, Berenfeld O, Anumonwo J, Pandit SV, Vikstrom K, Napolitano C, Priori SG, Jalife J (2007) Arrhythmogenic mechanisms in a mouse model of catecholaminergic polymorphic ventricular tachycardia. Circ Res 101:1039–1048

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Chan KH, Sy RW (2016) Catheter ablation of recurrent ventricular fibrillation: a literature review and case examples. Heart Lung Circ 25:784–790

    Article  PubMed  Google Scholar 

  6. Conte G, Dewals W (2014) Drug-induced Brugada syndrome in children: clinical features, device-based management and long-term follow-up. J Am Coll Cardiol 63:2272–2279

    Article  PubMed  Google Scholar 

  7. Delannoy E, Sacher F (2013) Cardiac characteristics and long-term outcome in Andersen-Tawil syndrome patients related to KCNJ2 mutation. Europace 15:1805–1811

    Article  PubMed  Google Scholar 

  8. Fujii Y, Itoh H, Ohno S, Murayama T, Kurebayahi N, Aoki H, Blancard M, Nagakawa Y, Yamamoto S, Matsui Y, Ichikawa M, Sonoda K, Ozawa T, Ohkubo K, Watanabe I, Guicheney P, Horie M (2017) A type 2 ryanodine receptor variant associated with reduced Ca2+-release and short-coupled torsades de pointes ventricular arrhythmia. Heart Rhythm 14:98–107

    Article  PubMed  Google Scholar 

  9. Gaita F, Giustetto C, Bianchi F, Wolpert C, Schimpf R, Riccardi R et al (2003) Short QT syndrome: a familial cause of sudden death. Circulation 108:965–970

    Article  PubMed  Google Scholar 

  10. Gaita F, Giustetto C, Bianchi F, Schimpf R, Haissaguerre M, Calo L (2004) Short QT syndrome: pharmacological treatment. J Am Coll Cardiol 43:1494–1499

    Article  CAS  PubMed  Google Scholar 

  11. Giustetto C, Di Monte F, Wolpert C, Borggrefe M, Schimpf R, Sbragia P, Leone G, Maury P, Anttonen O, Haissaguerre M, Gaita F (2006) Short QT syndrome: clinical findings and diagnostic-therapeutic implications. Eur Heart J 27:2440–2447

    Article  PubMed  Google Scholar 

  12. Igarashi M, Tada H, Kurosaki K, Yamasaki H, Akiyama D, Sekiguchi Y, Kuroki K, Machin T, Murakoshi N, Nakata Y, Kuga K, Nogami A, Aonuma K (2012) Electrocardiographic determinants of the polymorphic QRS morphology in idiopathic right ventricular outflow tract tachycardia. J Cardiovasc Electrophysiol 23:521–526

    Article  PubMed  Google Scholar 

  13. Kautzner J, Peichl P (2013) Catheter ablation of polymorphic ventricular tachycardia and ventricular fibrillation. Arrhythm Electrophysiol Rev 2:135–140

    Article  PubMed  PubMed Central  Google Scholar 

  14. Kimura H, Zhou J (2012) Phenotype variability in patients carrying KCNJ2 mutations. Circ Cardiovasc Genet 5:344–353

    Article  CAS  PubMed  Google Scholar 

  15. Leenhardt A, Denjoy I (2012) Catecholaminergic polymorphic ventricular tachycardia. Circ Arrhythm Electrophysiol 5:1044–1052

    Article  PubMed  Google Scholar 

  16. Leenhardt A, Lucet V, Denjoy I, Grau F, Ngoc DD, Coumel P (1995) Catecholaminergic polymorphic ventricular tachycardia in children: a 7-year follow-up of 21 patients. Circulation 91:1512–1519

    Article  CAS  PubMed  Google Scholar 

  17. Marquez MD, Nava S, Gómez J, Colin L, Iturralde P (2014) Lack of efficacy of radiofrequency catheter ablation in Anderesen-Tawil syndrome: are we targeting the right sport? Europace 16:1697–1698

    Article  PubMed  Google Scholar 

  18. Moss AJ, Zareba W, Hall WJ, Schwartz PJ, Crampton RS, Benhorin J, Vincent GM, Locati EH, Priori SG, Napolitano C, Medina A, Zhang L, Robinson JL, Timothy K, Towbin JA, Andres ML (2000) Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. Circulation 101:616–623

    Article  CAS  PubMed  Google Scholar 

  19. Murakoshi N, Aonuma K (2016) Catheter ablation for ventricular tachyarrhythmias in patients with channelopathies. J Arrhythm 32(5):404–410

    Article  PubMed  PubMed Central  Google Scholar 

  20. Napolitano C, Priori SG (2007) Diagnosis and treatment of catecholaminergic polymorphic ventricular tachycardia. Heart Rhythm 4:675–678

    Article  PubMed  Google Scholar 

  21. Priori SG, Schwartz PJ, Napolitano C, Bloise R, Ronchetti E, Grillo M, Vicentini A, Spazzolini C, Nastoli J, Bottelli G, Folli R, Cappelletti D (2003) Risk stratification in the long-QT syndrome. N Engl J Med 348:1866–1874

    Article  PubMed  Google Scholar 

  22. Priori SG, Gasparini M, Napolitano C, Della BP, Ottonelli AG, Sassone B, Giodano U, Pappone C, Mascioli G, Rossetti G, De Nardis R, Colombo M (2012) Risk stratification in Brugada syndrome. Results of the PRELUDE Registry. J Am Coll Cardiol 59(1):37–45

    Article  PubMed  Google Scholar 

  23. Sadek MM, Benhayon D, Sureddi R, Chik W, Santangeki P, Supple GE, Hutchinson MD, Bala R, Carballeira L, Zado ES, Patel VV, Callans DJ, Marchlinksi FE, Garcia FC (2015) Idiopathic ventricular arrhythmias originating from the moderator band: electrocardiographic characteristics and treatment by catheter ablation. Heart Rhythm 12:67–75

    Article  PubMed  Google Scholar 

  24. Santoro F, Di Biase L, Hranitzky P, Sanchez J, Santangeli P, Perini AP, Burkhardt JD, Natale A (2014) Ventricular fibrillation triggered by PVCs from papillary muscles. J Cardiovasc Electrophysiol 25:1158–1164

    Article  PubMed  Google Scholar 

  25. Shirai Y, Goya M, Ohno S, Horie M, Doi S, Isobe M, Hirao K (2017) Elimination of ventricular arrhythmia in catecholaminergic polymorphic ventricular tachycardia by targeting “catecholamine-sensitive Area”: a dominant-subordinate relationship betweeen origin sites of bidrectional ventricular premature contractions. Pacing Clin Electrophysiol. doi:10.1111/pace.13006

    PubMed  Google Scholar 

  26. Sy RW, Gollob MH (2011) Arrhythmia characterization and long-term outcomes in catecholaminergic polymorphic ventricular tachycardia. Heart Rhythm 8:864–871

    Article  PubMed  Google Scholar 

  27. Ten Sande JN, Postema PG, Boekholdt SM, Tan HL, van der Heijden JF, de Groot NM, Volders PG, Zeppenfeld K, Boersma LV, Nannberg EA, Christiaans I, Wilde AA (2016) Detailed characterization of familial idiopathic ventricular fibrillation linked to the DPP6 locus. Heart Rhythm 13:905–912

    Article  PubMed  Google Scholar 

  28. Viskin S, Lesh MD, Eldar M, Fish R, Setbon I, Laniado S, Belhassen B (1997) Mode of onset of malignant ventricular arrhythmias in idiopathic ventricular fibrillation. J Cardiovasc Electrophysiol 8:1115–1120

    Article  CAS  PubMed  Google Scholar 

  29. Viskin S, Rosso R, Rogowski O, Belhassen B (2005) The “short-coupled” variant of right ventricular outflow ventricular tachycardia: a not-so-benign form of benign ventricular tachycardia. J Cardiovasc Electrophysiol 16:912–916

    Article  PubMed  Google Scholar 

  30. Wolpert C, Parade U, Herrera-Siklody C, Strotmann C, Rüb N (2014) Ion channel diseases in children. Herzschrittmacherther Elektrophysiol 25:148–157

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Medizinische Klinik für Kardiologie, Nephrologie und Internistische Intensivmedizin, Klinikum Ludwigsburg, Posilipostrasse 4, 71640, Ludwigsburg, Deutschland

    Christian Wolpert, Mara Vogel, Christian Nagel, Claudia Herrera-Siklody & Norman Rüb

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  1. Christian Wolpert
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  2. Mara Vogel
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  3. Christian Nagel
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Correspondence to Christian Wolpert.

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C. Wolpert, M. Vogel, C. Nagel, C. Herrera-Siklody und N. Rüb geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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Wolpert, C., Vogel, M., Nagel, C. et al. Ventrikuläre Arrhythmien bei Ionenkanalerkrankungen. Herzschr Elektrophys 28, 169–176 (2017). https://doi.org/10.1007/s00399-017-0510-6

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  • DOI: https://doi.org/10.1007/s00399-017-0510-6

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