Seltene Herzerkrankungen am Elektrokardiogramm erkennen

Seltene Erkrankungen
  • 132 Downloads

Zusammenfassung

Eine Reihe seltener Herzerkrankungen kann am Elektrokardiogramm (EKG) erkannt werden. Der vorliegende Beitrag illustriert die klinische Bedeutung des EKG als zentrales Diagnostikum beim Wolff-Parkinson-White-Syndrom und bei Ionenkanalerkrankungen, die häufig erst spät diagnostiziert werden, nachdem der plötzliche Herztod in betroffenen Familien ein oder mehrere Opfer gefordert hat. Zu diesen Ionenkanalerkrankungen gehören das Long-QT-Syndrom, Short-QT-Syndrom, Brugada-Syndrom und katecholaminerge polymorphe Kammertachykardien. Darüber hinaus finden sich häufig typische EKG-Befunde bei Patienten mit idiopathischen Kammertachykardien, arrhythmogener rechtsventrikulärer Kardiomyopathie, Digitalisvergiftung, Hyperkaliämie oder akutem Cor pulmonale im Rahmen einer Lungenembolie, zudem bei ausgeprägter linksventrikulärer Hypertrophie, wie etwa bei hypertrophischer Kardiomyopathie.

Schlüsselwörter

Brugada-Syndrom Long-QT-Syndrom Wolff-Parkinson-White-Syndrom Repetitive monomorphe Kammertachykardie Arrhythmogene rechtsventrikuläre Kardiomyopathie 

Recognizing rare cardiac diseases by electrocardiogram

Abstract

A number of rare cardiac diseases can be recognized by electrocardiogram (ECG). This article illustrates the clinical importance of ECG as a key diagnostic tool to detect Wolff-Parkinson-White syndrome and channelopathies, which are frequently diagnosed late after one or more affected family members have become victims of sudden cardiac death. These channelopathies include long QT syndrome, short QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia. In addition, typical ECG findings are frequently present in patients with idiopathic ventricular tachycardia, arrhythmogenic right ventricular dysplasia, digitalis intoxication, hyperkalemia, acute cor pulmonale due to pulmonary embolism, as well as severe left ventricular hypertrophy as in hypertrophic cardiomyopathy.

Keywords

Brugada syndrome Long QT syndrome Wolff-Parkinson-White syndrome Tachycardia, ventricular, repetitive monomorphic Arrhythmogenic right ventricular cardiomyopathy 

Notes

Einhaltung ethischer Richtlinien

Interessenkonflikt

W. Grimm, A. Grimm, K. Grimm und E. Efimova geben an, dass kein Interessenkonflikt besteht.

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

Literatur

  1. 1.
    Schwartz PJ, Stramba-Badiale M, Crotti L et al (2009) Prevalence of the congenital long-QT syndrome. Circulation 120:1761–1767CrossRefPubMedPubMedCentralGoogle Scholar
  2. 2.
    Heart Rhythm Society, European Heart Rhythm Association, Asia Pacific Heart Rhythm Society, Wilde AA, Horie M et al (2013) Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Europace 15:1389–1406CrossRefGoogle Scholar
  3. 3.
    Priori SG, Blomström-Lundqvist C et al (2015) 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: The Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC). Eur Heart J 36:2793–2867CrossRefPubMedGoogle Scholar
  4. 4.
    Moss AJ, Schwartz PJ, Crampton RS et al (1991) The long QT syndrome. Prospective longitudinal study of 328 families. Circulation 84:1136–1144CrossRefPubMedGoogle Scholar
  5. 5.
    Ackerman MJ, Priori SG, Willems S et al (2011) Heart Rhythm Society (HRS); European Heart Rhythm Association (EHRA). HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies. Europace 13:1077–1109CrossRefPubMedGoogle Scholar
  6. 6.
    Gussak I, Brugada P, Brugada J et al (2000) Idiopathic short QT interval: a new clinical syndrome? Cardiology 94:99–102CrossRefPubMedGoogle Scholar
  7. 7.
    Rudic B, Schimpf R, Borggrefe M (2014) Short QT syndrome—review of diagnosis and treatment. Arrhythm Electrophysiol Rev 3(2):76–79CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Mazzanti A, Kanthan A, Monteforte N et al (2014) Novel insight into the natural history of short QT syndrome. J Am Coll Cardiol 63:1300–1308CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Brugada P, Brugada J (1992) Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. A multicenter report. J Am Coll Cardiol 20:1391–1396CrossRefPubMedGoogle Scholar
  10. 10.
    Bayés de Luna A, Brugada J, Baranchuk A et al (2012) Current electrocardiographic criteria for diagnosis of Brugada pattern: a consensus report. J Electrocardiol 45:433–442CrossRefPubMedGoogle Scholar
  11. 11.
    Serra G, Baranchuk A, Bayés-De-Luna A et al (2014) New electrocardiographic criteria to differentiate the Type-2 Brugada pattern from electrocardiogram of healthy athletes with r’-wave in leads V1/V2. Europace 16:1639–1645CrossRefPubMedGoogle Scholar
  12. 12.
    Fauchier L, Isorni MA, Clementy N et al (2013) Prognostic value of programmed ventricular stimulation in Brugada syndrome according to clinical presentation: an updated meta-analysis of worldwide published data. Int J Cardiol 168:3027–3029CrossRefPubMedGoogle Scholar
  13. 13.
    Hayashi M, Denjoy I, Extramiana F et al (2009) Incidence and risk factors of arrhythmic events in catecholaminergic polymorphic ventricular tachycardia. Circulation 119:2426–2434CrossRefPubMedGoogle Scholar
  14. 14.
    Grimm W, Ritter M, Alter P, Funck R, Maisch B (2005) Bidirectional ventricular tachycardia due to digitalis intoxication. Z Kardiol 94:79–80CrossRefPubMedGoogle Scholar
  15. 15.
    Antman EM, Wenger TL, Butler VP Jr et al (1990) Treatment of 150 cases of life-threatening digitalis intoxication with digoxin-specific Fab antibody fragments. Final report of a multicenter study. Circulation 81:1744CrossRefPubMedGoogle Scholar
  16. 16.
    Grimm W (1996) Elektrokardiographie tachykarder Rhythmusstörungen. Springer, Berlin Heidelberg, S 66–93CrossRefGoogle Scholar
  17. 17.
    Grimm W, List-Hellwig E, Hoffmann J et al (1997) Magnetic resonance imaging and signal-averaged electrocardiography in patients with repetitive monomorphic ventricular tachycardia and otherwise normal electrocardiogram. Pacing Clin Electrophysiol 20:1826–1833CrossRefPubMedGoogle Scholar
  18. 18.
    Grimm W, Menz V, Hoffmann J, Maisch B (2001) Reversal of tachycardia induced cardiomyopathy following ablation of repetitive monomorphic right ventricular outflow tract tachycardia. Pacing Clin Electrophysiol 24:166–171CrossRefPubMedGoogle Scholar
  19. 19.
    Prystowsky EN, Padanilam BJ, Joshi S, Fogel RI (2012) Ventricular arrhythmias in the absence of structural heart disease. J Am Coll Cardiol 59:1733–1744CrossRefPubMedGoogle Scholar
  20. 20.
    Grimm W, Alter P, Maisch B (2004) Image of the month. Cardiac arrest due to severe hyperkalemia. Herz 29:353CrossRefPubMedGoogle Scholar
  21. 21.
    Konstantinides SV, Torbicki A, Agnelli G et al (2014) Task Force for the Diagnosis and Management of Acute Pulmonary Embolism of the European Society of Cardiology (ESC). 2014 ESC guidelines on the diagnosis and management of acute pulmonary embolism. Eur Heart J 35:3033–3069CrossRefPubMedGoogle Scholar
  22. 22.
    Maron BJ, Ommen SR, Semsarian C et al (2014) Hypertrophic cardiomyopathy: present and future, with translation into contemporary cardiovascular medicine. J Am Coll Cardiol 64:83–99CrossRefPubMedGoogle Scholar

Copyright information

© Springer Medizin Verlag GmbH, ein Teil von Springer Nature 2018

Authors and Affiliations

  1. 1.Herzzentrum Marburg, Zentrum für Innere Medizin – SP KardiologiePhilipps-Universität MarburgMarburgDeutschland
  2. 2.Julius-Maximilians-Universität WürzburgWürzburgDeutschland
  3. 3.Justus-Liebig-Universität GießenGießenDeutschland
  4. 4.Abteilung für ElektrophysiologieHerzzentrum LeipzigLeipzigDeutschland

Personalised recommendations