Zusammenfassung
Die häufigste Ursache für den plötzlichen Herztod ist eine zugrundeliegende koronare Herzerkrankung. Nur selten sind scheinbar gesunde junge Menschen von einem plötzlichen Herztod betroffen. Bei diesen Patienten wird meist eine elektrische oder eine genetisch bedingte strukturelle Herzerkrankung diagnostiziert. Zu diesen seltenen, dem plötzlichen Herztod junger Menschen aber häufig zugrundeliegenden Erkrankungen bzw. Syndromen gehören vor allem die arrhythmogene rechtsventrikuläre Kardiomyopathie, die hypertrophe Kardiomyopathie, das lange und kurze QT-Syndrom, das Brugada-Syndrom, die katecholaminerge polymorphe Kammertachykardie und das Syndrom der frühen Repolarisation. Die Patienten fallen klinisch durch Synkopen oder ein Reanimationsereignis auf. Gelegentlich wird eine Erkrankung auch zufällig oder im Rahmen einer Familienuntersuchung diagnostiziert. Die Schwierigkeit besteht für die Primärprophylaxe darin, besonders gefährdete Patienten zu identifizieren. Anhand verschiedener Risikofaktoren muss das Risiko individuell abgeschätzt und gegen die Nachteile einer ICD-Implantation bei den überwiegend jungen Patienten abgewogen werden.
Abstract
The most frequent cause of sudden cardiac death (SCD) is underlying coronary artery disease. Healthy appearing young individuals are affected in a minority of cases. These individuals are usually diagnosed with electrical or genetically determined structural heart disease. Arrhythmogenic right ventricular cardiomyopathy, hypertrophic cardiomyopathy, long and short QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, and early repolarization syndrome are generally considered rare underlying causes of SCD in these young patients. Affected patients typically present with syncope or cardiac arrest. Occasionally, disease is diagnosed during family screening. Risk stratification is difficult in this patient population. Risk of sudden death has to be weighed individually against risks associated with an implantable cardioverter defibrillator (ICD) in these young patients.
Literatur
Antzelevitch C, Brugada P, Borggrefe M et al (2005) Brugada syndrome: report of the second consensus conference: endorsed by the Heart Rhythm Society and the European Heart Rhythm Association. Circulation 111:659–670
Antzelevitch C, Yan G-X (2010) J wave syndromes. Heart Rhythm ■
Basso C, Corrado D, Marcus FI et al (2009) Arrhythmogenic right ventricular cardiomyopathy. Lancet 373:1289–1300
Brugada J, Brugada R, Antzelevitch C et al (2002) Long-term follow-up of individuals with the electrocardiographic pattern of right bundle-branch block and ST-segment elevation in precordial leads V1 to V3. Circulation 105:73–78
Budzikowski AS, Daubert JP, Sesselberg HW et al (2007) Non-inducibility of ventricular tachycardia does not prevent low likelihood of appropriate therapy in patients with ARVD. Heart Rhythm 4:218–219
Buja G, Estes NAM, Wichter T et al (2008) Arrhythmogenic right ventricular cardiomyopathy/dysplasia: Risk stratification and therapy. Prog Cardiovasc Dis 50(4):282–293
Corrado D, Leoni L, Link MS et al (2003) Implantable cardioverter-defibrillator therapy for prevention of sudden death in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia. Circulation 108:3084–3091
Christiaans I, Engelen K van, Langen I van et al (2010) Risk stratification for sudden cardiac death in hypertrophic cardiomyopathy: systemativ review of clinical risk markers. Europace 12:313–321
Eckardt L, Kirchhof P, Loh P et al (2002) Electrophysiologic investigation in Brugada Syndrome: Yield of programmed ventricular stimulation at two ventricular sites with up to three premature beats. Eur Heart J 23:1394–1401
Eckardt L, Probst V, Smits JPP et al (2005) Long-term prognosis of individuals with right precordial ST-segment-elevation Brugada Syndrome. Circulation 111:257–263
Epstein AE, DiMarco JP, Ellenbogen KA et al (2008) ACC/AHA/HRS Guidelines for device-based therapy: Executive summary. Circulation 117:e350–e408
Gaita F, Giustetto C, Bianchi F et al (2003) Short QT syndrome: A familial cause of sudden death. Circulation 108:965–970
Giustetto C, Di Monte F, Wolpert C et al (2006) Short QT syndrome: Clinical findings and diagnostic-therapeutic implications. Eur Heart J 27:2440–2447
Goldenberger I, Moss A (2008) Long QT Syndrome. J Am Coll Cardiol 51:2291–2300
Gussak I, Brugada P, Brugada J et al (2000) Idiopathic short QT interval: a new clinical syndrome? Cardiology 94:99–102
Haissaguerre M, Derval N, Sacher F et al (2008) Sudden cardiac death associated with early repolarization. N Engl J Med 358:2016–2023
Kaufman ES, McNitt S, Moss AJ et al (2008) Risk of death in the long QT syndrome when a sibling has died. Heart Rhythm 5:831–836
Marcus FI, McKenna WJ, Sherrill D et al (2010) Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia. Proposed modification of the task force criteria. Circulation 121:000–000
Maron BJ, McKenna WJ, Danielson GK et al (2003) American College of Cardiology/European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathy: a report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiologs Committee for Practice Guidelines. J Am Coll Cardiol 42:1687–1713
Maron B, Spirito P, Shen WK et al (2007) Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy. JAMA 298(4):405–412
Maron BJ, Spirito P (2008) Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy. J Cardiovasc Electrophysiol 19:1118–1126
Maron B (2010) Contemporary insights and strategies for risk stratification and prevention of sudden death in hypertrophic cardiomyopathy. Circulation 121:445–456
Napolitano C, Priori SG (2007) Diagnosis and treatment of catecholaminergic polymorphic ventricular tachycardia. Heart Rhythm 4:675–678
Paul M, Gerss J, Schulze-Bahr E et al (2007) Role of programmed ventricular stimulation in patients with Brugada syndrome: a meta-analysis of worldwide published data. Eur Heart J 28:2126–2133
Pezawas T, Stix G, Kastner J et al (2006) Ventricular tachycardia in arrhythmogenic right ventricular dysplasia/cardiomyopathy: Clinical presentation, risk stratification and results of long-term follow-up. Int J Cardiol 107:360–368
Piccini JP, Dalal D, Roguin A et al (2005) Predictors of appropriate implantable defibrillator therapies in patients with arrhythmogenic right ventricular dysplasia. Heart Rhythm 2:1188–1194
Priori SG, Napolitano C, Gasparini M et al (2002) Natural history of Brugada syndrome: insights for risk stratification and management. Circulation 105:1342–1347
Priori SG, Napolitano C, Memmi M et al (2002) Clinical and molecular characterization of patients with catecholaminergic polymorphic ventricular tachycardia. Circulation 106:69–74
Priori SG, Schwartz PJ, Napolitano C et al (2003) Risk stratification in the long-QT syndrome. N Engl J Med 348:1866–1874
Probst V, Veltmann C, Eckardt L et al (2010) Long-term prognosis of patients diagnosed with Brugada Syndrome. Results from the FINGER Brugada Syndrome Registry. Circulation 121:635–643
Sarkozy A, Boussy T, Kourgiannides et al (2007) Long-term follow-up of primary prophylactic implantable cardioverter-defibrillator therapy in Brugada syndrome. Eur Heart J 28:334–344
Schimpf R, Bauersfeld U, Gaita F et al (2005) Short QT syndrome: successful prevention of sudden cardiac death in an adolescent by implantable cardioverter-defibrillator treatment for primary prophylaxis. Heart Rhythm 2:416–417
Schwartz P, Spazzolini C, Crotti L (2009) All LQT3 patients need an ICD: True or false? Heart Rhythm 6:113–120
Wilde AA, Bhuiyan ZA, Crotti L et al (2008) Left cardiac sympathetic denervation for catecholaminergic polymorphic ventricular tachycardia. N Engl J Med 358:2024–2029
Willems S, Eckardt L, Hoffmann E et al (2007) Leitlinie invasive elektrophysiologische Diagnostik. Clin Res Cardiol 96:634–651
Zareba W, Moss AJ, Daubert JP et al (2003) Implantable cardioverter defibrillator in high-risk long QT Syndrome patients. J Cardiovasc Electrophysiol 14:337–341
Erklärung zum Interessenkonflikt
Prof. Dr. Eckardt erhielt Reisekostenübernahmen und Honorare für Vorträge von Biotronic, Medtronic, St. Jude Medical, Sanofi. Dr. J. Köbe erhielt Reisekostenübernahmen und Honorare für Vorträge von Biotronic, Medtronic, St. Jude Medical, Boston Scientific und Sanofi. PD Dr. C. Pott erhielt Reisekostenübernahmen von Sanofi. Dr. K. Wasmer erhielt Reisekostenübernahmen und Honorare für Vorträge von Biotronic, Medtronic, St. Jude Medical und Sanofi.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Wasmer, K., Köbe, J., Pott, C. et al. ICD-Therapie zur Primärprävention. Herzschr. Elektrophys. 21, 117–122 (2010). https://doi.org/10.1007/s00399-010-0079-9
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00399-010-0079-9