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Primär elektrische Herzerkrankungen im Erwachsenenalter—

Elektrophysiologische Befunde und Therapie

Primary electrical heart disease in adults—Electrophysiological findings and therapy

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Summary

Sudden cardiac death accounts for 100,000 victims in Germany per year. Predominantely, patients with structural heart disease such as coronary artery disease or dilated cardiomyopathy are affected. However, approximately 5–10% of sudden deaths hit patients without structural disease of the heart. The proportion of young patients (< 40 years of age) in this group is even higher (10–20%). In younger patients significantly more diseases like hypertrophic cardiomyopathy, arrhythmogenic right ventricular dysplasia and primary electrical diseases of the heart could be observed such as long QT syndrome, short QT syndrome, Brugada syndrome and catecholaminergic polymorphic ventricular tachycardia. The primary electrical diseases are different concerning their electrocardiographical pattern, clinical triggers of arrhythmias, results of invasive diagnostics and therapy. Meanwhile, molecular genetic screening can reveal specific mutations of ion channels and can identify consecutive functional defects. The significance of programmed ventricular stimulation is at present unclear concerning risk stratification in patients with Brugada syndrome and short QT syndrome and of no significance in long QTsyndrome and catecholaminergic polymorphic ventricular tachycardias. The implantable cardioverter defibrillator is the therapy of choice in most symptomatic patients. With increasing knowledge as a result of sophisticated molecular genetic screening, identification of underlying ion channel defects and new details of the mechanisms of arrhythmogenesis, a potential genotype-guided therapy will gain more importance in the future.

Zusammenfassung

In Deutschland sterben pro Jahr mehr als 100 000 Menschen an einem plötzlichen Herztod. Bei einer Großzahl der verstorbenen Personen liegt ursächlich eine strukturelle Herzerkrankung wie beispielsweise eine koronare Herzerkrankung oder eine dilatative Kardiomyopathie vor. Etwa 5–10% der plötzlichen Herztodesfälle betreffen allerdings strukturell völlig herzgesunde Personen. Der Anteil jüngerer Menschen (< 40 Lebensjahre) beträgt in dieser Gruppe sogar 10–20%. Bei älteren Personen sind koronare Herzerkrankung und dilatative Kardiomyopathie für den überwiegenden Teil der plötzlichen Herztodesfälle verantwortlich. Bei jüngeren Patienten sind neben strukturellen Erkrankungen des Herzmuskels primär elektrische Erkrankungen des Herzens als wesentliche Differentialdiagnose in Betracht zu ziehen. Dazu gehören Long QT-Syndrom, Short QT-Syndrom, Brugada-Syndrom und die katecholaminerge polymorphe ventrikuläre Tachykardie. Diese Erkrankungen unterscheiden sich elektrokardiographisch, teilweise durch spezifische klinische Trigger der Arrhythmieentstehung, in der invasiven Diagnostik und im therapeutischen Vorgehen. Molekulargenetische Untersuchungen konnten bei einem Teil der jeweiligen Erkrankungen Mutationen spezifischer Ionenkanäle aufdecken. Der Stellenwert der programmierten Kammerstimulation in der Risikostratifizierung der Patienten wird derzeit beim Brugada-Syndrom diskutiert und könnte beim Short QT-Syndrom einen Beitrag leisten. Bei den anderen Erkrankungen ist sie obsolet. Der implantierbare Kardioverter- Defibrillator steht bei der Therapie der Erkrankungen in der Verhinderung des plötzlichen Herztodes an erster Stelle. Durch die zunehmenden Kenntnisse im Rahmen molekulargenetischer Untersuchungen mit Aufdeckung sowohl der zugrundeliegenden spezifischen Ionenkanaldefekte als auch der Mechanismen der Arrhythmogenese gewinnt eine medikamentöse Genotyp-spezifische Therapie an Bedeutung.

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Authors and Affiliations

  1. I. Medizinische Klinik, Universitätsklinikum Mannheim, Theodor-Kutzer-Ufer 1–3, 68167, Mannheim

    R. Schimpf

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  1. R. Schimpf
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  2. J. Kuschyk
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  3. C. Veltmann
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  4. M. Borggrefe
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  5. C. Wolpert
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Correspondence to R. Schimpf.

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Schimpf, R., Kuschyk, J., Veltmann, C. et al. Primär elektrische Herzerkrankungen im Erwachsenenalter—. Herzschr. Elektrophys. 16, 250–259 (2005). https://doi.org/10.1007/s00399-005-0492-7

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  • DOI: https://doi.org/10.1007/s00399-005-0492-7

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