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Arrhythmogene rechtsventrikuläre Kardiomyopathie

Teil 2: Risikostratifikation und Therapie

Arrhythmogenic right ventricular cardiomyopathy

Part 2: risk stratification and therapeutic management

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Zusammenfassung

Die arrhythmogene rechtsventrikuläre Kardiomyopathie (ARVC) ist eine genetisch bedingte Herzmuskelerkrankung und relevante Ursache ventrikulärer Tachyarrhythmien und plötzlicher Herztodesfälle bei jungen, scheinbar herzgesunden Patienten und Sportlern. Patienten mit ARVC sollten vom Leistungssport und intensivem Training ausgeschlossen werden. Frühzeitige Diagnosestellung und Risikostratifikation sind entscheidend für eine individualisierte Therapiestrategie. Diese ist neben einer genetischen Beratung vor allem ausgerichtet auf die Suppression ventrikulärer Tachyarrhythmien und die Prävention des plötzlichen Herztodes sowie auf die Verhinderung von Krankheitsprogression und manifester Herzinsuffizienz. Patienten mit geringem Risiko für plötzlichen Herztod benötigen je nach klinischer Manifestation der Arrhythmien entweder keine spezifische Therapie oder können mit β-Blockern oder Antiarrhythmika behandelt werden. Die Katheterablation hat bei ARVC einen symptomorientierten und palliativen Ansatz vor allem bei häufigen oder medikamentös therapierefraktären ventrikulären Tachykardien. Trotz guter Akuterfolge bestehen jedoch hohe Rezidivraten im Langzeitverlauf. Bei Patienten mit hohem Risiko für plötzlichen Herztod führt die Implantation eines Kardioverter-Defibrillators (ICD) zu einer deutlichen Verbesserung der Prognose durch Erkennung und Beendigung lebensbedrohlicher Arrhythmien durch den ICD. In der Primärprävention ist jedoch die kumulative Komplikationsrate der ICD-Elektroden im Langzeitverlauf bei den meist jungen Patienten mit ARVC zu berücksichtigen. Der hier vorgeschlagene aktuelle Algorithmus zum therapeutischen Management der ARVC befindet sich in ständiger Validierung, Weiterentwicklung und Optimierung.

Abstract

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetically determined heart muscle disease and a major cause of sudden cardiac death and ventricular tachyarrhythmia in young, apparently healthy individuals and athletes. Patients affected by ARVC should be excluded from competitive sports and vigorous training. To provide optimal treatment, early diagnosis and risk stratification are mandatory and genetic counseling of families is recommended. Tailored treatment strategies aim at the prevention of ventricular tachyarrhythmia and sudden death as well as the preclusion of disease progression and symptomatic heart failure. Patients with a low risk of sudden death need either no specific treatment or can be treated with beta blockers or antiarrhythmic drugs, depending on the clinical manifestation of the arrhythmia. Catheter ablation in ARVC constitutes a symptom-oriented and palliative approach for frequently relapsing ventricular tachycardia refractory to antiarrhythmic medication. However, despite good acute results of catheter ablation, there is a high incidence of recurrence during long-term follow-up. In patients with ARVC at high risk of sudden death, implantation of an implantable cardioverter defibrillator (ICD) improves long-term survival by detection and termination of life-threatening ventricular arrhythmias. In the long term, however, the cumulative incidence of mainly lead-related complications of ICD therapy must be considered in the young population with ARVC, particularly when the indications are for primary prevention of sudden death or life-threatening arrhythmias. The proposed algorithm of therapeutic management in ARVC is under constant validation, development and refinement.

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Einhaltung ethischer Richtlinien

Interessenkonflikt. T. Wichter und M. Paul geben an, dass kein Interessenkonflikt besteht. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Authors and Affiliations

  1. Klinik für Innere Medizin/Kardiologie, Herzzentrum Osnabrück/Bad Rothenfelde, Niels-Stensen-Kliniken, Marienhospital Osnabrück, Bischofsstr. 1, 49074, Osnabrück, Deutschland

    T. Wichter

  2. Klinik für Kardiologie, Department für Kardiologie und Angiologie, Universitätsklinikum Münster, Münster, Deutschland

    M. Paul

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  1. T. Wichter
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  2. M. Paul
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Correspondence to T. Wichter.

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Wichter, T., Paul, M. Arrhythmogene rechtsventrikuläre Kardiomyopathie. Kardiologe 8, 179–196 (2014). https://doi.org/10.1007/s12181-014-0556-1

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