Herz

, Volume 37, Issue 6, pp 657–663

Arrhythmogenic right ventricular cardiomyopathy

What is needed for a cure?
  • G. Thiene
  • I. Rigato
  • K. Pilichou
  • D. Corrado
  • C. Basso
Main topic

Abstract

There have been major advances in recent years in the clinical setting of arrhythmogenic right ventricular cardiomyopathy, including new diagnostic criteria, a changing spectrum of the disease with even left dominant forms, the role of cardiac magnetic resonance and electroanatomic mapping, the expanding use of genetic screening and the existence of overlapping phenotypes. Moreover, early diagnosis at pre-participation screening with sports disqualification and risk stratification for the indication of ICD have been shown to be life-saving. In addition to traditional therapies targeting arrhythmias and congestive heart failure, an effective treatment of the disease could be based on the discovery of the molecular mechanisms involved in the pathobiology of the disease in order to block the onset and progression of cell death.

Keywords

Arrhythmogenic right ventricular cardiomyopathy Pathogenesis Sudden death Therapy 

Arrhythmogene rechtsventrikuläre Kardiomyopathie

Was ist für die Therapie wichtig?

Zusammenfassung

Diagnostik und Management der arrhythmogenen rechtsventrikulären Kardiomyopathie (ARVC) wurden in den letzten Jahren erheblich verbessert. Dies betrifft die Präzisierung diagnostischer Kriterien, die Erweiterung des Krankheitsspektrums auf überwiegend linksventrikuläre Formen, die das klinische Bild dominieren können, den diagnostischen Beitrag der kardialen Magnetresonanztomograpie und des elektroanatomischen Mappings, die Umsetzung des genetischen Screenings in die Praxis und die Beobachtung, dass der klinische Phänotyp Überlappungen zu anderen Kardiomyopathieformen aufweisen kann. Die Früherkennung durch Screeninguntersuchung vor Wettkämpfen mit Ausschluss des Sportlers vom Wettkampfsport und ggf. prophylaktischer Defibrillatorbehandlung (ICD), je nach individueller Risikoeinschätzung, ist nachweislich lebensrettend. Neben der traditionellen Behandlung der Arrhythmie und der Herzinsuffizienz sollte eine zukünftige Behandlung auf diejenigen molekularen Mechanismen in der Pathobiologie der ARVC zielen, die die Auslösung oder Progression des myokardialen Zelltods verursachen.

Schlüsselwörter

Arrhythmogene rechtsventrikuläre Kardiomyopathie Pathogenese Plötzlicher Herztod Therapie 

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Copyright information

© Urban & Vogel, Muenchen 2012

Authors and Affiliations

  • G. Thiene
    • 1
  • I. Rigato
    • 1
  • K. Pilichou
    • 1
  • D. Corrado
    • 1
  • C. Basso
    • 1
  1. 1.Department of Cardiac, Thoracic and Vascular SciencesUniversity of PaduaPaduaItaly

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