Professional paper

https://doi.org/10.15836/ccar2019.12

Arrhythmogenic right ventricular cardiomyopathy

Juraj Jug orcid.org/0000-0002-3189-1518 ; University of Zagreb, School of Medicine, Zagreb, Croatia
Martina Lovrić Benčić orcid.org/0000-0001-8446-6120 ; University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia

Abstract

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare progressive autosomal dominant genetic disease characterized by structural abnormalities of the right ventricle of the heart and
the appearance of the reentry type arrhythmias. Of the 140 different genetic mutations, the most significant are those related to proteins in the intercalated discs and myocardial desmosomes. Myocyte degeneration and death, which are caused by disrupted myocyte growth and differentiation, are most obvious in the triangle of dysplasia and result in very non-specific symptoms. Arrhythmias caused by ARVC are
considered the main cause of death in persons below 40 years of age. Since there are numerous other diseases that resemble ARVC, the diagnosis of ARVC is established based on a combination of fulfilling the
working group criteria and magnetic resonance imaging, heart biopsy, and echocardiography findings. Despite all the diagnostic criteria, more than 50% of real ARVC cases remain inadequately diagnosed. This is why detailed and frequent health checkups from an early age are recommended in asymptomatic athletes with family history positive for ARVC. The main goal of treatment is the prevention of sudden cardiac death. Sotalol and amiodarone are favored in medication therapy, with the goal of preventing the development of malignant arrhythmias. In patients with a very high risk of such arrhythmias the implantation of a cardioverter defibrillator is absolutely indicated as part of primary prevention. The successfulness of radiofrequency ablation is lower in this disease due to the frequent appearance of new arrhythmic foci caused by fatty fibrotic changes in the myocardium and is considered a palliative treatment method. Definitive treatment for this diseases can be achieved only through heart transplantation.

Keywords

arrhythmogenic right ventricular cardiomyopathy; heart failure; sudden cardiac death; ventricular extrasystoles

Hrčak ID:

218121

URI

https://hrcak.srce.hr/218121

Publication date:

18.3.2019.

Article data in other languages: croatian

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