Arrhythmogenic Right Ventricular Cardiomyopathy - what do we know? A review of current knowledge state

RADWAŃSKI, Jan, PAWĘSKA, Wojciech, DOMINIK, Hanna, POLATOWSKA, Marika, GADOMSKA, Anna, KUREK, Justyna, GORZYŃSKI, Rafał, CZUDY, Zuzanna, MOLCZYK-SIEŃCZAK, Justyna and GARSTKA, Michał. Arrhythmogenic Right Ventricular Cardiomyopathy - what do we know? A review of current knowledge state. Journal of Education, Health and Sport. 2023;38(1):134-145. eISSN 2391-8306. DOI http://dx.doi.org/10.12775/JEHS.2023.38.01.009 https://apcz.umk.pl/JEHS/article/view/44963 https://zenodo.org/record/8211325 The journal has had 40 points in Ministry of Education and Science of Poland parametric evaluation. Annex to the announcement of the Minister of Education and Science of 17.07.2023 No. 32318. Has a Journal's Unique Identifier: 201159. Scientific disciplines assigned: Physical Culture Sciences (Field of Medical sciences and health sciences); Health Sciences (Field of Medical Sciences and Health Sciences). Punkty Ministerialne z 2019 - aktualny rok 40 punktów. Załącznik do komunikatu Ministra Edukacji i Nauki z dnia 17.07.2023 Lp. 32318. Posiada Unikatowy Identyfikator Czasopisma: 201159. Przypisane dyscypliny naukowe: Nauki o kulturze fizycznej (Dziedzina nauk medycznych i nauk o zdrowiu); Nauki o zdrowiu (Dziedzina nauk medycznych i nauk o zdrowiu). © The Authors 2023; This article is published with open access at Licensee Open Journal Systems of Nicolaus Copernicus University in Torun, Poland Open Access. This article is distributed under the terms of the Creative Commons Attribution Noncommercial License which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author (s) and source are credited. This is an open access article licensed under the terms of the Creative Commons Attribution Non commercial license Share alike. (http://creativecommons.org/licenses/by-nc-sa/4.0/) which permits unrestricted, non commercial use, distribution and reproduction in any medium, provided the work is properly cited. The authors declare that there is no conflict of interests regarding the publication of this paper. Received: 07.07.2023. Revised:30.07.2023. Accepted: 31.07.2023. Published: 08.08.2023. Arrhythmogenic Right Ventricular Cardiomyopathy - what do we know? A review of current knowledge state Authors 1. Jan Radwański MD, ORCID: 0009-0009-1441-8057, Provincial Specialist Hospital No. 4, Aleja Legionów 10, 41-902 Bytom, Poland, jan.radwanski97@gmail.com 2. Wojciech Pawęska MD, ORCID: 0009-0007-0836-4983, District Hospital, Krakowska 3, 32-700, Bochnia, Poland, wojciech.paweska@gmail.com 3. Hanna Dominik MD, ORCID: 0000-0003-0371-2276, University Hospital, Zyty 26, 65-046 Zielona Gora, Poland, hania.dominik31@gmail.com 4. Marika Polatowska, 5th year medical student, ORCID: 0009-0007-8191-4566, Collegium Medicum University of Zielona Gora, Zyty 28, Zielona Gora 65-046, Poland, marika85588@gmail.com 5. Anna Gadomska MD, ORCID: 0009-0004-4029-3925, University Hospital, Zyty 26, 65-046 Zielona Gora, Poland, anna.gadomska@hotmail.com 6. Justyna Kurek MD, ORCID: 0009-0000-3828-9303, Clinical Hospital No. 2, Lwowska 60, 35-301 Rzeszow, Poland, jotkurek@gmail.com 7. Rafał Gorzyński MD, ORCID: 0009-0001-0815-1247, Clinical Hospital, Collegium Medicum University of Poznan, Dluga ½, 61-848 Poznan, Poland, rafal.gorzynski2@gmail.com 8. Zuzanna Czudy MD, ORCID: 0000-0002-1619-4343, University Hospital, Collegium Medicum University of Zielona Gora, Zyty 28, 65-046 Zielona Gora, Poland, zuzanna.czudy@o2.pl 9. Justyna Molczyk-Sieńczak DMD, ORCID: 0009-0007-8037-6305 NZOZ Alicja Ulanecka, Mościckiego 14, 33-100 Tarnów, Poland, justymolz@gmail.com 10. Michał Garstka MD, ORCID: 0009-0007-6152-9388 Medical Center HCP, 28 Czerwca 1956 r. 194, 61-485 Poznań, Poland, lek.garstka@gmail.com Corresponding author Jan Radwański MD, ORCID: 0009-0009-1441-8057, Provincial Specialist Hospital No. 4, Aleja Legionów 10, 41-902 Bytom, Poland, jan.radwanski97@gmail.com Abstract Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is an inherited disorder that is responsible for a considerable number of sudden death cases in young athletes. Its pathological hallmark is a progressive loss of myocytes predominantly in the right ventricle and its simultaneous replacement by fibrous and fat tissue, which in turn leads to an increased risk of potentially lethal ventricular arrhythmias to occur. Over a dozen of mutations have been confirmed as genetic basis of ARVC with most of them affecting genes encoding desmosome proteins, however in up to half of all cases the exact etiology is still unknown. By number of studies, it is implied that physical activity is the most significant environmental factor that impacts the development and course of the disease. The main focus of treatment is to prevent sudden cardiac deaths and additionally to moderate arrhythmic events and heart failure. Materials and methods: databases including “Pubmed” and “Google Scholar” Keywords: Arrhythmogenic right ventricular cardiomyopathy, ventricular fibrillation, ventricular tachycardia, implantable cardioverter-defibrillator, sudden cardiac death, heart failure Abbreviations: ARVC - Arrhythmogenic right ventricular cardiomyopathy, VF - ventricular fibrillation, VT - ventricular tachycardia, ICD - implantable cardioverter defibrillator, SCD - sudden cardiac death, HF - heart failure, nsVT - nonsustained ventricular tachycardia, ARNI - angiotensin receptor-nephrilysin inhibitor, MRA - mineralocorticoid receptor antagonist, PVC - premature ventricular complex, RV - right ventricle, LV - left ventricle, QOL - quality of life