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3 . 2020

Myocarditis in right ventricular arrhythmogenic dysplasia/cardiomyopathy: frequency, role in phenotype development, results of treatment

Abstract

High incidence of inflammation in myocardium is well known in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/ARVC). However, the nature of myocarditis, the relationship with ARVD and its impact on the development of the phenotype, as well as the treatment options and benefits remain unclear.

The aim - to study myocarditis frequency in patients with various clinical forms of ARVD, its role in phenotype development and the effectiveness of immunosuppressive therapy.

Material and methods. The study included 54 patients [31 women (mean age 38.7±14.1 years, from 18 to 79)]. According to the revised 2010 Task Force Criteria, definite ARVD was diagnosed in 34 (63%) patients, probable - in 18 (33.3%), possible - in 2 (3.7%). Anti-heart antibodies level by indirect immunoassay, ECG, EchoCG, Holter ECG monitoring were evaluated, as well as by signal-averaged ECG (n=18), MRI (n=49) and cardiac CT with contrast enhancement (n=17) and myocardial morphological study (n=4). Genetic testing was performed in PKP2, DSG2, DSP, DSC2, JUP, TMEM43, TGFB3, PLN, LMNA, DES, CTTNA3, EMD, SCN5A, LDB3, CRYAB and FLNC genes. The average follow-up period was 21 [6; 60] months.

Results. Myocarditis frequency in patients with ARVD was 70.4% (incl. viral). Myocarditis frequency varied in different clinical forms of ARVD: 20 (74.0%) in latent arrhythmic form, 4 (36.4%) in manifested arrhythmic form, 7 (87.5%) in ARVD with progressive CHF and 7 (87.5%) in combination of ARVC with left ventricular noncompaction. Myocarditis was most significant in the formation of the disease pattern in patients with less severe genetic pre-conditions (including mutations in genes SCN5A, FLNC), as well as in the development of progressive CHF. ARVD patients with and without myocarditis did not significantly differ in the initial parameters and their dynamics. Immunosuppressive therapy (IST; hydroxychloroquine, steroids, azathioprine) was performed in 65.8% of myocarditis patients and resulted in a significant decrease in ventricular arrhythmias and lethality in comparison with the group without IST (4.0 vs 30.8%, p<0.05). Patients with ARVD and myocarditis without IST showed a tendency to decrease the left ventricular ejection fraction (from 64.3±8.8 to 57.2±9.4%, p=0.058), while in the IST group it remained stable (52.1±14.8 and 52.4±13.5%, p>0.05).

Conclusion. Myocarditis in patients with ARVD was detected in 70% сases and may have different nature: primary viral, infectious immune, secondary immune mediated. Regardless of its nature, myocarditis influences the formation of different ARVD phenotypes and requires active diagnosis. An IST of myocarditis significantly reduces the burden of ventricular arrhythmias and lethality and prevents a decrease in myocardial contractility.

Keywords:arrhythmogenic right ventricular dysplasia/cardiomyopathy, myocarditis, myocardial biopsy, anti-heart antibodies, immunosuppressive therapy

Funding. The study had no sponsor support.
Conflict of interests. The authors declare no conflict of interests.
For citation: Blagova O.V., Lutokhina Yu.A., Kogan E.A., Nedostup A.V., Zaidenov V.A., Kupriyanova A.G., Kadochnikova V.V., Donnikov A.E., Schestak A.G., Zaklyazminskaya E.V. Myocarditis in right ventricular arrhythmogenic dysplasia/cardiomyo-pathy: frequency, role in phenotype development, results of treatment. Clinical and Experimental Surgery. Petrovsky Journal. 2020; 8 (3): 59-72. DOI: https://doi.org/10.33029/2308-1198-2020-8-3-59-72 (in Russian)

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Sergey L. Dzemeshkevich
MD, Professor (Moscow, Russia)

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