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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