CLINICAL AND MORPHOLOGICAL APPROACH TO DIAGNOSIS OF "IDIOPATHIC" ARRHYTHMIAS AND DILATED CARDIOMYOPATHY SYNDROME AS A BASIS FOR DIFFERENTIATED THERAPY. Part I (Diagnostics)

P

Aim. To develop a comprehensive clinical and morphological approach to the nosological diagnosis and treatment of "idiopathic" arrhythmias (IA) and the syndrome of dilated cardiomyopathy (DCM).

Material and methods. Patients (n=320) with IA (n=190; 117 women, age 45.3±14.8 years) and DCM (n=130, 41 women, age 46.9±12.5 years) were included in the main group. 51 people (patients with ischemic heart disease; heart valve disease, hypertrophic cardiomyopathy, who underwent open-heart surgery; healthy volunteers) were included in the control group. Along with the standard tests evaluation of the level of anti-heart antibodies (185 patients with IA and 122 with DCM), viral serology (166 and 122), multispiral computed tomography (42 and 88), cardiac magnetic resonance imaging (41 and 22), coronary angiography (19 and 54), myocardial biopsy/autopsy (19/0 and 33/9) were performed.

Results. According to morphological study infectious-immune myocarditis was found in 78.9% of IA and 66.7% of DCM-patients, arrhythmogenic right ventricular dysplasia in 5.3% and 4.8% of patients, respectively. Other genetic cardiomyopathies, including combination with myocarditis were revealed in other patients. The frequency  of the viral genome detection in the myocardium in IA, DCM and the control group was 17.6%, 66.7% and 77.1%, respectively. However in the control group the incidence of myocarditis and anti-heart antibodies titers were significantly lower, while in the main group a strong correlation between myocarditis and anti-heart antibodies titers was found. The algorithm of noninvasive nosological diagnostics was developed; it allowed to verify diagnosis in 95% of IA patients and 89% DCM patients.

Conclusion. Nosological cause of IA and DCM syndrome can be diagnosed in most patients by using an integrated clinical and morphological approach.

1. ACC/AHA/ESC 2006 Guidelines for the Management of Patients with Atrial Fibrillation. Circulation 2006; 114(7): e257-354

2. Weijs B., Pisters R., Nieuwlaat R. et al. Idiopathic atrial fibrillation revisited in a large longitudinal clinical cohort. Europace 2012; 14(2): 184-90.

3. Ardashev AV, Sklyarov TF, Zhelyakov EG, et al. Noncoronary ventricular heart rhythm disorders: classification, clinical manifestations, diagnosis and treatment. Cardiology 2007; 47 (11): 62-72. Russian (Ардашев А.В., Склярова Т.Ф., Желяков Е.Г. и соавт. Некоронарогенные желудочковые нарушения ритма сердца: классификация, клинические проявления, диагностика и лечение. Кардиология 2007; 47 (11): 62-72)

4. Chugh S.S., Kelly K.L., Titus J.L. Sudden cardiac death with apparently normal heart. Circulation 2000; 102(6): 649-54

5. Tereshchenko SN, Alaeva EN, Narusov OYu, et al. Prevalence and diagnosis of dilated cardiomyopathy according to the Russian case. Cardiology 2012; 52 (7): 67-72. Russian (Терещенко С.Н., Алаева Е.Н., Нарусов О.Ю. и соавт. Распространенность и диагностика дилатационной кардиомиопатии по данным Российского регистра. Кардиология 2012; 52 (7): 67-72)

6. Poptsov VN, Voronina OV. Early period after orthotopic heart transplantation. In: Gauthier S., editor. Infection in transplantation. Moscow: OOO "Triada"; 2010: 175-214. Russian (Попцов В.Н., Воронина О.В. Ранний период после ортотопической трансплантации сердца. В: Готье С.В., редактор. Инфекции в трансплантологии. М.: ООО «Триада»; 2010: 175-214)

7. Frustaci A., Chimenti C., Bellocci F. et al. Histological substrate of atrial biopsies in patients with lone atrial fibrillation. Circulation 1997; 96(4): 1180-4

8. Egorov DF, Andrianov AV, Malkin EV. Endomyocardial biopsy of the right chambers in children with progressive disorders of heart rhythm and conduction. St. Petersburg.: Chelovek; 2006. Russian (Егоров Д.Ф., Адрианов А.В., Малкина Е.В. Эндомиокардиальная биопсия из правых камер у детей с прогрессирующими нарушениями ритма и проводимости сердца. СПб.: Человек; 2006).

9. Drago F., Mazza A., Gagliardi M.G. et al. Tachycardias in children originating in the right ventricular outflow tract: lack of clinical features predicting the presence and severity of the histopathological substrate. Cardiol Young 1999; 9(3): 273-9

10. Thongtang V., Chiathiraphan S., Ratanarapee S. et al. Prevalence of myocarditis in idiopathic dysrhythmias: role of endomyocardial biopsy and efficacy of steroid therapy. J Med Assoc Thai 1993; 76(7): 368- 73

11. Luk A., Metawee M., Ahn E. et al. Do clinical diagnoses correlate with pathological diagnoses in cardiac transplant patients? The importance of endomyocardial biopsy. Can J Cardiol 2009; 25(2): e48-54

12. Kühl U., Pauschinger M., Noutsias M. et al. High prevalence of viral genomes and multiple viral infections in the myocardium of adults with "idiopathic" left ventricular dysfunction. Circulation 2005; 111(7): 887-93

13. Dec G.W. Jr, Palacios I.F., Fallon J.T. et al. Active myocarditis in the spectrum of acute dilated cardiomyopathies. Clinical features, histologic correlates, and clinical outcome. N Engl J Med 1985; 312(14): 885-90

14. Hufnagel G., Pankuweit S., Richter A. et al. The European Study of Epidemiology and Treatment of Cardiac Inflammatory Diseases (ESETCID). First epidemiological results. Herz 2000; 25(3): 279-85

15. Caforio A.L., Calabrese F., Angelini A. et al. A prospective study of biopsy-proven myocarditis: prognostic relevance of clinical and aetiopathogenetic features at diagnosis. Eur Heart J 2007; 28(11): 1326-33

16. Kuethe F., Lindner J., Matschke K. et al. Prevalence of parvovirus B19 and human bocavirus DNA in the heart of patients with no evidence of dilated cardiomyopathy or myocarditis. Clin Infect Dis 2009; 49(11): 1660-6

17. Caforio A.L., Pankuweit S., Arbustini E. et al. Current state of knowledge on aetiology, diagnosis, management, and therapy of myocarditis: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J 2013; 34(33): 2636-48

18. Basso C., Corrado D., Rossi L., Thiene G. Ventricular preexcitation in children and young adults: atrial myocarditis as a possible trigger of sudden death. Circulation 2001; 103(2): 269-75

19. Frustaci A., Priori S.G., Pieroni M. et al. Cardiac histological substrate in patients with clinical phenotype of Brugada syndrome. Circulation 2005; 112(24): 3680-7

20. Mavrogeni S., Papavasiliou A., Spargias K. et al. Myocardial inflammation in Duchenne Muscular Dystrophy as a precipitating factor for heart failure: a prospective study. BMC Neurol 2010; 10:33

21. Fontaine G. The multiple facets of right ventricular cardiomyopathies. Eur Heart J 2011; 32(9): 1049-51

22. Koepsell SA, Anderson DR, Radio SJ. Parvovirus B19 is a bystander in adult myocarditis. Cardiovasc Pathol 2012; 21(6): 476-81