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Russian Journal Of Cardiology, 2018, 2 (23)

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Russ J Cardiol 2018, 2 (154): 5

CLINICAL MEDICINE NEWS

Clinical medicine updates: a review of international news

Russ J Cardiol 2018, 2 (154): 6

ORIGINAL ARTICLES

PREDICTIVE VALUE OF THE GLOBAL LONGITUDINAL STRAIN IN HYPERTROPHIC CARDIOMYOPATHY PATIENTS

Komissarova S. М.1, Zakharova Е. Yu.1, Sevruk Т. V.1, Ustinova I. B.1, Krasko О. V.2

Abstract

Aim. To evaluate prognositc role of the decrease of global longitudinal strain as a predictor of adverse outcomes in a large cohort or hypertrophic cardiomyopathy patients (HCMP).

Material and methods. Totally, 262 HCMP patients investigated (162 males, 100 females) at the age 16-79 y. o. (median 48 y. o.), who, together with routine echocardiographical parameters, underwent registration of 2D Strain and estimation of the value of global longitudinal deformity. Clinical endpoints included mortality related to chronic heart failure (CHF) and hospitalization for CHF progression.

Results. During the follow-up (median 2,5 (0,2...5,1) years) in 11 among 262 patients (4,2%) with HCMP, there were adverse outcomes registered: sudden cardiac death (SCD) in 3 patients, resuscitated SCD and implanted cardioverter (ICD) — 1 patient, ICD shocks due to sustained ventricular tachicardia — in 2 patients, fatal outcome due to progression of CHF to an end stage — 5 patients, hospitalization due to CHF progression to III FC — 16 patients. Multifactorial analysis showed that independent factors for fatal outcome and hospitalization were the following: decrease of global longitudinal strain <13% (HR 28,5; 95% CI 2,9-278, p=0,004), AF episodes (HR 3,3; 95% CI 1,2-8,9, р=0,02) and CHF of III-IV FC NYHA (HR 4,2; 95% CI 1,2-14,9, р=0,028).

Conclusion. The value of global longitudinal strain is associated with the development of adverse events related to progression of CHF in HCMP and might be applied in identification of patients with high risk of adverse CHF progression.

Russ J Cardiol 2018, 2 (154): 7–12

dx.doi.org/10.15829/1560-4071-2018-2-7-12

Key words: hypertrophic cardiomyopathy, global longitudinal strain, adverse events prediction.

1Republic Scientific and Practical Center “Cardiology”, Minsk; 2SSI United Institute of Informatics Problems, Minsk, Belarus.

GENDER DIFFERENCES OF CLINICAL MANIFESTATION AND CARDIAC REMODELING IN IDIOPATHIC HYPERTROPHIC CARDIOMYOPATHY IN ELDERLY P ATIENTS

Polyakova А.А.1,2, Baranova Е.I.1,2, Semernin Е.N.1,2, Krutikov А.N.2, Melnik О.V.2, Pyko S.А.2,3, Davydova V.G.2, Kostareva А.А.1,2, Gudkova А.Ya.1,2

Abstract

Aim. To study gender differences of clinical course and myocardial remodeling in elderly patients with idiopathic hypertrophic cardiomyopathy (HCM).

Material and methods. The study included 131 patients with idiopathic HCM. Patients underwent standard clinical, laboratory and instrumental diagnostics.

Results. In the elderly patients with idiopathic HCM, proportion of females was 63% (n=82), males — 37% (n=49). Mean age of females 69±7 y. o., males — 68±7 y. o. Coronary artery disease (CAD) was more common in males (32%) than in females (22%), but with no significant difference (p=0,2). Atrial fibrillation was more common in males (49% vs 29%, respectively, p=0,03). Size of the left atrium and enddiastolic size of the left ventricle in males exceeded those in females (51,2±9,0 mm versus 46,3±4,7 mm, 51,5±7,6 mm versus 45,6±5,7 mm, respectively, p=0,01). In males, symmetrical myocardial remodeling was found more often (42% vs 25%, p=0,04). Obstructive form of HCM was predominant in females (45% and 14%, p=0,01). Chronic heart failure (CHF) with NYHA class III was found in 29% in female group (n=24) and in 12% in male group (n=6), with a tendency to difference (p=0,06). In females, CHF with NYHA class III was mostly due to the left ventricle outflow tract obstruction (n=13) and dilatation phase (n=3). In males, almost all cases of CHF in NYHA class III-IV (8 of 9 patients) were a result of combination of HCM with CAD and previous myocardial infarction. In males, the ejection fraction was significantly lower (55,7±14,8% versus 62,2±10,9%, p=0,01).

Conclusion. Proportion of females was higher in elderly patients with idiopathic HCM. Females with HCM were characterized by a more severe course of the disease due to the left ventricle outflow tract obstruction and dilatation phase. In males with idiopathic HCM there were registered more often the following: atrial fibrillation, larger left atrium and end-diastolic size of the left ventricle, symmetrical myocardial remodeling, lower ejection fraction of the left ventricle, — probably associated with a combination of HCM with CAD and previous myocardial infarction.

Russ J Cardiol 2018, 2 (154): 13–18

dx.doi.org/10.15829/1560-4071-2018-2-13-18

Key words: idiopathic hypertrophic cardiomyopathy, elderly, gender differences.

1Pavlov First Saint-Petersburg State Medical University of the Ministry of Health, Saint-Petersburg; 2Scientific Medical Research Center of the Ministry of Health, Saint-Petersburg; 3V. I. Ulianov (Lenin) Saint-Petersburg State Electrotechnical University “LETI”, Saint-Petersburg, Russia.

CLINICAL TYPES (CLASSIFICATION) OF THE RIGHT VENTRICLE ARRHYTHMOGENIC DYSPLASIA: SPECIFICS OF DIAGNOSTICS AND MANAGEMENT

Lutokhina Yu. A.1, Blagova О. V.1, Nedostup А. V.1, Shestak А. G.2, Zaklyazminskaya Е. V.2,3

Abstract

Aim. To classify established clinical types of the right ventricle arrhythmogenic dysplasia (RVAD) taken a variety of genetic and inflammatory mechanisms, and to analyze the specifics of differential diagnostics and management of the respective types.

Material and methods. Main group consisted of 50 patients with evident (n=26), probable (n=13) and possible (n=11) RVAD diagnosis, mean age 38,1±14,6 y. o., males — 20 (40%), follow up time 13,5 [4; 34] months. Comparison group consisted of 58 patients with some of the RVAD criteria insufficient for evident diagnosis. All patients underwent ECG, Holter ECG 24 hours, EchoCG; in the main group additionally — DNA-diagnostics (n=46), cardiac MRI (n=44), high definition ECG (n=16), endomyocardial biopsy of the RV (n=2), autopsy (n=2). In comparison group, MRI was done in 32 patients, biopsy to 7, and in 1 case — autopsy.

Results. Based upon the clinical data and specifics of the disease course, 4 types of established clinical RVAD were selected, that do not tend to overlap: latent arrhythmic (50% patients), manifest arrhythmic (20%), RVAD with predominant biventricular chronic heart failure (CHF, 16%), and RVAD with non-compaction left ventricle myocardium (14%). The development of one or another type is based on genetic factors, as on comorbid myocarditis (in percent in the following, respectively). In diagnostics of the latent arrhythmic type (frequent right ventricle extrasystoly, VE and/or non-sustained right ventricular tachicardia, VT) the key role played female sex, syncopes in anamnesis (16%), family history of sudden death (12%), ECG-criteria and positive results of DNA diagnostics (24%). For manifest arrhythmic type (sustained VT) — sudden death family anamnesis (in 20%), MRIcriteria (enlarged RV with lower EF), ECG-criteria and positive DNA tests (50%). For RVAD with progressing CHF — sustained VT (50%), syncopes (37,5%), predominance of RV failure with its severely reduced EF (25,7±15,0%), major MRIand ECG-criteria, decreased QRS-voltage and positive DNA test (38%). Comorbidity of RVAD and non-compaction myocardium differ by frequent VE, aggressive VT (57,1%), syncope (42,9%) and CHF with significantly lower than in DCMP EF LV. Mortality rate in I-IV types was, respectively, 0%, 10%, 25%, 14,3%, and relevant shocks in 8 of 13 (61,5%) patients with ICD.

Conclusion. It is worthy to use the proposed RVAD classification in clinical practice to define the spectrum of diagnostical and management events and assess the individual patient prognosis.

Russ J Cardiol 2018, 2 (154): 19–31

dx.doi.org/10.15829/1560-4071-2018-2-19-31

Key words: arrhythmogenic dysplasia/cardiomyopathy of the right ventricle, ventricular extrasystoly, ventricular tachicardia, myocarditis, endomyocardial byopsy, chronic heart failure, non-compaction myocardium.

1V. N. Vinogradov Faculty Clinics of Therapy, I. M. Sechenov First Moscow State Medical University of the Ministry of Health, Moscow; 2V. B. Petrovsky Russian National Research Centre of Surgery, Moscow; 3N. I. Pirogov Russian National Research Medical University (RNRMU), Moscow, Russia.

GENETIC MARKERS OF MYOCARDIAL FIBROSIS: OPPORTUNITY TO P REDICT ADVERSE OUTCOMES IN AORTIC STENOSIS

Tipteva Т. А.1, Chumakova О. S.1,2, Brovkin А. N.3, Nikitin А. G.3, Reznichenko N. Е.2, Zateyshchikov D. А.1,3,4

Abstract

Aim. To evaluate the influence of genetic markers of inflammation and fibrosis on survival rate of patients with non operated aortic stenosis (AS). The search for novel prognostically adverse factors in calcinated AS patients with none surgical treatment remains actual.

Material and methods. During 2,1±0,11 years, 191 patient has been followed up (28,6% males, 78,0±0,60 y. o.) with a “natural course” of AS (aortic valve area ≤2,0 cm2). All fatal outcomes (FO) were collected. Then the clinical, biochemical and echocardiographic parameters were compared, genotypes frequencies and alleles of mononucleotide polymorphisms (MNP) G(-238)A and G(-308)A gene TNF, С(-592)A gene IL-10, Arg25Pro gene TGFβ1 in groups with FO and with none. Life duration towards FO was evaluated.

Results. Fatal outcomes were registered in 71 (37,2%) of patients. Death causes were: myocardial infarction in 5, stroke in 8, sudden death in 10, chronic heart failure (CHF) in 29, thromboembolism in 5, non-cardiac cause in 14. Independent factors associated with FO were: presence of CHF III-IV FC NYHA, creatinine level, aortic valve area, myocardial mass index of the left ventricle and carriage of allele Pro MNP Arg25Pro gene TGFβ1 (р<0,05). Associations of MNP G(-238)A and G(-308)A gene TNF, as MNP С(-592)A gene IL-10 with FO were not found. Mean life duration before death in Pro MNP Arg25Pro gene TGFβ1 carriers was 468,6±99,62 days versus 617,6±77,19 days in non-carriers (р<0,05).

Conclusion. The carriage of allele Pro MNP Arg25Pro gene TGFβ1 can be regarded as one more predictor of fatal outcome in patients with non-operated AS. It is clear that LV myocardial fibrosis, developing in AS patients, might be a factor significantly influencing prognosis.

Russ J Cardiol 2018, 2 (154): 32–38

dx.doi.org/10.15829/1560-4071-2018-2-32-38

Key words: aortic stenosis, prognosis, IL-10, TGFβ1, TNF, fibrosis.

1Central State Medical Academy of the President Office, Moscow; 2City Clinical Hospital № 17 of Moscow Department of Health, Moscow; 3Federal Scientific-Research Center of Specialized Medical Care and Medical Technologies of the Federal Medical Biological Agency, Moscow; 4City Clinical Hospital №51 of Moscow Department of Health, Moscow, Russia.

THE ROLE OF OSTEOPROTEGERIN SYSTEM /RANKL/RANK IN PATHOGENESIS OF AORTIC STENOSIS

Irtyuga O. B.1, Zhiduleva Е. V.1, Murtazalieva P. М.1, Sibagatullina Yu. S.1, Kruk L. P.2, Solntsev V. N.1, Shishkova А. А.1, Malashicheva А. B.1,2, Moiseeva О. М.1

Abstract

Aim. To assess the concentrations of osteoprotegerin (OPG) and soluble ligand of the receptor of transcription activator factor kappa-B (sRANKL) in the blood serum of patients with various grade of aortic stenosis (AS) severity.

Material and methods. Totally, 247 AS patients studied of various grade: 46 — mild, 53 — moderate and 149 — severe. Of those 132 (53%) with bicuspid aortic valve (BAV) and 115 (47%) with tricuspid (TAV). Controls were 58 patients with no valvular pathology or coronary heart disease. All patients underwent lipid profile measurement, serum C-reactive protein (CRP) and OPG, sRANKL.

Results. In all studied groups of AS patients there was increased level of sRANKL in blood serum, comparing to controls (BAV =0,37 [0,32;0,53] pM/L, TAV =0,38 [0,33;0,50] pM/L, controls 0,30 [0,21;0,39] pM/L; р<0,0001). Concentration of OPG was increased only in TAV: 6,99 [5,19;9,90] pM/L; comparing to 5,23 [4,30;7,09] pM/L in BAV (р=0,0008). A hypothesis proposed that OPG concentration increase is compensatory and takes place as a response to the increase of the concentration or due to loss of sensitivity to sRANKL.

Conclusion. Development of AS is related to disorders in OPG/RANKL system, and these revealed changes might have significant diagnostic and predictive value, especially in TAV.

Russ J Cardiol 2018, 2 (154): 39–43

dx.doi.org/10.15829/1560-4071-2018-2-39-43

Key words: aortic stenosis, calcinosis, biomarkers, osteoprotegerin, sRANKL.

1Federal Almazov North-West Medical Research Centre of the Ministry of Health, Saint-Petersburg; 2Saint-Petersburg State University, Saint-Petersburg, Russia.

MYOCARDITIS AS A LEGITIMATE P HENOMENON IN NON-COMPACTION MYOCARDIUM: DIAGNOSTICS, MANAGEMENT AND INFLUENCE ON OUTCOMES

Blagova O.V.1, Pavlenko Е.V.1, Varionchik N.V.1, Nedostup А.V.1, Sedov V.P.1, Kogan Е.А.1, Zaydenov V.А.2, Kupriyanova А.G.3, Donnikov А. Е.4, Kadochnikova V. V.4, Gagarina N. V.1, Mershina Е. А.5, Sinitsyn V. Е.5, Polyak М. Е.6, Zaklyazminskaya Е. V.6

Abstract

Aim. To evaluate the prevalence of myocarditis in adult patients with non-compaction myocardium (NCM) of the left ventricle (LV), and its influence on the disease course, results of treatment and outcomes.

Material and methods. To the study, 103 adult patients included, with NCM, 61 males, mean age 45,6±14,9 y. o. (from 18 to 78). Mean end diastolic LV size was 6,0±0,8 сm, EF LV 38,8±14,5%. Diagnosis of NCM had been done by echocardiography, multispiral computed tomography (n=81) and magnetic resonance tomography (n=39). DNA-diagnostics was performed by NGS method with further Senger sequencing. Pathogenic mutations were found in 9% of patients in the genes MYH7, MyBPC3, LAMP2, DES, DSP, TTN. The investigation also included anticardiac antibodies, genome of cardiotropic viruses by PCR, coronary arteriography (n=26), scintigraphy (n=25). Morphological assessment of the myocardium was done in 19 patients (12 endomyocardial biopsies), 1 intraoperation biopsy, 3 explanted hearts, incl. 2 after biopsy, and 5 autopsies. Mean follow-up 12 [2; 32] months.

Results. Myocarditis was found in 53,4%, incl. virus-positive in 32,7% of those, with morphology done for 19 patients (active myocarditis in 10, borderline in 6; with minimal signs of activeness in 3). Viral genome in myocardium found in 8 patients (42,1%). The prevalence of myocarditis 44,4% in an arrhythmic variant of NCM, 12,5% in chronic ischemic, 57,5% in dilation cardiomyopathy, 50,0% in NCM patients with other cardiomyopathies. Special cases were acute/subacute myocarditis in NCM (10,7% of all), acute necrosis (infarction) in 4,9%. Comorbidity with myocarditis in NCM led to significantly more severe LV dysfunction (CHF FC 2 [1; 3] v 1,75 [0; 2], p<0,01, EF 33,8±13,5 v 44,7±13,6%, p<0,001), more prevalent non-sustained ventricular tachycardia (67,3% v 29,3%, p<0,01), proper shocks (38,9% v 0, p<0,05), deaths (16,4 and 4,2%, OR 5,75, 95% CI 1,21-27,43, p<0,05) and transplantation (7,3% v 2,1%, p>0,05). Only in myocarditis patients, as a result of basis (antiviral, immune suppression) and cardiotropic therapy there was significant increase of EF (in acute myocarditis from 25,4±7,9 to 38,6±9,5%, p<0,01), decrease of LV size and pulmonary systolic pressure.

Conclusion. Myocarditis is typical phenomenon developing in patients with the primary, i. e. genetically verified NCM. The nature of myocarditis in NCM varies (primary infectious-autoimmune, secondary as a response on genetic/ischemic damage of cardiomyocytes), however regardless of this, it leads to significant worsening of structural and functional parameters, increase of the life-threatening arrhythmias rate, and outcomes (death + transplantation, proper shocks), demanding for active basic therapy.

Russ J Cardiol 2018, 2 (154): 44–52

dx.doi.org/10.15829/1560-4071-2018-2-44-52

Key words: non-compaction myocardium, myocarditis, endomyocardial byopsy, anticardiac antibodies, viruses, DNA-diagnostics, immune suppression therapy, prognosis.

1I. M. Sechenov First Moscow State Medical University of the Ministry of Health, Moscow; 2V. I. Shumakov FSC of Transplantology and Artificial Organs, Moscow; 3SRCI of Pediatrics of Pirogov Russian National Research Medical University, Moscow; 4SPC DNAtechnology, Moscow; 5Treatment and Rehabilitation Center of the Ministry of Health, Moscow; 6V.B. Petrovsky Russian National Research Centre of Surgery, Moscow, Russia.

COMPARATIVE STUDY OF SIGNIFICANCE OF NON-INVASIVE DIAGNOSTIC METHODS IN INFLAMMATORY DISEASES OF THE HEART

Titov V. A., Ignatieva E. S., Mitrofanova L. B., Ryzhkova D. V., Zverev D. A., Lebedev D. S., Moiseeva О. М.

Abstract

Aim. To evaluate diagnostic significance of non-invasive methods of myocarditis diagnostics.

Material and methods. To the study, 141 patient included with suspected myocarditis (104 males, 37 females; age 11-69, mean age 42,7±13,7 y. o.). All patients, together with clinical assessment, underwent standard echocardiography, magnete resonance tomography (MRI) of the heart with contrast enhancement and endomyocardial biopsy with histological and immunohistochemical analysis. Also, the levels of circulating cardiotropic autoantibodies, troponin I and C-reactive protein were measured.

Results. There were no significant differences between patients with myocarditis and dilation cardiomyopathy (DCM) in analysis of clinical, anamnestical and standard laboratory data. The differences found, in levels of circulating cardiospecific autoantibodies in myocarditis patients and DCM comparing to almost healthy donors. However the profile of autoantibodies in myocarditis and DCM patients differed only by the level of autoantibodies to cardiac myosin (χ2=6,0; р=0,014), cytoplasmic antigen of cardiomyocytes CoS-05-40 (χ2=10,2; р=0,001) and the adenine nucleotide translocator protein ANT (epitope EGS) (χ2=10,7; р=0,001). Sensitivity of MRI for myocarditis diagnosis is 67%. In acute myocarditis MRI sensitivity is significantly higher than in chronic inflammation: 85% and 63%, respectively. In chronic borderline myocarditis the sensitivity is no more than 55%.

Conclusion. Low diagnostic significance of clinical and anamnestic data confirmed, and the data of standard laboratory and instrumental investigation, in diagnostics of myocarditis. In patients with myocarditis and DCM there was a specific autoantibodies profile found, different from that of almost healthy donors. MRI shows the highest sensitivity for acute myocarditis. In chronic one, sensitivity depends on inflammation activeness, making necessary the biopsy.

Russ J Cardiol 2018, 2 (154): 53–59

dx.doi.org/10.15829/1560-4071-2018-2-53-59

Key words: myocarditis, dilation cardiomyopathy, biomarkers, MRI, diagnostics.

Federal Almazov North-West Medical Research Centre of the Ministry of Health, Saint-Petersburg, Russia.

THE ROLE OF TRANSFORMING GROWTH FACTOR B ETA-1 AND GALECTIN-3 IN FORMATION OF THE LEFT ATRIUM FIBROSIS IN P ATIENTS WITH P AROXYSMAL ATRIAL FIBRILLATION AND METABOLIC SYNDROME

Zaslavskaya Е. L.1, Morozov А. N.1, Ionin V. А.1,2, Ma I.1, Nifontov S. Е.1, Baranova Е. I.1,2, Yashin S. М.1, Shlyakhto Е. V.1,2

Abstract

Aim. To evaluate the prominence of fibrosis of the left atrium myocardium and to define the value of transforming growth factor beta 1 (TGF-beta1) and galectin-3 in development of myocardial fibrosis in atrial fibrillation (AF) patients with metabolic syndrome (MS).

Material and methods. Totally, 58 patients with AF included, of those 27 with MS. Controls were 50 almost healthy participants. Levels of galectin-3 and TGF-beta1 in blood serum were measured by immune enzyme assay. For fibrosis assessment, the

anatomical and amplitude charts of the left atrium (LA) were built up with nonfluoroscopic system of electroanatomical charting CARTO3 (Biosense Webster, USA) and catheter measurement of the contact power with myocardium of LA (Smart Touch Thermocool, Biosense Webster, USA). In “off-line” regimen the evaluation performed of the zones of low voltage in amplitude specters 0,2-0,5 mV and 0,2-1,0 mV with the area measurement by navigation software function “area measurement”.

Results. Volume of LA and volume index of LA in AF with MS were higher than in AF with no MS: 78,0±20,4 mL and 60,4±19,8 mL (p=0,005) and 37,8±9,5 mL/m2 and 30,4±9,0 mL/m2 (p=0,005), respectively. The percent of LA fibrosis area in AF with MS was higher than in AF with no MS (16,1 [12,8;20,5]% and 10,5 [7,3;16,2]%, respectively, p=0,028). The positive correlations revealed of the level of galectin-3 (r=0,410, р<0,001) and TGF-beta1 (r=0,594, р<0,001) in blood serum with the percentage of LA fibrosis in AF patients. By the linear regression, the influence found of galectin-3 levels (β=0,549, p<0,001) and of TGF-beta1 (β=0,297, p=0,025) on the area of LA fibrosis in AF patients.

Conclusion. The area of fibrosis in the LA myocardium in AF patients with MS is larger than in AF no MS patients. Myocardial fibrosis markers evaluation (galectin-3, TGF-beta1) in blood serum may have diagnostic significance in prediction of AF fibrosis severity in AF patients.

Russ J Cardiol 2018, 2 (154): 60–66

dx.doi.org/10.15829/1560-4071-2018-2-60-66

Key words: galectin-3, transforming growth factor beta-1, myocardial fibrosis, amplitude map, metabolic syndrome, atrial fibrillation.

1Pavlov First Saint-Petersburg State Medical University of the Ministry of Health, Saint-Petersburg; 2Federal Almazov North-West Medical Research Centre of the Ministry of Health, Saint-Petersburg, Russia.

OPINION ON A PROBLEM

EFFICACY OF CARDIOVERTER-DEFIBRILLATORS IN P REVENTION OF SUDDEN DEATH AND OVERALL MORTALITY DECREASE IN P ATIENTS WITH THE SYNDROME OF DILATION CARDIOMYOPATHY:

DIFFERENTIAL APPROACH

Blagova O. V.1, Nedostup A. V.1, Zaklyazminskaya Е. V.2

Abstract

Aim. To evaluate the exact efficacy of the implantable cardioverter-defibrillators (ICD) and combination devices (CRT-D), and to evaluate their influence on the rate of sudden death and overall mortality in patients with the dilation cardiomyopathy syndrome (DCMP), and to clarify the selection criteria for implantation.

Material and methods. Totally, 220 DCMP patients investigated: 66 (30%) of them (mean age 48,5±12,8 y. o., 47 males) underwent implantation of ICD (n=37) and CRT-D (n=29), 154 (70%) patients were in comparison group (mean age 47,1±12,4 y. o., 104 males). In 60 patients (93,9%) the devices were implanted for primary prevention of sudden cardiac death (SCD). Follow-up lasted for 16 [6; 37] months. As primary endpoints, the following parameters were used: “death+transplantation”, mortality, SCD, “SCD+shock” and “death+transplantation+proper shocks of the defibrillators”.

Results. Mortality in all DCMP patients was 19,1%, “death+transplantation” — 21,4%, SCD — 2,7%. There were no significant differences by these values in patients with both devices (19,7%; 22,7% and 1,5%), ICD (21,6%; 24,3% and 2,7%), CRT-D (17,2%; 20,7; and 0) and patients with no devices (18,8%; 20,8% and 3,2%). Significantly higher rates by “SCD+shocks” (18,2% v 3,2%, p<0,001) and “death+transplantation+shocks” (36,4% vs 20,8%, p<0,05) in patients with the implanted devices witness for real impact of the defibrillators in equality of overall mortality and SCD parameters. Among the patients with implanted devices, the genetic and mixed (genetic and inflammatory) nature of DCMP predominated (62,1 v 35,7%, p<0,001), there was significantly lower EF (26,3±9,2 v 30,7±10,3%, p<0,01), its end value (31,1±11,0 v 39,2±13,5%, p<0,01), significantly higher end diastolic size of the left ventricle (EDS, LV, 6,8±0,8 v 6,5±0,8 cm, p<0,05) and the grade of mitral regurgitation. In patients with the devices, rate of proper shocks was 18,2%. In isolated myocarditis there were no shocks (35,3±9,1% v 26,8±9,3%, p<0,05), EDS significantly lower (6,2±0,6 cm v 6,9±0,9 cm, p<0,01), ECG signs of LV hypertrophy were more rare (16,7% v 56,3%, p<0,05), but more commonly — the low voltage of QRS (33,3% v 10,6%, p=0,53); there were no differences in the rates of cardiotropic drugs prescription. Main predictors of the shocks were genetic origin of DCMP (isolated or with myocarditis, 25/75%, comparing with 20/33% in patients with no shocks, p<0,01, HR 1,58, OR 10,93, sensitivity 94,1%, negative predictive value 99,2%), and sustained (HR 18,0, sensitivity 98,1%) and non-sustained ventricular tachycardia (HR 1,43, sensitivity and negative predictive value 100%), decrease of QRS voltage and absence of the signs of LV hypertrophy on ECG (negative predictive value 92,8% and 95,6%).

Conclusion. In DCMP patients, implanting of ICD/CRT-D was performed with acknowledged additional criteria (genetic or mixed DCMP etiology, lower EF and worse response to treatment); due to more effective ICD therapy, the values of mortality, “death+transplantation” and SCD were not higher than in less severe patients with no such devices. Proper shocks developed in patients with significantly higher EF. As an independent SCD risk factor and a criteria for patients selection to defibrillator implantation, genetic origin of DCMP should be used, especially if comorbid with myocarditis. As the predictors for benign outcome — absence of non-sustained ventricular tachycardia, lower QRS voltage, signs of LV hypertrophy on ECG. An algorithm proposed of DCMP patients selection for ICD treatment.

Russ J Cardiol 2018, 2 (154): 67–79

dx.doi.org/10.15829/1560-4071-2018-2-67-79

Key words: dilation cardiomyopathy, myocarditis, sudden death, cardioverterdefibrillator, ventricular tachicardia.

1V. N. Vinogradov Faculty Clinics of Therapy, I. M. Sechenov First Moscow State Medical University of the Ministry of Health, Moscow; 2V. B. Petrovsky Russian National Research Centre of Surgery, Moscow, Russia.

PATHOLOGY OF THORACIC AORTA IN CONNECTIVE TISSUE DYSPLASIAS

Nechaeva G. I., Semenova E. V., Semenkin A. A., Druk I. V., Konev V. P., Chindareva O. I., Zhivilova L. A., Loginova E. N., Tkachenko T. V.

Abstract

The article is focused on a variety of aspects of aortic pathology in connective tissue dysplasias; approaches discussed to diagnostics and management of aortic dilation in dysplasias.

Russ J Cardiol 2018, 2 (154): 80–90

dx.doi.org/10.15829/1560-4071-2018-2-80-90

Key words: connective tissue dysplasia, aortic dilation, echocardiography.

Omsk State Medical University of the Ministry of Health, Omsk, Russia.

CLINIC AND PHARMACOTHERAPY

THE P REVALENCE OF CHRONIC KIDNEY DISEASE MARKERS IN ARTERIAL HYPERTENSION P ATIENTS AND RELATION WITH DIABETES: RESULTS OF EPIDEMIOLOGICAL STUDY KHRONOGRAPH

Kobalava Zh. D.1, Villevalde S. V.1, Bagmanova N. Kh.1, Batyushin М. М.2, Orlova G. М.3

Abstract

Aim. Under the framework of non-interventional observational multi-center program KHRONOGRAPH (Acquisition of additional data on the prevalence of declined glomerular filtration rate and albuminuria in systemic hypertension patients with and with no diabetes mellitus type 2 in Russian Federation) to evaluate the prevalence of chronic kidney disease (CKD) markers.

Material and methods. In 1363 patients with arterial hypertension (AH) and/or type 2 diabetes (DM2) the glomerular filtration rate (GFR) was calculated with CKD-EPI, and albuminuria (AU) assessed via albumin/creatinine ratio (ACr) in morning urine.

Results. In DM2 patients (n=779, 62,6% women, 63,8 y. o.) comparing to non-DM2 patients (n=584, 50,7% women, 60,5 y. o.) there was more common decrease of GFR <60 mL/min/1,73 m2 (38 and 31%), <45 mL/min/1,73 m2 (G3b-G5) (15,92 and 9,94%) and <30 mL/min/1,73 m2 (G4-G5) (4,24 and 2,06%), high and very high AU (36,6 and 28,1%), very high AU (А3) — (7,32 and 2,40%). In DM2 patients there was more common presence of at least one marker of CKD <60 mL/min/1,73 m2 and/or ACr >30 mg/g) (54,0 and 44,35%), isolated GFR decrease <60 mL/min/1,73 m2 (17,46 and 16,27%), isolated increase of ACr >30 mg/g (15,66 and 13,35%) and combination of decreased GFR and high/very high AU (20,93 and 14,71%). Among DM2 patients, the portion is lower of decreased combination risk of CKD progression and cardiovascular complications (45,2 and 53,6%), higher — in very high risk (28,5 and 20%). Most prevalent are the combinations related to early stages of CKD: G3аА1, G2А2 and G3аА2.

Conclusion. In Russian Federation, patients with AH, regardless of DM2, are characterized by high rate of CKD markers. In DM2 patients comparing to those with none, there was more prevalent GFR decrease <60 mL/min/1,73 m2, increase of ACr >30 mg/g isolated and in combination. In AH and DM2 the portion of patients with a very high risk of CKD progression and cardiovascular complications is higher.

Russ J Cardiol 2018, 2 (154): 91–101

dx.doi.org/10.15829/1560-4071-2018-2-91-101

Key words: arterial hypertension, diabetes, chronic kidney disease, glomerular filtration rate, albuminuria.

1Peoples’ Friendship University of Russia (RUDN University), Moscow; 2Rostov State Medical University of the Ministry of Health, Rostov-na-Donu; 3Irkutsk State Medical University, Irkutsk, Russia.

POSITIVE CLINICAL EFFECTS OF AN M-CHOLINOLYTIC IN IDIOPATHIC ARTERIAL HYPOTENSION

Baev V. M., Agafonova T. Yu.

Abstract

Aim. Evaluation of an m-cholinolytic influence on blood circulation, mental performance and fatigability, self-feeling in young women with idiopathic arterial hypotension (IAH).

Material and methods. The study object were 20 females with IAH defined as systolic BP ≤98 mmHg, age 19-32 y. o. The study subject — circulation parameters (SBP, DBP, HR), mental performance and fatigability by E. Kraepelin, self-rate of health (self-feeling). Experimental drug was taken once as one pill.

Results. In 1 hour post one dosage of the drug, there was SBP increase from 94 (92-96) mmHg to 108 (106-112) mmHg, and DBP and HR (p<0,05); increase of summations performed by 26% (p<0,01); improvement of self-feeling — in 45% of participants. In 24 h, 85% reported improvement of self-feeling.

Conclusion. In idiopathic arterial hypotension in young women one dosage of the m-cholinolytic shown the following positive effects: increase of SBP, DBP and mental performance (in 1 h) and improvement of self-feeling (in 1 h and 24 h).

Russ J Cardiol 2018, 2 (154): 102–106

dx.doi.org/10.15829/1560-4071-2018-2-102-106

Key words: cardiology, idiopathic arterial hypotension, m-cholinolytic.

E. A. Wagner Perm State Medical University of the Ministry of Health, Perm, Russia.

CLINICAL CASES

THE ALCAPA SYNDROME IN ADULTS

Vasiltseva O. Ya., Boshchenko A. A., Gorlova A. A., Gladkikh N. N., Zavadovsky K. V., Vintizenko S. I., Kozlov B. N.

Abstract

Syndrome of abnormal originating of the left coronary artery from pulmonary artery (ALCAPA), or Bland-White-Garland syndrome, is an inborn heart defect of atypical coronary arteries branching. In most cases this anomaly is revealed in infancy or adolescence. Opinions exist, that the anomaly is much more prevalent than it is diagnosed. Even more, it might be a cause of myocardial infarction and sudden death of 1st year newborns. In the article, a clinical case presented of first time revealed ALCAPA in 64 year old woman.

Russ J Cardiol 2018, 2 (154): 107–114

dx.doi.org/10.15829/1560-4071-2018-2-107-114

Key words: congenital heart defect, anomaly of coronary arteries branching.

SRI of Cardiology, Tomsk National Research Medical Center of RAS, Tomsk, Russia.

ACROMEGALIC CARDIOMYOPATHY WITH DYNAMIC OBSTRUCTION OF THE LEFT VENTRICLE OUTFLOW TRACT

Shlyakhto Е.V.1,2, Polyakova А.А.1,2, Semernin Е.N.1,2, Krutikov А.N.2, Oksas А.Е.1, Tsoy U.А.2, Dalmatova А.B.2, Belousova L.V.2, Kostareva А. А.1,2, Grineva Е. N.1,2, Gudkova А. Ya.1,2

Abstract

Aim. To explore genesis of the left ventricle hypertrophy in acromegaly patient, with the method of next generation sequencing.

Material and methods. Standard clinical and laboratory minimum was done, with electrocardiography, 24 hour ECG monitoring, echocardiography, magnete resonance tomography of the heart, new generation sequencing on the IlluminaHiSeq 2000 equipment with simultaneous analysis of 108 genes associated with idiopathic hypertrophic cardiomyopathy (HCMP) and phenocopies of HCMP.

Results. At the age 59 y. o. the female patient had beed first time diagnosed with asymmetric HCMP, non-obstructive type (interventricular septum 19 mm, posterior wall 11 mm, pressure gradient in outflow tract of the left ventricle (OTLV) — 25 mmHg). At the age 62 y. o. she developed HCMP with dynamic obstruction of OTLV (pressure gradient in OTLV up to 80 mmHg) with progressing dyspnea on exertion, and required non-surgical reduction of interventricular septum. By the computed tomography data, at the age 63 y. o. the patient was diagnosed with endocellar hypophysis microadenoma (a tumor 6,7*7,3 mm), somatotropic hormone — 53,39 mU/L (normal: 0,1-20 mU/L), insulin-like growth factor 1 — 359 ng/mL (normal: 118-314 ng/mL). However, with retrospective analysis of her photos, even from the age 40 y. o. there were enlarged hands, feet, nasal cartilages, ears, lips and eyebrow arcs that witness for long lasting disease course. With the new generation sequencing, there were no pathological mutations revealed.

Conclusion. The case represents hypertrophic cardiomyopathy as a leading clinical sign in acromegalic cardiomyopathy that imitated idiopathic HCMP. Patient management in such case should include on-time etiopathogenetic therapy that works against disease progression, and in some cases even for regression of the left ventricle hypertrophy.

Russ J Cardiol 2018, 2 (154): 115–120

dx.doi.org/10.15829/1560-4071-2018-2-115-120

Key words: acromegalic cardiomyopathy, new generation sequencing, elderly.

1Pavlov First Saint-Petersburg State Medical University of the Ministry of Health, Saint-Petersburg; 2Federal Almazov North-West Medical Research Centre of the Ministry of Health, Saint-Petersburg, Russia.

NON-VAL30MET-TRANSTHYRETIN AMYLOID CARDIOMYOPATHY. LITERATURE REVIEW AND CLINICAL CASE

Gudkova А.Ya.1,2, Polyakova А.А.1,2, Amelin А.V.2, Moiseeva О.М.1, Krutikov А.N.1, Tishkova V.М.1, Grozov R.V.1, Ryzhkova D. V.1, Makurova Т. V.1, Kostareva А. А.1,2, Semernin Е. N.1,2, Shlyakhto Е. V.1,2

Abstract

A literature review presented, with a clinical observation of non-Val30Met-transthyretin amyloid cardiomyopathy. Modern diagnostic and management algorithms discussed.

Russ J Cardiol 2018, 2 (154): 121–128

dx.doi.org/10.15829/1560-4071-2018-2-121-128

Key words: genetic transthyretin amyloidosis, transthyretin amyloid cardiomyopathy, Val30Met mutation.

1Federal Almazov North-West Medical Research Centre of the Ministry of Health, Saint-Petersburg; 2Pavlov First Saint-Petersburg State Medical University of the Ministry of Health, Saint-Petersburg, Russia.

GIANT CELL MYOCARDITIS IN HIV INFECTION: A FATAL TANDEM

Nikulina S. Yu., Shesternya P. A., Kirichenko A. K., Chernova A. A., Brusentsov D. A., Shulman V. A.

Abstract

Giant cell myocarditis (GCM) is a rare autoimmune disease with the leading role of T-cell disorder in its pathogenesis. The associations are known of this disease with various autoimmunities, tumors, drug hypersensitivity. In the literary data there are just single cases of HCM development in HIV infected patients. The article presents clinical case and provides some discussion on the pathogenesis of HCM, making to think of HIV infection as one of possible causes of its development.

Russ J Cardiol 2018, 2 (154): 129–133

dx.doi.org/10.15829/1560-4071-2018-2-129-133

Key words: giant cell myocarditis, HIV infection.

V. F. Voino-Yasenetsky Krasnoyarsk State Medical University, Krasnoyarsk, Russia.

LITERATURE REVIEW

ATORVASTATIN — 20 YEARS IN THE STRUGGLE FOR LIFE

Gogolashvili N. G.1,2

The efficacy and safety of atorvastatin discussed for the decrease of cardiovascular complications development.

Russ J Cardiol 2018, 2 (154): 134–149

dx.doi.org/10.15829/1560-4071-2018-2-134-149

Key words: statins, atorvastatin, atherosclerosis, cardiovascular diseases, clinical trials.

1SRI of Medical Problems of the North — Separate Division of FITZ KSC SB RAS, Krasnoyarsk, Russia; 2V.F. Voyno-Yasenetsky Krasnoyarsk State Medical University, Krasnoyarsk, Russia.

12 марта 2018 г.
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