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The problem of non-coronary diseases of myocardium (NCDM) and, particularly, the classification of them, is one of the most complicated in cardiology and internal medicine. Until recently, there is no unified official classification in Russia, and there are various in use for practical and scientific purposes: American (2006), European (2008) and the latest issue by Paleev N.R. and Paleev F.N. (2008). The article critically examines and compares these classifications. The analysis provided, of evolution of the term “cardiomyopathy” (CMP), European and Russian classifications of myocarditis and MOGE(S) of 2014; the principles for an “ideal” classification are proposed, at modern stage, and our original synthetic variant.

Main aspects of the proposed classification are introduction of syndromal approach (the selection of primary clinical syndrome) as a first step of nosological diagnostics when NCDM suspected; retaining of nosological principle with distinction of NCDM to myocarditis, CMPs and myocardiodystrophies; selection of combination NCDM; parallel application of etiological (incl. genetic) and structure-functional CMP classification; selection of CMP with mostly right chambers lesion; selection (together with genetic and idiopathic) of the special CMPs, showing combinational nature; combinations of non-rheumatic myocarditis with peri- and endocarditis; the grade of myocardium diagnosis reliability (if biopsy impossible) with the original criteria of its diagnostics; the level of myocarditis activeness — histological and immune; two types of infarction-like myocarditis (as well as ischemic); paraneoplastic myocarditis and myocarditis as non-classified (latent) systemic immune disorder; introduction of a detailed morpho- and virological characteristics of myocarditis into classification; distinction of myocardiodystrophies by the level of systolic function compensation; creation of clinical classification of arrhythmogenic right ventricle dysplasia and non-compaction myocardium syndrome (incl. in the presence and absence of myocarditis).

Aim. To evaluate diagnostic significance of biomarkers for inflammatory cardiomyopathy (ICM).

Material and methods. Totally, 35 patients included with suspected inflammatory cardiomyopathy, chronic heart failure of I-III functional class (FC), decreased systolic function of the left ventricle (EF LV <40%). All patients, together with routine assessments, underwent endomyocardial biopsy (EMB). Studying bioptates, the methods were used of histology and immunohistochemistry with monoclonal antibodies to monocytes/macrophages, to T-lymphocytes (CD 45RO, CD3+, CD4+, CD8+, CD68+).

Diagnosis of ICM was set if in myocardium there were inflammatory infiltrates related to degeneration or necrosis of myocytes of non-ischemic origin, and number of leucocytes was 14 or more (including up to 4 monocytes) in 1 mm2 of specimen, and at least 7 of them should be CD3+ T-lymphocytes. According to the results, patients were selected to the groups of ICM and non-inflammatory dilation ardiomyopathy (niDCM). In all patients we evaluated the following biomarkers: immunoglobulines A, M, G, high-sensitive C-reactive protein (hsCRP), matrix metalloprotease-9 (MMP9), transforming growth factor β (TGFβ), tumor necrosis factor α, (TNFα), C3 and C4 components of complement, eosinophilic catione protein (ECP).

Results. While comparing groups ICM and niDCM by the parameters of humoral immunity, it was revealed that levels of hsCRP, C3 and C4 complement, MMP9, TGFβ were significantly higher in ICM group. In ROC analysis it was found that only hsCRP, C3 and C4 complement and MMP9 have diagnostic significance. The levels of the biomarkers mentioned, were assessed with a threshold definition for optimum sensitivity and specificity: 1,13 mg/mL, 1,2 g/L, 0,256 g/L, 680 ng/mL, respectively. Using combined biomarkers hsCRP with C3, C4 complement sensitivity reached 61%, specificity 94%; in MMP6 with C3, C4 complement — 66% and 94%, respectively.

Conclusion. Combination of hsCRP and MMP9 with C3, C4 components of complement is worthy to use in diagnostics of ICM.

Aim. To study the radiation semiotics of various clinical and morphological types of myocarditis with magnetic resonance imaging (MRI).

Material and methods. Totally, 70 patients included, with suspected by clinical and lab data myocarditis (28 females, 42 males; mean age 37,0±13,1 y.o). All patients underwent endomyocardial biopsy (EMB) and MRI with intravenous contrasting. Sensitivity of MRI method was evaluated with relation to EMB. Exclusion criteria had been the comorbid ischemic heart disease.

Results. Among 70 patients with histologically verified myocarditis, only 47 had this diagnosis by MRI data, sensitivity was set as 67%. In acute active and acute borderline myocardities there is high sensitivity of MRI — 86%; in chronic active myocarditis — 74%, in patients with chronic borderline myocarditis — 55%. Three MRI-criteria of myocarditis were found in acute active myocarditis. Acute borderline myocarditis in most cases presented with 2 signs: edema and delayed contrast enhancement. Significant difference of active myocarditis from other clinical and morphological types is dominance of transmural pattern of delayed contrasting. Also, there is interdependence of inflammation activity by EMB and severity of myocardium edema (64,3% vs. 40,5%, p=0,005). The main differentiating factor of chronic myocarditis is collecting kind of the delayed contrasting areas, which spread more than the zone of myocardium edema.

Conclusion. Magnetic resonance imaging shows the highest sensitivity in revealing of acute myocarditis, and in cases of chronic myocarditis the sensitivity depends on activity of inflammation. The overlap of the areas of delayed contrasting and myocardial edema make it to differentiate acute and chronic process in myocardium. Dignostics of chronic borderline myocarditis is impossible unless EMB is done.

Aim. To study the role of contrast-enhanced multispiral computed tomography (MSCT) of the heart in diagnostics of myocarditis in patients with the syndrome of dilation cardiomyopathy (DCMP) comparing to morphological investigation of myocardium.

Material and methods. Into the main group of study, 127 patients included (92 males, 46,9±11,8 y.o.) with DCMP syndrome (mean end diastolic size (EDS) of the left ventricle (LV) 6,6±0,8 cm, mean ejection fraction (EF) 29,7±9,5%, 3 [2; 3] FC by NYHA). All underwent 320-slice MSCT of the heart with i.v. contrast, 50 underwent morphological investigation of myocardium (endomyocardial byopsy in 30, intraoperational in 7, autopsy in 9, explanted heart study in 4). Also, viral infection markers were studied, as the level of anticardiac antibodies, EchoCG (all patients), scintigraphy (n=42), magnete-resonance tomography (MRI) (n=21), coronary arteriography (CAG, n=48). Comparison group included 18 patients (12 males, 69,2±8,5 y.o.) with coronary atherosclerosis (stenosis form 40%) by MSCT and absence of DCMP criteria (mean EDS LV 4,7±0,5 cm, mean EF LV 59,3±4,9%, 0 [0; 2] FC by NYHA).

Results. By the data from complex investigation, myocarditis as the main cause of DCMP syndrome was diagnosed in 79 (62,2%) patients of the main group, its comorbidity with genetic cardiomyopathies — in 19 else (15%). In MSCT of the heart the areas of lower accumulation were found in 4 patients from main group (3,1%, type 1 by the proposed evaluation score), delayed accumulation of the contrast in myocardium — in 72 (56,7%) patients: in 12 subendocardial (type 2), in 4 intramyocardial (type 3), in 44 subepicardial (type 4), in 12 transmural (type 5); in 51 patient there was no delayed accumulation. Sensitivity and specificity of all types of delayed accumulation in diagnostics of myocarditis were 63,3% and 78,7%, positive and negative predictive value 86,1% and 50,7%, subepicardial and transmural types — 49,0%, 83,0%, 85,7%, 43,8%, respectively. While comparing the data of MSCT directly with morphological study of myocardium, diagnostic significance of all types of delayed accumulation in myocarditis revealing was 66,7%, 84,6%, 87,5%, 61,1%, subepicardial and transmural types — 52,4%, 92,3%, 91,7%, 54,5%, respectively.

By MSCT in the main group also the non-compaction myocardium was found (n=29, 22,8%), coronary atherosclerosis (n=33, 26,0%), confirmed by CAG in 16 patients. Presence/absence of delayed accumulation by our proposed score correlated with 1) diagnostical signs: duration of illness (r=-0,185, p<0,05), acute onset (r=0,196, p<0,05), connection of onset and infection (r=0,332, p<0,001); 2) functional signs as the class of heart failure (r=0,183, p<0,05), VTI (r=-0,303; р<0,05); 3) mortality rate (r=0,176, p<0,05).

Conclusion. MSCT with the evaluation of delayed contrast accumulation (and synchronic CT-angiography of coronary arteries) can be used for non-invasive diagnostics of myocarditis in patients with DCMP syndrome, including if MRI contraindicated. Delayed accumulation of contrast in myocardium correlates with myocarditis presence, the grade of functional disorder and prognosis.

Dilation cardiomyopathy (DCMP) remains a disease with poor prognosis.

Aim. To study the importance of risk stratification improvement for fatal outcomes, especially sudden cardiac death (SCC), with the aim for on-time prevention procedures, as the previously proposed non-invasive electrophysiological predictors — heart rhythm turbulence (HRT) and variability (HRV), microvolt alternation of T wave (mTWA), deceleration capacity (DC) and acceleration capacity (AC) in DCMP are not studied well enough.

Material and methods. During 4 years there was follow-up of 54 DCMP patients and sinus rhythm at the age 42 [30;58] year-old (36 males) and control group — 54 persons with no cardiovascular pathology (32 males, mean age 47 [27;64] y.o.). At baseline the Holter monitoring was done, of ECG with HRV, HRT, DC, AC, mTWA assessment, and echocardiography. Patients took standard treatment of chronic heart failure (CHF); part of cardioverter-defibrillator (CD) implanted was 18,5%.

Results. Mean ejection fraction (EF) in the main group was 32% [22;38], signs of CHF had 93% of patients. Those with DCMP differed from the main group by significantly lower values of SDNN, pNN50, DC, TO, TS, higher AC and mTWA in early morning. During 4 years there was 1 SCD, and 7 died from CHF progression; there was 1 adequate shock in CD patient (totally 9 deaths). Comparing to those survived, died patients had had lower EF, HRV, DC, maximal mTWA values, higher end diastolic volume, CHF class, AC, mTWA, number of episodes of non-sustained ventricular tachycardia (nsVT) in morning. Under monofactorial analysis there was significant influence on fatal outcome risk of the following (in order of declining significance): EF (odds ratio (OR) 32), SDNN (OR 21), DC (OR 9), AC (OR) 7, pNN50 (OR 6), nsVT (OR 5,2; p=0,05). In multifactor analysis the only independent predictor of fatal outcomes was the decrease of EF of the left ventricle less than 26% (sensitivity 80%, specificity 90%).

Conclusion. DCMP patients, comparing to persons with no cardiovascular pathology, have decreased HRV and DC, increased AC, more common pathological HRT, increase of mTWA in early morning, and in those died these specifics was more prominent. In monofactor analysis the non-invasive electrophysiological predictors associated with poor DCMP prognosis, were AC, SDNN DC, pNN50, nsVT. However the most significant and the only independent predictor of fatal outcomes in DCMP patients is the decrease of EF. If to apply the EF less than 26% as a criteria of high risk, it predicts the 32 times increase of fatal outcomes risk with sensitivity 80% and specificity 90%.

Regardless the developed algorithms for risk stratification in sudden cardiac death (SCD) among patients with hypertrophy cardiomyopathy (HCM) there are SCD cases with no common risk factors. The issue for prediction of SCD in this pathology does not lose its importance.

Aim. To study the risk factors for SCD in HCM patients.

Material and methods. During 14 years, 198 HCM patients studied (mean age — 60,0±13,8 y.o., 105 males) with ECG, EchoCG, Holter ECG, veloergometry done. During 14 years, 15 died. There was 7 SCD cases with 2 successful resuscitations.

Results. In HCM patients there was relation of SCD with the following clinical and instrumental parameters: age (r=-0,19, p=0,015), syncopes in anamnesis (r=0,17, p=0,03), high flow velocity in outflow tract of the left ventricle (LV) in exertion (Ex) (VmaxLVOT) (r=0,3, p=0,04), low Ex tolerance (r=-0,34, p=0,009), abnormal Ex response of blood pressure (ABPR) (r=0,36, p=0,00001), high minimal heart rate (HR) on Holter ECG (r=0,26, p=0,01). Applying the binary logistic regression with inclusion of the most significant predictors, the model was formulated for SCD prediction in HCM patients: SCD=22,28хABPR-6,9хEx Duration+12,5хVmaxLVOT+0,32хMinHR-0,4478хAge-14,7 If the result of equation more than 0 — the patient has high risk of SCD. In negative — SCD risk is minimal. Value of χ2 for the proposed equation is 19,3 (p=0,002).

Conclusion. Regardless the common (age, syncopes, severity of LVOT obstruction, ABPR in Ex) there are additional SCD risk factors in HCM patients: low Ex tolerance and high minimal HR on Holter ECG. the mathematic model proposed, making to, based on the age of patient and presence of ABPR in Ex, Ex tolerance, maximal flow velocity in LVOT in Ex, as minimal HR by Holter ECG, to reveal patients with high risk of SCD.

In amyloidosis AL-type the heart involvement is most common; this type is specific with predecessor proteins of light immunoglobulin chains, transthyretin (TTR), mutant and wild (senile) types. Most common clinical form is restriction cardiomyopathy.

Aim. To assess the recent specifics of diagnostics, due to broader abilities of novel methods.

Material and methods. Five 40-79-year old patients with various morphofunctional variants of heart involvement and typical ECG signs (low QRS voltage, QS, non-sufficient R increase, absence of left ventricle (LV) hypertrophy signs) to verify the suspected amyloidosis, underwent EchoCG, immunohistochemistry of blood and urine for light chains of immunoglobulines, biopsy of subcutaneous fat and mucose of the gum/gut; MDCT of the heart (n=3), MRI (n=1), scintigraphy with 99Tc-pyrophosphate with assessment in 1 hour after indicator injection (n=1), endomyocardial biopsy (n=2), titer of anticardial antibodies assessment (n=2), DNA-diagnostics (n=1).

Results. The diagnosis of amyloidosis was confirmed in all cases. Its morpho-functional types were RSMP with LV hypertrophy, hypertrophic cardiomyopathy (HCMP) with no restriction but progressing fall of ejection fraction (EF), dilation cardiomyopathy (DCMP), severe hypertrophy with low EF, minimal hypertrophy with no restriction and systolic dysfunction. There was AL-type diagnosed (n=2, one case with myopathy mimicking “dermatomyositis”), mutant TTR (n=1, novel mutation Thr40Asn) and wild TTR (n=2) types. The leading clinical signs were biventricular heart failure and atrial rhythm disorders: sustained atrial fibrillation in 3 patients (in one, before amyloidosis verification, the RF-modification was done as “labyrinth” surgery, isthmus block with no established efficacy) and frequent supraventricular extrasystoly in one another. To the patient with mutant ATTR the ICD was implanted with further replacement by CRT-D, and increase of EF from 24% to 31% was achieved (patient is followed-up for 8 years). As the morphological equivalent of severe systolic dysfunction in this patient the amyloid deposition in myocardial arteries could be suspected. MDCT revealed typical subendocardial delayed deposition in 2 from 3 patients, in one case there was also diffuse deposition of 99Tc-pyrophosphate in myocardium. Antibodies to the nuclei of cardiomyocytes (specific ANF) was found in a female patient with AL-type and DCMP, which made not to rule out myocarditis.

Conclusion. Cardiac amyloidosis might present as any structural and functional variant of cardiomyopathy, including DCMP. The most specific is diffuse hypertrophy with restriction and EF decrease, but with no LV dilation. Early fall of contractility, ischemia symptoms and LV dilation might be the result of amyloid lesion of small arteries. In the presence of any systemic presentations together with “HCMP”, “RCMP”, “DCMP” there must be amyloidosis ruled out. Myocardial scintigraphy with 99Tc-pyrophosphate is a method of use for the diagnosis of ATTR; MDCT of the heart — for any type of amyloidosis. Cardiac amyloidosis might be followed by significant type of the titer of specific ANF (secondary reaction or concomitance with myocarditis?).

Aim. To analyze the specifics of clinical course of pregnancy, delivery and postnatal period in women with disorders of aortic valve (AV) and aorta.

Material and methods. During the period from January 2012 to December 2014, under conditions of specialized perinatal center of FSBI Almazov Center in Saint-Petersburg, into prospective cohort study, 56 patients included, with structural pathology of aorta and AV. Mean age 29±4,5 y.o. (18-38 y.o.). All patients underwent echocardiographic study (EchoCG) on Vivid 7 GE equipment by standard protocol. The level of N-terminal brain natriuretic peptide (NT-proBNP) was measured by electrochemiluminiscent analyzer Cobas e411 (Roche, Swiss) at 34±6 week of pregnancy.

Results. The most common pathology in the analyzed group were aortic stenosis and aortic coarctation. The main cause of AV pathology was inborn defect (IHD) — bicuspid AV (BAV). Complications in cardiovascular functioning developed in 9 cases (16%) and were related to ventricular rhythm disorders of high grades and signs of heart failure with need of medication treatment (10,7%, n=6). Level of NT-proBNP was higher than 125 pg/mL in 12 patients (21,4%) and its mean value was 330,3±54,7 pg/mL; in women with increased NT-proBNP complications in cardiovascular system developed 3 times more often. In 25 patients (44,6%) various obstetric complications were found, mostly in the 3rd trimester of pregnancy. Regardless the clinically significant cardial pathology, women delivered in mature term, 38,6±1,5 weeks. After delivery there were no cases of heart failure progression or other cardiovascular complications development.

Conclusion. For women with aortic and AV pathology there is benign prognosis of pregnancy and delivery taken they are managed in specialized centers.

Aim. To find out the close mean-end relations of the false tendons (FT) topology in the left ventricle (LV), regional and global heart functioning, including its ability to endure exertion.

Material and methods. Totally, 90 healthy young persons included (48 females), age 20,2±2,9 y.o., actively participating in sports. Connective tissue dysplasia was confirmed according to National guidelines on Diagnostics and management of inherited connective tissues disorders (2012). The routine echocardiographic study was done with further digital processing and 3D modelling of the LV with regional myocardium function assessment. The ability of the heart to adapt exertion was measured by exercise test on thread-mill.

Results. Describing of FT topology in 3D-model of LV revealed the presence of 1 to 5 tendons by 1 LV, which mostly were located in upper parts of the ventricle, and oriented perpendicularly or at small angle to its longitudinal axis. Main values of global structure and function of the LV were at normal range, but in all participants, there was high level of mechanical asynchronicity at rest. In addition, there was high variability of parameters related to the ability of the heart to endure exercises. By methods of correlational, monofactorial and multifactorial analysis, it was revealed that: as many FT by 1 LV, as higher the level of baseline mechanical asynchronicity and lower the heart ability to adapt exertion.

Conclusion. Young healthy persons with FT in LV, participating systematically in sports, physical exercise or fitness, need strictly individualized regimen of exercise.

First time, myocardial clefty were described more than 20 years ago in autopsy of patients with hypertrophic cardiomyopathy (HCMP): the “fissure” along muscle fibers in interventricular septum. Currently, the precise diagnostical and practical value of this pathology unknown. What sort of diagnostic and practical impact such information brings into the management evaluation of patients? Previously, we reported on unexpected finding of myocardial fissure together with Barlow syndrome. This article continues analysis of the experience of clinical work and includes reports on 2 patients with diffuse-generalized form of HCMP and intramyocardial crypt.

In the article, a case presented of primary amyloidosis of the heart and pericardium, with severe resistant heart failure. That kind of case witnesses that diagnostics and management of patients with this disease, is a complicated issue in cardiology practice. That is related to its rarity, absence of key simptoms in clinical practice and delayed diagnostics with lack of expected efficacy of modern treatment.

The left ventricle non-compaction myocardium syndrome is relatively new diagnosis entered clinical practice with improvement of the heart visualization methods. There is plenty of studies on genetic heterogeneity of non-compaction myocardium syndrome and its impact on a variety of associated cardiomyopathies. However, there is no single opinion on pathogenesis of non-compaction, as there are no unified diagnostic criteria and no guideline on treatment and management of patients with this syndrome. Current review provides a summary of data on this pathology nature, its genetics, diagnostics and treatment.

Regardless the common consensus on the benefits of immunity suppression in eosinophilic, granulomatous, giant cell and lymphocyte myocarditis, associated with systemic connective tissue diseases, and in rejection of transplanted heart, role of immune suppression therapy (IST) in treatment of lymphocyte inflammatory cardiomyopathy remains controversial. Previous retrospective studies revealed clinical improvement in 90% of patients with virus-negative inflammatory cardiomyopathy and absence of response or worsening of heart function in 85% of virus-positive inflammatory cardiomyopathy after immunity suppression. Other studies identified the enhanced expression of HLA in cardiomyocytes as an additional indicator of sensitivity of inflammatory cardiomyopathy to IST. Recently, the singlecenter randomized study was performed with double blind application of prednisone and azatioprin as addition to maintenance therapy in 85 patients with virus-negative inflammatory cardiomyopathy. The results of the study showed significant improvement of the left ventricle ejection fraction and decrease of the left ventricle dimensions in 88% patients among 43 of treated comparing to 42 patients on placebo demonstrated worsening of cardiac functioning in 83% cases (the TIMIC study).

This data confirms the efficacy of immunity suppression in virus-negative inflammatory cardiomyopathy. Insufficiency of response in 12% cases undermines existence of non-revealed viruses and mechanisms of damage and inflammation, non-sensitive to immunity suppression. Restoring of cardiac function in patients actively responded to immunity suppression, was determined by inhibition of cardiomyocytes death and increase of cells proliferation with ad novo synthesized contractile material.

Meta-analytic data shows that addition of trimetazidine (TMZ) to standard therapy increases significantly exercise tolerance in ischemic heart disease (IHD) patients. In heart failure due to IHD, condition improves as well, administering TMZ. There is no strict evidence that TMZ is especially effective in people with comorbid diabetes. TMZ shows significant antianginal effect in combination with other medications that is, certainly, improves life quality of patients.