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Left ventricular hypertrophy, atrial fibrillation and chronic heart failure are often in the practice of a cardiologist. The etiology of these conditions is very important because the correct early treatment. We are presenting a case of a late diagnosis of amyloidosis in a 53-year-old man. Despite the complex therapy, the course of the disease was complicated by the development of bilateral pneumonia, sepsis, disseminated intravascular coagulation and the patient died. Autopsy confirmed the diagnosis of systemic AL-amyloidosis (type Kappa) with massive damage to the heart, kidneys, lungs, liver, spleen, adrenal glands, thyroid gland, pancreas, gastrointestinal tract, subcutaneous fatty tissue and arterial vessels of the bone marrow. Thus, screening for amyloidosis is necessary in idiopathic LV thickening, atrial fibrillation, and heart failure for timely intravital diagnosis and therapy.

Aim. To study the relationship of obstructive respiratory disorders during sleep with subclinical development of right ventricular dysfunction and pulmonary hypertension, as well as with the risk of an adverse course of chronic heart failure (CHF) with preserved left ventricular ejection fraction (LVEF). Materials and methods. The study included 86 men with moderate and severe forms of obstructive sleep apnea syndrome (OSAS) (with an apnea/hypopnea index (AHI) > 15 per hour). All patients had abdominal obesity and hypertension. Upon inclusion in the study, all patients underwent polysomnography and echocardiography according to the standard protocol with an additional assessment of the fractional area change in the right ventricular myocardium (ΔSRV) and the right ventricular stroke work index (RVSWI). Also, the content of the N-terminal brain natriuretic peptide precursor (NT-proBNP) in the blood serum was determined by enzyme immunoassay analysis. A six-minute walk test (6MWT) was performed after inclusion in the study and after 12 months of follow-up. Depending on the course of CHF during the follow-up, retrospectively, the patients were divided into 2 groups: with an unfavorable ( n = 33) and favorable ( n = 53) prognosis. Results. A significant relationship between AHI and ΔSRV, RVSWI, NT-proBNP, and 6MWT was revealed. Based on the results of one-way correlation analysis, it was found that ΔSRV (odds ratio (OR) 2.51; 95% confidence interval (CI) 2.42–3.24; p = 0.0009), NT-proBNP (OR 1.92; 95% CI 1.32–2.78; p = 0.003), and AHI (OR 3.93; 95% CI 2.87–4.11; p = 0.018) were predictors of an adverse course of CHF. In a multivariate analysis, it was found that AHI was an independent predictor of an adverse course of CHF (OR 3.49; 95% CI 2.17–11.73; p = 0.0008), while the addition of NT-proBNP improved risk stratification of an adverse course of CHF (OR 4.66; 95% CI 3.87–13.11; p < 0.0001). Conclusion . The fractional area change in the right ventricular myocardium (ΔSRV) can be considered as a non-invasive marker for determining the emerging right ventricular dysfunction and predicting adverse cardiovascular events in patients with preserved LVEF and OSAS. Moreover, the combined use of echocardiographic (ΔSRV) and laboratory (NT-proBNP) markers can improve risk stratification of CHF progression.

Cardiac amyloidosis (amyloid cardiomyopathy) is a disease damage to the heart caused by extracellular amyloid deposition. In some cases, there may be local damage to the structures of the heart, for example, the atria; more often, heart damage is part of a systemic (generalized) pathology. Depending on the amyloid precursor protein, 36 types of amyloidosis are described, among which hereditary and acquired forms are distinguished. Cardiac amyloidosis is diagnosed 1) in the case of the amyloid infiltration in the myocardial bioptates or 2) in the case of non-cardiac amyloid deposition and the left ventricular wall thickening >12 mm without arterial hypertension and other reasons. The heart is most often affected in AL-, ATTR-, AA-, AANF-types of amyloidosis. Cardiac amyloidosis should be considered in patients with a heart failure with an unclear etiology, especially with preserved left ventricular ejection fraction, refractory to treatment, with proteinuria and CKD 4-5, in patients with idiopathic atrial fibrillation and conduction disturbances, in patients with left ventricular wall thickening of unclear etiology, low ECG voltage, unexplained arterial hypotension and pulmonary hypertension. Screening for cardiac amyloidosis should include non-invasive methods such as electrophoresis and immunofixation of blood and urine proteins, the free light lambda and kappa chains of immunoglobulins, 99Tc-DPD scintigraphy, genetic testing (if hereditary variants of amyloidosis are suspected), as well as a histological examination of biopsy samples stained with Congo red and polarizing microscopy.

Aim. To determine the impact of polymorphic variant rs1739843 of the HSPB7 gene on clinical profile and outcomes in patients with hypertrophic cardiomyopathy (HCM).Material and methods. The study population consisted of 108 patients with HCM ≥45 years old. The control group included 192 healthy donors. The design of the study included an assessment of the clinical course, determining the outcome of HCM using a new methodological approach proposed by Rowin EJ, et al. (2017). Polymorphic variant rs1739843 of the HSPB7 gene was genotyped by allele-specific real-time polymerase chain reaction (PCR) assay.Results. It was found a significant increase in frequency of TT genotype of rs1739843 of the HSPB7 gene in patients with HCM — 20,4%, compared with control group — 4,2% (ТТ: ТС+СС, odds ratio (OR) =5,88, 95% confidence interval (CI) =2,52-13,75, pConclusion. HCM progression along 2 and more adverse pathways in patients ≥45 years old has been characterized with adverse outcome. The T allele and TT genotype of rs1739843 of the HSPB7 gene were more frequent in patients with HCM ≥45 years old, compared with control group. It was also found a significant increase in frequency of TT genotype and T allele of rs1739843 of the HSPB7 gene in HCM patients with oligosymptomatic HCM course, compared with control group.Allele T of rs1739843 of the HSPB7 gene is associated with 2 and more adverse pathways of HCM progression.

Rationale: More than half of patients with chronic heart failure have a preserved left ventricular ejection fraction. It has been found that sudden cardiac death is more frequent in this patient category. A reliable and easily available marker is needed that would predict the death outcome with a high probability. The use of the late ventricular potentials for this purpose could be reasonable.Aim: To assess the prevalence of the late ventricular potential and their association with the structural and functional parameters of the myocardium in patients with early stages of chronic heart failure with preserved ejection fraction.Materials and methods: We examined 77 patients with chronic heart failure (grade I to IIА, functional class I to III) with preserved ejection fraction (> 50%), aged 47 to 77 years (mean ± SD, 59.7 ± 7.8 years). Echocardiography (SonoScape 8000 and Vivid 3) at М-, В- and Doppler modes was performed in all patients. Ventricular ectopic activity and late ventricular potentials were registered during 24- hour ECG monitoring (Cardiotekhnika, INKART, St. Petersburg).Results: Late ventricular potentials were found in 13% (10 of 77) of patients. The following correlations were found in patients with concentric hypertrophy (n = 53): between the duration of the filtered QRS (TotORSF) and the left ventricular end diastolic diameter (r = 0.27, p = 0.049), TotORSF and the interventricular septum thickness (r = 0.28, p = 0.04), TotORSF and the left ventricular myocardial mass (r = 0.35, p = 0.01), mean square magnitude of fluctuations within the last 40 ms of ORS (RMS40) and the left ventricular end diastolic diameter (r = -0.42, p < 0.01), RMS40 and the left ventricular end-systolic diameter (r = -0.37, p < 0.01). In the patients with eccentric hypertrophy (n = 14), the following correlations were found: between RMS40 and the left ventricular myocardial mass index (r = -0.77, p < 0.01), between duration of the low amplitude signal (< 40 μV) at the end of QRS (LAS40) and the left ventricular myocardial mass index (r = 0.76, p < 0.01), as well as between LAS40 and RMS40 with the end diastolic diameter of the left ventricle adjusted to the body surface area (r = 0.76, p < 0.01 and r = -0.83, p < 0.01, respectively). The patients with a pseudo normal type of diastolic dysfunction (n = 27) showed an association between their NT-proBNP levels and TotORSF (r = 0.56, p < 0.01).Conclusion: The prevalence of late ventricular potentials among patients with early stage chronic heart failure and preserved left ventricular ejection fraction is not high. The parameters of electrophysiological myocardial instability in patients with concentric hypertrophy are mostly influenced by the size of heart chambers, whereas in those with the eccentric one, by the myocardial mass and volume parameters. The risk of sudden cardiac death is higher in patients with a pseudo normal type of diastolic dysfunction.

The paper reviews major biomarkers for determining the prognosis in patients with chronic heart failure and preserved ejection fraction. It also considers cystatin C, one of the novel and probably the most practically important biomarkers.Представлен обзор основных биомаркеров, определяющих прогноз у пациентов с хронической сердечной недостаточностью и сохраненной фракцией выброса. В том числе рассмотрен один из новых и, вероятно, наиболее практически значимый биомаркер - цистатин С.

For the long time the systolic myocardial dysfunction was traditionally associated with the severity of chronic heart failure (CHF). Increasing number of patients with symptoms of CHF but without systolic dysfunction has drawn the attention of specialists to so-called CHF with preserved ejection fraction. Prognosis in CHF with preserved ejection fraction may be as bad as in CHF with reduced ejection fraction. Significant changes in views on the pathogenesis of CHF led to the creation of new therapeutic approaches in the treatment of this disease. However, at present, convincing evidence base of mortality reduction in patients with CHF with preserved ejection fraction using well-known therapeutic agents is unavailable. It makes conduct active searches for new biological markers of diastolic heart function. Participation of proinflammatory cytokines, in particular GDF-15, in the process of elasticity reduction and relaxation disorders of left ventricular myocardium, may be of great importance in the development of new medical agents designed to delay the progression of CHF with preserved ejection fraction.