[AL-amyloidosis with cardiac involvement. Diagnostic capabilities of non-invasive methods].

There are presented the literature data and a description of the clinical course of the disease in isolated/predominant cardiac amyloidosis. Amyloid cardiomyopathy is the most common phenocopy of hypertrophic cardiomyopathy. The modern possibilities of non-invasive diagnostics using osteoscintigraphy for the differential diagnosis between amyloid cardiomyopathy caused by AL- and transthyretin amyloidosis are described in detail.

[Results of 3 years work of the Russian hospital register of chronic heart failure (RUssian hoSpital Heart Failure Registry - RUS-HFR): relationship between management and outcomes in patients with chronic heart failure].

AIM: To analyze management and outcomes in patients with CHF managed by specialists in heart failure (HF) or general cardiologists/physicians in real-life clinical practice. MATERIALS AND METHODS: Survival rate, rehospitalization rate, general health condition, and the administered therapy were evaluated for HF patients with reduced LV ejection fraction at three years of discharge from cardiological hospitals. These patients had been included in a prospective, multicenter, observational study, "The Russian Hospital HF Registry" (RUS-HFR). The first group consisted of patients who were managed at a specialized HF department of the Federal Center and followed up at the outpatient stage by a cardiologist specializing in HF (Group 1, St.­Petersburg; n =74). The other two groups (Group 2 and Group 3) included patients who were managed at other cardiological departments of the Federal Center (n=186) or the Regional Center (n=130) and subsequently followed up at the place of residence. RESULTS: After the discharge from the hospital, 58-95 and 12-19% of RUS-HFR patients were followed up by a cardiologist or a physician, respectively, on an outpatient basis while 5-23% of patients did not visit a doctor at all. In three years, the survival rate of Group 1, 2, and 3 patients was 80 vs. 78 (р>0.05) vs. 52% (р0.05) vs. 100% (p1,2.

[Noninvasive Electrophysiological Mapping in Patients With Complete Left Bundle Branch Block and Different Modes of Biventricular Pacing].

OBJECTIVE: to investigate possibilities of noninvasive electrophysiological mapping (NEM) during biventricular (BiV) pacing and to compare the results with data of 12 lead ECG and tissue Doppler echocardiography (TDI). MATERIAL AND METHODS: The study included 25 patients with complete left bundle branch block (LBBB) and implanted cardiac resynchronization therapy (CRT) system. Twenty-two patients were CRT responders, three other patients did not demonstrate a clear effect. NEM was performed with "Amycard 01C EP LAB" system and multi slice computed tomography. RESULTS: Qualitative analysis of isochronous maps at NEM showed that the most homogenous color coding of the left ventricle (LV) may correspond to the optimal BiV pacing mode. A statistically significant positive correlation between the width of the QRS complex during a set BiV pacing mode and the standard activation deviation time, measured at NEM, for 12 segments (SD12) LV (r= 0,88, p<0,0001) was observed. With the minimum value of SD12 and biventricular QRS width at the same time, three patients had intraventricular dyssynchrony. A comparative analysis of NEM and TDI methods in terms of interventricular dyssynchrony (IVD) at LBBB revealed statistically significant positive correlation (r=0,58, p=0,005). CONCLUSION: These data show that both minimal values of QRS and SD12 at BiV pacing are not accompanied by the effect of CRT in all patients. The lack of response from this type of therapy is associated with a non-optimal position of the LV electrode. Qualitative assessment of isochronous maps at NEM demonstrates intraventricular dyssynchrony and can help in selecting the optimal mode of BiV pacing.

[Results of Russian Hospital Chronic Heart Failure Registry in Three Subjects of Russian Federation].

Aim of the RUssian hoSpital Heart Failure Registry (RUS-HFR) was to analyze clinical and epidemiological characteristics, application of modern drug and non-drug therapy among hospitalized patients with systolic chronic heart failure (CHF) in 3 subjects of Russian Federation. MATERIAL AND METHODS: We conducted a prospective multicenter observational study in 3 cardiological centers (Petersburg, Orenburg, Samara) in which we recruited patients aged 18-75 years with left ventricular ejection fraction less or equal 40% without ongoing myocardial infarction or pulmonary thromboembolism. RESULTS: Between October 2012 and June 2014 we included 524 patients (mean age 60 years, >79% men) mostly with NYHA class III CHF. Main etiological factors were ischemic heart disease and arterial hypertension, which was not corrected by the time of admission in 20-40.7% of patients. Portions of patients who had undergo the myocardial revascularization, surgery because of valvular heart defects, and implantation of permanent pacemaker including device for cardiac resynchronizing therapy were 11-41, 0-4, and 0-19.5%, respectively. In up to 25% of patients therapy with one of main medications or diuretic was initiated after hospitalization. At discharge 82.3-8.3, 76.3-95.8, 65.9-81.1, 80.7-94.6% of patients received ACEI/ARB, -adrenoblockers, MRA, diuretic therapy, respectively. Implantation of cardioverter ICD/CRT, ICD and heart transplantation were considered indicated in 0-21.2 and 0-6.6% of patients, respectively. Duration of hospitalization was 26+/-14, 15+/-10, and 13+/-5 bed-days in Petersburg, Orenburg, and Samara, respectively. CONCLUSIONS: Compared with data obtained 10 years earlier portrait of a patient with in general remained unchanged. However portion of patients who had undergone cardio-surgical and electrophysiological interventions became tangible, and numbers of patients receiving therapy with ACEI/ARB, -adrenoblockers, and MRA became comparable with those in European registries. Significant deviations from guidelines on drug therapy were revealed at prehospital stage; and high-technology methods of therapy were insufficiently often recommended in participating hospitals. Portion of hospitalizations not related to acute decompensation was 5-37%, while duration of such hospitalizations as well as hospitalizations because of acute decompensation of CHF significantly exceeded this parameter in EU and USA. Absence of obligatory drug therapy prior to admission in a part of patients in RUS-HF raises questions on correction of patients compliance and on creation of a system of specialized ambulatory surveillance of CHF patients after discharge from hospital.