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OBJECTIVE: To establish the criteria for reversibility of myocardial contractility in patients with coronary artery disease (CAD) after coronary artery bypass grafting considering data of cardiac magnetic resonance imaging (MRI) and echocardiography. MATERIAL AND METHODS: We studied the results of coronary artery bypass grafting in 186 patients with CAD complicated by reduced left ventricular ejection fraction (<30%). All patients underwent cardiac MRI and echocardiography before surgery. Immediate and long-term results were evaluated according to echocardiography and MRI data. RESULTS: We confirmed the previously established predictors of improvement in left ventricular contractility: diastolic IVST ≥10.5 mm and PWT ≥9.5 mm, score of LV myocardium damage according to MRI with delayed contrast enhancement (p<0.05). Multivariate analysis makes it possible to calculate prognostic index and obtain information about further myocardial contractility after revascularization with an error of 6%. CONCLUSION: Echocardiography and contrast-enhanced cardiac MRI are valuable to assess morphological and functional state of the left ventricle in patients with ischemic cardiomyopathy and preoperatively determine functional reserve of the myocardium.

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POLG-associated diseases are rare causes of pharmacoresistant epilepsy and status epilepticus, especially in adult patients. Phenotypic and genotypic variability in these conditions causes the complexity of their diagnosis. In the study, we report a case of a 33-year-old female patient who developed recurrent convulsive status epilepticus with focal clonic onset at the week 22/23 of pregnancy. Intensive anti-seizure therapy was administered, including the use of valproic acid, as well as the treatment of somatic complications. Given the acute onset, the semiology of seizures, the presence of psychopathological symptoms, autoimmune etiology of the disease was initially suspected. A month after the withdrawal of valproic acid, the patient began to show signs of toxic hepatitis, which eventually led to death. According to the results of whole-exome sequencing obtained later, the patient was a carrier of a pathogenic homozygous variant c.2243G>C (p.W748S) in the POLG gene. The presented case highlights the importance of molecular genetic testing and the risk associated with valproic acid hepatotoxicity in patients with cryptogenic epileptic status.

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Objective To evaluate prospects for clinical use of circulating biomarkers for characterizing fibrotic changes in the myocardium of patients with hypertrophic cardiomyopathy (HCMP) with left ventricular (LV) outflow tract obstruction.Materials and Methods This was a prospective study with a 12-month follow-up period. The study included 47 patients (29 females and 18 males) with obstructive HCMP who were selected for septal reduction. Echocardiography (EchoCG), cardiac magnetic resonance imaging (MRI) and measurements of serum C-reactive protein, N-terminal pro-brain natriuretic peptide, and relevant circulating markers of fibrosis (TGF-ß1, MMP-2,-9, TIMP-1, galectin-3, sST2, CITP, PICP, and PIIINP) were performed for all patients. All patients were evaluated at baseline and at 7 days, 6 and 12 months following surgical treatment. Morphometrical analysis of intraoperative biopsy samples was performed for evaluation of the degree of fibrotic changes. Patients received beta-blockers (95.7%), angiotensin-converting enzyme inhibitors/angiotensin receptor blockers (34%), loop diuretics (68.1%), aldosterone antagonists (34%), and statins (66%).Results Women with HCMP were older and more frequently had additional risk factors (arterial hypertension). Men had a higher risk of sudden cardiac death. Histological study of intraoperative myocardial biopsy samples showed that the area of fibrotic changes was 13.9±6.9%. According to cardiac MRI mean area of delayed contrast enhancement was 8.7±3.3% of LV myocardial mass. No association was established between traditional cardiovascular risk factors and severity of myocardial fibrotic changes or levels of circulating fibrosis markers. Perhaps that was due to the modifying effect of the drug therapy received by HCMP patients. According to EchoCG maximum pressure gradient in the LV outflow tract before the surgical treatment was 88 (55; 192) mm Hg, and interventricular septal thickness was 22 (16; 32) mm. A considerable decrease (p=0.0002) in the LV outflow tract gradient was observed after myectomy in all patients. At the same time, the left ventricular dimension, which tended to decrease in the early postoperative period, returned to baseline values by the 6th month of follow-up.Conclusion The study confirmed the increase in relevant circulating markers of fibrosis in patients with obstructive HCMP. At the same time, no correlation was observed between levels of circulating biomarkers and severity of fibrosis according to data of histology and cardiac MRI, which was probably due to the modifying effect of drug therapy and limited sampling.

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OBJECTIVE: the aim of the study was to characterize the mechanical properties of the pulmonary arterial wall (PA) in patients with pulmonary arterial hypertension (PAH) using magnetic resonance imaging (MRI) of the heart, and to determine their diagnostic and prognostic value. MATERIALS AND METHODS: 57 patients with PAH were examined. The diagnosis of PAH was verified according to the recommendations of the ERS/ESC from 2015. All patients underwent a detailed echocardiographic (ECHO) study, MRI of the heart and right heart catheterization (RHC). To calculate the stiffness of the pulmonary artery wall, the MRI and RHC data were used. RESULTS: We identified a correlation between the functional class of PAH and the parameters of hemodynamic, physical performance, ECHO parameters of the right chambers. There were no differences in the stiffness of the pulmonary artery wall, depending on the functional class of PAH. Among the six stiffness indicators, only pulsation index was associated with the structural and functional parameters of the right ventricle and pulmonary vascular resistance. CONCLUSION: The MRI pulsation index is the simpleststiffness index of the pulmonary artery wall and the most promising one for evaluating the prognosis of patients with PAH.

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AIM: The aim of the study was to characterize the mechanical properties of the pulmonary arterial wall (PA) in patients with pulmonary arterial hypertension (PAH) using magnetic resonance imaging (MRI) of the heart, and to determine their diagnostic and prognostic value. MATERIALS AND METHODS: 57 patients with PAH were examined. The diagnosis of PAH was verified according to the recommendations of the ERS/ESC from 2015. All patients underwent a detailed echocardiographic (ECHO) study, MRI of the heart and right heart catheterization (RHC). To calculate the stiffness of the pulmonary artery wall, the MRI and RHC data were used. RESULTS: We identified a correlation between the functional class of PAH and the parameters of hemodynamic, physical performance, ECHO parameters of the right chambers. There were no differences in the stiffness of the pulmonary artery wall, depending on the functional class of PAH. Among the six stiffness indicators, only pulsation index was associated with the structural and functional parameters of the right ventricle and pulmonary vascular resistance. CONCLUSION: The MRI pulsation index is the simpleststiffness index of the pulmonary artery wall and the most promising one for evaluating the prognosis of patients with PAH.

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AIM: To carry out complex assessment of the right ventricular (RV) function with two-dimensional echocardiography (2D-EchoCG) for detection of most informative markers of the disease severity in patients with pulmonary hypertension (PH). MATERIAL AND METHODS: We examined 63 patients with PH (38 with idiopathic PH, 7 with corrected congenital heart defects, 6 with systemic scleroderma, 12 with chronic inoperable thromboembolic PY). Examination included right heart catheterization, 2D-EchoCG, and cardiac magnetic resonance tomography (MRT). RESULTS: 2D-EchoCG revealed dilation of right chambers of the heart, hypertrophy of RV anterior wall, increase of ratio of right to left ventricular end-diastolic dimensions (RV:LV), reduction of LV stroke volume, diminution of amplitude and velocity of tricuspid annular plane systolic excursion, and significant increase of myocardial performance index Tei. MRT data evidenced for lowering of RV ejection fraction. Canonical correlation was found between integral characteristic of 2D-EchoCG and integral hemodynamic characteristic (r=0.77; p=0.007). We also determined threshold values of RV: LV to be used for stratification of risk in patients with PH. CONCLUSION: In patienns with PH calculation of simple 2D-EchoCG parameters provides information important for determination of disease severity, selection of optimal method of treatment, and monitoring of patients condition.

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Takayasu arteritis is well-known rare form of large vessel vasculitis. Pulmonary involvement is observed in 50% of cases. However, pulmonary hypertension is a rare complication of pulmonary artery stenosis. In this article we present a rare clinical case of Takayasus arteritis with typical stenosis of subclavian, carotid arteries, and involvement of pulmonary arteries with development of severe pulmonary hypertension. The article also contains discussion of current methods of diagnosis and treatment of pulmonary artery stenosis in Takayasus arteritis with special focus on effectiveness and complications of angioplasty and surgery.

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