The article describes the rare clinical case of transthyretine amyloidosis in young patient, manifested by heart rhythm disturbances and biventricular heart failure.

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.

Вегетативный дисбаланс, развивающийся при инфаркте миокарда (ИМ) и характеризующийся активацией симпатической нервной системы и угнетением парасимпатической, является самостоятельным и независимым фактором риска внезапной смерти. Ввиду того, что возможности медикаментозного воздействия на вегетативный дисбаланс ограничены, представляется актуальным поиск альтернативных методов воздействия, одним из которых являются физические тренировки (ФТ).Цель. Оценить влияние ранних ФТ на вариабельность сердечного ритма (ВСР), как метода оценки вегетативного дисбаланса, у пациентов, перенесших ИМ.Материалы и методы. В исследование были включены 60 пациентов мужского пола с неосложненным Q -ИМ, которые были разделены на две группы: тренируемая (30 человек) и контрольная (30 человек). До начала исследования (10-18-е сутки ИМ) всем пациентам были выполнены ЭКГ, ЭхоКГ, суточное мониторирование ЭКГ, велоэргометрия, ВСР. Все исследования были проведены повторно через 4, 6 и 12 мес. Исходно все пациенты получали стандартную медикаментозную терапию. Пациенты контрольной группы проходили стандартную программу реабилитации амбулаторно, пациенты тренируемой группы через 1,5 мес после ИМ цикл ФТ, который длился 8 нед и состоял из 1-часовых занятий 3 раза в неделю.Результаты. В ходе обследования установлено, что у больных, перенесших ИМ, проходивших обычную систему реабилитации, к концу года наблюдается ухудшение ВСР за счет нарастания симпатических и снижения вагусных влияний. ФТ, проводимые в ранние сроки после ИМ, способствуют повышению ВСР и уменьшению вегетативного дисбаланса за счет снижения симпатикотонии и повышению тонуса вагуса. К концу 1-го года после ИМ ухудшения ВСР не наблюдается. ФТ у больных в ранние сроки ИМ приводят к снижению частоты сердечных сокращений, артериального давления, увеличению фракции выброса левого желудочка и повышению толерантности к физическим нагрузкам. ФТ способствуют уменьшению реактивной тревожности у больных, перенесших ИМ.Выводы. Ранние ФТ, проводимые по разработанной методике, позволяют уменьшать вегетативный дисбаланс, развивающийся при ИМ, и, таким образом, играют важную роль во вторичной профилактике ИМ и внезапной смерти.

Takayasu arteritis is a well-known rare form of large vessel vasculitis. This review details the clinical features, opportunities of disease activity assessment including new serum biomarkers and imaging diagnostics. Current evidence based treatments are presented and discussed. Particular attention is paid to the effectiveness and complications of interventional and surgical treatment in arteritis Takayasu.

A 31 years old male patient was admitted to a resuscitation unit with severe dyspnea (MMRC 4 grade). The patient had the dyspnea for 3 months.The diagnosis of chronic thrombo-embolic pulmonary hypertension with floating thrombi in the right heart ventricle was established. On the sixth day of admission after IVC filter insertion, the patient underwent thromboectomy with pulmonary endarterectomy. For an access to segmentary pulmonary arteries during operation SVC and the aorta had been cross-clamped. The duration of deep hypothermic (20 degrees C) circulatory arrest was 54 minutes. The patient was discharged in satisfactory condition on the 17th day. At the check-up at 3 months the dyspnea was absent and heart hemodynamic parameters had been normalized.

Aim. To investigate sudden cardiac death (SCD) predictors in patients after myocardial infarction (MI), to develop the strategy of risk stratification and SCD prevention. Material and methods. In total, 420 patients were examined at Day 10-14 after MI; follow-up period lasted for 1-4 years. General clinical examination, echocardiography, 24-hour electrocardiography (ECG) monitoring, late ventricular potentials (LVP) detection, active orthostatic test (AOT), heart rate variability (HRV) assessment at baseline and during functional tests, as well as psychological testing, were performed. Some participants underwent coronarography and endocardial electrophysiological examination. SCD risk was assessed by Cox multi-factor analysis and Kaplan-Meier survival curve method. Results. According to Cox multivariate regression analysis, the most important predictors included the following six parameters: LVP, HRV reduction, left ventricular ejection fraction <40 %, ventricular arrhythmias, previous MI, and hypotension in AOT Prognostic accuracy and positive predictive value of this model were high enough to assess SCD risk. Based on modeling results, in particular, on the presence of structural and trigger mechanisms of life-threatening arrhythmias, SCD risk stratification was performed. Differential preventive strategy was based on risk levels. Conclusion. In patients after MI, SCD risk stratification (very high, high, intermediate, and low risk), together with differential preventive measures, facilitated a two-fold reduction in sudden and cardiac death incidence, comparing to the control group.

Aim. To assess prognostic value of clinical and functional factors in regard to sudden cardiac death (SCD) risk in patients after myocardial infarction (MI). Material and methods. In total, 420 patients were examined at Day 10-14 after MI; follow-up period lasted for 14 years. General clinical examination, echocardiography, 24-hour electrocardiography (ECG) monitoring, late ventricular potentials (LVP) detection, active orthostatic test (AOT), heart rate variability (HRV) assessment at baseline and during functional tests, as well as psychological testing, were performed. SCD risk was assessed by Cox multi-factor analysis and Kaplan-Meier survival curve method. Results. According to Cox multivariate regression analysis, the most important predictors included the following: LVP, HRV reduction, left ventricular ejection fraction <40%, ventricular arrhythmias, previous MI, and hypotension in AOT. Prognostic accuracy and positive predictive value of this model were high enough to assess SCD risk. Conclusion. To assess SCD risk in MI patients, complex clinical and functional factors - SCD predictors – should be measured.

Aim. To assess prognostic value of clinical and functional factors in regard to sudden cardiac death (SCD) risk in patients after myocardial infarction (MI). Material and methods. In total, 420 patients were examined at Day 10-14 after MI; follow-up period lasted for 14 years. General clinical examination, echocardiography, 24-hour electrocardiography (ECG) monitoring, late ventricular potentials (LVP) detection, active orthostatic test (AOT), heart rate variability (HRV) assessment at baseline and during functional tests, as well as psychological testing, were performed. SCD risk was assessed by Cox multi-factor analysis and Kaplan-Meier survival curve method. Results. According to Cox multivariate regression analysis, the most important predictors included the following: LVP, HRV reduction, left ventricular ejection fraction <40%, ventricular arrhythmias, previous MI, and hypotension in AOT Prognostic accuracy and positive predictive value of this model were high enough to assess SCD risk. Conclusion. To assess SCD risk in MI patients, complex clinical and functional factors - SCD predictors - should be measured.

Prognostic significance of duration and dispersion of interval QT as well as relationship of these parameters to other clinical and instrumental signs were studied in 220 survivors of acute myocardial infarction (MI). These patients were examined on days 10-14 of MI and then followed for 12-36 months. Values of corrected QT (QTc) increased with increase of degree of severity of heart failure and derangements of left ventricular function, intensity of ventricular disturbances of rhythm. QT dispersion depended on left ventricular contractile function and rose in anterior localization of MI and non-Q MI. Dependence was observed between QTc duration and mortality of patients after MI, both total and cardiovascular including sudden. At multifactorial analysis QTc>440 ms appeared to be independent predictor of risk of total and cardiac mortality in patients with anterior non-Q MI. QT dispersion did not possess sufficient informative power for prognostication of outcomes.

Late ventricular potentials (LVP), heart rate variability (HRV) and dispersion of QT interval (QTd) were studied in 91 patients with myocardial infarction with various ventricular arrhythmias. Patients with episodes of sustained ventricular tachycardia (group 4) had the following characteristics: prevalence of LVP 73.7%, QTd 82.5 ms, standard deviation of RR intervals (SD) 26.5 ms; spectral analysis of HRV revealed preponderance of sympathetic influences and lowered vagal activity. Frequency of LVP detection, QTd and SD in patients with ventricular extrasystoles (Lown classes 3-5) (group 3) were 33.3%, 72.8 ms, and 42.8 ms, respectively. Patients of group 3 also had augmented sympathetic and lowered parasympathetic influences. These data significantly differed from those obtained in patients with Lown class 1-2 ventricular extrasystoles (group 2) and patients without extrasystoles (group 1). Groups 3 and 4 had significantly different prevalences of LVP and values of some HRV parameters but similar QTd. There was close correlation between presence of severe ventricular arrhythmias and some parameters of HRV and signal averaged ECG. Stepwise regression analysis showed that the following group of parameters was related to the presence of malignant ventricular rhythm disturbances: heart rate, SD and total QRS duration (p<0.05). Thus patients with life threatening ventricular arrhythmias were characterized by the presence of LVP and changes of some parameters of HRV and QTd. Registration of these parameters can apparently be used for prediction of potentially fatal ventricular arrhythmias in patients with myocardial infarction.

Aim. To assess prognostic value of clinical and psychological factors in regard to sudden cardiac death (SCD) risk in patients after myocardial infarction (MI).Material and methods. In total, 420 patients were examined at Day 10-14 after MI; follow-up period lasted for 1-4 years. General clinical examination, echocardiography, 24-hour electrocardiography (ECG) monitoring, late ventricular potentials (LVP) detection, active orthostatic test (AOT), heart rate variability (HRV) assessment, psychological testing, if necessary - coronary angiography and endocardial electrophysiological examination were performed.Results. In the first post-MI year, SCD incidence was 6,7%. SCD risk was predicted by anterior MI localization, left ventricular (LV) aneurysm, heart failure, pre-syncope in anamnesis, hypotension during AOT, sinus tachycardia, left bundle block, anxiety and depression symptoms, leukocytosis and monocytosis in acute AMI period, alcohol abuse in anamnesis, treatment features (no beta-adrenoblockers; diuretic therapy). In multivariate analysis, the most important clinical factors included previous MI, hypotension in AOT, LV ejection fraction, ventricular arrhythmias by 24-hour ECG monitoring, HRV and LVP assessment data.Conclusion. Due to high SCD rates in MI patients, SCD risk should be assessed as early as during hospitalization, using relevant clinical and psychological parameters. If needed, detailed examination and secondary SCD prevention measures should be performed.