Russian Journal of Cardiology 2016 11 (139)

Address to the readers

Russ J Cardiol 2016, 11 (139): 5

CLINICAL MEDICINE NEWS

Clinical medicine updates: a review of international news

Russ J Cardiol 2016, 11 (139): 6

ORIGINAL ARTICLES

THE CONDITION OF DISTAL AORTA AFTER PROXIMAL RECONSTRUCTION FOR DISSECTION IN MARFAN SYNDROME

Chernyavskiy A. M., Lyashenko M. M., Sirota D. A., Alsov S. A., Khvan D. S.

Abstract. Marfan syndrome is a disease associated with high prevalence of aorta-related morbidity and mortality. Dissections and ruptures of thoracal aorta (ThAo) lead to decreased life duration in persons with Marfan syndrome with mediana at 30 year-old.

Aim. To study the changes of distal aorta and clinical outcomes after surgical treatment for proximal aorta dissection in Marfan syndrome.

Material and methods. In the SRICP, among patients operated for proximal aorta dissection, 14 were selected, who had histologically proven Marfan syndrome. They were followed-up.

Results. In the long-term follow-up after the intervention on thoracoabdominal aorta (TAAo) was required in 2 patients. Reasons for transforming of the distal aorta were the age of the disease onset (p=0,04), acute stage of dissection (p=0,02). Changes in TAAo developed during first months after primary intervention, as in several years.

Conclusion. Surgery for proximal dissections in Marfan syndrome patients is related to acceptable levels of morbidity and mortality in early and post-operation periods. Regular follow-up is required of such patients in post-operation period for on-time diagnostics and performing of secondary interventions.

Russ J Cardiol 2016, 11 (139): 7–11

dx.doi.org/10.15829/1560-4071-2016-11-7-11

Key words: Marfan syndrome, aorta dissection, aorta aneurysm, surgery, dysplasia.

Center for Surgery of Aorta, Coronary and Peripheral Arteries, E. N. Meshalkin Novosibirsk Scientific-Research Institute of Circulation Pathology, Novosibirsk, Russia.

LONG-TERM RESULTS OF THE VALVE-SAVING SURGERY IN MARFAN SYNDROME PATIENTS

Khvan D. S., Chernyavskiy A. M., Sirota D. A., Alsov S. A., Lyashenko М. М.

Abstract. The main causes of mortality and morbidity in Marfan syndrome patients are cardiovascular complications. The Bentall DeBono operation for long time has been a gold standard treatment for this pathology.

Aim. To present the obvious benefits of valve-saving surgery stimulating for saving of aorta valve (AoV) in dysplasia of connective tissue.

Material and methods. A single-center retrospective analysis was done, of the results of valve-saving operations on the aorta root. All Marfan syndrome patients were included, who had undergone valve-saving operation on AoV in 2007-2015 y. As the valve-saving operation the David method was used, operation of reimplanting of aorta root into prosthesis and supracoronary prosthesing of ascending aorta.

Results. Nine-year freedom from aortic regurgitation more 2 grade in long-term follow-up was 64%. As the a causes for development of aortic valve insufficiency were progressing degenerations of AoV. There were no cases of bleedings and endocarditis. Expected cumulative survival was 63%.

Conclusion. Valve-saving operations are applicable in patients with the syndrome of connective tissue dysplasia. However, the worth of valve-saving operations in Marfan syndrome is questionable. Taken the known data, the studies demanded, with more number of patients and longer follow-up.

Russ J Cardiol 2016, 11 (139): 12–15

dx.doi.org/10.15829/1560-4071-2016-11-12-15

Key words: aorta, aortic regurgitation, valve-saving surgery, Marfan syndrome.

Center for Surgery of Aorta, Coronary and Peripheral Arteries, E. N. Meshalkin Novosibirsk Scientific-Research Institute of Circulation Pathology, Novosibirsk, Russia.

Afanasiev A. V., Zheleznev S. I., Bogachev-Prokofiev A. V., Nazarov V. M., Demin I. I., Astapov D. A., Karaskov A. M.

Abstract

Aim. To analyze the results of valve-securing surgery on mitral valve in connective tissue dysplasia.

Material and methods. From 2011 to 2014 y., totally 171 patient included, with isolated mitral regurgitation. Patients were randomized to 2 groups. Mean age in the Group D — ring, and Group C — flex, was 57 [42;65] and 54 [41;63] year old, respectively (p=0,092). Part of males was 69 and 67% in the groups, respectively.

Results. There were no cases of 30-day mortality. During 24 months of follow-up, in D ring and C flex groups, survival was 96,0±2,3% (95% CI 88,6-98,7%) and 94,3±2,8% (95% CI 85,5-97,9%), resp. (р=0,899); no re-operation — 97,0±2,1% (95% CI 88,4-99,3%) and 100%, resp. (р=0,044); no return of significant mitral regurgitation 80,8±6,5% (95% CI 64,0-90,3%) and 92,8±3,1% (95% CI 83,4-97,0%), resp. (р=0,002).

Conclusion. Application of flexible C flex rings in valve-securing operations makes it to achieve better results in middle-long-term post-operation period comparing to the usage of semi-hard support rings in patients with connective tissue dysplasia and severe mitral regurgitation.

Russ J Cardiol 2016, 11 (139): 16–21

dx.doi.org/10.15829/1560-4071-2016-11-16-21

Key words: mitral regurgitation, mitral insufficiency, connective tissue dysplasia.

E. N. Meshalkin Novosibirsk Scientific-Research Institute of Circulation Pathology, Novosibirsk, Russia.

Miroshnichenko E. P., Ushakov A. V., Kubyshkin V. F.

Abstract

Aim. To assess the dynamics of aldosterone in blood serum and structurefunctional parameters of the heart in myocardial infarction patients, developed on the background of non-differentiated connective tissue dysplasia.

Material and methods. Totally, 90 patients included with Q-myocardial infarction with non-differentiated connective tissue dysplasia and without it. The controls were almost healthy persons without cardiovascular pathology, comparison group — patients with connective tissue dysplasia but not having cardiovascular pathology. Clinical and phenotypic assessment was done, echocardiography, aldosterone levels measurement in serum at dynamics of the infarction course.

Results. All patients, independent of existence of connective tissue dysplasia, showed aldosterone elevation at 1st day of infarction with further decrease to the level of controls or comparison in 28 days. In the group of infarction patients with non-differentiated connective tissue dysplasia there was significant enlargement of the left ventricle size, reduced pumping function with tendency to less prominent myocardial mass index increase comparing with those not having dysplasia.

Conclusion. Presence of non-differentiated connective tissue dysplasia does not influence aldosterone levels and their dynamics in myocardial infarction, but is associated with adverse type of postinfarction remodeling of the heart presenting with the left ventricle dilatation and decrease of its pumping function.

Russ J Cardiol 2016, 11 (139): 22–26

dx.doi.org/10.15829/1560-4071-2016-11-22-26

Key words: myocardial infarction, undifferentiated connective tissue dysplasia, aldosterone, postinfarction remodeling of heart.

S. I. Georgievsky Medical Academy, V. I. Vernadsky Crimean Federal University, Simferopol, Russia.

Vaykhanskaya T. G.¹, Sivitskaya L. N.², Kurushko Т. V.¹, Danilenko N. G.², Melnikova О. P.¹, Frolov А. V.¹

Abstract. Search for effective methods of risk stratification in patients with higher risk of lifethreatening ventricular tachyarrhythmias (VTA) and sudden cardiac death is important task for applied healthcare and a priority scientific field.

Aim. To invent a mathematic model and algorithm of individualized risk assessment for sudden cardiac death (SCD) in dilation cardiomyopathy patients (DCMP).

Material and methods. Totally, 165 patients included, with verified DCMP (mean age 49,2±11,5 y; 135/81,8% males; NYHA class 2,67±0,45; LV ejection fraction 26,7±10,1%; follow-up 46,7±12,5 months). With an original software "Intecard 7", with the data of 7-minute ECG-12 registration, we evaluated markers of electrical instability of myocardium — microvoltage alternans of T-waves (mATW), turbulence of cardiac rhythm (TCR), intervals QT and JT dispersion, acceleration and deceleration of cardiac rhythm. As primary endpoints for multifactorial Cox-analysis we used sustained ventricular tachicardia (VT) or ventricular fibrillation, shocks of implanted devices and documented SCD. We analyzed clinical, electrocardiographic, echocardiographic data and results of molecular-genetic study of lamin A/C gene (LMNA).

Results. As result of multifactor regression analysis we found 2 cumulative independent predictors (HR 5,23; 95% CI 1,45-16,9; р=0,013) of life-threatening VTA events in DCMP patients: paroxysms of non-sustained VT (≥5 ventricular complexes with HR ≥150 bpm) and changes in LMNA gene (missense mutations and polymorhpism of 10 exon of rs4641). With binary logit-regression analysis of independent risk factors (VES, sVT, mATW, TCR, JTd and GLS LV) we built-up a model of binary regression (F=31,2; χ2=143,2; p=0,0000) and developed an algorithm of SCD risk evaluation that make it to classify with high prediction power up to 93,9%, cases of DCMP (OR 470; sensitivity 80,8%, specificity 99,1%).

Conclusion. The invented algorithm of SCD risk is non-ivasive, individualized, easily applicable and interpretable technology that makes it to stratify patients with higher risk of life-threatening VTA with standard clinical and instrumental methods of investigation (ECG, EchoCG, Holter ECG). Implementation of the oroginal risk-stratification model makes it to optimize tactics of DCMP patients treatment and strategy of selection of potential candidates for cardioverter-defibrillator implanting for primary SCD prevention.

Russ J Cardiol 2016, 11 (139): 27–35

dx.doi.org/10.15829/1560-4071-2016-11-27-35

Key words: sudden cardiac death, dilation cardiomyopathy, markers of electrical instability of myocardium, lamin A/C genes mutation, risk stratification.

¹SI Republic Scientific-Practical Center of Cardiology, Minsk; ²SSI Institute of Genetics and Cytology of National Science Academy of Belarus, Minsk, Belarus.

Frolova Yu. V.¹, Dymova O. V.¹, Babaev М. А.¹, Kim S. Yu.¹, Van Е. Yu.¹, Sinitsin V. Е.², Mershina Е. А.², Dzemeshkevich S. L.¹

Abstract

Aim. To evaluate informativity and relation of changes of galectin-3 (G3) and natriuretic brain peptide (BNP) in diagnostics of myocardium dysfunctions during analysis of surgical intervention in HCMP patients.

Material and methods. An analysis is presented, of 30 patients with diffusegeneralized type of hypertrophic cardiomyopathy (HCMP) (16 females and 14 males, mean age 53,7±2,8 y. o.), who during the time from 2009 to may 2016 in V. B. Petrovskiy Russian National Research Centre of Surgery, underwent the operation of extended myectomy. Before and after operation all patients underwent laboratory tests with measurement of the levels of BNP and G3 in serum, echocardiography, MRI/MSCT of the heart. Operations were done on-pump and pharmaco-cold cardioplegy.

Results. In transthoracal echocardiography there was significant decrease revealed, of interventricular septum thickness and gradient of ascending LV tractus, and nonsignificant enlargement of LV cavity during systole. In analysis of i. v. contrast MRI/ MSCT of the heart in 26 patients there was non-significant increase of LV cavity during systole and decrease of LV mass in diastole. In analysis of serum BNP level as an important marker of clinical course and treatment efficacy, there was significant decrease of the values comparing to baseline in pre-operation period.

Conclusion. The extended myectomy surgery with parietal resection of papillary muscles makes it to reach good effect on an interventricular pressure gradient elimination in the space of whole ascending tractus of the left ventricle. Comparing to BNP that make it possible to evaluate the effectiveness of treatment, G3 is not so effective in diagnostics and there is yet no convincing evidence to confirm the value of G3 concentration in blood as an instrument that can be applied in monitoring of surgery effect.

Russ J Cardiol 2016, 11 (139): 36–39

dx.doi.org/10.15829/1560-4071-2016-11-36-39

Key words: diffuse-generalized form of DCMP, extended myectomy, fibrosis, brain natriuretic peptide, galectin-3.

¹V. B. Petrovskiy Russian National Research Centre of Surgery, Moscow; ²Treatment and Rehabilitation Center of the Ministry of Health, Moscow, Russia.

Kochkina K.V.¹, Kochkina Т.А.¹, Mashtakova О.B.¹, Usik G.А.¹, Myznikov А.V.¹, Kulakov F.S.¹, Malyshkin D.А.¹, Sidorenko А.V.¹, Evtyagin S.Е.¹, Protopopov А.V.1,²

Abstract. Transcatheter implanting of aortic valve (TIAV) in critical aortic stenosis is a separate manipulation requiring thorough preparation. Among patients with critical aortic stenosis from a group of endovascular correction, there is a high prevalence of comorbid atherosclerotic lesions in coronary, carotid arteries, and other pathologies, manageable only interventionally.

Aim. To assess the efficacy and safety of single-moment performing of TIAV and additional endovascular interventions.

Material and methods. From the year 2011, in our clinic endovascular implanting of aortic valve was done for 125 patients with aortic valve dysfunction, in 51% (64 patients) had coronary, renal and carotid arteries lesion, aneurysmatic dilation of abdominal aorta. In 31 (48%) cases together with endovascular valve placement additionally endovascular interventions were done.

Results. Technical success was reached in all 125 cases. In-patient mortality from all causes was 7.2% (9 patients), with no significant difference between combination treatment group and other patients. In 2 cases there was stenting done, of coronary and carotid arteries (with 1 case of coronary, carotid and renal arteries), as single step. In one patient there was endovascular prevention done of ischemic complications of permanent atrial fibrillation by implanting of the system Watchman (Boston Scientific, USA) and stenting of coronary arteries; in one case a single step endoprosthesing of abdominal aorta was done and coronary stenting. In concomitant interventions group there was no myocardial infarction and no significant difference in cerebrovascular events between groups.

Conclusion. TIAV can be followed by single step addition of endovascular procedures if the surgical team and clinics has good experience, and with thorough planning of operation and post-surgery period.

Russ J Cardiol 2016, 11 (139): 41–46

dx.doi.org/10.15829/1560-4071-2016-11-41-46

Key words: concomitant interventions, transcatheter implanting of aortic valve.

¹Regional Clinical Hospital, Krasnoyarsk; ²V. F. Voino-Yasenetsky Krasnoyarsk State Medical University, Krasnoyarsk, Russia.

Kamenskaya O. V., Klinkova A. S., Chernyavskiy A. M., Lomivorotov V. V., Karaskov A. M.

Abstract

Aim. To evaluate the results of prosthetic replacement of ascending aorta and the arc, according to the methods of brain protection.

Material and methods. Totally, 63 patients included, 52 (42-60) year old with DeBakey type I chronic dissection. Group 1 (n=32) — with the method of antegrade brain perfusion (AP) within artificial circulation (AC) and hypothermia (23-24º C); group 2 (n=31) with retrograde perfusion (RP) within AC and profound hypothermia (18º C). With the cerebral oxymetry the frontal lobe oxygen supply was measured (rSO2, %) during the operation. Questionnaire SF-36 was in use for life quality assessment before and late after operation.

Results. In the group 1 the rSO2 during AC reduced less than 10% from baseline, and in the group 2 values of rSO2 reduced by more than 30%, (p<0,05). Neurological complications in group 1 were registered in 12,5% cases, in the group 2 — in 35,4% (p<0,05). Logistic regression showed that decrease of rSO2 during AC for more than 33% from baseline does raise the odds for neurological disorders development 5 times. Mental health parameter (MH) in long-term follow-up of operation was negatively correlated with the duration of AC (r=-0,46; p=0,003).

Conclusion. Antegrade perfusion of the brain under conditions of moderate hypothermia during reconstruction surgery of proxymal aorta demonstrated better cerebroprotection, which was showed by lower rate of neurological complications in early post-operation period comparing to patients after systemic hypothermic AC.

Russ J Cardiol 2016, 11 (139): 47–53

dx.doi.org/10.15829/1560-4071-2016-11-47-53

Key words: aorta prosthetic replacement, cerebral protection, neurological complications.

E. N. Meshalkin Novosibirsk Scientific-Research Institute of Circulation Pathology, Novosibirsk, Russia.

Zhiduleva E. V., Irtyuga O. B., Murtazalieva P. M., Karonova T. L., Moiseeva O. M.

Abstract. Aortic stenosis (AS) is of the most prevalent acquired heart valve defects; the role calcium is serious in its development. One of the key mediators of calcium metabolism is vitamin D.

Aim. To reveal the relation of the sufficiency of vitamin D with mineral bone density (MBD) and other biomarkers of calcification in AS patients.

Material and methods. Totally, 89 patients studied, with severe AS (aortic valve flow velocity more than 4 m/s) at the age 40-76 year old. The controls were almost healthy participants, blood donors (n=31). All patients underwent 25(OH)D in serum measurement, of osteopontin, C-reactive protein and lipid profile, as bienergetic x-ray absorptiometry of lumbar spine and of proximal femur.

Results. According to the data of absorptiometry, there was no osteoporosis or osteopenia in AS patients. However, the pressure gradient on aortic valve was related with MBD and T-criteria value. Vitamin D deficiency was found in 46% of patients. Content of 25(OH)D in serum of AS patients was lower and did not differ between bi- and tricuspid aortic valve patients. There was higher level of osteopontin in AS patients comparing to the controls. Decrease of 25(OH)D was associated with the decrease of osteopontin and atherogenety coefficient. There was no relation of the gradient on aortic valve and the level of vitamin D sufficiency.

Conclusion. Vitamin D deficiency might impact pathogenesis of AS regardless the anatomic specifics of aortic valve. Its action probably is mediated by the regulation with proinflammatory cytokines, such as osteopontin.

Russ J Cardiol 2016, 11 (139): 54–59

dx.doi.org/10.15829/1560-4071-2016-11-54-59

Key words: aortic stenosis, vitamin D, osteopontin.

Federal Almazov North-West Medical Research Centre of the Ministry of Health, Saint-Petersburg, Russia.

Simakova M. A., Maslyansky A. L., Naymushin A. V., Klyaus N. A., Moiseeva O. M.

Abstract

Aim. Pulmonary arterial hypertension (PAH) in systemic sclerodermia patients (SSD) is associated with poorer outcomes. Aim of current study is to assess the prevalence of PAH associated with SSD, among patients with newly diagnosed pulmonary hypertension (PH), included to the registry of Federal Almazov North-West Medical Research Centre and to conduct the analysis of applicability of the algorithms for earlier diagnostics of PH in SSD patients.

Material and methods. To comparative analysis we included patients with idiopathic PAH (iPAH) and PAH associated with SSD (SSD-PAH). All patients underwent thorough echocardiographic test (EchoCG), 6-minute walking test (6WT), spirometry. To confirm the diagnosis of PAH we applied the right chambers catheterization (RCC).

Results. Totally, 33 SSD patients included complaining on dyspnea, of those 14 had verified PAH and were taking specific treatment. With PHAROS algorithm we were able to separate additional risk subgroup of 6 patients for further study. Among participants, iPAH had 44%, and SSD-PAH — 11%. Most patients in both groups had III-IV FC (WHO) of PH: 55% of iPAH and 75% of PAH-SSD. In SSD patients there were lower values of the right ventricle systolic function by EchoCG [FAC=26±7% (р=0,028); TAPSE=15±3 mm (р=0,027); TAS'V=9±2 cm/s (р=0,023)]; values of lung diffusion capacity (DLco): 46±14% vs 62±16% in iPAH group (р=0,001) and distance of 6WT: 326±105 m vs 383±106 m (р=0,041). This data correlates with foreign registers and witness serious prognosis in PAH-SSD.

Conclusion. Our study demonstrated significance of novel algorithms development for earlier diagnostics of PAH and start of specific treatment according to severity of prognosis of SSD-PAH patients. Current guidelines for PAH diagnostics use DETECT algorithm as recommended for application in patients with SSD lasting for more than 3 years and DLco below 60%. We demonstrated the worth of inclusion in investigation algorithm for SSD the additional echocardiographic criteria for PH to increase specificity.

Russ J Cardiol 2016, 11 (139): 60–65

dx.doi.org/10.15829/1560-4071-2016-11-60-65

Key words: pulmonary hypertension, systemic sclerodermia, PAH.

Federal Almazov North-West Medical Research Centre of the Ministry of Health, Saint Petersburg, Russia.

Polonskaya Ya. V.¹, Kashtanova E. V.^1,3, Murashov I. S.², Volkov А. М.², Chernyavskiy А. М.², Ragino Yu. I.¹

Abstract

Aim. To assess some of biochemical markers of bone tissue metabolism, in vessel wall in males with coronary atherosclerosis; to evaluate the influence of these markers on vulnerability of atherosclerotic lesion and to figure out the specifics of their spread among unstable plaques of various kinds.

Material and methods. Totally, 65 males included at the age 46-79 y. o., admitted to the Clinics of E. N. Meshalkin Novosibirsk Scientific-Research Institute of Circulation Pathology for coronary bypass surgery. During operation, an endarterectomy was performed of coronary arteries. Each specimen of endarterectomy was symmetrically divided to fragments for histological and biochemical studies. By the results of histological analysis, in 193 specimens we found: 19 fragments of intact intima, 102 stable, 72 unstable plaques. Then the types of unstable plaques were established: 1) lipids'; 2) inflammatory-erosive; 3) distrophic-necrotic. In homogenates of the fragments we assessed: osteoprotegerin, calcitonin, osteocalcin, cholesterol. Statistics was done with SPSS (13.0).

Results. By the results of the performed histology, in unstable plaques there was higher level of calcification comparing to stable plaques. Biochemical studies showed increased levels of calcitonin and osteocalcin. If to assess the diverse types of unstable plaques, the highest level of these parameters was found in distrophic-necrotic plaques; lowest amount of calcitonin and osteocalcin was found in inflammatory-erosive type. Content of cholesterol in stable and unstable atherosclerotic lesions was about 4 times higher (p<0,01) in comparison with intact intima. Relation of cholesterol with calcitonin and osteocalcin was found, as well.

Conclusion. The data shows that increased osteoprotegerin aids the stabilization of lesion, and calcitonin and osteocalcin might be markers of plaque instability, caused by increased calcification.

Russ J Cardiol 2016, 11 (139): 66–69

dx.doi.org/10.15829/1560-4071-2016-11-66-69

Key words: osteoprotegerin, calcitonin, osteocalcin, cholesterol, atherosclerotic plaque.

¹Scientific-Research Institute of Therapy and Prevention Medicine, Novosibirsk; ²E.N. Meshalkin Novosibirsk Scientific-Research Institute of Circulation Pathology, Novosibirsk; ³Novosibirsk State Technical University, Novosibirsk, Russia.

Zheleznev S.I., Demidov D.P., Afanasiev A.V., Nazarov V.M., Demin I.I., Bogachev-Prokofiev A.V., Astapov D.A., Karaskov A.M.

Abstract. Pulmonary hypertension is an increase of pulmonary artery mean pressure for more than 25 mmHg at rest, measured by direct tensometry, and it includes a broad spectrum of diseases. Mechanisms of pulmonary hypertension are a connection of diverse vicious circles determining chronic progression of the disease, progradient worsening of patient's condition and serious decline in life quality, and premature death. Severe pulmonary hypertension significantly raises risk of perioperation mortality and closely correlates with long-term survival. We propose an original technics of pulmonary artery denervation during one-stage cardiosurgical interventions, that confirmed its safety. Radiofrequency ablation of pulmonary artery in patients with dysplasia of connective tissue and high combined (precapillary and postcapillary) pulmonary hypertension, undergoing surgery for severe sympthomatic mitral insufficiency, is an effective procedure. An assessment is needed for short- and long-term results. Further studies will make it to know, whether the achieved hemodynamical benefits do turn to better clinical outcomes.

Russ J Cardiol 2016, 11 (139): 70–72

dx.doi.org/10.15829/1560-4071-2016-11-70-72

Key words: pulmonary hypertension, mitral insufficiency, connective tissue dysplasia.

E. N. Meshalkin Novosibirsk Scientific-Research Institute of Circulation Pathology, Novosibirsk, Russia.

Klyshnikov R. Yu., Ovcharenko E. A., Kudryavtseva Yu. A., Barbarash L. S.

Abstract. Recently, the number of cardiac surgery in the world and particularly in Russia is raising, especially with utilization of biological prostheses; it is related to average population ageing too. Main disadvantage of such devices is restricted time period of work due to degrading of biomaterials, which demands additional surgery in longterm period (5-10 years). The technology of alternative reimplantation of bioprosthesis by method "prosthesis-into-prosthesis" using transcatheter low invasive devices is the most promising solution for the mortality risk reduction from operations on replacement of failing prosthesis. Current review includes an analysis of clinical results of application of that methodology for correction of acquired defects of aortic, mitral, tricuspid valves, and pulmonary artery valve. The results that are described, witness increasing interest of the world society in methodic as "prosthesis-into-prosthesis", presenting via attempts for systematization and structuring of the interventions results. However complications that occur, risk and procedure restrictions significantly slow wider spread of the method application.

Russ J Cardiol 2016, 11 (139): 73–80

dx.doi.org/10.15829/1560-4071-2016-11-73-80

Key words: cardiac valve, transcatheter, low-invasive, prosthesis failure.

Research Institute for Complex Issues of Cardiovascular Diseases, Kemerovo, Russia.

Bogachev-Prokofiev A. V., Afanasiev A. V., Zhuravleva I. Yu., Demidov D. P., Zheleznev S. I., Malakhova O. Yu., Syrtseva Ya. V., Karaskov A. M.

Abstract. Recently, the main cause of mortality and disability in the world are cardiovascular diseases. By the data from autopsies, valve defects have 4-7% prevalence, and most common are mitral valve defects. Valve prolapse is caused by in-born defect of connective tissue development. Reconstruction surgery continue becoming more common in mitral regurgitation (MR) correction and is more preferable than the valve replacement. Regardless the long history of successful management of MR, official data from most world cardiosurgery centers shows than reconstruction operations are done just in 10-20% cases. Issues on the improvement of the management of patients with connective tissue dysplasia are still open. There is enough literature on laboratory and experimental studies showing benefits and disadvantages of one or other methods, but there are no large clinical trials with sufficient evidence, to confirm or stronly deny differences in clinical outcomes depending on the selection of one or other method.

Russ J Cardiol 2016, 11 (139): 81–86

dx.doi.org/10.15829/1560-4071-2016-11-81-86

Key words: mitral valve, connective tissue dysplasia, acquired valve defects.

E. N. Meshalkin Novosibirsk Scientific-Research Institute of Circulation Pathology, Novosibirsk, Russia.

Fursov А.А.¹, Gordeev V.V.^1,3, Demko I.V.^2,3, Gordeeva N.V.^2,3, Kraposhina А.Yu.², Solovyova I.А.^2,3, Mosina V.А.^2,3, Sobko Е.А.^2,3

Abstract. Clinical presentation of myxomas is diverse, so it is not easy to diagnose this pathology even with available modern instrumental methods. With its "natural" course this pathology is associated with poor outcome, most patients die in several years from the first clinical signs onset.

Russ J Cardiol 2016, 11 (139): 87–89

dx.doi.org/10.15829/1560-4071-2016-11-87-89

Key words: myxoma, heart, clinical case.

¹Federal Center of Cardiovascular Surgery, Krasnoyarsk; ²V. F. Voino-Yasenetsky Krasnoyarsk State Medical University, Krasnoyarsk; ³Regional Clinical Hospital, Krasnoyarsk, Russia.

Vaykhanskaya T. G.¹, Sivitskaya L. N.², Danilenko N. G.², Kurushko Т. V.¹, Nizhnikova О. G.¹, Davydenko О. G.²

Abstract. Recent decades significantly increased the spectrum of monogenic diseases associated with mutations in the gene of lamin A/C (LMNA), that codes the proteins group performing important functions in the nucleus. This pathology presents with diverse systemic tissue involvement. Mutations of the gene LMNA are the cause of more than ten different inherited disorders — laminopathies. In clinical practice, there are cardial phenotypes common, i. e. dilation cardiomyopathy (DCMP), skeletal-muscular dystrophies (Emery-Dreifuss dystrophy, inherited and limbleveled) and more rare forms — lipodystrophies, progeroid phenotypes (acromandibular dysplasia, Hutchinson-Gilford progeria, atypical Werner syndrome), Malouf syndrome. Malouf syndrome, known nowadays as cardiogenital syndrome, is rare inborn pathology with DCMP phenotype and ovarial dysgenesis (females) or primary testicular failure (males), with cognitive delay and variety of skeletal abnormalities (usually facial dysmorphism and marfanoid signs). The arcticle presents a clinical case of female patient with primary amenorrhea, hypogonadism, DCMP, cognitive deficiency, hypodeveloped secondary gender signs, body mass deficiency, and facial dysmorphism. Radiation, viral parotitis, autoimmunity and Turner syndrome were ruled out. Signs if bone dysplasia typical for mandibular-acral dysplasia are absent. Relatives of the 1st line are normal. With sequencing method, we searched for mutations in the gene LMNA, but there were no mutations. Results make it to suggest that pathogenetic mechanisms of Malouf syndrome are not only of "lamin" nature but of other genetic causes too. The article also points on the key issues of diagnostics and treatment, presents differential criteria and clinical signs of an orphan disease.

Russ J Cardiol 2016, 11 (139): 90–94

dx.doi.org/10.15829/1560-4071-2016-11-90-94

Key words: lamin gene mutations А/С, dilation cardiomyopathy, hypergonadotropic hypogonadism.

¹Republic Scientific-Practical Center "Cardiology", Laboratory for medical information technologies, Minsk; ²State Scientific Institution of genetics and cytology of the National Science Academy of Belarus, Laboratory of nonchromosome inheritance, Minsk, Belarus.

Gomboeva S. B.^1,2, Ryabov V. V.^1,2,3, Shelkovnikova Т. А.¹, Usov V. Yu.¹, Markov V. А.^1,3, Karpov R. S.^1,3

Abstract. Clinical case demonstrates the necessity for multimodal visualization of the heart in differential diagnostics of acute coronary syndrome with ST elevation in patients with non-obstruction coronary heart disease. This made possible to be sure on the diagnosis of pseudocoronary variant of myocarditis, to prescribe etiotropic and pathogenetic treatment. On-time diagnostics of alternative causes of chest pain may help to avoid unnecessary treatments related to acute myocardial infarction.

Russ J Cardiol 2016, 11 (139): 95–96

dx.doi.org/10.15829/1560-4071-2016-11-95-96

Key words: acute coronary syndrome, non-obstructive atherosclerosis, myocarditis, cardiac MRI.

¹RI Cardiology, Tomsk; ²National Research Tomsk State University, Tomsk; ³Siberian State Medical University (SSMU), Tomsk, Russia.

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