[Use of dual-energy computed tomographic angiopulmonography in patients with chronic thromboembolic pulmonary hypertension before and after pulmonary artery thromboendarterectomy].

OBJECTIVE: To estimate the contribution of dual-energy computed tomographic (CT angiopulmonography t o a diagnostic algorithm in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and its role in the evaluation of postoperative changes. MATERIAL AND METHODS: CT angiopulmonography was performed on the 64-slice Discovery HD 750 system (GE Healthcare) using the dual-energy scanning mode (140 and 80 kV). The examination results in patients with CTEPH were analyzed. RESULTS: All the patients were found to have typical CT signs of CTEPH: abnormal contrast enhancement of the pulmonary arteries, their dilatation and tortuosity; signs of right cardiac overload: right ventricular dilatation and/or hypertrophy, bronchial artery dilatation, decreased peripheral vascular pattern, mosaic pulmonary perfusion, and wedge-shaped perfusion defects on the iodine maps. Mosaic pulmonary perfusion areas were also found in all the patients. Perfusion defects were more clearly visualized when dual-energy CT by constructing iodine perfusion maps was used. Analyzing the perfusion maps in 6 patients operated on revealed a 20-50% reduction in perfusion deficit. CONCLUSION: Information on the vascular bed and pulmonary perfusion may be obtained in patients with CTEPH within one investigation, which is important to plan surgical treatment. Construction of iodine pulmonary perfusion maps allows evaluation of perfusion recovery after artery thromboendarterectomy.

[The mitral valve dysplasia in adults: the choice of surgical tactics].

Early and long-term results of hereditary mitral valve dysplasia surgical treatment were obtained in 203 patients. All patients were divided in 2 groups: 73 (36%) patients after valve-preserving operations and 130 patients after universal chorda-preserving valve prosthetics. The choice of treatment modality depended on the type of anatomical changes and overall surgical volume. Hospital lethality rate was 2.46%. Surgery led to satisfactory functional results, thus, 83.3% of the operated patients have I-II NYHA functional class. Analysis of the own experience allowed to mark out factors, contraindicating the durable plastic mitral valve.

[The donor's heart 22 years after the orthotopic transplantation].

The orthotopic heart transplantation is an acknowledge method for the treatment of cardiomyopathies of various etiology. Specific vasculopathy of the transplanted heart is considered to be a significant problem of the long-term postoperative period and serves the reason of low 10-years survival rates (not more then 50%). The issue unites the experience of follow-up and intravital electronic microscopy of transplantated heart's biopsies from 20 patients. Previously unknown data can help the clarification of posttransplantational cardiomyopathy.

[Noncoronary dilated cardiomyopathy after reverse remodeling heart surgery].

We present here analysis of surgical treatment of 24 patients (5 women, 19 men, age 20-75, mean age 50.7 +/- 2.5 years) with dilated cardiomyopathy (DCM) operated during the period from 2008 to 2013. Duration of the disease ranged from 4 months to 12 years (mean 49.4 +/- 7.5 months). According to symptoms and results of 6-minute walk test 3 patients (13%) had heart failure NYHA class III and 21 patients (87%)--NYHA class IV. Average end-diastolic left ventricular size was 7.4 +/- 0.18 cm (6.0-9.2 cm), ejection fraction--26.7 +/- 2.1% (13-47%), mean pulmonary artery pressure 54.9 +/- 2.9 mm Hg (35-80 mmHg). All patients underwent organ-conserving surgery aimed at reverse remodeling of the heart. Surgery was accompanied with implantation of implantable cardioverter defibrillator in 3 patients and/or cardiac resynchronization therapy device in 6 patients. Two patients (8.3%) died during hospitalization of hemodynamically significant ventricular arrhythmias; seven patients (29.2%) died in the late postoperative period. The results of the analysis indicate that reverse-remodeling surgery may be effective in patients with DCM of any age group with preserved reserves of the liver, kidney, and lung function in the absence of active myocarditis. Further observations are needed to determine the place of this operation in the protocol of treatment of patients with DCM.

[Noncompaction myocardium as a primary phenomenon or consequence of myocardial dysfunction: clinical masks of the syndrome].

Noncompaction myocardium (NCM) is a genetic heterogeneous primary cardiomyopathy which affects both children and adults and can be either isolated or combined with other congenital heart disorders. It has common pathogenesis of symptoms but is distinguished by pronounced clinical polymorphism. We have observed 25 adult patients (15 men, 10 women aged from 20 to 62 years, mean age 42.9+/-13.3 years) with NCM syndrome. Heart failure have been found in 96% of patients (functional class [FC] I in 7, II - in 6, III in 7, and IV - in 4 patients). Ninety two percent of patients have ventricular extrasystoles, 32% - atrial fibrillation, 28% - FC I-III angina. Mean end diastolic left ventricular dimension is 6.5+/-0.8cm, ejection fraction 29.7+/-13.0%, mean pulmonary artery pressure - 42.6+/-13.5 mm Hg. Intracardiac thrombosis have been found in 24% of patients. In 7 patients morphological study of myocardium has been performed. NCM syndrome was diagnosed at initial investigation just in 1 case. We distinguished the following clinical masks (variants of diagnosis) of NCM: 1) clinically not manifest, is revealed at accidental examination (4%); 2) exists under mask of "idiopathic" rhythm disturbances (8%); 3) has a mask of ischemic heart disease; 4) is revealed in patients with acute or subacute myocarditis (12%); 5) has a mask of dilated cardiomyopathy (52%); 6) NCM in patients with other primary cardiomyopathies (hypertrophic, restrictive, genetic myopathy, arrhythmogenic right ventricular dysplasia). Combination of NCM with congenital heart defects has been found in 20% of patients. In 56% of cases myocarditis was diagnosed (it was viral in no less than 44%). Only in 32% of patients it is possible to consider presence of isolated NCM syndrome. This paper contains discussion of problems of diagnostics (including morphological) and treatment in the presented group of patients, significance of myocarditis for development of decompensation, role of NCM in patients with other primary cardiomyopathies, possibility of compensatory (secondary) character of NCM in severe systolic dysfunction.

[Radial diagnosis MRT and MSCT in assessing the outcomes of surgical correction for complicated congenital heart defects: discordant atrioventricular connections with double outlet right ventricle].

Discordant atrioventricular connection with double outlet right ventricle is a rare complicated congenital heart defect (CHD) requiring accurate diagnosis and appropriate correction. Magnetic resonance imaging (MRI) and multiple spiral computed tomography (MSCT) were used to assess long-term outcomes of the «classical¼ repair of the double outlet right ventricle in a patient presenting with discordant atrioventricular connection. Using a modified segmental approach provided all necessary anatomical evidence concerning the condition of the heart, major vessels, and an extracardiac pulmonary valve-containing conduit. MSCT made it possible to evaluate the degree of calcinosis and stenosis of the conduit and to visualize the coronary arteries. MRI was employed to assess the pressure gradient at the level of stenosis of the conduit, the relationship between the pulmonary and systemic circulation, transvalvular regurgitation, and ventricular contractility. Comprehensive use of present-day tomographic methods of imaging made it possible to obtain complete anatomical and functional information about the condition of the heart, vessels, extracardiac vascular transplant, as well as to reveal complications and determine the indications for a repeat operative intervention.

[Arrhythmogenic right ventricular dysplasia: polymorphism of clinical manifestations].

We observed 15 patients with arrhythmogenic right ventricular dysplasia (ARVD): 9 with definite and 5 with probable ARVD (modified European Criteria, 2010). Eight patients had typical ARVD (frequent right ventricular extrasystoles, nonsustained right ventricular tachycardia without heart failure with or without myocarditis). Five patients had ARVD with progressive heart failure (right- or biventricular with or without myocarditis). Two patients had full scale arrhythmic form (sustained right ventricular tachycardia without or with right ventricular dilation, with or without myocarditis). In 3 cases diagnosis was confirmed morphologically or with DNA-diagnostics. This material allowed us to highlight the following specific points related to diagnostics of ARVD. Detection of fat at MRT is not obligatory for diagnosis, fat can be detected by MSCT; ventricular arrhythmias can move backwards in the picture of the disease; leading clinical manifestation can be unexplained right ventricular insufficiency; ARVD can be combined with other genetic cardiomyopathies as well as with infectious immune myocarditis (up to 50% of patients); elevated titer of anticardiac antibodies is not characteristic for isolated ARVD; myocardial biopsy allows to verify both ARVD and concomitant myocarditis. The paper also contains discussion of the role of myocarditis in various forms of ARVD and possibilities of its diagnosis and treatments.

[Successful treatment of massive thrombosis of the vena cava inferior with nephrotic syndrome and chronic bilateral pulmonary artery thromboembolism in a patient with genetic thrombophilia].

A case is reported of a 23-year-old male patient who developed, after severe blunt injury of the lumbar region, massive thrombosis of the vena cava inferior (VCI), both renal veins, bilateral pulmonary artery thromboembolism (PATE), nephrotic syndrome (NS). In spite of anticoagulant therapy, the condition of the patient progressively aggravated for 1.5 year: thrombosis involved the ileac and femoral arteries on the right, thrombus floated in the right atrium with PATE recurrent episodes, pulmonary hypertension reached 120 mm Hg with formation of decompensated cor pulmnonale, proteinuria and hypoalbuminemia deteriorated, anasarca edema developed Multigenic thrombophilia was diagnosed (1 homozygous and 5 heterozygous mutations). A radical one-stage operation was successful: thromboectomy from the VCI, right ileac and left renal veins, thrombendarterectomy from the pulmonary arteries, suture of the interatrial septum defect, installation of cava-filter After the operation pulmonary pressure lowered to 40-45 mm Hg, right heart volume normalized, immunosuppressive therapy with prednisolone and cyclosporine led to nephropathy remission. The discussion covers mechanisms and factors (including genetic) of thrombosis progression, correlations between intravascular thrombosis, NS and chronic glomerulonephritis (possible NS development due to bilateral thrombosis of the renal veins and nephropathy role in thrombosis progression), approaches to conservative and surgical treatment of such patients. Global experience in conduction of pulmonary thrombendarterectomy and thrombectomy from VCI is reviewed (one-stage operations were not described earlier).

[Dilated cardiomyopathy as clinical syndrome: experience with nosological diagnostics with biopsy and treatment approaches].

AIM: To study possibility of nosological diagnosis in patients with dilated cardiomyopathy (DCMP) with use of myocardial biopsy. MATERIAL AND METHODS: The trial enrolled 62 patients (23 females) with DCMP syndrome (end diastolic left ventricular size > 5.5 cm, ejection fraction < 55%). Mean age of the patients was 46.0 +/- 12.8 years. The examination included diagnosis of viral infections (Herpes virus, parvovirus B19), measurement of anticardial antibodies titer, 99Tc-MIBI single photon emission computed tomography of the myocardium, multislice computed tomography, MRT of the heart, coronarography, morphological study of the myocardium (n=20) with application of polymerase chain reaction (PCR) for H.simplex viruses of types 1, 2 and 6, herpes zoster, Epstein-Barr, cytomegalovirus, parvovirus B-19, adenoviruses. The control group (20 operated patients with valvular heart disease and coronary heart disease) was examined for viral genome in the blood and myocardium. RESULTS: Complex examination of DCMP patients showed the following distribution by nosological entuities: myocarditis (n=41, 66.1%) including virus-positive (n=14), primary DCMP (n=16, 25.9%) including with non-compact myocarditis (NCM) in 3, with debute at delivery of the child--in 3. Arrhythmogenic right ventricular dysplasia combined with viral myocarditis (n=2), genetic myopathy (n=1) and Takayasu disease (n=1) combined with NCM, isolated NCM (n=1) were diagnosed in the rest cases. Morphological investigation of the myocardium was made in 20 patients: diagnosis of myocarditis and primary DCMP were made in 70% (including in 2 patients with CHD) and 20%. Detection of viral genome was 20 and 15% in the study and control group, respectively, in the myocardium--in 57.9 (test for parvovirus B19 was not made in 26%) and 65.0% (complete diagnosis). All the virus-positive patients with DCMP were diagnosed to have signs of active/borderline myocarditis. Diagnostic criteria and poor prognosis factors were defined. CONCLUSION: The nosological diagnosis of DCMP was made in all the examinees basing on the complex of clinical, case history and device evidence. The diagnosis was morphologically verified in 33.9% patients. Treatment approaches are developed.

[Foreign experience in registering and analyzing the poor outcomes of treatment].

Negative consequences of treatment are observed at least in 16% patients at multidisciplinary hospitals and may result from the causes that are independent of medical staff, but more frequently from errors and inadequate treatment standards due to human factors. Systematic improvement of professionalism, which should be started from some educational reforms at medical institutes, is needed.

[An enterosorbent with trimethylaminoethanol chloride. 2. Hypolipidemic action and effect on cholesterol absorption in the intestine (experimental data)]. / Enterosorbent s immobilizovannym khloridom trimetilaminoéthanola. 2. Gipolipidemicheskoe deistvie i vliianie na vsasyvanie kholesterina v kishechnike (éksperimental'nye dannye).

Enterosorbent containing trimethyl aminoethanol chloride administered per os into rabbits accelerated the rate of experimental hypercholesterolemia regression. Moreover, this substance decreased 4-14C-cholesterol absorption in the rat intestine.

[Evaluation of various experimental dyslipoproteinemia models in the study of hypolipidemic agents]. / Otsenka nekotorykh modelei éksperimental'noi dislipoproteidemii pri izuchenii veshchestv gipolipidemicheskogo deistviia.

Models of experimental dyslipoproteinemias have been developed by using non-inbred rats (males and females) and guinea-pigs. Administration of cholesterol (C) with fats and supplementation of 4-hydroxy-6-methyl-2-thiouracil to male rats and that of C in the mixture of fats to guinea-pigs have been demonstrated to lead to the development of atherogenic dyslipoproteinemias. Genfibrozil, cholestyramine and vasosan II, a new pectin-enriched formulation, promoted normalization of the impaired serum lipoprotein spectrum caused by exogenous C administration. Bilateral ovariectomy in female rats induced atherogenic dyslipoproteinemia. The use of the estrogen derivative methyl ether of 6-oxa-D-homo-8-isoestrone normalized lipid and lipoprotein metabolic disturbances.

[Enterosorbent with immobilized trimethylaminoethanol chloride. I. Hypolipidemic properties]. / Enterosorbent s immobilizovannym khloridom trimetilaminoétanola. I. gipolipidemicheskie svoistva.

Hypolipidemic properties of the synthesized enterosorbent, water soluble polymer containing covalently immobilized trimethylamine ethanol chloride, were studied. Content of cholesterol in blood was decreased by 29% and accumulation of cholesterol in liver tissue--by 22% after oral administration of the enterosorbent at a dose of 250 mg/kg into rats with hypercholesterolemia. Cholestyramine exhibited the similar effect only at a dose of 2.5 g/kg. Except for the hypocholesterolemic effect, the enterosorbent altered also the lipoprotein spectrum in rat blood serum.

[The hypolipidemic action of water-soluble enterosorbents of cholesterol and bile acids in an experiment]. / Gipolipidemicheskoe deistvie vodorastvorimykh énterosorbentov kholesterina i zhelchnykh kislot v éksperimente.

Experiments on rats, guinea pigs and rabbits with experimental dyslipoproteinemia have revealed that the new water-soluble high-molecular weight polymers containing digitonin (ED) or a quaternary nitrogen atom (EA) have a more marked hypolipidemic action than cholestyramine and normalize lipoprotein metabolism. The digitonin-containing enterosorbent has been shown to decrease the absorption of 4-14C-cholesterol, while the nitrogen-containing enterosorbent diminishes the absorption of 3H-cholic acid.

[Experimental and clinical study of Vasosan P, a new form of pectin-enriched cholestyramine]. / Eksperimental'noe i klinicheskoe izuchenie vasozana P--novoi formy kholestiramina, obogashchennogo pektinom.

The bile acid sequestering agent Vasosan P is a new form of pectin-enriched cholestyramine (20%), saccharose (5%) and sorbic acid (0.18%). In comparison with cholestyramine Vasosan P has a more marked hypolipidemic effect in experiment. Vasosan P is remarkable for good organoleptic properties. It is effective in treating patients with type IIa hyperlipoproteinemia and practically has no side effects as cholestyramine.