Modified chordal sparing mitral valve replacement as effective technique for both stenotic and insufficient mitral valves.

Background: Chordal apparatus preservation is important for preserving left ventricular (LV) function in the long-term perspective. We present results of originally modified chordal-sparing mitral valve replacement (MVR) successfully used in patients with mitral stenosis and mitral insufficiency.

Methods: The modified surgical method involves preserving only four strut chords with portions of the mitral valve leaflets, which are later fixed to the fibrous ring.

[Dilated phase of hypertrophic cardiomyopathy: high-risk reconstructive surgery as an alternative to heart transplantation].

The authors report the diagnosis and surgical treatment of 5 patients with dilated phase of hypertrophic cardiomyopathy (HCM). Features of these patients are progressive heart failure, double-level blood flow obstruction and the risk of apical aneurysms. Reconstructive remodeling surgery is a reasonable alternative to heart transplantation despite the existing risk.

Low mutation rate in the TTN gene in paediatric patients with dilated cardiomyopathy - a pilot study.

Idiopathic dilated cardiomyopathy (DCM) is a common cardiomyopathy with the prevalence of 1:250, and at least one-third of all the cases are inherited. Mutations in the TTN gene are considered as the most frequent cause of inherited DCM and cover 10-30% of the cases. The studies were mainly focused on the adult or mixed age group of patients with DCM.

[Myocardial Crypt of the Left Ventricle Combined With Barlows syndrome].

Myocardial crypts were initially described in patients with hypertrophic cardiomyopathy. Modern diagnostic data show that this structural abnormality can be found in patients with other diseases, or might represent the variant of normal heart development in healthy individuals. The prognostic significance of this finding is uncertain.

New intronic splicing mutation in the LMNA gene causing progressive cardiac conduction defects and variable myopathy.

Background: Most of mutations in the LMNA gene are unique and have been found in only a few unrelated families. The clinical interpretation of new genetic variants, especially beyond the coding area and canonical splice sites, is proving to be difficult and requires advanced investigation.

Methods: This study included patients with progressive cardiac conduction defects with neuromuscular involvement.

The role of mutations in the SCN5A gene in cardiomyopathies.

The SCN5A gene encodes the alpha-subunit of the Nav1.5 ion channel protein, which is responsible for the sodium inward current (INa). Since 1995 several hundred mutations in this gene have been found to be causative for inherited arrhythmias including Long QT syndrome, Brugada syndrome, cardiac conduction disease, sudden infant death syndrome, etc.

[Pharmacological and mechanical support of the myocardium in perioperative period in cardio-surgical patients with chronic heart failure].

The article deals with a retrospective study devoted to the combined methods of myocardial support in cardiosurgical patients with chronic heart failure (III-IY FC according to NYHA) and low myocardial reserves capacity (LVEF 28.3 +/- 9.4%).

[Dilated cardiomyopathy caused by p.E446K mutation in SCN5A gene].

Dilated cardiomyopathy (DCM) is myocardial disorder characterized by progressive heart chambers enlargement and impairment of myocardial contractility. This disorder is the most common cause of advanced heart failure requiring the heart transplantation. The prevalence of the disease is 36.

[The training concept of laparoscopic surgery in post-graduate education of doctors].

The active use of virtual devices and laparoscopic boxes for the control of the achieved skills level are the general idea of the suggested concept of the laparoscopic surgical training. The in vivo training make sense only after finishing the "virtual" course. The complete realization of these new concept of laparoscopic surgery training is possible only in frames of the endoscopic surgery department of the mighty hospital center.

[The multiple organ failure syndrome after cardiac surgery with artificial blood circulation].

The 10-year study of etiology, pathogenesis, diagnostic, treatment and prevention of the multiple organ failure syndrome (MOFS) after cardiovascular operations with artificial blood circulation was conducted in the SCS. 4383 patients, aged 16-75 years, were observed. Of them, MOFS was diagnosed in 206 (4.

[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.

[Clinic and genetic polymorphism of Brugada syndrome in Russian patients, caused by mutation in SCN5A gene].

Brugada syndrome (BrS) is an inherited cardiac arrhythmic disorder, characterized by ST-segment elevation in right precordial leads V1-V2>2 mm, pseudo right bundle branch block (RBBB), T-wave inversion and an increased risk of cardiac sudden death (SCD) due to molymorphic VT. It is estimated to be responsible for 12% of SCD cases and about 20% of deaths in patients with structurally normal hearts in autopsy. Mutations in the SCN5A gene account 15-30% of all cases.

[Noninvasive registration of turbulent flows in the left ventricle].

Observation results of 133 patients with chronic heart insufficiency and 29 healthy people, all aged 41 ± 6 years were analyzed. 51 patients had ischemic heart disease, 53 had various valvular diseases and 30 patients had dilated cardiomyopathy. Echocardiography was performed together with ECG.

[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.

[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.

[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.

The case of 17-year-old male with LEOPARD syndrome.

LEOPARD syndrome is a phenotypic expression of mutations in several genes: PTPN11, RAF1, and BRAF. All these genes are responsible for Ras/MARK signaling pathway, which are important for cell cycle regulation, differentiation, growth, and aging. Mutations result in anomalies of skin, skeletal, and cardiovascular systems.

[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).

[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).

[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%).

[Primary lymphoma of the heart: difficulties in diagnosis and treatment].

Primary lymphoma of the heart (non-Hodgkin's lymphoma primarily of B-cells affecting first the heart and pericardium) is a rare disease which accounts for 1.3-2.0% of all primary tumors of the heart.

[Decalcification of anterior cusp of mitral valve at marked calcification of aortic valve].

Short-term and long-term results of surgical treatment were analyzed in 108 patients with aortic valve disease, complicated significant calcinosis spreading on anterior cusp of mitral valve. Mean age of the patients - 53,5±10,8 years (from 25 to 88 years). All patients underwent aortic valve replacement and decalcification of anterior cusp of mitral valve.