[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 36.5 per 100 000 in population. About 20-30% of cases are familial. Disease is genetically heterogenous, there more than 100 genes when mutated can give rise a DCM. In 2004, the role of SCN5A gene mutations was shown in origin of DCM with cardiac conduction defects and arrhythmias. In this work we present a clinical case of dilated cardiomyopathy with cardiac arrhythmias and p.E446K mutation in SCN5A gene. We have observed DCM with mild left ventricular hypertrophy, progressive AV block, atrial fibrillation and congenital heart defect (atrium septal defect) in two generations. The congenital heart defect did not co-segregate with SCN5A mutation and DCM.

[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. Blood flows in left ventricle, myocardium shift velocity and pressure gradients during the heart cycle were measured. The study showed that systole leads to the directional change of blood flows within the heart cycle, accompanied by turbulence phenomenon with extremely high flow velocities. Detection of vortex flows and velocity vector characterizes changes of flow direction. All patients of the main group were operated on with the use of modern methods of heart remodeling. The principally novel method of surgical treatment, allowing to preserve the patient's heart, was worked out.

[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.

[Myocardial biomechanics in patients with coronary circulatory insufficiency].

The paper analyzes a new approach to evaluating myocardial systolic and diastolic function, which is based on recording of the rate of myocardial displacement in different phases of a cardiac cycle. Changes in the rate of vector movements in a period of isovolumic contraction and relaxation are shown on ample clinical materials in both healthy individuals and patients with coronary heart disease. There is evidence for a significant difference in the performance of a stress test by healthy individuals and patients with a low coronary circulatory reserve.

[Comparative evaluation of clinical use of cardioplegic solutions custodiol and konsol in the correction of cardiac valvular diseases].

The efficiency of myocardial protection with cold crystalloid cardioplegia (CC) during operations on the heart under total ischemia and extracorporeal circulation was studied. Two hundred and fifty patients who underwent prosthetic or plastic repair of the aortic and mitral valves or plastic repair of the tricuspid valve were examined. Group 1 comprised 218 patients in whom the myocardium was protected with Konsol solution ("Biofarm "research-and-production enterprise, Russia); Group 2 included 32 patients in whom Custodiol solution ("Dr Franz Kohler Chemia GMBH", Germany). The volume of the Konsol solution required for CC was 400-2000 ml, with the duration of myocardial ischemia (MI) being 75 +/- 33 min; that of the Custodiol was 2000-4000 ml, with the duration of MI being 80 +/- 22 min. The used algorithm of administration of the solutions provided a persistent cardioplegic effect in both groups. After aortic declamping, there was a spontaneous recovery of cardiac performance in 84 and 40% in Groups 1 and 2, respectively. In the reperfusion period (RP), no significant differences were found in the basic hemodynamic parameters (heart rate, blood pressure, and central venous pressure) in both groups. After defect correction, cardiac output equally increased in both groups. The groups did not differ in the rate of inotropic support either. In RP, transient cardiac rhythm and conduction disturbances occurred in 19 and 28% in Groups 1 and 2, respectively.