[Clinical case of the cardiovascular system involvement in a patient with Charcot-Marie-Tooth disease].

Hereditary motor and sensory type 1A neuropathy (known as Charcot-Marie-Tooth disease) is a disease of peripheral nerves characterized by symptoms of progressive polyneuropathy with preferential damage of distal extremity muscles. Damage to the cardiovascular system is extremely rare and heterogenous in this pathology. This disease is not included in the list of indications for interventional antiarrhythmic aid. We could not find in available literature a clinical description of the development of sinus node dysfunction associated with this pathology. The present clinical report presents a case of detection and successful treatment of a damage to the cardiovascular system that manifested itself as sinus node dysfunction/sick sinus syndrome in the tachy-brady variant. A combination treatment approach using radiofrequency catheter ablation, implantation of a permanent pacemaker, and antiarrhythmic therapy associated with drug and non-drug treatment of motor sensory neuropathy resulted in recovery and long-term maintenance of sinus rhythm as well as in beneficial changes in the patient's neurological status.

[Atrial Fibrillation and Mortality: Prognostic Factors and Direction of Prevention].

This review focuses on major causes and risk factors for death of patients with atrial fibrillation (AF). The authors analyzed current therapeutic strategies for managing patients with AF with respect of their effects on prediction and mortality. Special attention is paid to the strategy of rhythm control and the clinical significance of catheter ablation in the treatment of patients with AF and heart failure.

[Combined Approach for Management of the Chronic Heart Failure with Preserved Left Ventricular Ejection Fraction and Permanent Atrial Fibrillation: a Case Report].

The article described a clinical case of a patient with chronic heart failure (CHF) with preserved ejection fraction (CHF-PEF) and permanent normosystolic atrial fibrillation (AF). A 73 year-old man (body mass index, 26.4 kg /m2) with permanent normosystolic AF (duration, 10 years) was hospitalized for augmenting of CHF symptoms. The patient had NYHA II-III functional class CHF and a history of long-standing arterial hypertension. The patient received chronic therapy according to the effective guidelines (angiotensin receptor blockers, diuretics, beta-blockers, and new oral anticoagulants). Transthoracic echocardiography showed a normal ejection fraction (EF) (57 %), a moderate enlargement of the left atrium (48 mm), and moderate left ventricular (LV) hypertrophy. Radiofrequency catheter ablation (RFCA) of left atrial AF was performed. For preparation to the RFCA, the patient was administered propanorm two weeks prior to the procedure. Following external electrical cardioversion (ECV) after RFCA, sinus rhythm did not recover. The patient was prescribed amiodarone, and repeat ECV was performed in a month, which resulted in successful recovery of sinus rhythm. However, due to an increase in serum thyrotropic hormone, amiodaron was replaced with the sotalol therapy (240 mg/day). This resulted in development of symptomatic sinus bradycardia and AF relapse at 3 days after ECV. A dual-chamber cardioverter defibrillator was implanted to the patient; in another three months, repeat AF RFCA was performed with successful recovery of sinus rhythm. During the cardioverter testing for one year, the patient had one more AF episode, which was stopped by external ECV. Also, a 6-hour AF episode occurred at three months after the repeat RFCA. Symptoms of CHF disappeared by the 12th month. The combination therapy administered to the patient with normosystolic permanent AF and preserved EF, which included a pathogenetic therapy for CHF, antiarrhythmic drugs, implantation of a dual-chamber ECV, two sessions of AF RFCA, and repeat external ECVs, provided considerable improvement of CHF symptoms and stable sinus rhythm during a one-year follow-up. The return to sinus rhythm after 10 years of permanent AF necessitated changing the arrhythmia diagnosis to long-standing, persistent AF.

[Recommendations of the Eurasian Arrhythmology Association (EURA) for the diagnosis and treatment of patients with arrhythmias and conduction disorders during the COVID-19 pandemic].

The beginning of 2020 was characterized by the development of a new coronavirus pandemic (COVID-19). Information about the epidemiology, etiology, pathogenesis, clinical and laboratory diagnostics, as well as prevention and therapy for this disease is constantly being expanded and reviewed. The COVID-19 pandemic creates the need for the emergence of new conditions of specialized care for patients with heart rhythm and conduction disorders [1]. These recommendations are intended for general practitioners, internists, cardiologists, electrophysiologists/arrhythmologists, cardiovascular surgeons, functional diagnostics doctors, anesthesiologists-resuscitators, laboratory diagnostics specialists, health care organizers in the system of organizations and healthcare institutions that provide specialized care to patients with heart rhythm and conduction disorders.

[Use of Statins, Anticoagulants, Antiaggregants and Antiarrhythmic Drugs in Patients With COVID-19. The Agreed Experts' Position of Russian Society of Cardiology, Eurasian Association of Therapists, National Society on Atherothrombosis, Societies of Experts in Urgent Cardiology, Eurasian Arrhythmology Association].

This article discusses relevant aspects in the treatment of patients with COVID-19. Up-to-date information about principles for administration of statins, antithrombotics, and antiarrhythmics is presented. The authors addressed in detail specific features of reversing heart rhythm disorders in patients with coronavirus infection and the interaction of antiarrhythmic and antiviral drugs. Recommendations are provided for outpatient and inpatient antithrombotic therapy for patients with COVID-19. Issues of antithrombotic and antiviral drug interaction are discussed.

[The First in Russia Experience of Successful Implementation of Constant Neurostimulation of the Spinal Cord in the Complex Treatment of a Patient with Permanent Form of Atrial Fibrillation Combined with Spinal Stenosis].

This article describes for the first time in the domestic literature a clinical case of the therapeutic effect of neuromodulation on the permanent form of atrial fibrillation and chronic heart failure in an elderly patient with spinal stenosis which led to the development of pain syndrome and movement disorders. For the treatment of neurological pathology, at the beginning epidural administration of drugs was applied, followed by spinal cord stimulation trial and implantation of permanent neurostimulator. At each stage of treatment conducted by a functional neurosurgeon the patient had a spontaneous restoration of sinus rhythm, and during continuous neurostimulation a stable retention of sinus rhythm and regression of heart failure symptoms have been observed throughout a long observation period. The article also presents the data of a few experimental and clinical studies on the use of neuromodulation in cardiology, describes the method of implantation of spinal electrodes and analyzes possible mechanisms of modulation of the autonomic innervation of the heart, implemented by spinal cord stimulation.

Update of the Diagnostic Criteria of J-Wave Syndrome: New Concepts and Their Relevance to Cardiology Practice (According to Materials of J-Wave Syndromes Expert Consensus Conference Report: Emerging Concepts and Gaps in Knowledge (2016).

This review includes main positions of the revision of diagnostic criteria of "J-wave syndromes in the J-Wave Syndromes Expert Consensus Conference Report: Emerging Concepts and Gaps in Knowledge" (2016). The article, systematized according to the sections of the above-mentioned document, outlines the questions of terminology, new criteria for diagnosis of the Brugada syndrome (BrS) and early repolarization syndrome (ERS). The section devoted to ERS on the issues of new terminology and standardization of measurements, is supplemented with material from the Consensus Paper - The Early Repolarization Pattern (2015). The article also presents the issues of differential diagnosis in BrS, presents modulating factors, defines acquired Brugada-pattern and Brugada phenocopies. The similarities and differences between BrS and ERS are presented in a comparative aspect.

[Clinical Case of Severe Heart Failure Treatment, Produced due to the Development of Arrhythmogenic Cardiomyopathy in a Patient With Permanent Junctional Reciprocating Tachycardia (Coumels Tachycardia) With Additional Left Lateral Accessory Pathway].

Thirty two years old man had history of atrioventricular re-entry tachycardia (AVRT) with concealed left-lateral accessory pathway (AP), 3-5episodes per year, 30-40 minutes duration without hemodynamic compromise. Two years ago patient underwent ablation of concealed left lateral AP. After that tachycardia became malignant (high-frequent 150 beats-min, incessant (lasting up to 18 h/day), resistant to AAD, and led to development of tachycardia-induced cardiomyopathy (EF was 16%, and 2 episodes of pulmonary oedema). During redo EPS we verified AVRT with conduction via decremental retrograde left lateral AP which corresponded to the criteria of permanent junctional reciprocating tachycardia (PJRT). Ablation effectively ceased the arrhythmia. After 18 months of follow up there are no symptoms of heart failure and recurrence of arrhythmia. CONCLUSIONS: We present a case of effective treatment of severe heart failure in a patient with arhythtmogenic cardiomyopathy due to malignant course of incessant tachycardia AVRT with retrograde decremental conduction via left lateral AP. Ablation of AP eliminated of arrhythmia. After 18 months of follow up patient had signs of left ventricle reverse remodeling and had not heart failure symptoms.

[Endocardial Electrophysiological Study in an Asymptomatic Competitive Athlete With Ventricular Preexitation due to Conduction via Fasciculoventricular Accessory Pathway].

We present a clinical case of a rare form of the WPW phenomenon due to anterograde conduction over fasciculoventricular accessory pathway in 20-year-old competitive athlete. The patient had no history of palpitations or syncope. ECG revealed shortening of PQ interval (112 ms) and wide QRS complex due to conduction via accessory pathway. To address the question of participation in competitive activity and the need for ablation the patient underwent endocardial electrophysiological study in the course of which we verified conduction via fasciculoventricular accessory pathway. The result of the study was used in determination of strategy of further management.

[Comparison Clinical Data and Results of Mathematical Modeling in Radiofrequency Ablation of Permanent Atrial Fibrillation: Cardioversion Might Be the Final Stage in the Combined Treatment].

AIM: to compare the theoretical possibility of permanent atrial fibrillation (AF) elimination (on the model of the 6-wave re-entry) result.

[Successful restoration by radiofrequency ablation and maintenance of sinus rhythm in a patient with longstanding (for 21 years) persistent atrial fibrillation].

Clinical observation of a patient with atrial fibrillation persisting throughout 21 years is presented with discussion of results of radiofrequency catheter ablation.

[Approximation of mathematical scanning simulation data and clinical results f radiofrequency ablation of perimitral atrial flutter. Condition of existence of perimitral atrial flutter].

AIM: 1) to create Perimitral Atrial Flutter (PMAFL) model and estimate theoretical probability of elimination of perimital reetntry using left atrial geometry two-dimensional mathematical modeling and ablative formatting; 2) to compare clinical results of PMAFL ablation by means of mitral vs inferioseptal isthmus ablation and mathematical modeling data. MATERIAL AND METHODS: Clinical phase. Study was conducted on 24 pts (6 women, 57.1 ± 9.3 years) with PMAFL. Initially RF-lesions delivered in LA in the MI (endocardial approach to MI). Distal CS roof ablation (epicardial approach to MI) was a second step. As a third step linear RF-lessions of the inferioseptal isthmus (ISI)- from right pulmonary vein ostium to mitral annulus was performed (endocardial approach to ISI). As a fourth step RF-applications applied inside the proximal CS roof (epicardial approach to ISI). Mathematical phase. As the first step numeric reconstruction of the autowave process in excitable tissues of the left atrium was performed. Fitzhugh-Nagumo equation was used for simulation to enabled us to take into account the electrical inhomogeneity of the atria (pulmonary vein ostia). A special scanning method was used for calculating characteristics of autowave processes in a two-dimensional mathematical model of the atrium. As the second step simulation of linear ablation formatting which linked PV ostia and active medium boundaries (corresponding to mitral and inferioseptal isthmus ablation lines) was performed. RESULTS: Clinical phase. Left MI endocardial RFA terminated PMAFL in 6 cases, increased CL without changes of atrial hierarchy activation in 2 cases, and transformed PMAFL to AFib in 2 cases. Distal CS ablation terminated PMAFL in 2 pts. Endocardial ISI ablation of associated with SR restoration in 2 cases and increasing of PMAFL CL in 5 cases. Proximal CS-roof ablation terminated PMAFL in 12 pts. Follow up was 26.7 ± 12.4 mos. Endocardial and epicardial MI approach terminated PMAFL in 8 pts (36%). RFA of endocardial 39nd epicardial aspects of the ISI restored SR in 12 pts (64%) (p< 0.05). MATHEMATICAL PHASE: There are three definite conditions of PMAFL existing: 1) Initial autowave spreading between superior PV and boundary of medium (corresponding to patent conduction between superior PV ostia and mitral annulus); 2) Non-active medium existing between four PV ostia (corresponding to PV isolation after index ablation); 3) Refractory characteristics of medium (corresponding to posterior wall of LA) and medium between PV ostia and boundaries (corresponding to isthmus zones) have to differ each other. The linear ablation patterns (from PV ostia to boundary of medium) suppress PAMFL in two-dimensional mathematical modeling of the left atrium. CONCLUSION: There are definite conditions of PAMFL simulation by means autowave processing in a 2-D active medium using scanning algorithm. Those conditions may consistent with certain EP characteristics of LA after index ablation clinical results of PAMFL ablation.

[Long-term results of radiofrequency catheter ablation of long-lasting persistent atrial fibrillation: five years of follow-up].

AIM: To evaluate long-term results of radiofrequency catheter ablation (RFA) vs. rate-control strategy in patients with long-lasting persistent atrial fibrillation (AF) METHODS: We assessed 5-years results in 66 patients (53.3+/-12.3 years old, 8 women) with long-lasting persistent AF who underwent RFA (ablation group), as well as in age-gender-AF duration-matched patients who were treated with rate-control strategy (rate-control group).The ablation strategy consisted of wide-area circumferential lines around pulmonary veins, roof lines and extensive RFA of the left atrial substrate modification using a three-dimensional mapping system. Incidence of sinus rhythm (SR) maintenance, death, stroke, myocardial infarction (MI), worsening of heart failure (NYHA) were evaluated after 5 years of follow-up. RESULTS: After 5 years of follow up SR was present in 38 (56%) of 42 who were under follow up patients of ablation group and all patients had AF in rate-control group (95% CI 0.02247-0.3598; p=0.0001). Seventeen (27%) patients of ablation group continued to take atniarrhythmic drugs. 29 (44%) patients of ablation group vs. 48 (73%) patients of rate-control group received warfarin (95% CI 0.442-1.1; p=0.046) at five years of follow up. After five years of follow up the incidence of MI, stroke, worsening of heart failure functional class, and death in the ablation/rate-control groups were 0%/7.5% (95% CI 0.05247-0.30898; p=0,006), 0%/9% (95% CI 0.08903-0.32561; p=0.001), 6%/25% (95% CI 0.147-0.894; p=0.006), and 0.02%/0%, respectively. CONCLUSIONS: In patients with long-lasting persistent AF ablation strategy results in stable SR in the majority of patients, and decreased incidence of cardiovascular events compared with rate-control strategy during up to 5 years of follow-up.

[Comparison of effectiveness of radiofrequency catheter ablation of left arterial isthmuses for treatment of postablational perimitral atrial flutter].

AIM: To compare results of radiofrequency (RF) ablation (A) of mitral and inferior septal isthmuses in the left atrium for the treatment of perimitral atrial flutter (AF) in patients with atrial fibrillation previously subjected to RF isolation of pulmonary veins and modification of the left atrial anatomical arrhythmia substrate. MATERIAL AND METHODS: We treated 20 patients (3 women, mean age 57.1+/-9.3 years) with recurrent postablational perimitral AF verified by 3-dimensional reconstruction of left and right atrial activation by nonfluoroscopic mapping and pacing techniques of entrainment into tachycardia cycle. At first stage RFA was carried out in the region of mitral isthmus (its endocardial parts). At second stage RFA was performed in the region of coronary sinus (CS) roof (epicardial portions of the mitral isthmus). At the third stage RFA influences were applied in the region of inferior septal isthmus (endocardial portions of the inferioseptal isthmus). At the fourth stage RFA was applied in the region of roof of proximal CS (epicardial portions of inferioseptal isthmus). RESULTS: Application of RF influences to endocardial parts of the mitral isthmus resulted in sinus rhythm (SR) restoration in 6 cases. Change of length of the tachycardia cycle was noted in 5cases. SR restoration was observed after RFA in the region of the roof of distal CS in 3 cases and change of length of the tachycardia cycle was noted in 2 more cases. RFA of epicardial parts of the inferioseptal isthmus resulted in SR restoration in 3 cases and in increase of atypical AF cycle length in 6 cases. In 12 cases SR was restored during RFA application in the region of the roof of proximal CS parts. RF influences in the region of endocardial (stage 1) and epicardial (stage 2) parts of the mitral isthmus resulted in SR restoration in 9 cases while continuation of RFA in the region of endocardial (stage 3) and epicardial (stage 4) parts of the inferioseptal isthmus led to SR restoration in the remaining 15 cases (p<0.05). CONCLUSION: RFA of endocardial and epicardial parts of the inferioseptal isthmus significantly more often led to termination of perimitral atypical AF in patients previously operated because of atrial fibrillation.

[Comparison of the effectiveness of pulmonary veins isolation vs linear radiofrequency ablation in paroxysmal atrial fibrillation patients using either mathematical scanning or clinical approach].

AIM: to compare clinical results of linear ablation vs. PVI approach in patients with paroxysmal AF and to estimate theoretical probability of 4-waves re-entry to eliminate as a results of the both ablative techniques formatting by means left atrial geometry two-dimensional mathematical modeling. MATERIAL AND METHODS: Clinical phase. Study was conducted on 20 pts (6 women, 51,4±13,6 years of age) with paroxysmal AF underwent index RFA. All pts were divided into two age-sex-arrhythmia history duration-antiarrhythmic drugs (AAD)-matched groups. The first group consisted of 10 pts (3 women, mean age - 51,1±11,9, history of arrhythmia - 3,2±1,2 years) in whom ablation strategy consisted of PVI using LASSO technique. The second group concluded of 10 pts (3 women, mean age - 51,1±12,9, history of arrhythmia- 3,1±1,1 years) in whom ablation strategy consisted of wide-area circumferential lines application around pulmonary veins, combined to roof line and mitral isthmus RFA using three-dimensional mapping system. Mathematical phase. As the first step numeric reconstruction of the autowave process in excitable tissues of the left atrium and the simulation of AF was performed. Fitzhugh-Nagumo equation was used for simulation to enabled us to take into account the electrical inhomogeneity of the atria (pulmonary vein ostia). A special scanning method was used for calculating characteristics of autowave processes in a two-dimensional mathematical model of the atrium. As the second step simulation of circular (corresponding to LASSO approach) and linear ablation (corresponding to 3D approach) was performed. RESULTS: Clinical phase. There were no complications associated with RFA. 7 pts of the first group vs 4 pts of the second subgroup had early recurrences of arrhythmia. AAD free sinus rhythm incidence in the first/second groups was 80%/20% at 12 months respectively (p=0,003). Mathematical phase. While circular LASSO-like ablation pattern was used, there was no elimination of 4-waves re-entry turning around the pulmonary veins ostia and vortex waves caused by them in a distributed two-dimensional atrial medium after time period equal to the re-entry period. Thus, the circular ablation formatting does not affect peripulmonary veins re-entry and, therefore, does not suppress AF. In contrast, linear ablation patterns suppress arrhythmias caused by 4-waves re-entry in two-dimensional mathematical modeling of the left atrium. CONCLUSION: Mathematical approach using linear ablation to simulate suppressed 4-waves re-entry more effectively comparing to PVI only. Clinical results are consistent with ablation formatting data obtained by means of 4-waves re-entry simulation in a two-dimensional mathematical modeling of the left atrium.

[The rare case of Bland-White-Garland syndrome in adult patient].

We present analysis of a case history of a patient with rare congenital heart disorder - Bland-White-Garland syndrome. The 25 years old women was first diagnosed with this disorder during an examination in the cardiological department of the Moscow clinical hospital No83. The paper contains discussion of difficulties of diagnosis and peculiarities of management of adults with this pathology as well as of a problem of the choice of further therapeutic approaches.

[Evaluation of the radiofrequency ablation effectiveness in patients with the Wolff-Parkinson-White syndrome].

A one-year prospective study involved 22 patients with the Wolff-Parkinson-White syndrome (WPW) and 20 healthy people. Means age of patients was 34.3 +/- 16.3 years. All 22 patients were successfully treated with radiofrequency ablation (RFA) of additional pathways. RFA effectiveness was evaluated with the help of clinical questionnaire, data of ECG, EchoCG, heart rate variability (HRV), frequency response and nonlinear dynamics. Cardiac rhythm disturbances were verified using Holter monitoring applied to all patients. Positive clinical effect was achieved in all the WPW patients, as RFA arrested cardiac arrhythmias completely. Holter monitoring did not register cardiac disturbances which points to high RFA effectiveness in WPW patients. HRV, frequency response and nonlinear dynamics reassumed their normal patterns.

[Antitachycardia pacing in patients with implanted cardioverter defibrillators].

Efficacy and safety of the use of antitachycardia pacing (ATP) function in 54 patients (age 18-54, mean age 62.2 +/- 11.5 years, 6 women) with cardioverter defibrillators implanted for prevention of sudden death. Duration of follow-up was 6-64 months (mean 27.3 +/- 8.6 months). Ischemic heart disease (IHD) was etiopathogenetic basis for development of ventricular rhythm disturbances in 38 (70.4%) patients. Arrhythmogenic right ventricular dysplasia (ARVD) was diagnosed in 8 (14.8%), hypertrophic cardiomyopathy--in 3, dilated cardiomyopathy--in 2, the Brugada syndrome--in 2 patients, and long QT syndrome--in 1 patient. Three regimens of ATP were studied--burst, ramp, ramp(+)--which were activated in 22 patients (17 with IHD and 5 with ARVD). We registered 776 episodes of ATP occurring because of ventricular tachycardia (VT) in 20 of 22 patients with activated ATP. Overall efficacy of burst ATP was 49.1%; VT was not terminated or was accelerated in 43.2 and 7.6% of cases, respectively. Ramp ATP was effective in 44.3, ineffective--in 33.9, and caused VT acceleration in 21.8% of cases, what called forth fulfillment as a next step of shock therapy (cardioversion or defibrillation). ATP led to successful termination of VT in 67.5% of patients with IHD and to acceleration of arrhythmia--in 6% of episodes. Most effective type was ramp ATP which effectively terminated VT in 76.3% of cases, while efficacy of burst ATP was 61.4% (p < 0.01). In patients with ARVD total efficacy of ATP was 25%, acceleration of VT during ATP was observed in 24% of cases; optimal was burst ATP, which efficacy was 33.9%--significantly higher than efficacy of ramp and ramp+ ATP (19.4 and 24.4%, respectively, p < 0.005).

[Echocardiographic parameters in patients with Wolf-Parkinson-White syndrome before and during one year after radiofrequency catheter ablation of accessory atrioventricular junction].

We present in this paper results of assessment of morphofunctional state of myocardium in patients with the Wolf-Parkinson-White syndrome before and during one year after radiofrequency catheter ablation (RFA) of accessory atrioventricular junction (AAVJ) and comparison of them with analogous parameters of the group of healthy volunteers as well as in dependence on electrophysiological properties of AAVJ and its localization. One hundred sixty patients took part in the conducted study: main group comprised 160 patients (80.7%) with WPW syndrome (114 men [81.4%], 26 women [18.6%], mean age 39.5+/-15.3 years), comparison group comprised 20 practically healthy persons (15 men [75.0%], 5 women [25%], mean age 41.9+/-5.3 years). All main group patients were subjected to endocardial electrophysiological investigation and RFA of AAVJ. Transthoracic echocardiography (EchoCG) was carried out in patients of main group before and in 2, 6, and 12 months after operation of RFA of AAVJ, and once in control group. Analysis of parameters of central hemodynamics according to data of transthoracic EchoCG in patients with WPW syndrome before RFA of AAVJ demonstrated that before conduct of operative intervention no significant differences were revealed in the studied parameters compared with analogous characteristics of the clinical comparison group. During whole period of dynamic observation (2, 6, and 12 months after fulfilled RFA of AAVJ) in patients with WPW syndrome the studied parameters of central hemodynamics did not undergo substantial changes compared with initial characteristics. We failed to establish significant differences of EchoCG parameters in patients with WPW syndrome in dependence on electrophysiological properties of AAVJ (concealed, manifest) and on AAVJ localization (right, left, septal). According to EchoCG data in patients with WPW syndrome so called "minor" anomalies of development of connective tissue of the heart were diagnosed in 69 (49.3%) patients while in control group - in 2 (10%) patients.