The article outlines historical milestones of the Kuzbass Cardiology Center. It presents the areas of expertise, stages of development and the main achievements. 

Aim To assess the diagnostic accuracy of long QT syndrome in children and to estimate the results of the follow-up.

Methods High-risk groups of children with bradycardia less than the second percentile and/or a family history of sudden death syndrome, and children with syncope diagnosed with the ECG testing were included in the study. All patients underwent routine medical examination, molecular genetic testing and were followed-up for 3,5–10 years.

Results The majority of children haves transient corrected QT prolongation secondary to therapy, requiring ECG monitoring. High-risk group screening reports higher rates of idiopathic LQTS. ECG testing shows its efficiency among asymptomatic children with a normal heart rate. Patients present with syncope at the outpatient settings require the exclusion of a wide range of diseases, both congenital and acquired heart disease. The clinical status of the examined patients does not always correspond to the known LQTS variants. Molecular genetic analysis provides relevant information on the genetic heterogeneity of the disease, including new mutations, both pathological and beneficial ones.

Conclusion Regardless of the presence or absence of molecular genetic confirmation of LQTS, beta blocker therapy in some cases combined with implanted cardioverterdefibrillator prevents the development of the adverse events in the long-term period and ensures normal emotional, intellectual and physical development.

Aim To estimate the prevalence of functional cardiovascular system disorders and immunological parameters in children living in the Irkutsk Region, a technogenic region of Southern Siberia.

Methods A set of clinical, laboratory and instrumental, statistical and analytical methods were used in the study. Immunological parameters and the presence of functional cardiovascular system disorders were assessed in 63 children aged 5–11 years living in the technogenic region with aluminum exposure. The study group included children (n = 31) with functional changes in the cardiovascular system. Children (n = 32) without any functional changes in the cardiovascular system were enrolled in the control group. The functional status of the cardiovascular system was evaluated with the electrocardiography and physical examination. The immune parameters were measured with the flow cytometry and enzyme allegrosorbent testing. The analytical study of the chemical changes in the biomedia induced by aluminum exposure followed the national guidelines.

Results Children permanently residing in the technogenic region with aluminum exposure suffer from mild anomalies of the heart development. These anomalies are associated reliably with the diseases of the blood and blood-forming organs involving the immune mechanism. The analytical study of the chemical changes in the biomedia showed that the study group has significantly elevated aluminum levels in blood and urine. Immune alterations have been found in the study, including a significant increase in the TNFR receptor, Bcl-2 protein, Treg cell differentiation clusters (CD4+CD25+CD127-) and CD3+CD25+. A significantly increased level of specific sensitization by the IgG criterion was established.

Conclusion The study reported that functional cardiovascular system disorders among children living in the technogenic region with aluminum exposure are associated with alteration in the immune system. These alterations suggest the presence of the imbalance between apoptotic regulation and the initiation of adjuvant sensitivity to man-made haptens (aluminum).

Aim To evaluate the effects of parenting behaviours on physical activity of toddlers after total repair of septal heart defects.

Methods 80 patients who underwent total repair of CHD in the infancy were recruited in the study. The mean age at the interviewing and medical examination was 33.5±4.28 months (boys – 32 (46.0%), girls – 48 (60.0%). The functional status of the cardiovascular system was estimated using the exercise stress testing (10 squats per 20 seconds). Parenting behaviours were evaluated using the Varga-Stolin questionnaire.

Results Adverse response to the exercise stress testing prevailed among toddlers with CHD. Recovery time did not exceed 5 minutes in both groups. None complaints in the study and control groups were recorded. Most parents of children with CHD followed authoritarian hypersocialization pattern (p = 0.046) demanding unconditional obedience and discipline as compared to parents of healthy children. More than a third of the families in the study group (38.1%) followed this behavior pattern and only six families in the control group (14.8%). Five families (18%) in the control group followed democratic behavior pattern given independence to their children. Physically inactive toddlers were more commonly found in the families with authoritarian behavior pattern.

Conclusion Authoritarian hypersocialization pattern prevailed among parents of toddlers who suffered from septal heart defects. This type of parenting behaviour affected physical activity of toddlers.

Aim To compare the effects of continuous regional perfusion methods for infant aortic arch repair on the incidence of acute renal dysfunction and mortality in the early postoperative period.

Methods A total of 121 infants who underwent aortic arch reconstruction in the period from June 2007 to December 2017 were included in the study. Patients were 1:1 propensity score matched using the nearest-neighbor methodology. Sixty-two patients (median age 14 (interquartile range (IQR) [8; 23]) days) were selected for the retrospective analysis and divided in two groups: those who underwent repair with selective antegrade cerebral perfusion (SACP) (SACP group, 31 patients) at 23–26°C and those who received whole brain perfusion and additional lower body perfusion (double arterial cannulation (DAC) group, 31 patients) at 28–32°C.

Results In-hospital mortality was 3.2% in the DAC group (3.2%) and 12.8% in the SACP group, respectively (p = 0.03). More patients from the SACP group required an open chest after operation (p = 0.013). Patients from the DAC group had shorter open chest duration (p = 0.002) and shorter length of stay in the intensive care unit (ICU) (p = 0.035). There were no differences in the need for renal replacement therapy (p = 0.20) and rate of creatinine level progression during the first three postoperative days between the groups (p = 0.53). The body mass of less than 2 kilos predisposes patients to the onset of acute renal dysfunction (p = 0.013), which was found to be a predictor of early mortality (p = 0.038).

Conclusion Lower body perfusion reported similar results to SACP in terms of the onset of acute renal dysfunction, but was superior in terms of lower mortality and shorter lengths of stay in the ICU.

Aim To evaluate cardioprotective effects of combined intracoronary phosphocreatine and succinic acid administration after primary percutaneous coronary intervention (PCI) in patients with ST elevation acute coronary syndrome who were present within 12 hours of symptom onset.

Methods Seven patients with STEMI presenting within 12 hours of onset of symptoms and thrombotic occlusion of the left anterior descending artery were included in the study. Evaluation of efficiency and safety was performed with the recording of major adverse cardiac events, evaluation of the procedure success, and the rate of composite endpoints.

Results Six (85.7%) patients after primary PCI showed TIMI grade 3 flow on control coronary angiography. None complications had been reported after intracoronary administration of phosphocreatine and succinic acid. Acquired left ventricular aneurysms were found in two patients in the in-hospital period. One patient was readmitted with acute coronary syndrome without ST-segment elevation. Coronary angiography reported restenosis of the previously implanted stent. No definite increase in the left ventricular ejection fraction had been found in the in-hospital and long-term period.

Conclusion There were no evidences on definite increase in myocardial contractility. The obtained results might partially be explained by the median symptom-to-balloon time over 400 minutes.

Highlights The findings of this original study ensure the detection of violations in the humoral regulation of the maternal immune interactions with semiallogeneic fetus, considered as a risk factor for developing sporadic conotruncal heart malformations in the next generation.
Aim To study the role of female autoserum blood in limiting allogeneic interactions in short-term lymphocyte cultures of parents having children with conotruncal heart malformations.
Methods 21 married couples (the study group) with children suffering from conotrucnal heart malformations (Tetralogy of Fallot) without chromosomal diseases were examined. The control group consisted of 21 families with three or more healthy children. The immune response in a mixed lymphocyte culture of parents was assessed by the increase in HLA-DR expression in the mixed culture with respect to spontaneous lymphocyte cultures. Primary staining of female and male lymphocytes with monoclonal antibodies to CD45, conjugated with various fluorescent dyes (PC-5 and PC-7), allowed assessing the immune response of female lymphocytes to male and vice versa.
Results The effects of female autoserum on the mixed lymphocyte culture of parents were assessed. The obtained results reported that the birth of children with conotruncal heart malformations is associated with the interfering effect of female autoserum on HLA-DR expression on subpopulations of female lymphocytes (CD3+, HLA-DR+) and the activating effect on subpopulations of female lymphocytes (CD3-, HLA-DR+). The observed role of female autoserum in the study group may be associated with the absence of HLA-DR-blocking autoantibodies and high synthesis of cytokines by T2 and T3 helper lymphocytes.
Conclusion The effects of female autoserum on allogeneic lymphocyte interactions of parents may be observed in short-term mixed lymphocyte cultures. The evaluation of the activating and interfering effects ensures timely identification of any violations in the humoral regulation of the maternal immune interactions with the HLA semiallogenic fetus, considered as a risk factor for developing sporadic conotruncal heart malformations in the next generation.

Highlights. Bioabsorbable vascular scaffolds for percutaneous coronary interventions in multivessel coronary
artery disease patients and concomitant type 2 diabetes mellitus may increase the effectiveness of endovascular treatment and improve the long-term prognosis. 

The review article discusses recent data and evidences on the efficacy and safety of various stent generations for treating patients with coronary artery disease. The main complications commonly occurred following the implantation of drug-eluting stents in the long-term period are summarized, suggesting the rationale for the use of the next generation bioabsorbable vascular scaffolds in routine clinical practice. Bioabsorbable vascular scaffolds for endovascular treatment of patients with coronary artery disease show comparable efficacy compared with conventional drug-eluting stents. Particular attention is paid to the technical approaches of bioabsorbable vascular scaffold implantation, the use of intavascular imaging to control the implantation, as well as the timing of dual antiplatelet therapy in the postoperative period. The final term of the first-generation scaffold absorption, dual antiplatelet therapy, the effectiveness of bioabsorbable vascular scaffold-treated distal lesions and safety in patients with type 2 diabetes are highlighted. Future perspectives of using the next generation bioabsorbable vascular scaffolds that may improve the efficiency of percutaneous coronary interventions are presented.

Congenital heart disease is the most common fetal abnormality resulting in high pediatric disability and mortality. Congenital heart disease is a heterogeneous group of cardiac abnormalities including atrial septal defect, valvular defects and cardiac outflow tract anomalies. Genetic, epigenetic and ecological factors leading to the development of congenital heart defects in each particular case remain poorly understood. Nevertheless, multifactorial and polygenic mechanisms underlying the disease may be suggested. Moreover, advanced genetic technologies including single nucleotide polymorphism testing, copy number variation and next-generation sequencing ensure early detection of genetic causes of heart abnormalities. Recent studies suggested the contributing role of small non-coding RNA (miRNA) in the pathogenesis of congenital heart defects. miRNA is known to coordinate the development of heart and stimulate such pathological processes like fibrosis, hypertrophy and impaired angiogenesis. Thus, the study of miRNA and its impact on the pathogenesis of various heart diseases has demonstrated its promising potential for therapeutic targets in regenerative medicine. The review presents recent genetic findings, miRNA functions, signaling pathways and evidences on its role in the development of certain congenital heart defects.

This review discusses the role of autoimmune mechanisms in the development of heart rhythm disturbances and conduction disorders of various origins. The search was performed using PubMed, Medline and Google Scholar. Specific cardiovascular diseases (dilated cardiomyopathy, myocarditis, conduction disorders) developing in childhood and adolescence are associated with an increase in titer to intracellular proteins specific to myocardiocytes and cells of the cardiac conduction system. Candidate autoantibodies markers for autoimmune response have been selected. The rationale for analyzing the immune status of heart rhythm disturbances in children and adolescents has been provided.

Venous stroke in cerebral venous sinus thrombosis is a rare pathological condition with uncertain diagnostic algorithm to ensure prompt treatment by neurologists and radiologists. All the methods are not obligatory in the workup of acute stroke, except computed tomography (CT) and transcranial Doppler. Researchers and clinicians are commonly guided by their own experiences. Therefore, the diagnosis of venous stroke is an unstable process resulting in its underestimation in the general stroke population. The optimal use of neuroimaging methods within the existing standards of care for patients with stroke, determination of the necessary and sufficient set of diagnostic procedures is an organizational and methodical problem. In addition, there is no complete description of venous ischemic stroke patterns in the medical literature. The article reviews existing evidence-based data on the diagnostic algorithms for venous stroke and discusses the probability of detecting radiologic symptoms based on the diagnostic accuracy and effectiveness of such methods as conventional CT and magnetic resonance imaging (MRI), CT and MR angiography, CT and MR perfusion, transcranial Doppler (TCD) and ultrasound of the brachiocephalic arteries stated in the national and international guidelines, as well as our own experience. Limitations of their use and options to overcome major drawbacks including the introduction of teleradiology are considered.

High prevalence of internal carotid atherosclerosis and recent advances in its diagnosis result in an increase in the annual number of carotid endarterectomy procedures to restore carotid patency. Various randomized prospective studies as well as meta-analyses show a decrease in the number of perioperative and postoperative complications following carotid endarterectomy using patches compared with primary arterial closure. Despite the fact that the autologous vein is the material of choice for carotid patch angioplasty, xenogenic and synthetic patches are also widely used in the clinical practice. Furthermore, tissue engineering technologies and the development of novel biomaterials have recently emerged and may encourage manufacturing of vascular patches capable to promote a regenerative potential of the body and restore vascular wall tissues.

Medical records of a patient presented with severe aortic and mitral regurgitation and connective tissue dysplasia were reviewed. Clinical, laboratory and instrumental findings were collected at baseline and after the surgery. Long-term outcomes, including quality of life, were estimated.

The article presents a long-term follow-up of the patient who underwent an intracardiac electrophysiological study (EPS) and radiofrequency ablation (RFA) of focal tachycardia at the age of 2 months. 12 years after the indexed procedure, Wolff-Parkinson-White (WPW) syndrome was diagnosed and required repeat RFA procedure. The atrial map of the first ablated zone was reconstructed using non-fluroscopic mapping system. EPS reported the absence of myocardial electrical activity reduction zones. There were no damages after the indexed ablation. Our findings suggested the normal electrical activity of the atrial tissue in the long-term period following the indexed RFA. This clinical case reports the absence of post-ablation necrosis and successful restoration of the electrical activity of the myocardium with the child’s growth.

The article is aimed to analyze the causes of delayed diagnosis of congenital heart disease. A rare case of abnormal originating of the left coronary artery from pulmonary artery in an adolescent is reported. Both, clinical manifestations of circulatory insufficiency and diagnostic challenges, are described. All routine medical records and data were reviewed. Cardiac anatomy and age-related clinical symptoms are described with the subsequent discussion of the surgical treatment. The approach to differential diagnosis is presented. The factors contributing to the delayed diagnosis of the CHD are analyzed.