Objective: to analyze the factors affecting enteral feeding ability of neonates with congenital heart defects (CHD) who underwent cardiac surgery.

Patients and methods. The study included 214 full-term newborns with CHD who underwent cardiac surgery: 63 children with palliative correction of CHD and 151 children with radical correction of CHD. The characteristics of the early postoperative period, the timing of bottle feeding with additional gavage feeding, the achievement of the full volume of bottle feeding, the incidence of breastfeeding after surgery, and the duration of hospitalization were analyzed.

Results. The use of cardiopulmonary bypass (CPB) was associated with a longer duration of mechanical ventilation and inotropic therapy after cardiac surgery. Positive correlations (r > 0.6) were revealed between the duration of mechanical ventilation, inotropic therapy, of stay in the intensive care unit and the timing of the bottle feeding ability (including without additional gavage feeding). Newborns with palliative correction were able to feed from bottle significantly later than children with radical correction: there was a higher frequency of supplementary gavage feeding by the time of discharge (15.7 % vs. 1.8 %, p = 0.0016), a longer length of stay in hospital. Children who underwent the Norwood procedure were able to bottle feeding significantly later than newborns after other types of surgery.

Conclusion. Newborns with CHD require a personalized approach to enteral feeding, depending on the types of cardiac surgery undergone. Factors influencing the feeding of newborns were the use of CPB, the duration of mechanical ventilation and inotropic therapy after surgery.

Introduction. TAPVC is a condition that requires diagnosis and surgical treatment in a short period of time. As the result of an absence of the direct connection between pulmonary veins and left atrium, systemic and pulmonary venous return drainage to the right heart with volume and pressure overload. At the same time, left heart preload is depending on atrial septal defect. The hemodynamic features of the TAPVC create conditions for abnormal spread of electric impulses, whereas necessity of surgical reconstruction on the atrial level — reasons for rhythm and conduction disturbance in postoperative period.

Aim of the study: detection of specific EKG markers of TAPVC for differential diagnosis of critical conditions in children, analysis of basis preand postoperative EKG to determine EKG pattern in children with TAPVC, registration of EKG changing in early and late postoperative period to define the potential rhythm and conduction disturbance in patients with TAPVC.

Materials and methods. The data of biventricular TAPVC patients were reviewed. We estimated characteristic of P, PQ, QRS, QT, rhythm and conduction disturbance and evaluated preand postoperative EKG data, with their comparison in 30 patients. Results. Preoperative EKG (9,3 ± 6,5 days) showed sinus rhythm in all cases, P wave was 62,6 ± 10,6 (40–80) msec, PQ — 104,1 ± 15 (80–160) msec, QRS — 64,3 ± 13,5 (40–100) msec, QT — 253,1 ± 29,5 (210–350) msec, electric axis of the heart — 130,9 ± 44,4 (-70 ± 185). Postoperative rhythm was sinus in 83,3 % cases, atrial ectopic — 13,3 %, AV node rhythm — 3,3 %. The comparison of preand postoperative EKG data showed significant (p < 0,05) difference in P, QT, and electric axis of the heart measurements. In late postoperative period we detected non-pathological differences in P, PQ, QRS, QT compared early posleoperative period, but sinus node dysfunction frequency was high in late postoperative period.

Conclusion. There are no specific ECG markers in preoperative TAPVC patients. The standard EKG showed right heart volume and pressure overloud with positive changing of it after surgical correction of TAPVC. The early postoperative EKG did not showed “typical” rhythm and conduction disturbance after surgery at the atrial level, but patients require a long-time dynamic observation after TAPVC surgery because of higy frequency of sinus node dysfunction in late postoperative period.

Early diagnosis of cardiovascular diseases in people involved in sports, and dynamic monitoring of them are important for the primary prevention of sudden cardiac death. Our study presents clinical and instrumental information about the health status of young athletes with various cardiovascular abnormalities. Continued long-term prospective follow-up of underage athletes will contribute to the adoption of optimal decisions in the individual assessment of the athlete’s condition, aimed mainly at protecting the health of a young athlete, as well as developing his sports career.

Arrhythmogenic cardiomyopathy (ACM) is a genetically determined disease with a high risk of sudden cardiac death (SCD). The spectrum of genetic causes of this disease is quite wide and includes mutations in both desmosomal and non-desmosomal genes. A positive result of a genetic examination is used as a diagnostic tool in patients with ACM, so its correct interpretation is one of the key factors in a personalized approach to managing a patient with this pathology. The review presents generalized modern ideas about the genetic nature of ACM.

Recently, the number of reports and clinical observations of various immune-mediated diseases in children that occur after COVID-19, including those with central nervous system involvememnt, has increased. This review briefly highlights the current knowledge about neurological diseases in children after COVID-19 with a description of a rare post-сovid-19 case of the PANS syndrome in our center.

The article provides a brief review of the etiopathogenetic aspects of congenital central hypoventilation syndrome with an emphasis on genotype-phenotypic correlations, and highlights approaches to ventilatory support. Based on the data of the local register, the experience of Almazov National Medical Research Centre is summarised. A systematic multidisciplinary approach to the treatment and rehabilitation of children with congenital central hypoventilation syndrome that has been implemented provides the most favorable results in preserving neurological and intellectual potential and quality of life.

Background. The problem of managing children with hypertrophic cardiomyopathy (HCM) remains relevant due to the high risk of sudden cardiac death (SCD). Registers of patients with HCM contribute to a better understanding of the course of the disease and its outcomes.

Objective. To study the structure of CMP with a hypertrophic phenotype, as well as to identify clinical, molecular and genetic features of the course of HCM and outcome in children based on anamnestic, clinical and instrumental data from an electronic database.

Design and method/ Currently, the database includes complete information on 80 children from 0 to 18 years of age inclusive with a hypertrophic phenotype of CMP. The study was performed on the basis of the Department of Pediatric Cardiology and Medical Rehabilitation of the V.A. Almazov” of the Ministry of Health of Russia. The database contains the history of the disease and family history, the results of clinical and laboratory-instrumental examination, the results of molecular genetic research. The register is dynamically updated.

Results. The mean age of onset of HCM was 1.75 [0.02; 10.00] years. There were more boys than girls — 54 (67.5 %). The main complaints were decreased exercise tolerance in 35 (43.8 %) children and shortness of breath in 30 (37.5%) children. All children had signs of heart failure (HF): 53 (66.3 %) had signs of functional class 2 HF, 26 (32.5 %) — functional class 1 and 1 (1.2 %) — functional class 3. Ventricular arrhythmias were recorded in 23 (29 %) children, unstable ventricular tachycardia in 2 (2.5 %) children. Myocardial fibrosis of the left ventricle according to the results of MRI of the heart was found in 60.7 %. A lethal outcome was registered in 5 % (n = 4) of cases on average at 241 ± 117 days of life. All the deceased had phenocopies of HCM.

Conclusion. Maintaining an electronic database of patients with HCM will allow a better understanding of the influence of factors, including genetic ones, on the course, outcomes, and prognosis of the disease in the pediatric population.

Today’s personalized medicine represents an integral model for the selection of diagnostic, therapeutic agents and preventive measures that are optimal for a particular individual, taking into account his genetic, physiological, biochemical, psychosocial and other characteristics.

The presence of a special relationship between the cardiovascular system and the kidneys are confirmed by a significant difference in mortality from malformations and in the population. The possibility of prenatal diagnosis of malformations sets the task of a special approach to the management of such patients, which can provide a personalized approach.

The article presents the experience of a personalized approach to the management of children with cardiorenal syndrome (combined congenital heart and kidney defects).

The problem of nutrition during pregnancy is relevant all over the world because of globalization. Globalization has brought fast food, sweets and improper nutrition. As a result of such nutrition, hunger is quenched, and saturation of the body with the necessary nutrients does not occur. As a result, the number of meals and their volumes increase, which leads to a vicious circle. Mom gains weight, and the fetus suffers even more: receiving a signal about insufficient intake of nutrients, it activates the genes for storing these nutrients and becomes obese after birth. Therefore, starting from the early stages of pregnancy, it is necessary to evaluate the eating behavior of a pregnant woman and make recommendations for its correction. The necessity and importance of nutritional support for gestation, adequate preparation for pregnancy is undeniable.

Small for gestational age and fetal growth restriction (FGR) are the leading causes of perinatal morbidity and mortality. It has been established that FGR may be a late manifestation of impaired invasion and growth of the placenta. It has been proven that a change in the maternal biochemical marker’s concentration during first-trimester screening at 11⁺⁰–13⁺⁶ weeks of gestation helps to identify pregnant women with a high risk of abnormal karyotype. The deviations in the maternal marker’s concentration in chromosomally and morphologically normal fetuses are associated with various obstetric complications, such as miscarriage, FGR, preeclampsia, and preterm birth. This review has predictive value in identifying pregnant women at high risk of developing FGR based on the results of a combined first screening.

HCMP is a severe heart disease characterized by high rates of sudden mortality, especially in young people. Optimization of the tactics of treatment of children with HCMP in order to improve the prognosis remains one of the urgent tasks of cardiology. A clinical case of a 15-yearold boy with moderate myocardial hypertrophy and an asymptomatic course of HCMP is presented, in connection with which the disease was not diagnosed for a long time. The boy was engaged in professional sports, during the competition he suddenly lost consciousness, clinical death occurred. Only timely resuscitation measures allowed to save the patient’s life. Implantation of a cardioverter defibrillator was performed as a secondary prevention of SCD (class 1 indications). The importance of timely examination of asymptomatic athletes with myocardial hypertrophy for differential diagnosis between HCMP and myocardial remodeling against the background of intense physical exertion (“athlete’s heart”) is emphasized. The importance of solving the issue of the inadmissibility of intense physical exertion, even with an asymptomatic course of HCMP, is shown. It should be remembered that it has been established that intense sports loads act as a trigger for cardiac arrest in patients with HCMP. Retrospective calculation of SCD risk based on HCM risc-SDD calculator/The BCC risk calculator at GCMP showed 3.19 %, which corresponded to a low risk. The possibility of SCD in patients with HCMP is emphasized, even with an estimated low risk, which requires further research to find predictors of an unfavorable outcome.

A prospective observation for 4 years (from 14 to 17 years) is presented for a girl with Danone’s disease. The early onset of the disease was noted, the clinical picture was dominated by syncopal and syncopal conditions. According to the clinical picture, presyncopal conditions were of a vasovagal nature, which was verified by the results of the Tilt test when the vasodepressor variant was detected. The disease was characterized by isolated cardiac disorders in the form of concentric hypertrophic cardiomyopathy, widespread fibrous changes in the myocardium. According to the standard ECG and daily ECG monitoring, ventricular preexitation syndrome was detected. Electrophysiological examination established the nodo-ventricular tract. Myopathy, cognitive impairment and visual changes were absent. A mutation in the heterozygous state was detected in exon 5 of 9 exons of the LMP2 gene, leading to the acquisition of a premature stop codon, was identified. X:g.119581719G>A ENST00000434600.2: c.718C>T. The mutation was verified by Sanger. The case in the pedigree is sporadic. As a prevention of sudden cardiac death, implantation of a cardioverter-defibrillator was performed. Effective relief of induced stable ventricular fibrillation (Burst stimulation protocol) was achieved only with the use of a ventricular electrode with two shock coils.

Introduction. Epispadias is an extremely rare disease. Epispadias in women is 5–6 times less common than in men. The incidence of female epispadias ranges from 1 in 160,000 to 480,000 live births. Epispadias can be diagnosed by a thorough examination of the genitals. Treatment of epispadias is only surgical, which is quite difficult and requires experience. The literature that discusses female epispadias is very limited. In this article, we would like to report on the surgical treatment of isolated epispadias in a 3-year-old girl at the Almazov National Medical Research Center.

Clinical case. A 3-year-old girl presented with the main complaint of urinary incontinence since birth, during the day and at night. Urinary incontinence was not caused by physical activity and was not aggravated by eating/drinking. Physical examination showed that the external genital organs of the patient have underdeveloped labia minora, abnormal urethra, bifurcated clitoris. Laboratory results were within the normal range. Voiding cystourethrography revealed urine leakage during the filling phase. The bladder wall was normal, there was no vesicoureteral reflux. The patient underwent simultaneous surgical interventions, consisting in plastic surgery of the urethra and reconstruction of the bladder neck, plastic surgery of the clitoris and labia minora. There were no intraoperative and postoperative complications. After 1 week and 6 months of observation, the patient achieved urinary retention, the surgical wound healed primarily.

Discussion. Epispadias is a rare condition that can vary in severity from mild to severe. Severely, splitting of the entire urethra occurs, involving the bladder neck, causing permanent urinary incontinence in the patient. Cases of epispadias are quite difficult to diagnose. The doctor should dilute the labia majora and carefully conduct a physical examination. The objective goals of the surgical treatment of epispadias are to achieve urinary retention, restore the anatomy, function and cosmetic appearance of the urethra and genitals. One-stage reconstruction is a modern surgical technique used to treat isolated epispadias in women.

Conclusion. Epispadias in women is a rare congenital anomaly that is often not detected in childhood. For isolated epispadias in a girl, one-stage surgical correction is currently preferred.

A case of clinical observation of a child having with) dilated cardiomyopathy in the structure of Becker’s progressive muscular dystrophy, complicated by chronic heart failure of the second A degree, the second functional class. The etiology, pathogenesis, clinic, diagnostics, features of differential diagnostics and new approaches to the treatment of progressive neuromuscular dystrophies, which are relevant in pediatric cardiology practice, are reflected.

The article presents a rare case of a combination of Ebstein’s anomaly and primary cardiomyopathy in a 14-year-old teenager. The clinical case demonstrates diagnostic difficulties due to an unusual clinical picture, as well as difficulties in interpretation and choice of treatment tactics.

The incidence of myocarditis in children ranges from 0.026 to 2 cases per 100,000 children. It is known that approximately 0.05 % of all hospitalizations of children are associated with myocarditis. The total mortality of children from myocarditis in the acute period reaches 7–17 %, and in the fulminant course — up to 30 %. Currently, there is a tendency to decrease mortality, which is associated with the acquisition of new knowledge and the introduction of new methods of treatment of myocarditis, according to modern clinical recommendations. This report presents a case of a favorable outcome of fulminant myocarditis in a 13-year-old 4-month-old girl who became acutely ill. Clinical manifestations of the onset of the disease, issues of differential diagnosis, treatment tactics at the stages of routing are described.