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Медичні науки 56Український журнал медицини, біології та спорту -№ 3 (5)Мета роботи -аналіз результатів застосування ендоваскулярних методів лікування при синдромі гіпоплазії лівих відділів серця (СГЛВС). Критеріями відбору були діаметр висхідної аорти менше 2 мм та мала вага пацієнтів. Прооперовано 15 пацієнтів: 4 жіночої (26,7%) та 11 (73,3%) чоловічої статі. Се-редній вік пацієнтів склав 9,1±2,4 днів, маса у сере-дньому менше 2,24±0,14 кг. У всіх випадках була проведена гібридна операція з білатеральним зву-женням легеневої артерії (ЛА), доповнена стенту-ванням відкритої артеріальної протоки як перший етап лікування. Після оперативного втручання з 15 пацієнтів четверо (26,7%) були виписані у відпо-відні стаціонарні клініки для продовження лікування.В результаті досліджень встановлено, що, не-зважаючи на високу летальність, гібридні операти-вні втручання необхідно виконувати, оскільки це єдиний шанс на порятунок цих пацієнтів. Ендовас-кулярні оперативні втручання при СГЛВС є опера-ціями вибору, які дозволяють підготувати пацієнта до наступного етапу лікування. Ендоваскулярні втручання при СГЛВС є малотравматичними, що збільшує шанси пацієнтів малої ваги та з ускладне-ною анатомією на виживання, не дивлячись на важкий їх стан при надходженні в стаціонар. Складність оперативного втручання та невелика частота поширення серед усіх інших ВВС потребує концентрувати таких хворих в одному високо-спеціалізованому кардіохірургічному центрі з ме-тою накопичення досвіду, та поступовим покра-щенням результатів лікування. Проведення подібних оперативних втручань потребує досвіду, ретельної підготовки, злагодженості на всіх етапах та усіх служб, що приймають участь у лікуванні даної категорії пацієнтів: діагностичної, транспортної, анестезіологічної, хірургічної, реанімаційної та післяопераційної лікувальної і реабілітаційної.Ключові слова: вроджена вада серця, синд-ром гіпоплазії лівих відділів серця, ендоваскулярні методи.Вступ. Лікування критичних вад серця є однією з найбільш складних проблем в серцево-судинній хірургії. Це обумовлено: незрілістю усіх систем новонародженого, і в першу чергу серцево-судинної [1], яка окрім того ще й ускладнена вро-дженою вадою серця (ВВС); стресовою ситуацією для новонародженого, організм якого перейшов з комфортних умов існування внутрішньоутробно до самостійного життя. Хоча вже сама критична вада -це така патологія розвитку серця, яка не дозволяє забезпечити адекватний серцевий викид з достатнім для життя тиском і насиченням киснем, що призводить до смерті в перші дні життя за від-сутності екстреного хірургічного втручання [3]. При цьому з перших годин життя у пацієнтів розвива-ється серцево-судинна недостатність з гіпоксемі-єю, життєвозагрозливими аритміями, і навіть їх поєднання. Прояв артеріальної гіпоксемії -ціаноз -у більшості випадків є центральним, і тому включає слизові оболонки та є результатом право-лівого шунтування венозної крові і її надходженням до аорти, зниженим легеневим кровотоком.Крім того, звичайне оперативне втручання су-проводжується значною травмою для такого осла...

Aim: To analyze the experience of treatment of congenital heart disease. Identify the proportions and ratios of surgical and endovascular treatments. Development of diagnostic service. To determine the impact of industry funding mechanisms on the development of endovascular surgery for congenital heart disease. Materials and methods: The tendencies of cardiac surgery development of congenital heart defects on the basis of the analysis of endovascular and surgical methods of treatment are considered. Own results of 10 years of experience in the treatment of congenital heart disease are presented. Results: An increase in the proportion of endovascular methods and a decrease in surgical treatments. The obtained data show a rapid increase in the share of medical procedures (from 48.9% to 89.4%) and a sharp decline in the share of diagnostic procedures (from 51.1% to 10.6%) in endovascular surgery. Early mortality after cardiac surgery in children is <5%, mortality after edovascular interventions - <1%. Ultrasound examination (ultrasound) of the heart - allows to detect 95% of all congenital heart defects and is shown to every citizen as a screening method. The influence of industry financing mechanisms on the development of endovascular surgery of congenital heart defects is shown. Expensive equipment began to be purchased at public expense, the number of equipment representatives on the market increased and, as a result, the number of endovascular procedures increased. Over the past 5 years, public funding for the needs of the industry has increased from 16 to 75%. Conclusions: Today, pediatric cardiology and cardiac surgery are reaping the fruits of the golden age in medical science. With the development of cardiac surgery, the number of operated patients increases every year. The successful outcome of treatment of patients with CHD depends on a comprehensive multidisciplinary team (Heart Team). New evidence-based approaches in resuscitation management allow to recover as soon as possible after the intervention. We see further development of the industry in the development of multidisciplinary teams to help patients with the CHD. Finally, we expect an increase in the regulatory burden and the cost of developing new treatments and diagnostics. Key words: congenital heart defects, endovascular methods, open heart operations, minimally invasive interventions, critical heart defects.

Ebstein’s anomaly (EA) is a rare and complex congenital heart disease, with a prevalence of 4 to 7 cases per 100,000 live births. At this stage of the development of EA surgery, significant progress has been made in our understanding and treatment of EA, but the question of the tactics of treating complex forms, especially in the period of infancy and early childhood, remains open. Due to significant phenotypic heterogeneity, the treatment of EA varies greatly from patient to patient and depends on many factors that require a comprehensive approach. The choice of the method of surgical correction directly depends on the anatomical features: the degree of dysplasia, rotation and displacement of the tricuspid valve, the degree of atrialization of the right ventricle, the combination with other congenital heart diseases, and the severity of hemodynamic disorders. The aim. To evaluate perioperative characteristics in patients with complex forms of EA, immediate and long-term results after hemodynamic correction. Materials and methods. In the period from 1996 to 2022, at the National Amosov Institute of Cardiovascular Surgery of the National Academy of Medical Sciences of Ukraine, one and a half ventricle repair was performed in 18 patients with complex forms of EA. There were 12 (67%) male patients, 6 (33%) female patients. The median age of the patients at the time of surgery was 30 months [3.5; 312]. The main diagnostic method in determining the defect and assessing the immediate and remote results was echocardiography and probing of the heart cavities. In order to qualitatively evaluate the perioperative, immediate and long-term results to determine the optimal tactics and terms of surgical intervention, prevention of possible complications, all the patients were divided into two age groups: I group up to 2.5 years including 9 patients (50%), II group older than 2.5 years including 9 patients (50%). Results. In the postoperative period, 2 (11%) deaths were noted in both groups. In all the deceased patients, the cause of death was acute heart failure and acute respiratory failure. The analysis of the postoperative period showed normal results in such a cohort of highly complex patients of both groups, but a more stable course was noted among patients of the I group, as indicated by statistically significant values for the duration of mechanical ventilation, stay in the intensive care unit, stay in the hospital, the average dose of sympathomimetic support and its duration, the total duration of exudation in the comparison groups. An uncomplicated course of the early postoperative period was observed in 8 (44%) patients. The remaining 10 (56%) patients of both groups had: I group, 3 (33%) patients: 7 complications; II group, 5 (55%) patients: 13 complications. In 5 patients (2 patients in I group and 3 patients in II group), attention-grabbing acute heart failure was observed, which required high doses and long-term sympathomimetic support, and in 2 patients caused death. The average observation period was 45 months [6; 190]. No deaths were observed during the observation period among 13 (72%) patients of both groups, contact was lost with 3 (17%) patients. There were no violations in the functioning of the right ventricle, but the recovery of the systolic function of the left ventricle occurred only in 8 (44%) patients. Tricuspid insufficiency was minimal in 5 (28%) patients, moderate in 6 (33%), severe in 2 (11%) patients. Conclusions. Determining the tactics of treatment of complex forms of EA is a serious problem in patients of various ages, especially in newborns and infants, due to pronounced dilatation and systolic dysfunction of the left ventricle, a sharp decrease in systemic output due to changes in the geometry of the left ventricle. The technique of hemiventricular correction shows very promising results and may become the technique of choice for patients with severe forms of EA and pronounced right ventricular insufficiency.

Tricuspid atresia (TA) is a rare cyanotic congenital heart disease (CHD) first described by Kreysig (1817). Bidirectional cavopulmonary anastomosis (BCPA) is part of the hemodynamic correction of such a complex combined CHD. In patients after BCPA, blood flow through the superior vena cava, providing efficient pulmonary blood flow, is the most important factor influencing blood oxygen saturation. The aim. To evaluate perioperative characteristics of the patients, immediate and long-term results after BCPA with TA. Materials and methods. In the period from January 1996 to April 2022, 50 patients with TA and multiple concomitant CHD underwent hemodynamic correction at the National Amosov Institute of Cardiovascular Surgery of the National Academy of Medical Sciences of Ukraine. There were 27 male patients (54%) and 23 female patients (46%). The mean age of the patients at the time of surgery was 31.6 ± 17.2 months (from 6 to 144 months). The main method of diagnosis in determining the defect and assessing the immediate and long-term results was echocardiographic examination and probing of the heart cavities. Among the 50 patients surveyed, 17 children were older than 2.5 years, so all the subjects were divided into 2 age groups: group I (n = 33) up to 2.5 years, group II (n = 17) older than 2.5 years to determine the optimal time of hemodynamic correction based on immediate and long-term results. Results. In the early postoperative period, there were 2 (4%) deaths among the patients of group II. The cause of death was acute heart failure in one case and infectious factor which led to the development of sepsis in another. There was a very interesting trend of increase in the duration of the operation (min): 239 ± 73.3 in group I, 251 ± 68.5 in group II; duration of mechanical ventilation (hours): 5.3 ± 1.4 in group I, 11 ± 3.7 in group II; duration of exudation (hours): 67 ± 22.9 in group I, 76 ± 26.8 in group II. There was significantly higher average dose of sympathomimetic (μg/kg/hour): 5 ± 2.1 for 60 ± 21.4 (hours) in group I; 7 ± 4.5 for 84 ± 29 (hours) in group II, hospital stay (days): 18 ± 5.7 in group I, 22 ± 11.3 in group II. Significantly lower mean systemic saturation at discharge 81 ± 4.6 (%) which was reported in children of group II compared to those of group I 88 ± 3.4 (%) clearly shows a decrease in the contribution of superior vena cava flow into the systemic circulation depending on the patient’s age, therefore, the clinical effect of performing BCPA as a stage of single-ventricular correction in patients with TA is much better when performing surgery in early childhood. Early postoperative period was uneventful in 27 (54%) patients. The other 23 (46%) patients of both groups had 16 and 18 complications in 10 (20%) patients of group I and 13 (26%) patients of group II, respectively, due to adverse factors of preoperative condition and specific changes in blood circulation characteristic of the BCPA. During the observation period lasting 49 ± 23.5 months, from 6 to 180 months, the final stage of hemodynamic correction was examined and performed: total cavopulmonary anastomosis was created in 31 (65%) patients. No deaths were reported after the final stage of hemodynamic correction. Conclusions. The contribution of superior vena cava flow to total cardiac output is directly related to the patient’s age and gradually decreases in the elderly, which indicates a decrease in systemic saturation, so the clinical effect of BCPA as a stage of single ventricular correction in patients with TA is much better when performing surgery in early childhood.

Background. The choice of surgical approach for the reconstruction of aortic arch hypoplasia is a rather controversial issue. Traditionally, left lateral thoracotomy is used to correct aortic coarctation with hypoplasia of one or more arc seg-ments. However, in recent years, more and more clinics have begun widespread use of median sternotomy for the surgical treatment of aortic arch hypoplasia. The purpose of the research was to analyze the immediate and long-term results of surgical treatment of isolated hypoplasia of the aortic arch in infants through left thoracotomy. Materials and methods. From 2010 to 2019, 159 infants with coarctation and hypoplasia of one or more segments of the aortic arch underwent reconstruction of the aortic arch at the National Amosov Institute of Cardiovascular Surgery of the NAMS of Ukraine and Ukrainian Children’s Cardiac Center. The study included only patients with biventricular physiology and subsequent biventricular correction. There were 108 male patients (68%) and 51 female patients (32%). The mean age of the patients was 2.4 ± 0.9 months, the mean body weight was 4.7 ± 2.0 kg. The main method of diagnosis, i.e. determining the defect and assessing the immediate and long-term results, was echocardiographic examination. The study group consisted of 155 patients (97.5%) in whom the reconstruction of the aortic arch was performed through left thoracotomy, in the remaining 4 patients (2.5%) the intervention was performed through median sternotomy.   Results. In-hospital mortality was 0.6% (n = 1). The average time of the operation was 126 ± 40 minutes, the aortic cross-clamp time was 24.7 ± 5.1 minutes. The mean duration of mechanical ventilation after the surgery was 41 ± 15.9 hours. The mean follow-up period ranged from 1 month to 13 years. There were no deaths in the long-term period. Ten (6.4%) patients developed aortic arch restenosis in the postoperative period. In 8 patients, the narrowing was treated by endovascular dilatation. Two patients underwent repeated aortic arch repair using median sternotomy. There were no cases of compression of the trachea, main bronchi, neurological complications in the follow-up period.   Conclusions. Reconstruction of the aortic arch hypoplasia through left thoracotomy is an effective and safe treatment option in infants associated with good immediate and long-term results. The study indicates that this surgical strategy can be used in most patients with hypoplasia of the isthmus and distal aortic arch.

Objective. To analyze the immediate and late follow-up results of the aortal arch plasty, using anastomosis «end to side». Materials and methods. In 2010 - 2019 yrs period in Amosov National Institute of Cardio-Vascular Surgery and Scientific-Practical Medical Centre of Pediatric Cardiology and Cardiosurgery in 75 new-born babies, suffering coarctation and hypoplasia of one or several segments of aortal arch its reconstruction was done, using ‘end-to-side” anastomosis. Into the investigation only the patients with a two-ventricular physiology and further two-ventricular correction were included. There were 41 (54.6%) boys and 34 (45.4%) girls. Average age of the patients was (2,5 ± 1,2) mo, and average body mass - (3.3 ± 0.6) kg. The main diagnostic method for the failure determination and immediate and late results studying was echocardiographic investigation. Results. Hospital lethality have constituted 6.6% (5 patients died). The lethality causes were not connected with the aortal arch reconstructive procedure, using anastomosis «end to side». Duration of follow-up was 1 mo - 9.3 yrs, (2.8 ± 2.5) yrs at average. In late follow-up period 1 patient died. In 11 (14.6%) patients in postoperative period the aortal arch restenosis have developed. In 7 patients restenosis was eliminated endovascularly, using balloon dilatation. In 4 patients the aortal arch surgical replasty was done. Tracheal and the main bronchi compression, neurological complications in late follow-up period were not observed. Conclusion. After plasty for the aortal arch hypoplasia, using anastomosis «end to side» in new-born babies good immediate and late follow-up results were noted, what witnesses its efficacy. In accordance to results of the investigation accomplished such kind of anastomosis applied may be used in patients, suffering hypoplasia of proximal aorta or in tubular hypoplasia of all aortal arch.

Background. The optimal tactics of surgical treatment of aortic arch hypoplasia combined with other congenital heart defects remain controversial. In recent years, the leading clinics of the world prefer one-stage repair of such a combination, however, two-stage treatment is still used by cardiac surgeons and remains one of the variants of surgical tactics. The aim. To analyze immediate and long-term results of a two-stage repair of aortic arch hypoplasia with other con-genital heart defects in newborns and infants. Materials and methods. The study included 138 infants who were selected for a two-stage repair of aortic arch hy-poplasia with other congenital heart defects. There were 79 (57.2%) male and 59 (42.8%) female patients. The mean age of the patients was 1.4 ± 0.7 months, mean body weight was 4.1 ± 2.0 kg. The main method of the defect diagnosis and evaluation of immediate and long-term results was echocardiographic examination. The aortic arch segment was consid-ered hypoplastic if the Z-score deviation was less than –2.0. Results. The hospital mortality rate was 5.8% (n = 8). Two patients (1.4%) had delayed sternal closure in the early postoperative period. As a result of severe myocardial failure, two patients underwent extracorporeal membrane oxy-genation (ECMO). According to echocardiography before discharge, the mean pressure gradient at the site of aortic arch repair was 16 ± 6 mmHg. There was one death in the follow-up period. In 16 (11.5%) patients, aortic arch restenosis developed in the postoperative period. In total, 20 re-interventions were performed: 11 endovascular balloon dilatations and 9 surgical re-interventions. In the early postoperative period, aortopexia was performed in two patients (1.4%) due to compression of the left main bronchus. Conclusions. Two-stage repair of aortic arch hypoplasia with other congenital heart defects in newborns and infants is effective and safe treatment with good immediate and long-term results. This study shows that this surgical strategy can be effectively used in this complex pathology.