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.

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.

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In patients after bidirectional cavapulmonary anastomosis, blood flow through the superior vena cava (SVC), providing effective pulmonary blood flow, is the most important factor influencing blood oxygen saturation. Blood flow through the inferior vena cava recirculates into the systemic bloodstream. The study of the ratio of these flows will provide better understanding of the physiology of blood circulation after anastomosis and determine systemic oxygen saturation of blood and optimal time to perform surgery.   The aim. To determine volumetric blood flow in the SVC, calculate pulmonary to systemic blood flow ratio in children after bidirectional cavapulmonary anastomosis, and evaluate its contribution to cardiac output and oxygen saturation in systemic blood flow. Materials and methods. In the period from January 2010 to June 2021, 51 patients with congenital heart defects with depleted pulmonary blood flow underwent hemodynamic correction at the National Amosov Institute of Cardiovascular Surgery of the National Academy of Medical Sciences of Ukraine. There were 29 male patients (57%) and 22 female patients (43%). The mean age of the patients at the time of the surgery was 34 ± 18.2 months (2 to 120 months), the mean age of patients at the time of examination was 43.5 ± 28.4 months (12 to 134 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. To evaluate the optimization of pulmonary/systemic blood flow we used equations obtained using the Fick method. Pulmonary to systemic blood flow ratio was calculated separately for 35 patients. Among the examined 35 patients, 18 children were older than 2.5 years, so all the examined patients were conveniently divided into 2 age groups: I group (n = 17) up to 2.5 years, II group (n = 18) older than 2.5 years to assess the contribution of SVC to the systemic circulation depending on age. Results. Pulmonary to systemic blood flow ratio was calculated for 35 patients Qp/Qs = (82% – 66%) / (97% – 66%) = 0.52. The calculated cardiac index according to echocardiography was 4.0 ± 0.85 L/min/m2 which corresponds to the SVC saturation (r = 0.60, p = <0.001). The flow in the superior vena cava = 2.08 L/min/m2. There was a very interesting trend towards decrease in the average rate of systemic saturation in patients after bidirectional cavapulmonary anastomosis depending on age and duration of surgery. Thus, in 17 patients of group I, the calculated Qp/Qs was (84% – 67%) / (97% – 67%) = 0.57. In patients of group II, the average systemic oxygen saturation was 78 ± 2% (from 65% to 81%). Calculated Qp/Qs for 18 patients of group II = (78% – 66%) / (97% – 66%) = 0.39, which indicates a decrease in pulmonary to systemic ratio with the growth of the child. Decreased systemic saturation after bidirectional cavapulmonary anastomosis in patients with increasing age and body surface area is associated with a decrease in the proportional flow from the superior vena cava. Therefore, in our clinical material, we confirmed the phenomenon of change in pulmonary to systemic ratio depending on age, which was described by Salim et al. according to a study conducted on healthy babies. Conclusions. The contribution of SVC flow to total cardiac output after bidirectional cavapulmonary anastomosis is directly associated with the patient’s age and gradually decreases in older patients, as indicated by a decrease in systemic saturation, so the clinical effect of bidirectional cavapulmonary anastomosis may be significantly better when performing surgery in early childhood.

Background. Aortic arch hypoplasia is a congenital anomaly of the development of the aortic arch characterized by hemodynamically significant narrowing of one or more segments of the aortic arch. This defect occurs either as an isolated condition or combined with other congenital heart defects. The materials used to enlarge aorta for successful repair and prevention of postoperative complications range from the patient’s own aortic tissue (in most cases) to other materials (autopericardium, xenopericardium, pulmonary artery tissues, synthetic patches). An autologous pericardial patch is the most common, available and cheap option. The purpose of this work is toanalyze immediate and long-term results of our experience using autologous pericardium to reconstruct the aortic arch. Materials and methods. From 2011 to 2019 at the National M.M. Amosov Institute of Cardiovascular Surgery of the NAMS of Ukraine and Ukrainian Children’s Cardiac Center 16 infants underwent aortic arch repair using autologous pericardial patch. The group included 6 (37.5%) male and 10 (62.5%) female patients. The mean age of the patients was 1.3 ± 0.9 months (from 0.06 to 4.5 months), the average body weight was 3.8 ± 1.6 kg (from 2.2 to 8.7 kg). The average body surface area was 0.24 ± 0.05 m?. Antegrade selective cerebral perfusion was performed in all the patients during the aortic arch reconstruction. Results. Hospital mortality was 6.2% (n = 1). The cause of death was not related to the reconstruction technique. The average duration of cardiopulmonary bypass was 142.5 ± 38.5 minutes, the aortic cross-clamp time was 76.9 ± 33.7 minutes, and the time of selective cerebral perfusion was 50.4 ± 25.4 minutes. Six patients (37.5%) in the early postoperative period had delayed sternal closure. According to echocardiographic data at discharge, the average pressure gradient at the site of aortic arch reconstruction was 15 ± 5.5 mm Hg, the left ventricular ejection fraction was 65.9 ± 5.9%. The mean follow-up period ranged from one month to 6.1 years (mean 2.8 ± 2.3 years). There were no deaths in the long-term period. Four (25%) patients developed aortic arch restenosis in the postoperative period. One patient presented with an aortic arch aneurysm 1 month after the initial operation. There were no cases of compression of the trachea, main bronchi, neurological complications in the follow-up period. Conclusions. The use of autologous pericardium in aortic arch reconstruction is an effective and safe procedure for infants with good immediate and long-term results. The study showed that autologous pericardium may be an acceptable alternative to reconstruct the aortic arch. A large percentage of reinterventions encourages more thorough removal of ductal tissues and determining the size of the autopericardial patch.

The aim. This study aims to determine the reintervention rate in infantsundergoing aortic arch repair and to analyze risk factors and evaluate the results of reinterventions. Materials and methods. This retrospective study examines 445 infants with aortic arch hypoplasia who under-went aortic arch reconstruction between 2011 and 2019. The study included only patients with two-ventricle physiology and subsequent two-ventricle repair. Techniques for primary repair included extended end-to-end anastomosis (n = 348), end-to-side anastomosis (n = 611), autologous pericardial patch repair (n = 16). Results. The overall mortality in the entire study group was 3.3 %. Follow-up period ranged from 1 month to 9.4 years (mean 2.8 ± 2.5 years). Restenosis at the site of aortic arch repair was identiϐied in 47 (10.5 %) patients. Of these, 12 patients underwent surgical reconstruction of the aortic arch, 27 patients underwent balloon angioplasty, and in 8 patients both methods were used. Freedom from reintervention was 89.4 % at 1-year follow-up and 87.5 % at 4-year follow-up. The most determining factorsfor restenosis were related to hypoplastic proximal aortic arch and body weight less than 2.5 kg. Conclusions. Surgical treatment of aortic arch hypoplasia in newborns and infants is effective and shows good immediate and long-term results. Anatomical correction of reobstruction at the level of the aortic arch is safe with both endovacular and surgical methods with low mortality and incidence of repeated interventions. Identified risk factors for mortality and recurrent aortic arch interventions help to improve the treatment of aortic arch hypoplasia in patients under 1 year of age.

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Aortic arch surgery in neonatal patients remains problematic despite the constant evolution and improvement of treatment methods. Even after successful correction, complications associated with aortic arch reconstruction are more common in young children. This is especially true for newborns and infants with concomitant complex congenital intracardiac abnormalities. Despite the risk of postoperative complications, the risk of surgical treatment of aortic arch hypoplasia is lower than the cumulative hazards associated with the natural course of this defect. That is why pediatric cardiologists are obliged to constantly monitor the operated patients in order to timely identify and treat complications. The aim. To analyze the complications in the early and remote postoperative periods after the reconstruction of the aortic arch in the neonatal period. Materials and methods. The work is based on a study of 445 patients under 1 year of age, who underwent surgical treatment of aortic arch hypoplasia from 2010 to 2019. The criterion for inclusion in the study group was the presence of isolated hypoplasia of the aortic arch and combination with other defects, which were corrected by two-ventricular repair. The majority of the treated patients were male (284 [63.8% of the total number of patients]). The median age of the patients was 0.7 months (0.3; 2.7). The median body weight of the patients was 3.7 kg (3.25; 4.59). Results. At the hospital stage, 12 patients died, which accounted for 3.1% of the entire cohort of operated patients. Complications occurring at the hospital stage were recorded in 75 (16.8%) patients. In the long term, a complicated course was observed in 72 (16.6%) children. Among them, the most frequent complications were: respiratory failure requiring prolonged mechanical ventilation (36.9%), dilated sternum due to severe heart failure (17.4%), diaphragmatic paresis (8.7%), chylothorax (5.4%). In the long term, 47 (10.5%) patients developed aortic arch restenosis, which required reinterventions. The proportion of patients without reoperations in the follow-up period according to the KaplanMeier analysis was 93.4% after 1 year, 91.2% after 4 years, and 76.5% after 9 years. Residual hypertension requiring medical treatment was reported in 59 (13.2%) patients. Conclusions. Aortic arch hypoplasia is a complex congenital heart disease; its surgical treatment is accompanied by the development of complications both in the early and in the long-term period. The main complications at the hospital stage were respiratory and heart failures which were associated with the presence and correction of concomitant congenital heart defects. The main complication of the follow-up period was reobstruction at the level of the aortic arch. Anatomical correction of reobstruction is safe with both endovascular and surgical treatments.