Aim: to demonstrate a clinical observation of the use of hemodynamic treatment in a newborn with hypoplasia syndromeof the left heart.Methods. A newborn with a congenital heart disease that affects normal blood flow through the heart and is characterizedby critical underdevelopment of the left heart (left heart hyperplasia syndrome) underwent the Norwood procedure. Italso provides observation during the inter-stage period and information on the procedure of bidirectional cavopulmonaryanastomosis.Results. Hypoplasia syndrome of the left heart is 1.4-3.8% among all congenital heart defects and 16% among criticalcongenital heart defects. Mortality in large cardiac surgery centers currently does not exceed 15%. However, mortality amongpatients with low body weight is up to 51% after the first stage of palliative treatment. In our clinical case, we describe a childwith hypoplasia of the left heart and low body weight, as well as hemodynamic treatment (Norwood procedure, inter-stageperiod and bidirectional cavopulmonary anastomosis procedure).Conclusion. Palliative hemodynamic correction in patients with hypoplasia syndrome of the left heart and low bodyweight can be performed with good early and long-term results.

The purpose : This study evaluates long-term results of modified method of reverse subclavian flap aortoplasty, of infants with coarctation of the aorta combined with hypoplasia of the distal aortic arch. Materials and methods. 64 patients under the age of 1 year, correction of aortic coarctation with hypoplasia of the distal arch department was performed. Patients were divided into 2 groups: the 1st group included patients, whose correction was made by modified method of reverse subclavian flap aortoplasty of the left subclavian artery (n=32); the 2nd one included patients, whose correction was made by the method of extended anastomosis (n=32). Average diameter of aorta at the level of distal aortic arch was 5.1±0.1and 5.5±1.8 (р=0.51), peak gradient at the level of isthmus was 46.6±31.03 and 48.4±32.01(р=0.7). All patients underwent CAT scanning of aorta together with opacification, and Z score of aortic arch was calculated to clarify the level of hypoplasia. Results . Four-year cumulative survival rate was 95.9% in the 1st group and 95.6% in the 2nd group. In the distant period, isthmus diameter in the 2nd group was significantly different from the one in the 1st group, and was 0.98±0.4 mm and 1.2±0.86 mm correspondingly (р=0.003). For patients from the 1st group, freedom from recoarctation in distant period was 98.2%, while it was 96.3% for patients from the 2nd group, and it was not fairly different from statistical point of view. While comparing the long-term results, statistically significant prevalence of hypertension in the 2nd group (р=0,0034) was observed; pressure gradient in the 1st group at the level of isthmus was 12.2 ±1.06 and in the 2nd group it was 15.5±1.89 (р=0.002); cases of hypertrophy of myocardium of the left ventriculus -10 (40%) in comparison with the 1st group, where they were 2 (8%) (р=0,003). Conclusions . Modified method of reverse subclavian flap aortoplasty of the subclavian artery exhibits results, which can be compared to those of the method of extended anastomosis.

Patients with postoperative complications after complete repair of Tetralogy of Fallot from 2005 for 2011 in pediatric department of Meshalkin State Research Institute of Circulation Pathology were analyzed. It was revealed that the most frequent yearly postoperative period complication after complete repair of Tetralogy of Fallot is ventricular septal defect recanalization, and for long-term one is right ventricle outflow tract stenosis. There are results and methods listed below describing correction of these complications.

Objective. The study was designed to assess the morphology and structural changes in the walls of the left subclavian artery in neonates and infants with coarctation and aortic arch hypoplasia. Methods. The study included 27 patients with hypoplasia of the aortic arch at the age of 64 (27; 89) days, average body weight of 3.6±1.1 (from 1.8 to 6.9 kg), who were operated at the Center of Pediatric Surgery of Academician Ye. Meshalkin Research Institute of Circulation Pathology in the period from 2013 to 2014. All patients underwent off-pump surgery of hypoplasia of the aortic arch by using modified reverse plasty with a patch from the left subclavian artery. The patch was taken intraoperatively to determine the ratio of elastin and collagen. The results were compared with those of the control group. Results. Significant differences were found in the content of collagen and elastic fibers in patients with aortic arch hypoplasia. Patients with hypoplasia had a significantly higher content of thin collagen and a lower content of elastic fibers in the wall of the subclavian artery. Conclusion. The morphology of the subclavian artery in children with aortic arch hypoplasia is characterized by an increase of collagen and a decrease of elastic fibers due to the proliferation of smooth muscle cells, myofibroblasts, fibroblasts and the change in their relationship. These features determine the changes in elastic characteristics of the vascular wall, i.e. increased rigidity.

Objectives. The objective of the study was to investigate the influence of the main right ventricular (RV) anatomical components on global RV function by using CMRI after tetralogy of Fallot (TOF) repair based on various surgical approaches. Materials and Methods. A total of 52 asymptomatic, clinically stable patients (28 boys and 24 girls) aged 7.5 (5.5; 9.5) years were retrospectively examined after TOF repair to assess RV dysfunction in the Pediatric Cardiac Surgery Department of the Novosibirsk Research Institute of Circulation Pathology n.a. acad. E.N. Meshalkin. All patients were divided into 2 groups depending on surgery technique of TOF repair: transannular plasty (group 1, 26 pts) or TOF repair with pulmonary annulus preservation (group 2, 26 pts). Results. Right ventricular ejection fraction for the entire ventricle and for its parts was significantly higher in group 2 whereas RV end diastolic volume (EDV) for the entire ventricle and for its parts and stroke volume (SV) were significantly higher in group 1. Pulmonary regurgitation was also higher in group 1: 36.7 (32; 44) versus 13.2 (3; 14) (p>0.01). Odds ratio for pulmonary regurgitation was lower in group 2 in comparison with group 1: OR (95% confidence interval, CI) 0.19 (0.04–0.72), р=0.02. Conclusions. Right ventricular function after TOF repair depends on the type of right ventricular outflow tract (RVOT) reconstruction. Through separate functional analysis of the anatomical RV components, this study demonstrated that the global RV function is decreased in patients after transannular plasty. Long term RV dysfunction in group with transannular plasty was associated with significant pulmonary regurgitation and the presence of large akinetic RVOT region involving the sinus part in the contraction.

Congenital left ventricular diverticulum is an extremely rare heart defect. Here, we report on a case of successful modified endoventriculoplasty by using the Dacron patch in an infant with one-year follow up.

Long-term results where evaluated for different methods of pulmonary artery banding (PAB) in 25 patients with univentricular congenital heart defects with used three methods. Hospital mortality was 8%. Second stage palliation was performed in 61% of patients. More tight PA banding in patients with univentriclar gave opportunity to perform 2nd stage of operation in 100% of patients, decrease complications and mortality rate, modify pulmonary circulation effectively.

Objective: To evaluate post-operative elastic properties of the aorta and prevalence of arterial hypertension and left ventricular (LV) hypertrophy in infants who underwent surgery for aortic coarctation during the first 2 months of their lives. Methods: Main group (n=32) consisted of patients who underwent surgery for isolated aortic coarctation (extended anastomosis and classic end-to-end anastomosis technique). Control group (n=19) included healthy children. Echocardiography was done in all patients upon admission, immediately after surgery, before discharge, and 1 year after surgery. We also evaluated the elastic properties of the aorta such as distensibility (D) and the aortic wall stiffness index (SI). Results: Patients with aortic coarctation, who underwent surgery during the first 2 months after birth, had different aortic elastic properties compared with those in healthy children. We found that the elastic properties remained decreased in the long-term follow-up period. One year after surgery, despite the absence of the signs of re-coarctation in the main group, the mean pressure gradient (according to echocardiography) was 16.4+9.6 and 29.1% of patients still required antihypertensive medications; LV hypertrophy was documented in 45.8% of patients. Conclusion: Among patients, who underwent surgery for aortic coarctation during the first 2 months of life, 30% had arterial hypertension and 50% had LV hypertrophy one year after surgery. These results were independent from the correction method of aortic coarctation. The study showed that anatomically radical operation was not physiologically and functionally radical in one third of our patients.