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. The combination of simple transposition of the great arteries (TGA) and obstruction at the level of the aortic arch is not very common. However, when transposition is combined with the VSD, Taussig-Bing anomaly, this combination is more common. The degree of obstruction at the level of the aortic arch may vary from discrete coarctation of the aorta, tubular hypoplasia of the aortic arch, to interruption. Despite the improvement in the results of surgical treatment of this pathology in recent years, the question of the stage of treatment remains debatable.; The objective of the study was to analyze immediate and long-term results of the correction of transposition of the great arteries and aortic arch hypoplasia.; From 2010 to 2019 at the "Amosov National Amosov Institute of Cardiovascular Surgery of the National Academy of Medical Sciences of Ukraine" and "Scientific Center For Pediatric Cardiology and Cardiac Surgery of the Ministry of Health of Ukraine" 76 infants underwent repair of TGA and aortic arch hypoplasia. The study included only patients with two-ventrical physiology and subsequent two-ventrical repair. There were 49 (64%) male patients and 27 (36%) female patients. The mean age of patients was 1.1 ± 0.5 months, mean body weight was 3.7 ± 1.4 kg. The average body surface area was 0.23 ± 0.05 m². Patients were divided into two groups: group I involved 52 (68%) individuals - patients who underwent one-stage repair of TGA and aortic arch hypoplasia, group II included 24 (32%) patients with two-stage repair, consisting of aortic arch reconstruction and pulmonary banding at the first stage and correction of TGA at the second stage. Antegrade selective cerebral perfusion was performed in 24 (46%) patients during reconstruction of the aortic arch.; The in-hospital mortality rate was 5.3% (n= 4). In group I, in-hospital mortality was 3.8% (n=2), and was significantly lower compared to the group II - 8.3% (n=2), p<0.05. Eight patients (14.5%) had delayed sternal closure in the early postoperative period. The average long-term follow-up was 3.7±2.8 years (from one months to 9.1 years). One late death occurred in group II. Aortic arch restenosis developed in 11 (14.4%) patients: 7 (13.4%) patients in group I and 4 (16.6%) patients in group II. Right ventricle outflow tract and pulmonary artery obstruction occurred in 9 (11.8%) patients: in 2 (3.8%) patients from group I and in 7 (29.1%) patients from group II. In the long term follow-up there were no neurological complications and compression of the trachea.; Surgical treatment of TGA with aortic arch hypoplasia is effective in infants with good immediate and long-term results. The study indicates that one-stage repair has better immediate and long-term results, compared to two-stage treatment and may be the method of choice in the treatment of this complex pathology.
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