Correction of the aortic arch hypoplasia in newborn babies, suffering complete form of atrio-ventricular septal defect

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Objective. Tactics of treatment and results of the hypoplasia of the aortic arch correction in newborn babies with complete form of atrio-ventricular septal defect were studied.
 Маterials and methods. During period from 2011 tо 2019 yrs in 21 patients correction of hypoplasia of aortic arch and atrio-ventricular septal defect were performed. The investigated group consisted of patients with a two-ventricular physiology. The patients, suffering imbalanced form of atrio-ventricular septal defect and with variants of hypoplasia in left parts of the heart, were excluded from the investigation. There were 9 (42.9%) boys and 12 (57.1%) girls. Average age of the patients have constituted (2.3 ± 1.1) mo (from 0.06 до 7.7 mo). Average body mass of the patients have constituted (3.8 ± 1.0) kg (from 2.0 до 5.9 kg). The aortic arch segment was considered a hypoplastic one if a Z-score (Standard Score - standard estimation) shift have constituted lesser than 2.0.
 Results. Hospital lethality have constituted 14.2%. Three newborn babies died: 2 - after first stage of correction, and 1 - after second stage of correction. All the patients, to whom a one-staged correction was done, have survived. The causes of lethality were not connected with method of the aortic arch reconstruction. While performance of echocardiography before discharge from the hospital the pressure gradient on place of the aortic arch plasty have constituted (13.6 ± 6.8) mm Hg at average. Average follow-up period have constituted (2.6 ± 2.1) yrs (from 2 mo tо 8.1 yr). In late follow-up period lethality was absent. In 2 (9.5%) patients in postoperative period reoperations on aortic arch and mitral valve were performed. Tracheal compression as well as the main bronchi compression, neurological complications in late follow-up period were absent.
 Conclusion. For the patients, suffering complete form of atrio-ventricular septal defect in conjunction with hypoplasia of aortic arch the staged correction constitutes a more favorable option, owing good immediate and late results. A staged approach in the treatment is indicated for newborn babies, suffering hypoplasia of distal aortic arch and competent atrio-venticular valves or with small regurgitation. Оne-stage correction is indicated in hypoplasia of aortic arch, pronounced regurgitation on common atrio-ventricular valve and pronounced heart failure.