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Thirty-nine patients with a diagnosis of common arterial trunk and high pulmonary hypertension were examined. Radical correction of the anomaly was performed in 22 patients with a hospital lethality of 36%. Residual pulmonary hypertension was the cause of death in all cases. Results of surgical treatment as well as the indexes of hemodynamics and biopsy and autopsy data were analyzed during the investigation.

The paper provides the results of a modified subclavian pulmonary anastomosis by employing Gore-Tex vascular prostheses in 91 babies with Fallot's tetrad whose age was 10 days to 32 months and weight was 3.6 to 15 kg. In 89 patients, the operation was performed by using a prosthesis, 4-6 mm in diameter, without crossing the subclavian artery, whereas in 2 patients, this was done by lengthening the crossed short subclavian artery. In the early postoperative period, 2 (2.2%) patients died, anastomosis thrombosis requiring a reoperation, occurred in 3 (3.3%) patients; other non-fatal complications were observed in 8 (8.8%) patients. A clear-cut effect of the operation was seen in all patients. SaO2 rose from 54 +/- 18 to 80.0 +/- 8%. In the long-term (6-60 months) postoperative periods, a total of 77 patients were examined: anastomosis function proved to be impaired in 4 (5.2%), all the vascular prostheses, 6 mm in diameter, were patent up to 60 months of surgery. The thrombosis probability for anastomosis, 5 mm in diameter, was 5% during 12 months of operation and 16% during 24-60 months. Three patients needed therapeutical management of heart failure. Other serious complications, including deformity of the pulmonary artery or upper extremity, were not found in the examinees. In the palliative treatment of Fallot's tetrad, subclavian pulmonary anastomosis via the Gore-Tex vascular prosthesis can be the method of choice in the first-year-old infants and has some substantial advantages over the classical Blelock-Taussig anastomosis.

The work generalizes the experience of the Bakulev Institute of Cardiovascular Surgery in balloon valvuloplasty of valvular pulmonary stenosis in 58 children of the first 3 years of life: 8 were 1 to 6 months of age, 10 were 7 to 12 months of age, 18 were 13 to 24 months old, and 22 were 25 to 36 months old. Cyanosis was found in 27 of them. As the result of balloon valvuloplasty, the systolic pressure gradient between the right ventricle and the pulmonary artery reduced from 114.7 +/- 12.6 to 31.4 +/- 7.2 mm Hg in children under 1 year of age, from 143 +/- 12.6 to 40.1 +/- 8.3 mm Hg in children aged from 1 to 3 years. In patients with cyanosis, saturation of arterial blood with oxygen increased to 92%. The late-term results were studied in follow-up periods of 6 to 36 months in 35 patients, by means of catheterization and angiocardiography in 15 of them. Analysis showed balloon valvuloplasty to be effective. Balloon valvuloplasty was repeated in 5 patients with critical stenosis because a hemodynamic effect was not achieved by the first dilatation.

In the 1980-1988 period, Mustard's operation was used to treat transposition of great vessels at the Department of Younger Children, A. N. Bakulev Institute of Cardiovascular Surgery. Seventy nine patients were operated on: 56 with simple transposition, 9 (16%) patients died; 23 with complex transpositions of great vessels (18 with concurrent ventricular septum defect, 5 with Taussig-Bing's disease), 5 (22%) patients died. Rhythm disturbances and obstruction of the venae cavae were most common postoperative complications. Six patients underwent Mustard's operation with closure of ventricular septal defect. In 18 patients this operation was performed as a palliative procedure due to high pulmonary hypertension. Lung biopsy showed the majority of patients with concurrent transposition and interventricular septal defect to have grade III-IV changes (by Hit-Edwards' classification) in the pulmonary vessels. Long-term hemodynamic studies showed good effect of palliative Mustard's operation.

Among 111 infants with coarctation of the aorta, fibroelastosis of the left ventricular endocardium was found in 14 cases comprising 12.6%. Such a combination usually caused an extremely poor state of the patients and served as an indication for surgery. A resection of the coarctation of the aorta in cases of fibroelastosis provides for a significant reduction of the workload on the left ventricular myocardium, for an improvement of the patient's state permitting to survive the critical first three years of life. The problems of intravital diagnosis of fibroelastosis in patients with coarctation of the aorta are presented, the importance of chronic ischaemia of the left ventricle in the development of fibroelastosis is emphasized. The details of surgical tactics and postoperative care of such patients are discussed.

Thirty-seven children who had been operated on for tetralogy of Fallot during the first year of life were examined in remote postoperative periods (in 5 years and 5 months, on the average). They all underwent palliative operations. The condition of 78.4% of them in remote postoperative periods was found to be good or satisfactory; in 8 patients (21.6% the result of the operation was poor. With prolongation of the follow-up period, the effect of the operation tends to be less favourable than in the initial period. The causes aggravating the patients, condition in the remote periods are: an anatomically unfavourable variant of the anomaly, the development of hypo- or hyperfunction of the anastomosis, faults in the surgical techniques of the operation.

Alpha 1-antitrypsin rare variants' distribution in a group of 196 families with developmental malformations of newborns was investigated. Significantly increased frequencies of rare variants were noted in groups of probands and their mothers, as compared to the control groups. Preferential transmission of rare alleles from mothers to probands is demonstrated.