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Total anomalous drainage of the pulmonary veins into the coronary sinus is a complicated congenital heart disease which takes a particularly severe course at early age. Its diagnosis is based on the discovery of overfilling of pulmonary circulation on radiography in a cyanotic patient, atrial septal defect and distended coronary sinus on echocardiography, and, in some cases, anomalous drainage of the pulmonary veins into the coronary sinus. The final diagnosis may be established during selective pulmonary arteriography. Operations were performed on 12 patients with this pathological condition under extracorporeal circulation and hypothermia with good results. In patients over one year of age the operation consisted in widening the atrial septal defect with resection of the part of the interatrial septum between the defect and the superior border of the coronary sinus and closure of the newly formed defect with a graft and transposition of the coronary sinus and the abnormally draining pulmonary veins into the left atrium. In patients under one year of age the interatrial septum was not resected.

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Radical correction of Fallot's tetralogy by means of an approach through the right atrium and pulmonary artery without ventriculotomy (10 patients) or with minimal ventriculotomy (23 patients) was carried out in children whose ages ranged from 13 to 50 months and body weight from 7.9 to 15 kg. Lethality was 12%. The death of 3 patients was not connected with the peculiarities of the surgical tactics, the death of the fourth patient was due to insufficient experience in development of the surgical techniques of the first operations. The operative techniques applied are described in detail and the possibility of adequate correction of Fallot's tetralogy by it is proved principally. The late-term operative results are good in 10 patients. The first results of the suggested method of surgical correction of the anomaly are claimed by the authors to be promising, which allows them to continue its further elaboration.

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In the period between 1978 and 1989 operations were conducted on 56 patients with symptoms of the vascular ring (VR). The diagnosis of the disease was established on grounds of the clinical picture and the results of esophagography and angiocardiography. Double arch of the aorta (DAA) was recognized in 28 patients, anomalies of the right arch of the aorta (RAA) with VR formation in 25, and anomalies of the left arch of the aorta (LAA) in 3 patients. In 29 patients VR was combined with another congenital heart disease (CHD): with Fallot's tetralogy (FT) in 9 patients, with ventricular septal defect (VSD) in 4, and with coarctation of aorta (CA) in one patient. RAA anomalies were combined with FT in 6, with VSD in 4, and with CA in one patient. LAA anomalies were combined with VSD in 3 and with CA in one patient. VR separation was carried out through left anterior posterolateral thoracotomy in 91% of cases and through right thoracotomy in the remaining cases. In combination of VR with CHD the VR was corrected in one stage and then the CHD was corrected under extracorporeal circulation. Combination of RAA with FT allowed anastomosis with the use of a divided proximal stump of the left arch of the aorta to be established in 6 cases. In the group of 56 patients one died from hemorrhage during the operation. The late-term results are good.

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The authors studied the angiocardiographic picture in different variants of criss-cross atrioventricular connections in 24 patients. This is the greatest number of cases described in the world literature. The tactics of surgical treatment in various types of a criss-cross heart is substantiated on the grounds of analysis of hemodynamics and concomitant congenital heart diseases. Operation on a patient with a criss-cross heart should consist in correction of the concomitant anomalies and, if necessary, it should be supplemented by intracardiac correction of the blood flow (Mustard's operation). Fontana's operation is the only method of surgical treatment of patients with a complicated complex of anomalies.

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The article discusses the appraisal of the degree of pulmonary hypertension and surgery of a ventricular septal defect with pulmonary hypertension in 108 patients whose ages ranged from 8 months to 31 years. The hemodynamic and morphological manifestations of pulmonary hypertension were found to increase with age. The hemodynamic manifestations of the anomaly may be similar in different types of changes of the vascular wall. A sharply hypertrophied middle muscular coat may be the cause of residual high pressure after closure of the defect, but with gradual reduction in contrast to the stable increase in sclerosis of the vascular lumen. This predetermines lung biopsy as an obligatory method for verifying the severity of pulmonary hypertension, which together with the hemodynamic data allows the indications for closure of the ventricular septal defect to be determined.

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In the period of 1980 to February 1988, 60 patients aged 1 year 7 months to 23.5 years were operated on for concordant atrioventricular linkage and origin of the aorta and pulmonary artery from the right ventricle. Sixty-two heart specimens were examined to study the defect anatomy. The left ventricular outlet opened up in the subaortic cone (Type A defect) in 47 (78.3%) patients, in the common subaortic and subpulmonary cone (Type B) in 8 (13.4%) patients, in the subpulmonary cone (Type C) in 3 (5%) patients; 2 (3.3%) subjects exhibited nonconcomitant ventricular septal defect (Type D). Thirty-four (56%) patients had pulmonary stenosis. Concomitant heart diseases were found in 78%. Fifteen patients (25%) underwent radical operation after prior palliative interventions. The total fatal cases were 13.7% (out of 58 operated patients, 8 died) in the group of radically operated patients. The fatal outcomes were observed in Type A defect. The analysis showed that defect correction techniques, age, prior palliative interventions, presence or absence of pulmonary stenosis, concurrent heart diseases, and restrictive ventricular septal defect failed to affect the immediate result of surgeries. An inaccurate preoperative diagnosis and lack of surgical experiment were factors increasing the risk for correction from 1980 to 1986. In the 2 years, the death rates decreased from 17.9% (7 dead patients per 39 operated ones) to 5.3% (1 dead patient per 19 radically operated ones).

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The effect of the Blalock-Taussig operation on the growth of the pulmonary arteries (PA) in infants with tetralogy of Fallot (TF) was studied in late-term periods. Thirty-eight patients were examined, angiography and study of hemodynamics were conducted 31 to 144 months after the Blalock-Taussig operation. The obtained data provide evidence that as the result of the operation all segments of the hypoplastic PA grew to such a size that radical correction of the anomaly could be accomplished. In patients with an initially normal size of the PA growth of the vessel was not encountered. Iatrogenic complications and deformities of the PA occurred in 13.2% of cases. These complications developed most frequently (7.9%) in patients in whom the anastomosis was established in the first year of life. The degree of severity of the infundibular stenosis grew in all patients. Severe obstruction of outflow from the right ventricle developed in 10.5% of patients; in 7.9% of children it was completely obstructed. In view of that prolonged delay of the second stage of surgical treatment is inexpedient.

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The results of operations for correction of ventricular septal defects (VSD) in 97 young children (from 12 to 48 months of age) were evaluated. In 57 children the operation was conducted through a transventricular (group 1) and in 40--through a transatrial approach (group 2). The results of the operations in the two groups did not differ in essence. Essential differences were revealed in the cardiac index (CI) value and the requirements in cardiotonics: the CI was much lower and the doses of adrenalin higher in group 1 than in group 2. A more pronounced stability of hemodynamics on the first postoperative day and lesser possibility of the development of acute cardiac failure are important advantages of the transatrial approach and allow the authors to recommend it as the method of choice in closure of perimembranous VSD, particularly if they are complicated by high pulmonary hypertension.

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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.

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With the aim to establish quantitative characteristics of the pulmonary artery in order to use them in planning operations on congenital heart diseases, the data of morphometric investigations on the pulmonary artery has been studied in 83 hearts of newborns, children and mature persons died from causes not connected with any cardiovascular disorders. The regression equations are calculated; they reflect certain dependence between the quantitative characteristics of the pulmonary artery and the area of the body surface.

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Forty-three lung biopsies taken from 42 patients with various congenital heart malformations with high pulmonary hypertension (aged from 8 months to 34 years) were studied by morphological and morphometric methods. Changes in the pulmonary vessels were evaluated according to Heat-Edwards' classification and morphometric classification of Rabinovitch , et al. The morphometric data give some additional information on the growth and development of peripheral vessels, the degree of hypertrophy of tunica media muscularis, a decrease in the size of arteries. The demonstration of considerable reduction in the number of peripheral arteries by morphometry constitutes a serious prognostic sign of further progression of sclerotic changes in the pulmonary vessels even in the early correction of the heart malformation.

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