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The history of surgical treatment, clinical features, and main variants of correction of Taussig-Bing anomaly in children during the first year of life is briefly reviewed including original data obtained in the Department of Urgent Surgery of newborn and first-year infants, A. N. Bakulev Research Centre of Cardiovascular Surgery for the period of 2000-2006.

The article presents the results of urgent surgical correction of ventricular septal defects (VSDs) and a range of associated congenital heart diseases (CHDs) in 123 children of the first 6 months of life, hospitalized between 1988 and 2002 in a severe or critical condition. In 102 patients the progression of clinical symptoms was caused by a big single VSD and in 21 patients--by multiple VSDs. 70 patients had significant associated CHDs. Significant extracardiac anomalies were revealed in 75 patients. The condition of 55.3% of children had deteriorated due to pneumonia. Correction of defects with cardiopulmonary bypass was performed after urgent examination and intensive therapy. Hemodynamically significant VSD were closed in 117 (95.2%) patients. Two patients were reoperatied due to the presence of residual VSD. Partial or full simultaneous correction of associated CHDs was performed in 68 patients. 93.5% of patients with isolated VSDs survived the operation. The survival rate of patients with associated CHDs and extracardiac anomalies was 81.5%. Postoperative mortality risk factors were found to be critical condition at hospitalization and preoperative activity of infectious process (P < 0.01 and < 0.05, respectively). Accumulation of experience and introduction of a special approach allowed an 8-time decrease of the hospital mortality, which has not exceeded 3.4% within last 3 years. Good and excellent follow-up results were obtained in 98% of the survived patients within 10 to 67 months after operation. Precise diagnostics of associated CHDs and extracardiac anomalies, intensive preoperative treatment and weighed estimation of indications and risk factors allow significant improvement in the results of primary correction of VSD and accompanying cardiovascular anomalies in children of the first months of life in a heavy and critical condition upon admission.

The paper discusses the influence of lung anomalies on the progress of congenital heart diseases (CHD) in infants, as well as diagnostic value of radiography and ultraspeed computed tomography (CT). The four-year experience of the authors includes preoperative examination of 178 infants with CHD (mean age 5.8 +/- 0.6 months, mean weight 5.2 +/- 0.3 kg). Lung anomalies were determined in 24 patients (13.5%) on plain films and in 85 patients (47.8%)--by means of CT. The stenosis of main bronchi in combination with lobar emphysema and lung hypoplasia were found to be the most frequent and severe predictors of respiratory disorders. The obtained data suggest that CT examination may be recommended in infants with CHD for precise diagnostics of associated lung anomalies and further prevention or early management of respiratory complications.

The efficiency of peritoneal dialysis (PD) was evaluated in newborns and infants with multiple organ dysfunction (MOD) after cardiosurgery. In 1996-2001 at A. N. Bakulev Cardiovascular Surgery Center, Russian Academy of Medical Sciences, PD was used as permanent renal replacement therapy (RRT) in 19 newborns and infants aged under 1 year after radical correction of congenital heart diseases. The mean age of infants was 6.2 +/- 4.3 months, body weight 6.1 +/- 1.7 kg. Radical correction of congenital heart diseases was carried out in all patients. The immediate postoperative period was complicated by MOD in all patients. Pronounced cardiac, respiratory, and renal insufficiency was observed in all cases. The main indication for the beginning of PD was oligouria, hypervolemia, edematous syndrome with progressive cardiorespiratory insufficiency. Multiple-modality intensive care including PD resulted in positive changes in the clinical status of infants by days 4-5 of PD: hemodynamic values, gaseous exchange function of the lungs, and laboratory values improved. Hence, PD as an RRT method in newborns and infants subjected to radical cardiosurgery proved to be effective in the complex of intensive care measures for MOD. This sufficiently simple method, requiring no expensive equipment, adequately corrects hyperhydration and hypervolemia, metabolic and electrolyte disorders, and other MOD symptoms in newborns and infants.

Material of intrasurgical biopsies of the right ventricular (RV) myocardium of 28 patients with Fallot's tetrad was studied with light and electron microscopy. RV cardiomyocytes were hypertrophic and showed signs of growth and differentiation. Myofibrillogenesis intensity directly correlated with the pressure gradient between RV and pulmonary artery. At the same time there was a structural alteration of mitochondria and formation of a specialized population of intermyofibrillar mitochondria. Continuing myofibrillogenesis in cardiomyocytes may be one of the causes of decreased myocardial contractility.

Modified ultrafiltration (MUF) was used in radical correction of complex congenital heart disease in 61 newborns and infants. The children were divided into 2 groups: group 1, 46 patients subjected to MUF, and group 2, 15 children operated on without MUF. Hemodynamic, hematological, biochemical, and immunological parameters were monitored. MUF in the newborns and infants helped adequately correct the hemohydrobalance due to ultrafiltration in the patient and hemoconcentration of the remaining volume in artificial ventilation device; it normalized the hematocrit values without additional infusion of donor blood. Elimination of inflammation mediators and cytokines recommends MUF for the treatment of newborns and infants.

The authors analyze the experience gained in catheter operations performed at their Department over the recent 28 years. A total of 500 balloon (Rashkind) and 35 knife (Park) atrioseptostomies were performed in critical patients with different "blue" congenital diseases over this period. An immediate hemodynamic effect at the operation table was attained in 95% of patients. Transluminal balloon valvuloplasty (TLBVP) was performed in 372 patients with valvular stenosis of the pulmonary artery. The results of this operation depend on the patient's age, anatomy of the defect, and correct choice of the diameter of balloon catheter. This intervention is particularly effective in patients aged under 1. In patients with combined valvular and infundibular stenosis the results of TLBVP depend on the severity of stenosis and age of patient. An attempt at TLBVP of congenital aortal stenosis was undertaken in 67 patients. Valvuloplasty was carried out in 57 patients. The operation was effective in 13 (62%) out of 21 patients aged under 1. The mortality in this group was 5.5%. Balloon valvuloplasty of the pulmonary artery was carried out in 71 patients with cyanotic congenital heart diseases. The intervention helped eliminate the critical state, rise the systolic pressure in the pulmonary artery, improve blood saturation with oxygen, and evade the operation for creation of a systemic-pulmonary anastomosis. Isolated and postoperative stenoses of pulmonary arteries were removed in 65 patients. Six Johnson and Johnson stents were effectively implanted to 3 patients with rigid postoperative stenoses; in 32 patients transluminal balloon angioplasty (TLBAP) for coarctation and recoarctation of the aorta brought about a satisfactory immediate hemodynamic effect. TLBAP of Blalock-Taussig's stenosed anastomosis were performed in 60 patients with various cyanotic congenital heart diseases. Its results were good in 39 (65%) patients, satisfactory in 19 (31.7%), and unsatisfactory in only 2 (3%) cases. Of the novel endocardial interventions, dilatation of the conduit following Rastrelli's operation, creation of a defect of the atrial septum after Fontain's operation, and embolization of the coronary-cardiac fistulas and of patent ductus arteriosus were carried out. This review demonstrates wide use of endocardial surgery methods in the treatment of some congenital heart diseases; in many cases such treatment may be an alternative to surgical interventions.

In the period March 1992 to January 1993, ten patients with transposition of great arteries (TGV) whose age ranged from 9 days to 8 months and body weight 3.2 to 7.2 kg were operated on using the arterial switch. A single-stage anatomic correction was made in 2 patients with patent arterial duct, 4 with ventricular septal defect (VSD), one with multiple VSD. Three patients with common TGA underwent correction in 2 stages: arterial switch followed prior constriction of the pulmonary artery switch followed prior constriction of the pulmonary artery and subclavian-pulmonary anastomosis. In all cases of single VSD, it was closed with a flap made from the Gore-Tex material. In the whole series of operations, 2 patients died: one being on the operating table due to anesthesiological errors, the other due to coronary blood flow disorder 3 hours after surgery. Six of the 8 discharged patients were followed 3-8 months after surgery. Two patients needed digitalis. No sign of myocardial ischemia was detected in antibody. All the patients were found to have normal left ventricular pump function. These surgeries are the first in our country and so our little experience may be of interest for national specialists.

Analysis of electrocardiograms in 55 children who underwent operation for Fallot's tetralogy at early age showed that the frequency and severity of postoperative disorders of rhythm and conductivity were determined by the degree of trauma inflicted by the used surgical techniques. Data obtained in the early and late-term postoperative periods confirm the expediency of performing surgical correction of the anomaly as early as possible by means of surgical methods which cause little injury. The results of the study are recommended for use in choosing the surgical tactics, for functional appraisal of the operative results.

Main trace posthyperbaric and postdecompression responses to respiration, circulation and oxygen profiles, dynamics of their reduction in 99 aquanauts at pressure of 46 kgs/cm2 (0.46 mPa) during 14-30 day period have been studied. The respiratory changes were found to be characterized by: ventilatory function disorders which followed the obstructive-restrictive pattern; discoordination of ventilation regulation and slowing down of respiratory gas transport through aerohematic barrier. The hemodynamic shifts have been manifested as cardiac rhythm instability, heart pump function followed by decreased cardiac contractions on exercise exposures. The economy and efficiency of body oxygen profiles in a respiration loop become lower than that in hemodynamic loop. The rate of the alveolar oxygen supply is decreased to the levels lower than the rates of oxygen supply by arterial and mixed venous blood. The long-term functional rearrangement of respiratory gas transport followed by the phase changes combined with the specific and nonspecific reactions persisting over a period of 30-60 days and more longer after decompression, is existed.

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.

Parameters which characterize lung volumes, absolute and relative flow rates, breathing reserve, were found unchanged with increasing age and record of active dive service, and in some deep-divers these parameters were growing up. This was disclosed due to comparative analysis of lung ventilatory function changes in divers through certain amount of years of professional occupation. At the same time a significant decrease in small to middle calibre bronchi patency was noted. In 30% of experienced deep-divers the mean maximal forced expiratory flow at 75% forced vital capacity (FEF75) decreased to 39% of predicted value at one-second forced expiratory volume (FEV1) being normal. Maximal expiratory flow at 50% forced vital capacity (FEF50) were in inferior normal limit (52% of predicted value).

Two-dimensional echocardiography was conducted in 24 patients, aged from 14 days to 12 months, suffering from coarctation of the aorta (CA) with the left ventricular (LV) ejection fraction (EF) less than 30%. The symptom of LV fibroelastosis was found in only 16.7% of patients. In the first 7 days after correction of CA the LV end-systolic volume reduced by 20% and the EF increased by 1.5 times, which may be explained by removal of LV post-loading. The values of the LV pumping function improved in the late periods due to restoration of myocardial contractility. In infants operated on in the first 3 months of life the dynamics was more favorable, the EF reached 68 +/- 3.8% while in those operated on after the age of 3 months it reached only 50.8 +/- 2.5% (p < 0.05). The authors discuss problems of compensatory hypertrophy and myocardial contractile capacity as the main mechanisms responsible for LV pumping function in CA.

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.

The results are presented which confirm applicability of the Valsalva test as a functional exercise for the cardiovascular system while studying changes of the circulation regulation under high ambient pressure. Methods of transthoracic tetrapolar impedance plethysmography, electro- and intervalocardiography and mean arterial pressure measurement by means of the oscillometric servosystem are shown as adequate and highly informative under these conditions.

It is found out that when reaching critical density of gas medium (20.4-24.0 kg/m3), the relative power of oscillations on the "flow-volume" curves and pneumotachograms sharply increases. At the same time the maximal rate of exhale, rates of expiratory flows in large bronchi decrease significantly and the rates of expiratory flows in bronchi of middle and small calibers reduce to zero. Within the range of densities of different gas medium (from 1.3 to 32.7 kg/m3) the changes in the relative power of oscillations of respiratory flows correlate well with the changes in permeability of upper respiratory tracts (r = -0.932 +/- 0.054). A regression equation describing this dependence is obtained. A detailed description is given to symptoms of high-density breathing syndrome: a decrease in rates of gas flows in tracheobronchial tree, early development of dynamic compression of airway, tremor of breathing muscles and appearance of respiratory flow oscillations, pronounced dyspnea and hypoventilation of alveoli. A hypothetic scheme of its development is presented. The functional significance of respiratory flow oscillations as a mechanism improving gas exchange in gas media with extremely high density is under discussion.

Evaluation of changes in respiration circulation and respiratory gas transport has shown that long-term influence of hyperbaria gives rise to phase changes and promotes a shift to a new level of the functional state of aquanauts. It is established that the postdecompression syndrome is characterized by the lability of main parameters of breathing and circulation, depression of gas exchange, disorders of bronchial permeability, relaxation of the cardiac pump function, decrease of physical workability against the background of inhibition of the adrenal cortex and weakening of the non-specific immunoresistance. Examination of 145 deep divers has revealed regularities of readaptation dynamics and has permitted a conclusion on an increase of unfavourable tendencies in the ventilatory pulmonary function and cardiac pump function under repeated effect of saturated diving. The normative values of external respiration are worked out as applied to divers. Some new approaches to the technology of medical providing of deep divings and correction of non-specific professional pathology of acquanauts are physiologically substantiated proceeding from tendencies of shifts in the periods between divings and diving norms.

The dynamics of forced inhale (I series) and exhale (II series) parameters with additional external artificial resistive load was studied under normobaric conditions. The artificial resistance to breathing increased stepwise using removable diaphragms with sequential decrease of hole diameter from 25, 17, 13, 9, 7.5, 4.5 to 3 mm. While studying forced inhale the diaphragms were set up at Fleish pipe airflow input. In the case of forced inhale the diaphragms were set up at the pipe output. A phenomenon is revealed which consists in appearance of respiratory flow oscillations on the "flow-volume" curves during forced breathing with an increase of resistive load. Frequency maxima of the oscillations were located within the range of 6-15 Hz. The possible mechanisms for appearance of respiratory muscle tremor and respiratory flow oscillations are under discussion.

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.

The data presented in the recent literature and the authors' results allow formulating basic positions of the conception on the phase adaptation to the increased density of gas mixtures (nitrogen, helium) with complete saturation. Each of the three phases is shown to depend on the intensity of hyperbaric stress and the organism state. A hypothesis is advanced that formation of the system functional and then a structural trace in possible in response to the repeated influences of prolonged hyperbarism. Cross deadaptation and decompensation of the systems which endure the highest functional load under hyperbarism are shown to be possible. A general syndrome of high pressures which unite nerve, respiratory, circulatory and metabolic components is characterized. Phases of the readaptation period are determined. Changes in main systems depending on periods after decompression are described.

The article generalizes the experience in surgical treatment of Fallot's tetrad in 20 infants. Radical correction of the anomaly was carried out under extracorporeal circulation and deep hypothermia with reduced volume rate of perfusion. Plastics of the conus arteriosus alone was performed in 6 patients, transanulus and the pulmonary trunk in 4 patients. Two patients died, the hospital lethality was 10%. A clinical effect was produced in all the other patients. The relation of systolic pressure in the left ventricle to that in the right ventricle was 0.56 +/- 0.1, the systolic pressure gradient between the right ventricle and the pulmonary artery was 18 +/- 4.2 mm Hg. Various degree of regurgitation at the pulmonary valve was revealed after the operation in all patients. It was maximum (34 +/- 10%) in transanulus plastics.

The work analyses the results of repeated clinical and intracardiac examination of 13 patients with tetralogy of Fallot, conducted at the age of 23.1 +/- 9.9 and 42.3 +/- 13.1 months. The severity of the clinical condition grew markedly during natural course of tetralogy of Fallot (TF): the SaO2 value reduced from 71.8 +/- 7.2 to 65.0 +/- 8.0% and the blood hemoglobin level grew from 165 +/- 28 to 194 +/- 30 g/l. The data presented are evidence that in TF following a natural course a distinct tendency is displayed to growth of end diastolic volume of both ventricles in relation to the normal value and their dysfunction progresses in the form of reduction of the ejection fraction and increase of right-ventricular diastolic "rigidity". The results of the present work may be considered a substantiation of the tactics of early TF correction in infants and young children before the development of serious morphological changes and functional disorders.

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.

A late congenital anomaly--double arch of the aorta (DAA)--was combined with Fallot's tetrad (FT) in 4 patients whose ages ranged from 21 months to 11 years. The diagnosis of DAA and FT was established before the operation in 3 patients, in one patient DAA was discovered during the operation. In three children DAA was manifested clinically by dysphagia and stridor. A palliative operation (FT) was carried out through a left posterolateral approach along the third intercostal space. One-stage separation of the vascular ring by cutting the smaller left arch of the aorta at its junction with the descending aorta and formation of anastomosis between this arch and the left pulmonary artery by the end to side method were conducted. In deficient length of the cut arch of the aorta the left subclavian artery was divided; in equal diameter of both arches the lumen of the arch was reduced to 0.5 cm with stitches before formation of the anastomosis so as to prevent hyperfunction of the shunt. In the case in which DAA was not recognized before the operation, an attempt to dilate the hypoplastic segment of the left arch led to rupture of the thinned wall and the patient died from hemorrhage. The advantages of one- and two-stage radical correction of both developmental anomalies (DAA and FT) are still debatable.

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.

The administration of nicotinic acid and aspirin in a sufficient measure to prevent reocclusion in complete recovery of the arterial blood flow. Under conditions of non-recovered blood flow the anti-thrombotic therapy is hopeless. So its use is restricted mainly by cases of a partial recovery of the blood circulation in main vessels. A continuous intravenous infusion of heparin, rheopolyglukin, nicotinic acid and trental is believed to be the most rational method of the postoperative anti-thrombotic therapy.

The main principle of the antithrombotic therapy in acute thrombosis of major veins is to eliminate the thrombotic state of the homeostasis system by means of a simultaneous correction of pathological changes in all the three main links of this system. The authors believe the joint application of anticoagulants, antiaggregants and fibrinolysis activators to be necessary for the elimination of the state mentioned. The doses should be chosen in order to normalize the parameters of hemocoagulation, fibrinolysis and aggregation of thrombocytes.

Statistical background characteristics and impulse activity of some fibres of the optical tract evoked by light flashes were compared before and after stimulation of the preoptic hypothalamic region in listenin-immobilized rabbits. It was shown that the influence of the high frequency preoptical hypothalamus stimulation correlates with the pattern of responses to light flashes observed when recording from the optical tract fibres. In most cases the preoptical hypothalamus stimulation caused an increase of the background activity and facilitation of responses to light flashes. Phasic response to a light flash with short latency (42 ms), consisting of a long train of pulses and tonic responses with long latency (155 ms) were facilitated to a greater extent. The facilitation of short-latent (35 ms) phasic responses to a light flash consisting of a short train of phases after the preoptical hypothalamus stimulation was not clearly pronounced or was inhibited.

Analysis of the results of using fibrinolysis activators in 196 patients with massive embolism of the pulmonary arteries was undertaken to choose the optimum method of thrombolytic therapy. High doses of fibrinolysis activators (from 5,000,000 to 7,000,000 U) were used in 127 cases and low doses (from 125,000 to 3,000,000 U) in combination with heparin and disaggregating agents in the remaining 69 cases. A cava-filter was implanted in 161 patients before beginning treatment. Obvious advantages of thrombolytic therapy of massive pulmonary embolism with high doses of fibrinolysis activators were revealed in the process of treatment. They consist in quicker restoration of continuity of the pulmonary arteries, which leads to rapid removal of hypertension in pulmonary circulation and reduction of the number of deaths from acute cardiopulmonary failure. Treatment with such doses does not lead to an increase of the number of hemorrhagic complications, which makes possible surgical prevention of recurrent pulmonary embolism by cava-filter implantation.

The system of the haemostasis in acute occlusion of the main arteries is characterized by a combination of hypercoagulation, arrest of fibrinolysis, and increase of the adhesive-aggregation function of the formed elements of the blood. The age, aetiology, localisation and the character of arterial occlusion, the degree, and the duration of ischaemia of the tissues influence the degree of the above disorders. The above factors must be taken into consideration in choosing methods of the antithrombotic therapy of thromboembolic lesions of the main arteries. The main principle of such therapy is simultaneous corrections of the disorders of haemocoagulation, fibrinolysis, adhesion, and aggregation of the formed elements of the blood by the combined use of anticoagulants, fibrinolytic and antiaggregation drugs.