[Simultaneous correction of congenital heart defect, atrial fibrillation and coronary artery disease in a 62­year-old patient].

Long-term, natural course of congenital heart defect often results in secondary hemodynamic and functional disorders in adult life. In such cases, first clinical manifestations are generally due to age-related accompanying or secondary pathology rather than with the defect itself. In the presence of concomitant ischemic heart disease (IHD), leading manifestations may be those of angina, which mask manifestations of the heart defect and complicate the diagnosis. Not infrequently in practice, patients refuse surgery in a younger age, which is usually due to their satisfactory condition and an absence of complaints. However, the wait and see tactics is not always justified since over time, hemodynamic disturbances progress, complications of the defect may develop, coronary arteries lesions join with age, and their correction requires an integrated approach. The presented clinical case describes a possibility of successful one-stage surgical correction of an atrial septal defect, tricuspid valve regurgitation, atrial fibrillation, and IHD in a 62-old female patient.

[Stenting in obstructive pathology of pulmonary artery in patients with various forms of congenital heart failure].

A total of 124 stents were implanted to 91 patients with various forms of congenital heart failure for the resolution of obstruction of 111 segments in pulmonary arteries. All these patients were treated in A. N. Bakulev Research Centre of Cardiovascular Surgery in the period from 1994 to 2007. Analysis of observations in early and late postoperative periods indicates that the efficiency of stenting of obstructed pulmonary arteries was 96.4% and the restenosis rate in the follow-up period 3.9%. It is concluded that stenting of pulmonary arteries is an effective and safe method for the management of obstruction problems. In the majority of patients it allows to avoid surgical intervention on pulmonary arteries.

[Two-directional cavopulmonal anastomosis in the surgical treatment of congenital heart failure].

World-wide experience with the use of two-directional cavopulmonal anastomosis (TDCPA) for the surgical treatment of congenital heart failure (CHF) is summarized together with the results of analysis of immediate and late outcomes of this operation. It is concluded that TDCPA in certain complex forms of CHF and in patients at risk of poor outcome of Fontan surgery may be used as the final stage of hemodynamic correction.

[Causes of second operations after surgical treatment of congenital tricuspid valve abnormalities].

Main causes necessitating replacement of tricuspid valve (TV) prosthesis were calcinosis and infectious endocarditis of the bioprosthesis. Second operations after primary reconstructive surgery for the replacement of TV prosthesis were needed when medical indications did not fully justify the use of valve salving surgery by the Danielson's method and in case of procedural errors during surgery by the method of Carpentier in the initial period of mastering these approaches.

[One and a half ventricular correction in the surgical treatment of congenital heart failure].

Fifty four operations of one and a half ventricular correction were made for the treatment of congenital heart failure (CHF) in 1999-2006. Age of the patients varied from 1 yr 3 mo to 29 yr. Indications to the use of this method are discussed along with analysis of its role in the surgical treatment of CHF. Two-directional cavopulmonal anastomosis in certain complex forms of CHF reduced volume load on the right ventricle and thereby promoted success of its radical correction. One and a half ventricular correction involved the hypoplastic and functionally compromised right ventricle and pulmonary circulation and therefore created more favourable conditions for circulation.

[The problem of follow-up period after Fontan operation].

The study was designed to analyse the frequency and structure of complications after Fontan operation depending on the form of congenital heart failure (CHF), method of surgery, patients' clinical and hemodynamic characteristics in the preoperative period. Results of surgical and endovascular treatment of different complications and strategies of conservative therapy are discussed. The most common complications of Fontan surgery were circulatory failure, disturbances of heart rhythm, and protein-losing enteropathy. In the majority of patients, they developed in the first 2-3 years after surgery.

[Fontan right heart bypass as a corrective procedure for congenital heart disease with single-ventricle hemodynamics].

Treatment of patients with complex cyanotic congenital heart disease is one of the most difficult problems in heart surgery. In a number of cases, when normal introcardiac hemodynamics is impossible to reestablish, the operation of choice consists in hemodynamic correction, which implies excluding the right heart from pulmonary circulation by rerouting venous blood directly to the lungs. The analysis of the outcomes of the operation, performed in various modification, shows that the most optimal method to date is extracardial bypass of the right heart with a valveless tubular prosthesis. This method is associated with a relatively rare early and long-term complications. Improvement of outcomes can be achieved mainly via dividing the correction procedure into stages, including bidirectional cavapulmanal anastomosis, and via thorough selection and preoperative preparation of patients.

Comparative assessment of Fontan operation in modifications of atriopulmonary and total cavopulmonary anastomoses.

OBJECTIVES: The optimal technique of the Fontan operation remains disputable. This investigation was aimed at the comparison of atriopulmonary and total cavopulmonary anastomoses. METHODS: The results of 81 operations of total cavopulmonary and 69 operations of atriopulmonary anastomosis, performed from 1983 to 1995 were analysed. A control study was carried out 1-10 (3.7 + 0.2) years after the operation in 80 patients (36 after total cavopulmonary and 44 after atriopulmonary anastomoses). 70 patients were studied several times. Cardiac catheterization was done in 70 patients. In 78 patients central hemodynamical indices were studied during exercise. RESULTS: Preoperative hemodynamical indices were not reliably different in the two groups. Early mortality after total cavopulmonary anastomosis was 15%, after atriopulmonary anastomosis 23%. The highest mortality was seen when the criteria of Choussat et al. (Choussat et al. Pediatric Cardiology. Edinburgh: 1977:559-566) were surpassed. In the early postoperative period after total cavopulmonary anastomosis circulatory insufficiency was less marked, transsudation from pleural cavities was reliably lower (15.3 + 1.2 versus 25.5 + 1.8 ml/kg/day, P < 0.01). Arrhythmias were more common after total cavopulmonary anastomosis (18.5 versus 12%). Late mortality after total cavopulmonary anastomosis was 6%, after atriopulmonary anastomosis 12%. 82% of patients after atriopulmonary anastomosis and 81% after total cavopulmonary anastomosis were in NYHA classes 1 and 2.7% of patients after total cavopulmonary anastomosis and 11% after atriopulmonary anastomosis were reoperated. Physical tolerance rose stepwise in both groups and by the third post-operative year reached 75% of normal level. At this time we saw the most optimal hemodynamical indices during exercise. However, the best hemodynamics during exercise were seen after atriopulmonary anastomosis. CONCLUSIONS: Total cavopulmonary anastomosis is accompanied by lower mortality rate and a more favourable course of early postoperative period. However, the best long-term functional results are obtained after atriopulmonary anastomosis.

Surgical reinterventions after modified Fontan operations.

BACKGROUND: Surgical reinterventions after Fontan operations are still associated with high mortality. This investigation aims to summarize our experience with such repeated operations and to assess their efficacy. METHODS: In 1983 through 1995 we performed 162 different modifications of Fontan operation. Repeated interventions were needed in 15 cases (9%). An analysis of all these cases is presented. RESULTS: Repeated operation consisted of the closure of residual interatrial communication (3 patients), atrioseptostomy (4), subaortic stenosis resection (1), pulmonary balloon valvuloplasty (1), embolization of residual right ventricular-pulmonary arterial communication (1), pericardectomy (2), pleurectomy (1), pacemaker implantation (1), and takedown of the Fontan operation (1). The results of operation were judged as good in 6 cases (40%) and as satisfactory in 5 (33%); 4 patients (27%) died. CONCLUSIONS: Repeated interventions aimed at the elimination of technical errors of the Fontan operation are successful in most cases. Atrial septostomy gives good results in the absence of anatomic causes of cardiac failure such as subaortic obstruction and atrioventricular valve insufficiency.

[Catheter therapy of congenital cardiovascular defects]. / Kateternaia terapiia vrozhdennykh porokov serdtsa i sosudov.

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.

[Attempt at allo-aortal conduits in the correction of congenital heart defects]. / Pervyi opyt ispol'zovaniia alloaortal'nykh konduitov pri korrektsii vrozhdennykh porokov serdtsa.

In the period September 1990 to April 1993, an allo-aortic conduit was used in 16 patients during reconstruction of the right ventricular outflow duct. The patients age ranged from 2 years 8 months to 14 years, their body weight was 11.8 to 26 kg. Seven patients were diagnosed as having pulmonary atresia with ventricular septal defect, 4 had Fallot's tetrad and 5 transposition of great vessels with pulmonary stenosis and ventricular septal defect. The allo-aortic conduit made by the procedure developed at the A. N. Bakulev Institute of Cardiovascular Surgery, Russian Academy of Medical Sciences, was used in all cases to reconstruct the right ventricular outflow duct. The allografts were sterilized in a solution containing culture medium RPMI-1640 and mixture of antibiotics. After sterilization, the prostheses were frozen in liquid nitrogen vapours and kept at -150 to -160 degrees C till their clinical application. In the immediate postoperative period, 2 patients died, which amounted to 13%. Two deaths were caused by excessive bleeding, but in one case the implanted conduit wall bled. The residual right ventricle-pulmonary artery gradient was no more than 23 +/- 17 mm Hg. A discharge echocardiographic examination of patients found no valvular dysfunction of the implanted conduits on days 12 to 26 days after surgery. The findings suggest that the allo-aortic conduits prepared at the A. N. Bakulev Institute of Cardiovascular Surgery, Russian Academy of Medical Sciences, can be regarded as the prostheses of choice in the surgery of congenital cardiac malformations.

[Immediate and late results of transluminal balloon angioplasty of Blalock-Taussig stenosing anastomosis in cyanotic congenital heart defects]. / Neposredstvennye i otdalennye rezul'taty transliuminal'noi ballonnoi angioplastiki stenozirovannogo anastomoza Bleloka-Taussig pri tsianoticheskikh vrozhdennykh porokakh serdtsa.

Transluminal balloon angioplasty of Blalock-Taussig's stenotic anastomosis was performed in 37 patients with various congenital heart diseases in 1989 to April 1993. The patients' age ranged from 3 to 16 years (mean 8.4 +/- 0.5 years). Arterial blood oxygen saturation varied from 38 to 74% (mean 62.4 +/- 1.5%). Hemoglobin amounted to 14 to 28.4 g% (mean 19.0 +/- 8.4 g%). Phonocardiography recorded no or low-amplitude systolic-diastolic anastomosis murmur in points IV-V in all patients. After balloon anastomosis dilatation, arterial blood oxygen saturation increased, on an average, from 62.4 +/- 1.5% to 81.2 +/- 1.2%. Anastomosis angiometry performed prior to and following balloon dilatation showed its significant dilatation (on an average, from 3.39 +/- 0.19 to 5.26 +/- 0.24 mm). There was a distinct systolic-diastolic murmur of anastomosis on the PhCG. Post-balloon-dilatation complications occurred only in 3 cases: femoral thrombosis in 2 patients and pulmonary edema in 1. The late results were studied in 18 patients at months 2 to 40. None of them showed deterioration of clinical results after balloon dilatation. Eight patients were radically operated on. The best results were achieved in patients with discrete anastomosis stenoses and when balloons whose diameter corresponded to that of the subclavian artery and equal to 0.92-1.2 mm were used. A good effect following balloon angioplasty was observed in 24 (64.9%), satisfactory and poor results were found in 12 (32.4%) and 1 (2.7%), respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

[Pulmonary sling in a patient with anomalous intrathoracic heart displacement (diagnosis and surgical treatment)]. / Lëgochnaia petlia u bolnogo s anomaliei vnutrigrudnogo raspolozheniia serdtsa (diagnostika i khirurgicheskoe lechenie).

The pulmonary sling is the origin of the left pulmonary artery from the right one, this is a rare abnormality giving a marked clinical picture. The paper presents a case of solely rare combination of the pulmonary of the pulmonary sling with abnormal cardiac placement--dextroversion. A three-year-old child underwent explorative thoracotomy for erroneously diagnosed mediastinal cyst. The thoracotomy revealed a suspected pulmonary sling. To make an accurate diagnosis, a comprehensive study (chest X-ray with esophageal contrasting, tracheobronchography, echocardiography, digital subtraction angiography) was performed. Echocardiography clearly showed the absence of bifurcation of the pulmonary trunk and the origin of the left pulmonary artery from the right one proved to be most informative, as evidenced by colour Doppler mapping. The paper deals with the specific features of surgical treatment of this pathology (the patient was successfully operated on through lateral sternotomy by using extracorporeal circulation). The good functional result of the operation was confirmed by echocardiography and angiography.

[The percutaneous transluminal balloon valvuloplasty of valvular stenosis of the pulmonary artery in treating tetralogy of Fallot as an alternative to the surgery of applying a systemic-pulmonary anastomosis]. / Chreskozhnaia transliuminal'naia ballonnaia val'vuloplastika klapannogo stenoza legochnoi arterii pri lechenii tetrady Fallo kak al'ternativa operatsii nalozheniia sistemno-legochnogo anastomoza.

From January 1991 to May 1992 transluminal balloon valvuloplasty [correction of vulvoplasty] of severe valvular stenosis of the pulmonary artery was performed in 14 patients with Fallot's tetrad at the Institute of Cardiovascular Surgery named after A. N. Bakulev. Five of them had pulmonary artery hypoplasia, 2 had stenosis and hypofunction of the systemic-pulmonary anastomosis applied by the Blalock-Taussig, which made them perform balloon angioplasty of the narrowed distal portion of the anastomosis. The patients' age ranged from 11 months to 14 years (mean age 6.8 +/- 1.1 years). Arterial blood oxygen saturation varied from 51 to 72% (mean 69.2 +/- 2.4%). The diameter of a valvular ring was 8-13 mm. The diameter of a balloon catheter exceeded that of the valvular ring by 20-25%. After dilation, arterial blood oxygen saturation increased from 69.86 +/- 2.5 to 85.1 +/- 1.2%. The angiometry performed after pulmonary artery valvuloplasty in patients with pulmonary hypoplasia showed an increase in sizes of all pulmonary arterial segments on an average by 22.5 +/- 4.1%. Later on 5 patients were radically operated on. Thus, with the ultimate goal of preparation of patients with severe Fallot's tetrad for radical surgery, transluminal balloon valvuloplasty in some patients with severe valvular stenosis of the pulmonary artery may be alternative to a systemic-pulmonary anastomosing operation.

[The immediate results of the radical correction of the tetralogy of Fallot in adults]. / Neposredstvennye rezul'taty radikal'noi korrektsii tetrady Fallo u vzroslykh.

In the period from 1982 to 1991, 74 radical corrections of tetralogy of Fallot were carried out on patients 18 to 40 years of age. In 37 of them the correction was preceded by various palliative interventions. The course of the disease was distinguished by a lesser frequency of episodes with dyspnea and marked cyanosis than in the younger age group (up to 18 years). The radical correction of the anomaly included only infundibulectomy in 7% of cases, infundibulectomy and plastic distention of the outflow tract of the right ventricle in 28%, while in 65% of cases reconstruction of the pulmonary trunk or its branches was conducted in addition. The hospital mortality was 9.3% and did not differ essentially from that in the younger age group. Mortality was somewhat higher in primary correction of tetralogy of Fallot than after earlier performed palliative interventions (10.8 and 8%, respectively). It is concluded that the patients' age is not a contraindication for radical correction of tetralogy of Fallot.

[Bidirectional cava-pulmonary anastomosis in complex congenital heart defects]. / Dvunapravlennyi kavapul'monal'nyi anastomoz pri slozhnykh vrozhdennykh porokakh serdtsa.

An operation for bidirectional cava-pulmonary anastomosis was performed in 11 patients. Anatomical correction could not be made due to the multiple pattern and complexity of the malformation. None of them was, however, an ideal candidate for hemodynamic correction either. The authors took into account not only anatomical and hemodynamic contraindications for Fontana's operation, but the latter was not performed when the patient's status was grave (severe hypoxemia). The operation was made under extracorporeal circulation and hypothermia. In addition to bidirectional cava-pulmonary anastomosis, dilation of the intraatrial junction, pulmonary artery plasty, etc. were conducted in some cases. Bilateral bidirectional cava-pulmonary anastomosis was performed when an accessory vena cava superior was present. Nine patients benefited from the operation. There was a significant health improvement and a substantial increase in arterial blood oxygen saturation. Four patients underwent a repeated examination one year after surgery. The anastomosis was found to function well.

[The transluminal balloon angioplasty of a stenosed Blalock-Taussig systemic-pulmonary anastomosis in congenital heart defects of the blue type]. / Transliuminal'naia ballonnaia angioplastika stenozirovannogo sistemno-legochnogo anastomoza po Bleloku--Taussig pri vrozhdennykh porokakh serdtsa sinego tipa.

Dilatation of a stenosed Blalock-Taussig anastomosis was performed in 15 patients with blue-type congenital heart diseases at the Bakulev Institute of Cardiovascular Surgery, AMS USSR, from March, 1989 to May, 1991. Seven of these patients had a severe form of Fallot's tetralogy, 4--double origin of the great vessels from the right ventricle with stenosis of the pulmonary artery, 2--atresia of the right atrioventricular orifice with stenosis of the pulmonary artery, 1--atresia of the right atrioventricular orifice with atresia of the pulmonary artery, and 1 patient had corrected transposition of the great vessels with stenosis of the pulmonary artery. The patients' ages ranged from 4 to 13 years (9.45 +/- 8.1 years on the average). Saturation of arterial blood with oxygen varied from 38 to 77% (64.2 +/- 2.9% on the average). A low-amplitude to-and-fro sound of the anastomosis was either recorded or absent on the phonocardiogram. The diameter of the balloons used ranged from 4 to 13 mm. Inflation of the balloon lasted from 5 to 60 sec. Dilatation was conducted 3 to 9 times. After dilatation saturation of arterial blood with oxygen increased to 81 +/- 2.7% on the average. A clearly defined to-and-fro murmur of the anastomosis was recorded on the phonocardiogram. Angiometry of the anastomosis performed before and after dilatation showed it to be widened considerably from 3.5 +/- 0.2 mm to 5.7 +/- 0.4 mm on the average. One patient underwent a radical operation 8 months after balloon dilatation.