[Primary delayed growth of the right and left atrial myxoma]. / Pervichno otsrochennyi vo vremeni rost miksom pravogo i levogo predserdii.

There is more than 58-year experience of surgical treatment of patients with intracardiac myxomas at the Petrovsky National Research Center of Surgery. Primary delayed growth of the right and left atrial myxoma after 21 years and 5 months was observed only in 1 (0.36%) patient. Transthoracic echocardiography was used for diagnosis of tumors. Tumors were totally resected including adjacent parts of myocardium or subendocardial tissue with subsequent cautery. In-hospital mortality was absent. There were no recurrent myxomas throughout subsequent 10-year follow-up.

[Diagnostics and surgical tactics for papillary fibroelastomas of the heart]. / Diagnostika i khirurgicheskaya taktika pri papillyarnykh fibroelastomakh serdtsa.

AIM: To investigate clinical, diagnostic and surgical aspects of papillary fibroelastomas of the heart. MATERIAL AND METHODS: Clinical, diagnostic and surgical aspects of papillary fibroelastomas of the heart were analyzed in 12 patients. RESULTS: Diagnosis was obtained using two-dimensional echocardiography and transesophageal echocardiography or computed tomography. Surgical treatment involved isolated excision of the tumor or combined with valve repair or replacement in case of valvular involvement. Hospital mortality was absent. CONCLUSION: Successful surgical treatment implies urgency, optimal exposition under hypothermic perfusion and pharmaco-cold cardioplegia, simple excision of the tumors and correction of comorbidities if it is necessary.

[Solid lesions of heart valves].

AIM: To analyze clinical-diagnostic and surgical aspects of cardiac valve solid lesions as well as long-term results of surgical treatment. MATERIAL AND METHODS: Clinical, diagnostic and surgical aspects of treatment of 51 patients with tumorous and pseudotumorous lesions of cardiac valves were analyzed. RESULTS AND DISCUSSION: Hospital complications were observed in 17.7% of patients. Mortality rate was 3.9%. 1- and 28-year overall survival was 100% and 94.1% respectively. Quality of life was good, satisfactory and unsatisfactory in 73.5%, 20.4% and 6.1% of cases respectively. CONCLUSION: Timely diagnostics of tumorous and pseudotumorous lesions of cardiac valves is very difficult but possible based on short history, positional dependence of symptoms, embolic events without rhythm disturbances. Additional instrumental methods including TTE, TEE, CT and MRI are necessary to define diagnosis. Intraoperative diagnostics and immediate histological survey are useful to determine volume of surgery in each case. Successful surgical treatment implies urgency, hypothermic perfusion and pharmaco-cold cardioplegia during operation.

[Clinic and genetic polymorphism of Brugada syndrome in Russian patients, caused by mutation in SCN5A gene].

Brugada syndrome (BrS) is an inherited cardiac arrhythmic disorder, characterized by ST-segment elevation in right precordial leads V1-V2>2 mm, pseudo right bundle branch block (RBBB), T-wave inversion and an increased risk of cardiac sudden death (SCD) due to molymorphic VT. It is estimated to be responsible for 12% of SCD cases and about 20% of deaths in patients with structurally normal hearts in autopsy. Mutations in the SCN5A gene account 15-30% of all cases. Clinical, instrumental and genetic analyses were performed for 25 Russian probands with BrS (19 males and 6 female). Phenotype-genotype correlation was studied in SCN5A-positive and SCN5A-negative patients. Rare genetic variants in SCN5A gene were found in 7 of 21 Russian probands (28%). Two variants affect protein splicing (c.IVS16DS-5A>G and c.IVS24AS+1G>A), three missense mutations (p,Y87C, p.R893H and p.S1787N), one in-frame deletion p.del848l, and one non-sense-mutation p.E553X. All mutations were unique for each family. There were no clinical or instrumental parameters were found to be effective in prediction of SCN5A mutations. The protocols of genetic counceling for SCN5A-positive and SCN5A-negative families were established.

[Echocardiography of cardiac cysts].

Experience of ultrasound diagnosis of cardiac cysts of various origin accumulated by the authors during 30 years of practice is described. For the first time in the literature of this country and in foreign literature attention is attracted to a wide spectrum of possible cardiac cysts and there main diagnostic signs are presented. Eleven patients aged 9-55 years were examined. Intraoperative and histological verification of the described pathology was conducted.

[Phospholipase A2, group IIa, as an earlier unknown immunohistological marker of cardiac myxoma].

Phospholipase A2, group IIA, gene expression has been analyzed in primary heart tumors. High expression has been demonstrated through several ways: reverse-transcriptase chain polymerase chain, Northern blotting hybridization at the RNA level and immunoblotting, immunohistochemical assay at the protein level. Human cardiac myxoma exhibits highly positive phospholipase A2, group IIA, immunophenotype (100% positive cases). The immunophenotype is unique among human primary cardiac tumors. Phospholipase A2, group IIA, can be proposed as a tissue marker for pathological examination after heart tumor resection.

[Clinical, diagnostic and surgical aspects of heart space-occupying lesions at children and youths].

Clinical, diagnostic and surgical aspects of primary heart tumors, pseudotumor and morphologically unidentified heart space-occupying lesions were analyzed at 52 childhood and adolescence patients. Difficulty of early diagnosis, more aggressive clinical course in majority cases, and individual approach to the choice of surgical approach has been demonstrated. Principle of oncological determinant has been ensured the success in surgical treatment of patients with non-malignant intracavitary and intramuscular neoplasms, and also with pseudotumor space-occupying lesions. Postoperative lethality was low (2.2%), and there were no true recurrences of disease in long-term period after surgery.

[Family myxoma of the heart with metachronic multiple growth]. / Semeinaia miksoma serdtsa s metakhronnym mnozhestvennym rostom.

A case of familial heart myxoma in 4 members of one family combined with myxomic (hamartomatous) syndrome is reported. The tumor in a proband was characterized with a multiple growth in various heart chambers. Five operations were made in the proband for 16 years and seven myxomas of different locations were removed. Myxomas originating from the endocardium of the interatrial septum oval hole were removed in 3 members of his family. The case confirms the conception of heart myxoma origin from endothelial hamartia of the endocardium.

Gene expression analysis to identify mRNA markers of cardiac myxoma.

cDNA expression arrays were used to identify mRNA expression markers for cardiac myxoma. The RNA profile analysis suggests that cardiac myxoma should be considered as a stand-alone tissue rather than a pathological modification of particular normal tissue. The analysis reveals a set of genes which are highly and steadily expressed in cardiac myxomas and can serve as an mRNA expression markers of the tumour. Marker status of selected genes was confirmed by reverse transcriptase polymerase chain reaction analysis. Genes MIA (melanoma inhibitory activity) and PLA2G2A (phospholipase A2, group IIA) show the highest specificity as cardiac myxoma markers, since they have more than 10-fold higher RNA level in cardiac myxomas than in any one of 15 normal tissues tested. Among markers of myxoma at least three are participants of phospholipid metabolism: ANXA3, PLA2G2A, and phospholipid transfer protein. Tissue inhibitor of metalloproteinase 1 and secretory leucocyte protease inhibitor are inhibitors of proteases degrading extracellular matrix proteins and participating in cell proliferation regulation. MIA, SPP1, fibromodulin are modulators or participants of the interaction between extracellular matrix proteins and their cell surface receptors. SOX9 is a transcription factor required for chondrocyte differentiation. Calretenin (CALB2) is an intracellular calcium-binding protein with poorly understood function.

[PRKAR1A gene mutations in two patients with myxoma syndrome (Carney complex)]. / Mutatsii v gene reguliatornoi sub"edinitsy 1-alpha tsiklo-AMF-zavisimoi proteinkinazy A (PRKAR1A) u dvukh bol'nykh s miksomnym sindromom (kompleksom Carney).

Carney complex is an autosomic dominant disorder initially described as the association of cardiac myxomas, spotty skin pigmentation and endocrine overactivity and considered as a multiple neoplasia and lentiginosis syndrome. Mutations in the tumor suppressor gene PRKAR1A, coding for the type 1-alpha regulatory subunit of cAMP-depended protein kinase A have been previously identified in about half of the Carney complex kindreds. In this paper we report identification of the molecular defect in PRKARIA gene in two Carney complex patients. A new mutation (403delAC) located in a 3rd exon of PRKARIA gene has been observed in one case, and a previously described mutation in exon 7 (847delTC) in the second case.

[Heart myxoma histogenesis in the light of its histological and ultrastructural features]. / Predstavleniia o gistogeneze miksomy serdtsa v svete ee gistologicheskikh i ul'trastrukturnykh osobennostei.

In 168 cases of heart myxoma histological and electron-microscopic examination have revealed signs of embryonal endothelium capable of vasoformation and glycoprotein and glycosaminoglycanes synthesis in myxoma cells. Neither differentiation of myxomas cells up to mature endotheliocytes no signs of malignant transformation was found. According to the authors, heart myxoma is a true benign dysontogenetic tumor originating from embryonal endothelium. The term myxoma does not reflect morphological entity of the tumor and the authors suggest to call it embryonal endocardial endothelioma.

[Short and long-term results of surgical correction of the aortal valve dysplasia]. / Blizhaishie i otdalennye rezul'taty khirurgicheskoi korrektsii displazii aortal'nogo klapana.

Nearest and long-term results of surgical treatment of 108 patients with dysplasia of aortal valve were analyzed. Necessity of individual approach to choice of method of surgical correction (prosthesis or valvuloplasty of aortal valve) depending on degree of dysplasia and/or secondary valvular pathology, it anatomic variants, presence of concomitant infectious endocarditis and age of patients is noted. This permitted to reduce hospital lethality from 8.8 to 3.9%. Survival in 1, 5 and 10 years after prosthesis and valvuloplasty of aortal valve was 92.3 +/- 3.3, 86.8 +/- 4.8 and 85.7 +/- 5.7% versus 100, 94.1 +/- 4.1, 94.1 +/- 4.1% respectively. Quality life in remote period was evaluated as good in 80.3% operated patients, as satisfactory--in 11.1% and unsatisfactory--in 8.6%.

[Electrical stimulation of the spinal cord in cardiovascular disease]. / Elektricheskaia stimuliatsiia spinnogo mozga pri serdechno-sosudistoi patologii.

The early and late outcomes of electrical stimulation of the spinal cord are analyzed in chronic critical ischemia of the lower extremities and coronary heart disease with peripheral involvement of coronary arteries. The clinical capacities of this treatment that has enjoyed little use in our country due to its high cost showed atraumaticity of electrical stimulation of the spinal cord; a few cases of complications and no hospital mortality; a rapid positive clinical effect after the initiation of electric neurostimulation, and better life quality in patients in the postoperative periods.

[Surgical correction of incomplete form of open atrioventricular canal in adults]. / Khirurgicheskaia korrektsiia nepolnoi formy otkrytogo atrioventrikuliarnogo kanala u vzroslykh.

Clinical and anatomo-hemodynamic features of adult patients with incomplete form of the open atrioventricular canal are presented, policy of surgical treatment and optimal degree of surgical intervention on mitral valve are determined. The study of variant anatomy of the defect, revealed two variants of left atrioventricular valve formation (with "functional" and with "anatomical" septal commissure). In the first anatomic variant the mitral insufficiency was hemodynamically important and required surgical correction. In the second variant, the mitral valve had good closure function, that permitted to avoid the routine suturing of anterior velum's splitting.

[Primary tumors of heart]. / Pervichnye opukholi serdtsa.

The analysis of 36 years' experience in surgical treatment of 162 patients with benign and malignant primary tumors of the heart, verified by morphological examination, is presented. The possibility of their timely and intra vital revealing is noted in cases of clinical alertness which makes possible to suspect this disease and purposefully examine these patients using preferably echocardiography and computed tomography. The scope of the operations and their success depend on morphological characteristics of the tumors and the extent of neoplastic process. However, by now short- and long-term results of surgical treatment evidence, that predominant part of this category of patients is potentially curable, provided that these neoplasms of the heart were revealed at an initial stage.

[Diagnostic and prognostic aspects of myxoma syndrome]. / Diagnosticheskie i prognosticheskie aspekty miksomnogo sindroma.

The analysis is presented of clinicomorphological and laboratory characteristics of myxoma syndrome which are of prognostic value. Early diagnosis of the syndrome is essential in making decision on the intraoperative and long-term postoperative treatment policy.

[Surgical treatment of heart echinococcosis]. / Khirurgicheskoe lechenie ékhinokokkoza serdtsa.

Echinococcosis of the heart is a rare but dangerous for life disease, very difficult for diagnosis. 5 patients with echinococcosis of the heart were examined, 2 were operated. Introduction into clinical practice of complex echocardiography contributed to precise diagnosis. The viability of the parasite was determined after application of the latex-test. Defection of the living echinococcus parasites in the heart was indication for surgery. Removal of the echinococcus from the myocardium of the left ventricle in 1 patient was carried out without artificial circulation and was accompanied by a significant risk, the complete excision of fibrous capsula being impossible. Intraventricular location of the echinococcus cyst in the other patient has required artificial circulation and cardioplegia. Intraventricular cyst was successfully and radically removed after preliminary sterilization by glycerine solution.

[Primary heart neoplasms]. / Pervichnye opukholi serdtsa.

The work analyses the anatomomorphological characteristics of primary tumors of the heart in 106 patients; in 101 patients the tumor was benign (myxoma, neurinoma, rhabdomyoma) and in 5 malignant (angiosarcoma, liposarcoma). Surgical treatment was conducted in 99 patients; hospital lethality was 7.1%. Six patients who refused an operation died at other therapeutic institutions, one patient died on admission to the clinic from occlusion of the left atrioventricular orifice by the tumor. It is pointed out that the diversity and the various degree of the clinical manifestations of the disease are predetermined by the localization and morphological characteristics of the new growths, their size, and mobility. The authors emphasize that knowledge of the anatomomorphological features of primary tumors of the heart and their early diagnosis are conducive to a differential approach in each concrete situation and to the choice of the optimal volume of an urgent surgical intervention.

[Myxoma syndrome]. / Miksomnyi sindrom.

The paper provides the follow-up findings of patients with cardiac myxomas according to the presence or absence of the myxomal syndrome. It is shown that it is a multiorgan abnormality wherein the cardiac myxomas that are prone to primarily multiple growth, extraseptal and/or multifocal fixation are associated with the changes in the hypothalamus-pituitary-adrenal and renin-angiotensin-aldosterone systems, skin spotty pigmentation, neoplasms in the viscera, skin, and fat. These specific features emphase the predictive value of the myxomal syndrome, which predetermines that its early diagnosis should be made in routine clinical practice.