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Mitral valve prolapse (MVP) is a pathological sagging (bending) of one or both mitral valve leaflets into the left atrium during left ventricular systole. Statistical data. MVP is found in 3–8% of people in the general population (apparently, the data is overestimated). Manifestations of MVP are first recorded at the age of 10–16 years; after 10 years, girls are observed 2 times more often.

Etiology. MVP can be primary or secondary • Primary MVP •• A disease inherited in an autosomal dominant manner with myxomatous deformation of the mitral valve leaflets •• MVP is also observed in patients with Marfan syndrome and other congenital connective tissue diseases, such as Ehlers-Danlos syndrome, elastic pseudoxanthoma, osteogenesis imperfecta •• In the occurrence of MVP, exposure to toxic agents on the fetus on the 35–42nd day of pregnancy may also be important • Secondary MVP can occur with: •• IHD (ischemia of the papillary muscles) •• rheumatism (post-infectious sclerotic changes) • • hypertrophic cardiomyopathy (disproportionally small left ventricle, change in the location of the papillary muscles).

Pathogenesis • Primary MVP •• Myxomatous degeneration of collagen leads to excessive accumulation of mucopolysaccharides in the middle spongy part of the mitral valve leaflets and its hyperplasia, which causes the appearance of break areas in the fibrous part of the valve. Local replacement of the elastic fibrous tissue of the valve leaflet with a weak and inelastic spongy structure leads to the fact that during systole, under the influence of blood pressure from the left ventricle, the leaflet bulges towards the left atrium (prolapses) •• In the occurrence of primary MVP in Marfan syndrome, it is also important dilatation of the annulus fibrosus of the mitral valve - it does not decrease by 30% in systole, as is normal, which leads to protrusion of one or both leaflets into the cavity of the left atrium • Secondary MVP occurs as a result of thinning and elongation of the tendon threads or their separation or dilatation of the fibrous ring. Lengthening of the tendon threads, separation of part of them lead to the fact that the leaflet is not held in place and begins to prolapse into the left atrium • If the mitral valve leaflet is excessively bowed, mitral regurgitation may occur with dilatation of the left atrium and left ventricle. It should be noted that MVP can be combined with prolapse of other valves: tricuspid valve prolapse in 40% of cases, pulmonary valve prolapse in 10%, aortic valve prolapse in 2%. In this case, in addition to mitral valve insufficiency, manifestations of insufficiency of the corresponding valve will occur. There is often a combination of MVP with other congenital anomalies of the heart - ASD, additional conduction pathways (usually left-sided).

Clinical manifestations • Complaints •• In most cases, MVP is asymptomatic and is detected by chance during a preventive examination •• With more severe prolapse, patients complain of palpitations (ventricular extrasystole, paroxysmal supraventricular tachycardia, less often ventricular tachycardia) •• A common complaint is pain in the chest. It can be either atypical or typical anginal due to spasm of the coronary arteries or ischemia as a result of tension of the papillary muscles •• Shortness of breath during physical exertion, fatigue are also noted by patients with MVP •• Extremely rare manifestations are visual impairment as a result of thromboembolism of the retinal vessels, and also transient ischemic attacks as a result of cerebral thromboembolism. In the occurrence of embolic complications, importance is attached to the separation of fibrin threads located on the atrial side of the mitral valve •• Often the above-described complaints are accompanied by psycho-emotional lability • Upon examination, concomitant congenital abnormalities of the chest shape can be identified - kyphoscoliosis, funnel-shaped chest, pathologically straightened back, reduced anteroposterior chest size cells or signs of Marfan syndrome • Auscultation of the heart (a “silent” form of MVP is possible) •• The main auscultatory sign of MVP is a short mid-systolic high-frequency click (pathognomonic). It appears as a result of sagging of the mitral valve leaflets in systole into the cavity of the left atrium and their sharp tension •• The systolic click may be followed by a mid- or high-frequency late systolic murmur, better heard at the apex of the heart •• To clarify the manifestations of MVP, dynamic auscultation of the heart is used •• • Changes in left ventricular end-diastolic volume result in changes in the timing of the click and murmur. All maneuvers that contribute to a decrease in end-diastolic volume, an increase in heart rate, or a decrease in resistance to ejection from the left ventricle lead to MVP appearing earlier (the click noise approaches the first sound). All maneuvers that increase blood volume in the left ventricle, reduce myocardial contractility or increase afterload increase the time from the beginning of systole to the appearance of a click noise (moves back to the second sound) ••• In the supine position, the click occurs later, the noise is short ••• In the position standing, the click occurs earlier, and the noise is longer ••• In the squatting position, the click occurs later, and the noise is shorter (may even disappear). Instrumental data • Usually no changes are detected on the ECG in patients with MVP. Of the detected deviations, depression of the ST segment or negative T waves in leads III, aVF are most often noted. These changes may reflect ischemia of the inferior wall of the left ventricle as a result of tension in the posterior papillary muscle due to leaflet prolapse. In patients with ECG changes, cardiac arrhythmias also occur. Some patients experience prolongation of the QT interval. Recording an ECG after taking beta-blockers increases the specificity of this method • EchoCG •• In one-dimensional mode, the “hammock” symptom is detected - sagging of one or both leaflets in systole by more than 3 mm •• In two-dimensional mode, sagging of the mitral valve leaflets into the cavity of the left atrium in systole is detected left ventricle, thickening of the leaflets more than 5 mm in diastole, lengthening of the tendon filaments, lengthening of the leaflets, dilatation of the fibrous ring •• There are three degrees of MVP, determined in a four-chamber section ••• I degree (minor) - sagging of the leaflets into the cavity of the left atrium up to 5 mm ••• II degree (moderate) - sagging of the leaflets into the cavity of the left atrium 5-10 mm ••• III degree (severe) - sagging of the leaflets into the cavity of the left atrium more than 10 mm •• Doppler examination can reveal a regurgitation jet in the left atrium. With severe MVP, dilatation of the left atrium and left ventricle occurs, detected in one- and two-dimensional modes. It should be remembered that in the presence of typical auscultatory signs of MVP, its EchoCG signs may be absent in 10% of patients. When conducting the study, you should remember about other congenital heart defects (in particular, ASD). Differential diagnosis • Mitral valve insufficiency of rheumatic origin • Isolated aneurysm of the interatrial septum • Isolated tricuspid valve prolapse • VSD.

TREATMENT Management tactics • Treatment of the underlying disease in secondary MVP • Risk groups for the development of complications (patients with severe systolic murmur, thickened prolapsed mitral valve leaflets, left ventricular hypertrophy, rhythm disturbances, fainting) undergo regular ECG, echocardiography • Prevention of endocarditis is indicated for persons with severe systolic murmur. Treatment of various options • For asymptomatic MVP without signs of mitral valve insufficiency, there is no need for treatment •• The patient should be given recommendations for normalizing lifestyle, optimizing physical activity (a decrease in the tone of the sympathetic nervous system can lead to a decrease in valvular dysfunction) •• EchoCG is recommended -control once every 1–2 years •• It is necessary to avoid drinking strong tea, coffee, alcohol, as well as smoking • For severe MVP •• If there are symptoms of MVP such as tachycardia, palpitations, chest pain,  is prescribed -adrenergic blockers in small doses (for example, propranolol at a dose of 30–60 mg/day) •• In case of dilatation of the left atrium and left ventricle, prolongation of the QT interval, a history of fainting, dilatation of the initial part of the aorta, physical activity is prohibited •• Prevention is recommended infective endocarditis using amoxicillin •• For symptoms of embolization, acetylsalicylic acid is prescribed at a dose of 80–325 mg/day •• With significant changes in hemodynamics, increasing symptoms of mitral valve insufficiency, mitral valve replacement or annuloplasty is performed.

Prognosis and complications . Typically, MVP is benign. Complications of MVP most often occur in patients with systolic murmur, thickened, elongated mitral leaflets, or enlarged left ventricular cavity or left atrium. Complications include: • separation of tendon threads • severe mitral valve insufficiency (0.06%) • fibrin deposition on the mitral valve leaflets • cardiac arrhythmias • cerebrovascular pathology (0.02%) • infectious myocarditis (0.02%) • sudden cardiac arrest death (0.06% of cases with severe mitral valve insufficiency). Synonyms • Systolic click-murmur syndrome • Barlow's syndrome • Inflated mitral valve syndrome. Reduction. MVP - mitral valve prolapse.

ICD-10 • I34.1 Prolapse [prolapse] of the mitral valve

The International Statistical Classification of Diseases and Problems, 10th revision (ICD-10), currently in use, is a normative document that ensures the formation of unified statistical control over the activities of medical institutions, assessment of the epidemiological situation in the country, and allows for the unity and comparability of incoming medical data [1] . ICD-10 is one of the important methodological tools for processing statistical data, which is extremely necessary for automating basic planning, regulatory and management work [2]. Unfortunately, this classification contains errors, both in the structural design of clinical sections and in the coding of a number of diseases. Some of the diagnoses that exist in it sound somewhat absurd; there is a confusion of placement of individual polyetiological diseases and syndromes in sections, which are often located in chapters that have a very indirect relationship to this nosology. In addition, many nosological forms, syndromes and symptoms that are currently common are not included in the accepted classification, which undoubtedly significantly limits the practical application of ICD-10.

The variety of different forms of diseases complicates the structure of the classification, so the latest revision has become much larger in volume and was accompanied by extensive guidelines, unfortunately, often without taking into account clinical practice. As a result, some of the clinical diagnoses ended up within the limits of unrefined conditions or conditions that were not sufficiently differentiated, and not in the headings or subheadings of the corresponding sections of the classification.

A number of difficulties arise with regard to the topographic classifications and terms used in ICD-10, many of which are imprecise, contradictory and do not correspond to clinical practice. Unfortunately, it should be noted that ICD-10 cannot currently be recommended as a model for terminology and recording of clinical diagnoses in medical records and other medical records.

To solve the problem of uniform standardization of medical diagnoses and statistical records, back in the late 70s of the last century, the idea arose of creating a “family” of classifications of diseases and health problems based on the ICD, adapted to a specific specialty, taking into account modern achievements of medical science.

Classification options for dentists, oncologists, dermatologists, rheumatologists and orthopedists, pediatricians, psychiatrists, and general practitioners have been published and recommended by WHO. Unfortunately, in the field of andrology there is still no unified classification [2].

Currently, the Research Institute of Urology is developing printed and electronic versions of a similar classification for urology and andrology, which is based on three-digit and four-digit ICD-10 codes. The coding system for urological and andrological diseases is presented in the form of a tree, where each subnode refines the information of the previous node. When forming a subnode, a number from zero to nine is added to the main code through a dot. The proposed system also provides for taking into account the localization of the pathological process, its stage and/or phase [3-5].

The creation of a printed and electronic version of a new unified clinical and statistical classification of andrological diseases will allow:

• facilitate data collection and improve the analysis of statistical material on andrology by increasing the reliability of statistical information and reducing the number of errors due to inconsistency of clinical diagnoses with ICD-10 diagnoses;
• provide a detailed assessment of the complexity of clinical cases and final treatment results when providing specialized (including high-tech) medical care;
• standardize the formulation of andrological diagnoses in medical documents and computer information systems of health authorities of the Russian Federation;
• improve the economic and statistical analysis of the provision of medical care in the field of andrology in the interests of healthcare of the Russian Federation, individual medical institutions and compulsory health insurance (CHI);
• optimize costs for diagnosis and treatment of andrological diseases.

It should be taken into account that andrological diseases, as a rule, are multi-etiological and many of them do not fit only into the framework of the block “Diseases of the male genital organs”. The modern concept of andrology is, of course, based on interdisciplinary interaction and is at the intersection of related medical disciplines (urology, endocrinology, psychiatry, genetics, neurology, therapy, etc.). Therefore, andrological diseases are heterogeneous in nature and are widely represented in different sections of ICD-10 (Table 1).

Table 1. General classification of andrological diseases according to ICD-10 and proposals for its correction

This summary table shows those symptoms, syndromes and nosological forms according to ICD10 that a practicing specialist in the field of andrology encounters in real practice. At the same time, in our opinion, some of them no longer correspond to the modern understanding of pathophysiological processes and require terminological and classification clarification (right column).

Naturally, the above classification needs to be supplemented and refined, and fundamental amendments be made taking into account interdisciplinary interaction. With our publication, we would like to provoke a discussion among specialists in the field of andrology, the main goal of which would be to create a single, integral classification of andrological diseases. This is undoubtedly valuable and convenient for medical practitioners, health authorities, scientific research, and insurance companies. We will be grateful for your comments and remarks, which can be sent to the email address

Key words: ICD-10, andrological diseases, clinical and statistical classification.

Keywords : ICD-10, andrology diseases, clinical and statistical classification.

Literature

1. International classification of diseases and related health problems. WHO, 1992
2. Order of the Ministry of Health of the Russian Federation dated May 27, 1997 No. 170 (as amended on January 12, 1998) “On the transition of health authorities and institutions of the Russian Federation to the international statistical classification of diseases and health-related problems of the X revision.”
3. Sivkov A.V., Kakorina E.P., Keshishev N.G. Unified clinical and statistical classification of urological diagnoses // Materials of the XI Congress of Urologists of Russia. M. 2007. pp. 598-599.
4. Unified clinical and statistical classification of urolithiasis / Apolikhin O.I., Dzeranov N.K., Sivkov A.V., Kakorina E.P., Keshishev N.G. // Urology. 2008. No. 6. P. 3-6.
5. On the creation of a unified clinical and statistical classification of oncological urological diseases using the example of kidney tumors / Apolikhin O.I., Sivkov A.V., Chernyshev Yu.V., Kakorina E.P., Keshishev N.G., Zhernov A.A. // Experimental and clinical urology. 2010. No. 1. pp. 23-28.