AYERSA SYNDROME

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AYERSA SYNDROME (A. Ayerza, the Argentina therapist, 1861 — 1918) — set of clinical signs of a sclerosis of system of a pulmonary artery (sharp diffusion cyanosis, a polycythemia, an asthma and high pulmonary hypertensia with a considerable hypertrophy of the right half of heart) which weight does not correspond to degree of ventilating insufficiency at bronchopulmonary diseases or the last in general are absent (primary pulmonary hypertensia).

Priority of intravital diagnosis of a sclerosis of a pulmonary artery at the patient with hron. a bronchopulmonary inflammation belongs to Ayersa (And. Ayerza, 1901), pupils to-rogo Arrillaga (F. Pages of Arrilaga) and P. Escudero in the subsequent in detail described this syndrome, having called it «Ayersa's disease». This term is applied sometimes as a synonym of primary pulmonary hypertensia — a peculiar disease, at Krom of Ampere-second. is the main clinical manifestation since bronchial tubes and lungs at this disease are not affected. However such use of the term is not justified historically (Ayersa the sclerosis of pulmonary arteries at the patient with primary damage of bronchial tubes and lungs distinguished), klassifikatsionno since the described syndrome is not a separate disease, it is not even specific to any one nosological form and is observed at a number of bronchopulmonary defeats.

Etiology and pathogeny. Major factor in development of a sclerosis of pulmonary arteries is hron. increase in pressure in them (see Hypertensia of a small circle of blood circulation, the Pulmonary heart). Most often lead diffusion pneumonias with a pneumosclerosis to it and hron. bronchial obstruction at which hypertensia develops in connection with reflex (because of hypoventilation of alveoluses) a hypertension of pulmonary arterioles and owing to thrombosis of small vessels and an inflammatory sclerosis of their walls in zones of an inflammation. At a peribronchial pneumosclerosis and diffusion hron. pneumonia the sclerosis of pulmonary arteries is noted at/5 patients, and from the dead in this group — at 1/3.

The etiology and a pathogeny of primary pulmonary hypertensia are a little studied. Attach significance to inborn inferiority of pulmonary arterioles, reflex (the different nature) to increase in their tone, pulmonary vasculites. In branches of a pulmonary artery of large and average caliber find fibrous and lipoid plaques, in small arteries — a thickening of an intima, a fibrinorvdny necrosis, a hyalinosis, vasculites and fibrinferments.

Leading sign of Ampere-second. — the sharp general cyanosis which is not corresponding to extent of ventilating disturbances is connected with disturbance of diffusion of gases in the sclerosed alveolocapillary membranes, formation of arteriovenous shunts, the polycythemia (promoting increase in blood of the recovered hemoglobin) and delay of a blood-groove at a decompensation of heart.

Clinical picture. The cyanosis and short wind accruing at the slightest exercise stress dominate. Complaints of patients (on weakness, dizzinesses, cough, a pneumorrhagia, etc.), and also many these inspections are not specific to Ampere-second., they often correspond to a basic disease of lungs. At a half of patients the pains in heart caused by restretching of a pulmonary artery and, perhaps, insufficiency of blood supply of a hypertrophied myocardium are noted.

Fig. Expansion of arterial vessels of roots of lungs at Ayersa's syndrome

Objective signs actually Ampere-second. (without symptoms of bronchopulmonary defeat) come down to a picture of a vascular pulmonary heart, as at primary pulmonary hypertensia: over lungs radical shortening of a percussion sound comes to light, limits of relative cordial dullness are expanded to the right (if there is no emphysema), the shaking cardiac impulse (a hypertrophy of a right ventricle) is defined; strengthening of the second cardiac sound and its sharp accent over a pulmonary artery is listened (hypertensia in a small circle of blood circulation), sometimes over it systolic and diastolic noise are heard (due to relative insufficiency of valves). Disturbances of blood circulation develop on right ventricular type (increase in venous pressure, swelling of cervical veins, increase in a liver, hypostases). Blood tests reveal a hyperglobulia and a polyglobulia, an anoxemia and often a hypercapnia (see). On an ECG — signs of the expressed hypertrophy and an overload of the right departments of heart. Functional trials of lungs reveal at primary pulmonary hypertensia only disturbances of diffusion of gases, and at bronchopulmonary defeats — the disturbances of ventilation corresponding to them. At catheterization of heart: high systolic pressure in a right ventricle and a pulmonary artery (it is higher than 35 mm of mercury.). On the angiogram — narrowing of small pulmonary vessels.

Radiodiagnosis. Radiological signs depend on the nature of a bronchopulmonary disease, and at its absence correspond to a picture of primary pulmonary hypertensia. At roentgenoscopy the strengthened pulsation of a pulmonary trunk and large branches of a pulmonary artery is visible and, as well as on roentgenograms (fig.), protrusion of an arch of a pulmonary trunk, increase in caliber of arteries in roots and radical zones of lungs, «amputation» of shadows of segmented vessels and lack of the pulmonary drawing in peripheral departments of pulmonary fields are defined. In the right front slanting situation protrusion of a pulmonary cone and increase in diameter of a round shadow of the left pulmonary artery in its orthogonal projection are noted. In the left front slanting situation «aortal window» it is closed by a shadow of an expanded pulmonary trunk; usually, as well as in side situation, there are signs of increase in a right ventricle.

In-depth X-ray inspection (tomography, elektrokimografiya, angiocardiography) reveals at Ampere-second. expansion only of arterial vessels while pulmonary veins are not expanded.

The anamnesis and lack of clinical and radiological signs of heart disease facilitate the differential diagnosis between primary pulmonary hypertensia and hypertensia at inborn heart disease, and the accompanying signs of damage of lungs (emphysema, a peribronchial pneumosclerosis) help to establish connection of pulmonary hypertensia and a hypertrophy of the right half of heart with a specific disease of lungs.

Forecast adverse.

Average age of the dead with a diffusion pneumosclerosis in the presence of Ampere-second. is 10 years less, than at its absence.

Treatment includes therapy of a basic disease (see Bronchitis, chronic; Pneumonia, chronic; Emphysema of lungs), correction of the available disturbances of ventilation (appointment bronchodilatory, expectorants and use of the auxiliary respiratory equipment according to indications); the oxygenotherapy is carried out; the drugs reducing pressure in a small circle of blood circulation (see Hypertensia of a small circle of blood circulation) — phentolamine, Reserpinum, ganglioblokiruyushchy means, spasmolysants are appointed. The individual choice of these means is carried out by comparative assessment of their hypotensive action more often by indirect methods, napr, on dynamics of a loop of RVKG or tooth of REKG (see Eufillinovaya test).

Bibliography: Zamotayev I. P. Chronic pneumonia with a diffusion pneumosclerosis, Sverdlovsk, 1969, bibliogr.; Mukharlyamov N. M. and Ryff I. M. Some questions of clinic and pathology of primary pulmonary hypertension, Rubbed. arkh., t. 46, No. 2, page 27, 1974, bibliogr.; Nesterov V. S. To clinic and a pathogeny of primary pulmonary hypertension, Klin, medical, t. 50, No. 3, page 55, 1972, bibliogr.; Ayerza A. Maladie d'Ayerza, sclérose secondaire de l'artére pulmonaire (cardiaques noirs), Sem. méd. (B. Aires), t. 32, p. 43, 1925; Anderson E. G., Simon G. Reid L. Primary and thromboembolic pulmonary hypertension, quantitative pathological study, J. Path., v. 110, No. 4, p. 273, 1973, bibliogr.; Barrett A. M. a. Cole L. Pulmonary vascular sclerosis with right ventricular failure, Brit. Heart J., v. 8, p. 76, 1946, bibliogr.; Lorimier A. And., Moehring H. G. a. Hannan J. R. The lung and the cardiovascular system emphasizing differential considerations, p. 141, Springfield, 1955.

Radiodiagnosis of Ampere-second. — Rabkin I. H. X-ray semiotics of pulmonary hypertensia, page 181, M., 1967; Radiodiagnosis of heart diseases and vessels, under the editorship of M. A. Ivanitskaya, page 299, M., 1970; Roesler H. Clinical roentgenology of the cardiovascular system, p. 346, Springfield, 1946.

I. P. Zamotayev; M. A. Ivanitskaya (rents.).

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