TAUSSIG-BINGA SYNDROME

From Big Medical Encyclopedia

TAUSSIG-BINGA SYNDROME (N of V. of Taussig, sort. in 1898, an amer. cardiologist; R. J. Bing, sort. in 1909, an amer. doctor; synonym: transposition of an aorta and left position of a pulmonary trunk, incomplete transposition of the main vessels with an otkhozhdeniye of a pulmonary trunk from two ventricles) — inborn heart disease, at Krom an aorta departs from a right ventricle, and a pulmonary trunk from both ventricles at highly located defect of an interventricular partition.

Formation of T. — The B. page is connected with the fact that in the course of embryonic development of an arterial cone there is no shift of a bulb of heart to the centerline. As a result of underdevelopment of a bulbous and ventricular ledge defect of an interventricular partition is at the same time formed.

the Diagrammatic representation of heart and the main vessels on a frontal section at a syndrome of Taussig — Bing to (and) and later a definitive repair of defect (by Smith's method): 1 — a pulmonary trunk; 2 — the left auricle; 3 — defect of an interventricular partition; 4 — a left ventricle; 5 — a right ventricle; 6 — the right auricle; 7 — an aorta; 8 — a klapansoderzhashchy prosthesis of a pulmonary trunk; 9 - an anastomosis of the resected piece of a pulmonary trunk and an aorta the end sideways; the direction of the movement of a venous blood is shown by dark shooters, an arterial blood — light shooters.

The main manifestations of defect are defined by features of the hemodynamics which is created at the same time: blood from a right ventricle comes to an aorta, and also to a pulmonary trunk (fig., a). Pressure in a right ventricle and an aorta identical, and in a left ventricle pressure equally or is a little less, than in right. Idiosyncrasy of T. — The B. page is the persistent pulmonary hypertensia (see Hypertensia of a small circle of blood circulation) in the absence of a valve stenosis of a pulmonary trunk leading to an angiosclerosis of a small circle of blood circulation and reduction of efficiency of a pulmonary blood-groove. Saturation of blood oxygen in an aorta at T. — The B. page is lower, than in a pulmonary trunk that leads to an anoxemia of various degree. Diameter of a pulmonary trunk at T. — The B. page is 2 — 3 times more than diameter of an aorta.

Children with T. — B. pages considerably lag behind in development. At patients cyanosis since the birth, deformation of distal (nail) phalanxes in the form of drum sticks, the expressed asthma, however without odyshechno-tsianotichesky attacks is noted. Borders of heart are perkutorno moderately increased; systolic trembling in the second mezhreberye to the left of a breast is noted and is slightly lower. The accent of the II tone and pansystolic noise are defined by Auskultativno. Radiological most often heart of the normal sizes, the pulmonary drawing on the periphery it is grown poor, roots of lungs are expanded. At the left sharply the arch of a pulmonary trunk, a pulsation eminates to-rogo it is strengthened. On an ECG — right axis deviation, a hypertrophy of a right ventricle.

Diagnosis of T. — The B. page is confirmed by data of catheterization of cardial cavities (see. Catheterization of heart ) and angiocardiography (see), allowing to reveal high pressure in a right ventricle and a trunk of a pulmonary artery, high oxygenation of blood in it. At the selection angiography in two projections early and intensive contrasting of an aorta is defined, at the right ventrikulografiya — simultaneous, but weaker contrasting of a pulmonary trunk; an arrangement of an aorta of a kpereda from a pulmonary trunk, wider diameter of a pulmonary trunk in comparison with an aorta.

Treatment — operative measure. Operations generally palliative — at early age narrowing of a pulmonary trunk according to Müller, creation of artificial defect of an interatrial partition for arterialization of blood of the right departments of heart; connection of the lower vena cava with the left auricle, and the right pulmonary veins — with the right auricle (Baffes's operation). Palliative methods however do not eliminate pulmonary hypertensia, finally leads of edges to death of the patient.

Smith (E. E. Smith) et al. in 1982 offered a method of a definitive repair by means of a klapansoderzhashchy prosthesis of a pulmonary trunk (fig., b). Operation consists in creation of adequate outflow tracts of blood from a right ventricle. For this purpose the pulmonary trunk is cut at bifurcation and anastomose with an aorta the end sideways, the mouth of an aorta is taken in; the blood stream from a right ventricle is sent to a small circle of blood circulation through a klapansoderzhashchy prosthesis, anastomosing it with a distal piece of a pulmonary trunk. This original and difficult operation admits perspective, its new techniques are developed. There are no materials on an outcome and the long-term results of this operation, certainly, yet.

Forecast at T. — B. page adverse. Life expectancy depends on weight of anatomic changes, expressiveness of pulmonary hypertensia and the general hypoxia. The main reason for death — right ventricular insufficiency.

See also Heart diseases inborn .



Bibliography: Banks. Inborn heart diseases and large vessels, the lane with English, page 87, M., 1980; Konstantinov B. A. Anomaly of Taussig — Bingl, in book: Chastn. hir. heart troubles and vessels, under the editorship of. V I. Burakovsky and S. A. Kolesnikov, page 230, 'M., 1967; In a f f e s T. G. A new method for surgical correction of transposition of aorta and pulmonary artery, Surg. Gynec. Obstet., v. 102, p. 227, 1956; Go or D. A. a. Lillehei C. W, Congenital malformations of heart, p. 203, N. Y. a. o., 1975; Smith E. E. a. o. A new technique for correction of the Taussig — Bing anomaly, J, thorac. cardiovasc. Surg., v. 83, p. 901, 1982; Taussig H. B. a. Bing R. J. Complete transposition of aorta and levoposition of the pulmonary artery, Amer. Heart J., v. 37, p. 551, 1949; Van Praagh R. What is the Taussig — Bing malformation? Circulation, v. 38, p. 445, 1968.


L. P. Cherepenin.

Яндекс.Метрика