130. Changes in the heart, systemic and pulmonary circulation in aortic malformations.

Aortic valve defect occupies the second place in frequency after mitral valve and usually occurs on the basis of rheumatism, less often — atherosclerosis, septic endocarditis, brucellosis, syphilis. In rheumatism, sclerosis of the semilunar flaps and valve defect develop in connection with the same processes that form the mitral defect. The flaps are spliced together, thickened, and lime is present in the sclerosed flaps. This leads in some cases to the predominance of valve insufficiency, and in others to stenosis of the aortic opening. In atherosclerosis, calcification and sclerosis of the flaps are combined with lipoidosis and liposclerosis, and the changes are more pronounced on the surface of the flaps facing the sinuses. In infectious endocarditis and brucellosis, there is a sharp destruction of the dampers and their deformation due to pronounced petrification. Syphilitic aortic malformation is usually combined with mesaortitis; due to the expansion of the aorta, valve insufficiency prevails in these cases.

The heart in aortic malformations is hypertrophied, mainly due to the left ventricle. With insufficiency of the aortic valves, the heart mass can reach 700-900 g — a "bull's heart" occurs. The endocardium of the left ventricle is thickened and sclerosed. As a result of hemodynamic disorders, formations resembling semilunar flaps — additional valves - sometimes appear below the valve opening.

(this is just in case, you don't have to dictate )

1. Fallot's Tetrad: (1) DMJP; (2) obstruction (stenosis) of the pulmonary trunk; (3) aortic dextraposition; (4) right ventricular hypertrophy.

Morphology: the heart is enlarged (especially in the apical region), the DMF is large, the aortic valve is located above both ventricles, stenosis of the pulmonary artery valve may accompany

Clinic: 1) if the pulmonary stenosis is moderate, it resembles isolated breast cancer (may be without cyanosis); 2) with an increase in pressure in the right ventricle, venoarterial bypass surgery with cyanosis. The heart increases in size, the obstruction increases.

2. Transposition of the main arteries (associated with ventricular-arterial discordance: the aorta originates from the right ventricle and is located in front and to the right of the pulmonary artery, which exits the left ventricle).

Morphology: 1) Hypertrophy of the right ventricle (provides systemic circulation); 2) the wall of the left ventricle atrophies, because it provides blood flow with low pulmonary vascular resistance

3. Persistent common arterial trunk (as a result of incomplete separation of the embryonic common arterial trunk into the aorta and pulmonary artery.

4. Tricuspid valve atresia (due to the unequal separation of the AV channel, the connection of the right atrium with the right ventricle is not formed)

Morphology: 1) the mitral valve is larger than normal 2) hypoplasia of the right ventricle is observed. 3) blood circulation is maintained due to atrial communication (DMPP or open oval window) and VHD, providing a connection between the left ventricle and the pulmonary artery. 4) cyanosis is present almost from birth, there is a high mortality rate.

6. Total abnormal drainage of the pulmonary veins (the direct connection of the pulmonary veins with the left atrium is disrupted, it is formed in the embryonic period when the common pulmonary vein does not develop or becomes atretic). There is an open oval window or DMPP, allowing blood from the pulmonary vascular bed to flow into the left atrium.

131. Congenital heart defects: principles of classification. Hemodynamic changes in the circulatory organs in the presence of defects in the interatrial and interventricular septa, non-closure of the ductus arteriosus.

Congenital heart defects occur as a result of a violation of the formation of the heart and the vessels leaving it.

Classification

1. According to anatomical features:

CHD with abnormal communication between the large and small circulatory circles – at the level of the atria, ventricles, main vessels (Def. Interventricular septum, atrial, their combination, absence of septa – three-chamber heart)

Defects with narrowing or obliteration of some part of the CCC (LA stenosis or atresia, aortic stenosis)

Defects with vascular transposition (aortic separation from the pancreas, LA from the LV, anomalies in the discharge of coronary vessels and large veins)

2. According to clinical signs (severity of hypoxia):

The blue ones are cyanotic

Whites are acyanotic

3. According to the degree of blood filling:

With reduced blood flow

With increased blood flow

With normal blood flow

Non–saturation of the arterial duct – Blood discharge from left to right, hypertension of the small circulatory circle, pancreatic hypertrophy, with a high degree of hypertension, blood discharge from right to left.

With small defects of the interventricular septum, the clinical picture does not differ from that described. With large defects, the blood is largely mixed in the ventricles, which gives the clinic a predominance of signs of heart failure. Cyanosis appears gradually and at a later date. The size of the heart increases significantly. The heart murmur is of a systolic nature.

Transposition with a wide open arterial duct is clinically manifested by congestive heart failure, while general cyanosis may be absent.

DMPP - Right-left shunt due to a small defect in the upper part of the MPP, overload of the right ventricle, its hypertrophy, mild course, long-term compensation. A large defect in the lower part of the septum causes concomitant insufficiency of the MC. Severe course and early decompensation.