Obliterating brachiocephalic arteritis (synonyms: pulseless disease, Takayasu’s disease ) is a non-specific inflammatory lesion of the aortic wall and large major vessels with their subsequent obliteration. Obliterating brachiocephalic arteritis has been described by Japanese physician Takayasu . Most often, young women get sick. The etiology is not known. A connection with repeated infections (tonsillitis, flu, typhus), hypothermia, trauma, and pregnancy is assumed.
Obliterating brachiocephalic arteritis belongs to the group of allergic vasculitis . The hyperergic nature of inflammation is confirmed by the presence of immune complexes at the site of the lesion and anti-arterial antibodies in the serum.
With obliterating brachiocephalic arteritis, panarteritis is observed with an initial lesion of adventitia and intima with their exudative infiltration, disorganization, spreading to the middle membrane, subsequent fibrinoid organization, sclerosis and the development of parietal thrombosis.
The disease is slowly progressive in nature. Most often, obliterating brachiocephalic arteritis begins with common symptoms: malaise, fever, arthralgia, night sweat, gradual asthenia . As the limbs ischemic , a feeling of numbness, an increase in weakness in the arm, a one-sided or symmetrical absence of a pulse on the affected limbs appear. Often “eye” symptoms (vision loss, pain in the eyes) associated with the formation of arteriovenous retinal aneurysms and optic atrophy join in . In most cases, hypertension associated with damage to the abdominal aorta and renal arteries is noted. Less characteristic (up to 20% of cases) damage to the pulmonary and coronary vessels. With obliteration of the carotid arteries, neurological symptoms associated with cerebral ischemia develop . Laboratory data are not characteristic. Possible anemia, ESR increased to 50 mm / h. Factor rheumatoid and antinuclear antibodies are not determined. System complement components are normal. The diagnosis is confirmed by arteriography .
Long-term administration of glucocorticosteroid drugs, indomethacin , delagil , drugs that improve peripheral blood flow ( trental , compliance , angiotrophip , prodectin ), heparin and indirect anticoagulants is indicated . In recent years, reconstructive surgery of affected vessels has been proposed.
This defect is based on the abnormal position of the ascending aorta and the trunk of the pulmonary artery. There are 2 main types of this anomaly: complete and corrected transposition of the aorta and pulmonary artery. Complete transposition of the great vessels makes up 4.5–20.8% of all congenital heart defects. Together with tetralogy of Fallot, this vice takes a leading place among the “blue” vices. Its hemodynamics boils down to the fact that the aorta departs from the anatomically right ventricle, and the pulmonary artery from the left. As a result, blood circulation occurs in two divided circles. In the absence of communication between them, the newborn dies immediately after birth due to the lack of conditions for blood oxygenation . Therefore, a prerequisite for viability is communication between these independent circles of blood circulation. Usually they are represented by defects of the septa, OAA, aortopulmonary fistula, abnormal venous drainage, or combinations thereof. In this case, cross blood circulation is formed, which determines the severity of the course of the defect. It is determined by the degree of overflow of one and “robbing” of another circle of blood circulation. The heart in these conditions works with overload. Nevertheless, the larger the shunt, the more favorable the course of the defect. So, the most favorable combination of septal defects with moderate stenosis of the pulmonary artery, which prevents excessive pulmonary blood flow. Corrected transposition of the great vessels makes up 1–1.4% of all congenital heart diseases. In this case, the aorta and pulmonary artery are transposed, but the right ventricle receives blood from the left atrium, and the left from the right. Thus, arterial blood enters the large circle of blood circulation, and venous blood enters the small circle. If there are no other concomitant anomalies, hemodynamic disturbances are absent.
Shortness of breath, cyanosis, fatigue, finger changes in the form of drumsticks, peripheral circulatory disorders, lag in physical development are noted. The auscultatory picture is diverse and is determined by the shape of the shunt between the circles of blood circulation. Along with the presence of pronounced systolic murmur along the left edge of the sternum, noise is often absent.
X-ray examination early reveals cardiomegaly due to both parts of the heart. More often the heart has a spherical shape. There is an increase in vascular pattern of the lungs, pronounced vascular pulsation on the periphery. An ECG allows you to identify only signs of overload of various parts of the heart, single-beam blockade of the conduction system. The most complete information about anatomical and hemodynamic malformations is provided by Doppler ultrasound, heart sounding, and angiocardiography .
Hereditary hemorrhagic telangiectasia (synonyms of the disease: Rendu- Weber- Osler disease) is a disease caused by thinning and dilatation of the walls of arterioles and capillaries. Hereditary hemorrhagic telangiectasia is transmitted by an autosomal dominant type, manifested in adults. The alleged defect is associated with impaired collagen synthesis in the vascular wall. Patients on the skin and mucous membranes of the nose, mouth, and gastrointestinal tract have characteristic vascular “spiders” of red color that disappear with pressure, which later rise above the surface and form nodules; in the lungs, arteriolovenous aneurysm of ism develops . Rupture of a dilated vessel (with mechanical compression, spontaneous) can cause hemorrhages.