Atrezium tricuspid valve

Atrezium tricuspid valve - this is the absence of tricuspid valve, combined with hypoplasia of the right ventricle. Combined anomalies occur frequently, including the defect of the atrial septum, the defect of the interventricular septum, the open arterial duct and the transposition of the main vessels. Symptoms of tricuspid valve atresia include cyanosis and manifestations of heart failure. II tone is single, the nature of the noise depends on the presence of concomitant defects. The diagnosis is based on echocardiography or cardiac catheterization. Radical treatment is surgical correction. It is recommended to prevent endocarditis.

Atrezium tricuspid valve accounts for 5-7% of all congenital heart defects. In the most common type (approximately 50%), an interventricular septal defect (VSD) and pulmonary artery stenosis are present, and the blood flow in the lungs is reduced. At the level of the atrium, an adequate discharge of blood develops, causing the appearance of cyanosis. In the remaining 30% there is a transposition of the main vessels with a normal pulmonary valve, and blood in a small circle of circulation comes directly from the left ventricle, as a result, usually develops heart failure.

[1], [2], [3], [4], [5]

Symptoms of tricuspid atresia

At birth, pronounced cyanosis is usually observed. Symptoms of heart failure may appear at the age of 4-6 weeks.

Physical examination usually reveals a single II tone and a systolic or early systolic noise of intensity 2-3 / 6 of the defect of the interventricular septum in III-IV intercostal space to the left of the sternum. Systolic jitter is rarely determined in the presence of pulmonary artery stenosis. Diastolic murmur at the top is heard, if pulmonary blood flow is increased. With long-lasting cyanosis, fingers can be formed in the form of tympanic rods.

Diagnosis of tricuspid atresia

The diagnosis is made on the basis of clinical data, taking into account the chest radiograph and ECG, an accurate diagnosis is established based on two-dimensional echocardiography with color Dopplercardiography.

In the most common form on the roentgenogram, the heart is of normal size or slightly enlarged, the right atrium is enlarged and the pulmonary pattern is impoverished. Sometimes the shadow of the heart reminds one of the tetralogy of Fallot (heart in the shape of a boot, narrow waist of the heart due to the segment of the pulmonary artery). Pulmonary pattern can be strengthened, as well as cardiomegaly in infants during transposition of the main vessels. The ECG is characterized by a deviation of the electric axis of the heart to the left and signs of left ventricular hypertrophy. Hypertrophy of the right atrium or hypertrophy of both auricles also occur frequently.

Cardiac catheterization is usually necessary to clarify the anatomy of the blemish before surgery.

[6], [7], [8]

Treating tricuspid atresia

Newborns with pronounced cyanosis are given an infusion of prostaglandin E1 [0.05-0.10 μg / (kgxmin)] in order to reopen the arterial duct prior to planned cardiac catheterization or surgical correction.

Balloon atrial septostomy (Rashkind surgery) can be performed as part of the primary catheterization to increase the discharge of blood from the right to the left if the interatrial message is inadequate. Some newborns with transposition of major vessels and signs of heart failure require drug treatment (eg, diuretics, digoxin, ACE inhibitors).

Radical treatment of tricuspid atresia includes a gradual correction: shortly after birth, the anastomosis of BlalockTaussig (connection of the artery of the large circulation and pulmonary artery by means of the tube GoreTex); at the age of 4-8 months, a bi-directional bypass surgery is performed - Glenn operation (anastomosis between the superior vena cava and right pulmonary artery) or hemiFontan operation (creating a bypass of the blood flow between the superior vena cava and the central part of the right pulmonary artery by forming an anastomosis between the right eye lobe atrium and pulmonary artery with the help of a patch sutured in the region of the upper right atrium); By the age of 2, a modified Fontan operation is performed. This approach increased early postoperative survival to more than 90%; Survival after 1 month is 85%, 5-year survival is 80% and 10-year survival is 70%.

All patients who have tricuspid valve atresia, regardless of whether a correction of the defect was performed or not, should receive endocarditis prophylaxis before dental or surgical procedures in which bacteraemia may develop.