Tricuspid valve anomaly (Ebstein's anomaly): symptoms, diagnosis, treatment

Ebstein's anomaly (an anomaly of the tricuspid valve) is a congenital pathology of the tricuspid valve, characterized by the displacement of the valves (most often septal and posterior) into the cavity of the right ventricle, which leads to the formation of the atrialized part of the right ventricle. As a result of the displacement of the valves of the tricuspid valve, the cavity of the right ventricle is divided into two parts. As the right ventricle, only the lower part, located under the valve, consisting of the trabecular and excretory divisions, functions. With moderate shifts of valves and regurgitation, circulatory disturbances are minimal, and the defect can be asymptomatic for a long time. Possible discharge of blood through the defect of the interatrial septum or through an open oval window. This causes symptoms of arterial hypoxemia. Volumetric overload of the right divisions leads to a swelling of the interventricular septum to the left, which limits the filling of the left ventricle, possibly the development of congestive heart failure. In the natural course of the disease, the prognosis depends on the degree of dysfunction of the tricuspid valve, and also on the degree of hypoplasia of the right ventricle. About a quarter of children die within the first month of life, in unoperated patients the cause of death is progressive heart failure. The frequency of the defect is 0.4% among all congenital heart anomalies. Family cases of the Ebstein anomaly are possible.

Symptoms of the anomaly of the tricuspid valve (Ebstein's anomalies) depend on the degree of hemodynamic disturbance. In the presence of a defect of the interatrial septum, a leading sign is cyanosis, the severity of which depends on the pressure in the right atrium and the volume of blood discharged through the atrial atrial communication. Over time, signs of heart failure increase, tolerance to physical activity decreases. Possible formation of a heart hump due to the large size of the right atrium and right ventricle. Auscultative changes are not bright. With tricuspid insufficiency, a severe systolic murmur is heard, in the presence of stenosis of the right atrioventricular orifice, there is presystolic or mesodiastolic murmur at the right edge of the sternum. The intensity of noise increases in the inspiratory phase, which indicates their connection with the lesion of the tricuspid valve. With a significant dilatation of the right atrium and ventricle, there are violations of the heart rhythm. Paroxysmal supraventricular tachycardia is detected in 25-50% of patients, Wolff-Parkinson-White syndrome - in 14%.

Electrocardiographic examination often helps in the diagnosis of Ebstein's anomaly. As a rule, the electric axis of the heart is sharply deflected to the right, show signs of incomplete or complete blockade of the right leg of the bundle with a low amplitude of the teeth R and S.

On the roentgenogram of the chest, the heart has a globular configuration, increased mainly due to the right atrium and the "atrialized" part of the right ventricle. The pulmonary pattern is normal or poor.

With echocardiography in the projection of four chambers, attention is drawn to the displacement of the septal flap into the cavity of the right ventricle. An open oval window or an atrial septal defect detects in 85% of cases. With the help of doppler echocardiography, the magnitude of tricuspid insufficiency is revealed.

Cardiac catheterization is performed to assess the pulmonary bed and electrophysiological examination for heart rhythm disturbances.

Treating the anomaly of the tricuspid valve (Ebstein abnormality)

Patients with moderate or minor tricuspid regurgitation require constant monitoring of the cardiologist, electrocardiographic control. Surgical treatment is indicated for heart failure, arterial hypoxemia, refractory to heart rhythm disorders.

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