Ebstein's anomaly
Contents:
Description:
Ebstein's anomaly belongs to rare inborn heart diseases. It meets frequency of 1% from all inborn malformations of heart. For the first time it was described in 1866 by the pathologist Ebstein.
Pathogeny:
At this defect of a shutter of the right atrioventricular valve are displaced in a cavity of a right ventricle. The right ventricle because of it has the reduced size that leads to reduction of amount of the blood which is thrown out by it and to depletion of a blood-groove in lungs. At the same time a part of a venous blood through an opening in the changed valve gets to the right auricle. The right auricle increases in sizes and extends. If at the same time still there is an opening in interatrial partitions a part of a venous blood gets to the left auricle and mixes up from arterial. It leads to reduction of oxygen in blood and oxygen insufficiency of bodies and fabrics. Sometimes the right ventricle reaches the sizes to 2,5 liters.
Hemodynamics at Ebstein's anomaly
Symptoms of anomaly of Ebstein:
Gradually heart failure which becomes a cause of death of the patient accrues. Very often this defect is followed by disturbances of a rhythm of cordial reductions. About 30% of patients die at early children's age. Up to 50 years live only 5%. More than a half of patients die suddenly because of the developed disturbances of a rhythm. At newborns small cyanosis (cyanosis) and increase of breath is observed. Later cyanosis decreases. At achievement of age of 20 years all patients complain of pains in heart. More than 80% of patients have disturbances of a rhythm of cordial reductions. "drum sticks" and "hour glasses" are found.
Diagnosis:
When listening heart find weakening of cardiac sounds, sometimes "cantering rhythm" - three tones instead of two. This defect can be diagnosed already according to the electrocardiogram, it by the main method serves an echocardiography.
Treatment of anomaly of Ebstein:
If the patient does not show complaints and a state its good, operation is not shown. At emergence of complaints or symptoms of heart failure operation is made. Do to children up to 15 years valve plastics. To patients 15 years are more senior make prosthetics of the right atrioventricular valve. Operation is carried out in the conditions of the cardiopulmonary bypass.
Mortality at these operative measures makes from 8 to 50% and depends on degree of manifestation of defect and experience of the surgeon. Results of operational treatment good in 90% of cases. In a year after operation recovery of working capacity at the patient is possible.
