Ebstein's anomaly

Ebstein 's anomaly ( Ebstein 's disease ) is a very rare congenital heart malformation. The septal and often the posterior leaflets of the tricuspid valve ( between the right atrium and right ventricle = ventricle ) are displaced toward the heart tip and the valve leaflets are misshapen. Show large differences in the structure. The valve ring is not displaced. Due to the low position of the flaps is the ventricle that serves the pulmonary circulation, reduce and enlarge the atrium. In addition, there is usually an open connection to the atrium level in the form of a Atriumseptumdefektes (ASD ) or a patent foramen ovale.

Effects

By flaps malformation occurs in an incomplete valve closure ( tricuspid regurgitation ) with a return flow of blood into the right atrium. This blood is missing the one in the pulmonary circulation and loaded the other hand, in terms of volume in both the atrium and the ventricle. As a result, there is an enlargement of the right heart and to a reduction in cardiac function ( right heart failure ). At the same time existing ASD oxygen-poor blood flows into the left atrium and leads to cyanosis.

Cardiac arrhythmias can occur as a complication, but sometimes are congenital ( Wolff- Parkinson -White syndrome ).

Clinic

The clinical signs of right heart failure are dependent on the severity of valve malformation, the function of the right ventricle, the size of the ASD and the cardiac arrhythmias frequently occur. The children are short of breath, suffer from poor feeding and failure to thrive. It can edema, a swelling of the liver and ascites occur.

Diagnostics

The diagnosis is first used echocardiography and electrocardiogram ( ECG) for the detection of right heart strain. To further diagnostic cardiac catheterization is often performed.

Causes

The exact causes of malformation are still unclear, for example, took you a long time that a taking lithium preparations during pregnancy poses an increased risk to the fetus. Recent analyzes have this relationship but no longer clearly confirm.

Therapy

Drug therapy is the main treatment. With progression of the disease signs the tricuspid valve is corrected using a heart -lung machine that a functional tricuspid valve is formed. In some cases, a patient an artificial heart valve or a homograft (human donor valve) must be used. Timing and extent of surgery will be determined individually for each patient, in very rare cases is already an operation in the neonatal period is necessary.

On a careful endocarditis is important for life.