Ebstein anomaly

Ebstein's anomaly (ICD-10: Q22.5) is a congenital cyanotic heart defect characterized by a change in the position of the tricuspid valve (shifted to the cardiac tip), papillary muscle defects, and is often associated with atrial septal defect. The insertion of the septal tip of the tricuspid valve is shifted to the cardiac tip. This leads to enlargement of the right atrium and reduction of the right ventricle, atrialization of the right ventricle, tricuspid valve is insufficient, which eventually leads to right-sided heart failure.

In the diagnosis, echocardiography (prenatal and postnatal) serves as a method of choice, but some ECG changes are also typical and there is an auscultative finding: systolic regurgitation murmur above the lower sternum from tricuspid regurgitation.

Therapy
Asymptomatic patients must be monitored, surgical intervention is not required. Treatment of the defect in symptomatic patients (i.e. developing right-sided cardiac insufficiency, development of arrhythmias, worsening symptoms such as dyspnoea) is surgical. Its essence is the closure of the atrial septal defect and the replacement or plastic surgery of the tricuspid valve.

Related Articles

 * Congenital heart defects
 * Acquired heart defects
 * Heart murmurs