Valve defects: Ebstein’s anomaly

Lesions valvulars: anomalia d'Ebstein a Vall d'Hebron

It is a congenital heart defect characterised by the tricuspid valve sitting lower than normal over the ventricular myocardium, caused by the corresponding atrioventricular ring. In this case, the right atrium greatly increases its volume and the right ventricle greatly reduces in size and lung flow is not sufficient.

Ebstein’s anomaly encompasses a wide range of defects characterised by different grades of displacement and adherence of the septal valve of the tricuspid valve to the right ventricle chamber. It is a very variable disease that may be severe or mild.

Description

Symptoms

The most serious cases are accompanied by severe cyanosis and congestive heart failure.

In less serious cases, the disease results in a transient period of cyanosis.

Who is affected by Ebstein's anomaly?

This is considered a rare disease and only represents 3% of congenital heart defects. It affects one in every 20,000 live births.

Diagnosis

An echocardiogram is used to detect the disease, and to find out if it is associated with anatomical or functional atresia of the pulmonary valve.

Typical treatment

The defect is treated with surgery which varies depending on the clinic, the anatomical form of the defect and the age of the patient.

In the case of new-born babies, for example, it is necessary to operate as soon possible. In adolescents or adults with symptoms, it is advisable to repair or replace the tricuspid valve with a prosthetic. If patients have no symptoms the best option is to wait.

Following surgery, 80% of patients diagnosed as teenagers or adults have a very good short to mid term prognosis as their ability to function has improved.