Ebstein malformation

The Ebstein anomaly describes a congenital heart defect characterized by a twisted displacement of the tricuspid valve leaflets that cause it to reach into the right ventricle.

The symptoms vary according to the extent of attachment of the antero-superior sail to the normal opening of the valve and the severity of dysfunction of the right ventricle.

Patients who have a mild anomaly often remain symptom-free or have inspecific symptoms such as exercise dyspnoea, palpitations, fatigue, and heart murmurs. Severe affected patients suffer from cyanosis, arrhythmia and heart failure.

Very severe malformations are manifested as early as neonatal, as children often have wall-to-wall hearts. Later, supraventricular tachycardias may occur.

In some cases, Ebstein malformation is associated with Wolff-Parkinson-White syndrome and other heart defects such as pulmonary stenosis, atrial or ventricular septal defect or open ductus arteriosus botalli.

The cause of the heart defect is not known yet, but in some cases it has been linked to the mother's lithium intake during pregnancy.

The prognosis is very good for patients with mild malformations. In all other cases, there is an increased risk of heart failure due to heart failure.