Eletrocardiograma na Doença de Ebstein Electrocardiogram in Ebstein DiseaseElectrocardiogram in Ebstein Disease

Ebstein’s anomaly is a rare congenital heart disease occurring in ~1:200,000 live births and accounting for <1% of all causes of congenital heart disease. It is a malformation of the tricuspid valve and the right ventricle characterized mainly by the downward (apical) displacement of the tricuspid valve, dilation of the atrialized portion of the right ventricle with various degrees of wall thinning and hypertrophy, and dilation of the right atrioventricular junction (true tricuspid annulus) Two thirds of hearts with Ebstein’s anomaly show dilated right ventricles. The ECG is abnormal in most patients with Ebstein’s anomaly and may display tall broad P waves as a result of right atrial enlargement, as well as complete or incomplete right bundle branch blocks. The R waves in lead V1 and V2 are small. Bizarre morphologies of the terminal QRS pattern result from infra-Hisian conduction disturbances and abnormal activation of the atrialized right ventricle.