Ebstein's Anomaly in a Young Nigerian Lady

Ebstein's anomaly (EA) is a rare congenital heart disease with a wide spectrum of presentation from the womb to adulthood characterized by apical displacement of tricuspid valve leaflets into the right ventricle. This is due to defects in embryological processes of uncertain etiology.

A 28-year-old woman who presented with palpitations from childhood occurring in short paroxysms daily and limiting her from strenuous activities. Physical examination findings revealed a small stature with no associated birth defects. The cardiovascular system revealed tachycardia, a small volume irregular pulse with a split S2 heart sound. ECG showed premature atrial complexes, fragmented QRS with Sodi-Pallares sign, and echocardiographic features in keeping with atrialization of right ventricle with apical displacement of septal leaflet of the tricuspid valve into the right ventricle in keeping with EA. The patient was placed on anti-arrhythmics and anticoagulants.

The diagnosis of EA in adults is often delayed in low-resource settings for multifaceted reasons. However, medical therapy offers a conservative measure to preserve life while surgical correction is advocated for the majority of cases when picked up early.