Late Diagnosis of Ebstein’s Anomaly Following Ventricular Septal Defect Repair in Infancy

Ebstein’s anomaly (EA) is a rare congenital heart defect characterized by apical displacement of the tricuspid valve (TV) leaflets into the right ventricle, leading to atrialization and dysfunction. While typically diagnosed in infancy or childhood, a subset of patients may present with symptoms only in adulthood. We report a case of a 23-year-old woman who presented with palpitations and was diagnosed via echocardiography with significant EA (3 cm displacement) with moderate regurgitation and frequent atrial and ventricular ectopy on electrocardiogram. Uniquely, she had a history of perimembranous ventricular septal defect (VSD) successfully repaired via thoracotomy at one year of age, with no mention of TV abnormality in the perioperative records. This timeline suggests the initial EA may have been very mild or "occult," progressing to hemodynamic significance over two decades following VSD repair. This case underscores the necessity of lifelong, specialized cardiac follow-up for adults with repaired congenital heart disease, even when the initial repair was considered "complete." Furthermore, we discuss the potential embryological link between VSD and EA, the diagnostic challenges of late-presenting EA, and the management strategies for associated arrhythmias. This report aims to increase awareness of EA diagnosed de novo in adulthood, particularly in patients with a history of prior cardiac surgery.