Ebstein Anomaly With Right Atrial Clot

Ebstein anomaly (EA) is a rare congenital malformation of the tricuspid valve (TV), often associated with other cardiac malformations, especially atrial septal defect/patent foramen ovale (PFO) which is present in 80-90% of patients and predisposes to paradoxical embolization. We describe the case of a 17-year-old female, who presented with worsening exertional dyspnea, fatigue and pedal edema and atrial fibrillation (AF). Transthoracic echocardiography showed EA with severely dilated right atrium (RA), small functional right ventricle (RV), low velocity flow across TV with spontaneous echo contrast and giant clot in RA. Fortunately for the patient, contrast and transesophageal echocardiography revealed an intact interatrial septum with no PFO preventing any paradoxical embolism from large clot in RA, more so in the background of AF. Important differential diagnosis of congenitally unguarded TV orifice was ruled out due to presence of septal and anterior leaflets of TV and associated chordae.