Abstract
BACKGROUND: Ebstein anomaly (EA) is a rare congenital abnormality of the tricuspid valve which can lead to progressive right heart dilatation and arrhythmias. While often seen in conjunction with other congenital cardiac lesions, such as atrial septal defects, it is not normally associated with atrial myxomas. CASE SUMMARY: We present a case report of an incidental finding of a right atrial myxoma in the context of undiagnosed EA, in a 16-year-old male who presented with appendicitis. Subtle cardiomegaly on routine chest X-ray prompted further investigation, which demonstrated characteristic findings of both conditions and culminated in surgical repair using the Cone procedure. At 4-month follow-up, the patient was asymptomatic, and transthoracic echocardiography demonstrated a mean gradient of 4.5 mmHg across the tricuspid valve with mild regurgitation. DISCUSSION: The combination of EA with right-sided myxoma is exceedingly rare, and, in this case, it may be that the apical displacement of the tricuspid valve was protective against right atrioventricular obstruction. We are reminded that although subtle abnormalities on routine investigations can be of limited significance, they can also indicate more serious underlying pathology and so consideration should be given to an appropriate cascade of further investigations to yield a timely diagnosis and enable prompt treatment.