Atrialised right ventricular myxoma in a patient with Ebstein’s anomaly

Ebstein’s anomaly is a rare entity affecting around 1 in 200,000 live births and accounts for less than 1% of congenital heart diseases. Ebstein’s anomaly with an associated right-sided myxoma is extremely rare, with only one other case report found in the literature. Previous reports have also noted cases of Ebstein’s anomaly associated with left-sided myxomas. We describe a female patient with, to our knowledge, the first case of a histopathologically confirmed right ventricular myxoma in the setting of Ebstein’s anomaly. LEARNING POINTS: Tall p waves (Himalayan) are associated with Ebstein’s anomaly. Large a-waves on clinical evaluation are typical of tricuspid valve obstruction or restrictive right ventricular filling. The presence of a stalk is suggestive of a myxoma. Before undertaking surgery, careful evaluation of involved structures including valvular morphology and function is essential.