Biatrial Myxoma in a Young Male Patient

Abstract

A 22-year-old man presented with low-grade fever and hemoptysis of four month duration, and a single episode of syncope. Physical examination revealed variable intensity of the first heart sound, and there was a low-pitched mid-diastolic murmur of grade 3/6, more pronounced in the tricuspid area on leaning forward. Transthoracic echocardiography revealed an echogenic mass in the left atrium, measuring approximately 33×18 mm, exhibiting a to-and-fro movement during different phases of the cardiac cycle. It was attached to the interatrial septum (IAS) by a stalk. Another similar echogenic mobile mass measuring approximately 63×28 mm was detected in the right atrium and was attached to the IAS as well (Figs. 1 and ​and2).2). He was diagnosed as a case of biatrial myxoma. Evidence of myxoma elsewhere, skin lesions and endocrine overactivity were not found. The patient was operated on an emergency basis. His postoperative recovery was uneventful. Histopathology revealed a soft tissue mass, suggestive of myxoma (Figs. 3 and ​and4).4). Echocardiographic screening in the first-degree relatives of this patient was within normal ranges. Fig. 1 Fig. 2 Fig. 3 Fig. 4 Myxoma is the most common primary cardiac tumour. Left atrium is the most common site (75%), followed by right atrium (18%), right ventricle (4%) and left ventricle (3%). Biatrial myxoma is found in less than 2.5% of all myxoma cases.2) Presence of biatrial myxoma should raise suspicion of familial myxomas, especially the Carney's complex, which was previously known as the NAME syndrome (nevi, atrial myxoma, myxoid neurofibroma, and ephelides), and the LAMB syndrome (lentigines, atrial myxoma, and blue nevi).3) Carney's complex is characterized by: 1) familial recurrent myxomas; 2) pigmented skin lesions, schwannomas, and multiple recurrent mucocutaneous myxomas; and 3) various endocrinal overactivity and neoplasms.3) If a myxoma syndrome is suspected, screening echocardiography is recommended for all first-degree relatives, particularly if the index patient is young, has multiple tumors, or has typical noncardiac features of the genetic syndrome.4)