Right Atrial Myxoma

 A 40 year-old woman presents with intermittent episodes of platypnea, as well as low grade fever, malaise, anorexia, and abnormal heart sounds. Her erythrocyte sedimentation rate (ESR) and C reactive protein (CRP) levels are elevated, and she has a polyclonal IgG hyperglobulinemia. A CBC is normal except for a microcytic anemia. Her serum iron and ferritin levels are normal. A recording of her internal jugular pulsations is shown below

giant A wave         heart

Recording of the patient's jugular vein pulsations showing a giant A wave         Right atrial myxoma (outer dashes) and the tricuspid valve (arrows)

DIAGNOSIS: Right atrial myxoma. The giant A wave is due to the tumor obstructing the tricuspid valve during atrial systole. Platypnea (dyspnea on assuming an erect position) can occur in patients with pedunculated atrial myxomas (particularly when left-sided) since gravity allows the tumor to drop onto or into the tricuspid or mitral valve, thereby diminishing preload. Myxomas can cause a systemic inflammatory response by producing interleukin-6 (IL-6), a pro-inflammatory cytokine; thus the findings of anemia of chronic inflammation (microcytic indexes with normal iron stores), elevated ESR and CRP, and polyclonal hyperglobulinemia in this patient. Systemic embolization of  tumor fragments occurs in up to 50% of patients with left atrial myxomas. The heart recording is that of a "tumor plop". Note that both S1 and S2 are not pure sounds (bop) but rather contain two sounds (blop (S1) - blop (S2). This is due to the tumor bobbing in and out of the right ventricle. Click here to hear the recording. The treatment is surgery.