Case Presentation: A 31-year-old female with a history of intravenous drug use presented to the hospital with bilateral lower extremity pain for 3 days. She also noted mild chest discomfort and shortness of breath. On exam, she had mottled bilateral lower extremities with decreased coloration to her toes. She underwent a computerized tomography angiogram of the abdomen with run off to the legs, which demonstrated abrupt cut off at the right popliteal artery, as well as occlusion of the left tibioperoneal trunk. Given the patient’s age, history of intravenous drug use, and the abrupt occlusions of the vessels, there was concern for a proximal embolic source. Subsequently, she was started on a heparin drip, and an echocardiogram was performed to evaluate for cardiac vegetations. The echocardiogram demonstrated multiple echodensities in the left atrium suggestive of thrombus, with severe global hypokinesis and a reduced ejection fraction of 18%. A bilateral lower extremity arteriogram revealed thrombi in the right popliteal and left posterior tibial arteries. Local thrombolytic therapy was initiated with the placement of a thrombolytic catheter; however, this therapy proved unsuccessful and was discontinued after 24 hours. A minimal hypercoagulable workup was pursued, which included testing for lupus anticoagulant and anticardiolipin antibody, the results of which were negative. The patient underwent open-heart surgery with removal of a large gelatinous mass attached to the posterior wall of the left atrium, measuring approximately six by three centimeters. Pathologic examination revealed an atrial myxoma. Three days later, an open thrombectomy of the bilateral popliteal arteries was performed. Histologic and immunohistochemical analysis of the excised masses confirmed embolized myxoma. Repeat echocardiogram showed dramatic improvement with normal systolic function and an ejection fraction of 60-65%. She improved clinically over her hospital course and tolerated guideline-directed medical therapy for heart failure.
Discussion: Primary cardiac tumors are extremely rare, with atrial myxomas being the most common, accounting for approximately 50%. Presenting symptoms are often similar to infective endocarditis. Although considered benign tumors, secondary complications, including embolization, can be devastating. Embolization occurs in up to 30% of atrial myxomas. Peripheral embolization resulting from tumor fragmentation can give rise to emboli large enough to cause vascular occlusion and acute peripheral artery ischemia. This presentation, with a known history of intravenous drug use, led to high clinical suspicion for endocarditis with septic emboli, however, in a younger female patient presenting with acute arterial ischemia, the diagnosis of embolic myxoma should be considered.
Conclusions: Atrial myxomas can often mimic other cardiovascular diseases and demand a high degree of suspicion for diagnosis. This is particularly important when younger patients present with acute peripheral artery ischemia in the absence of known cardiovascular risk factors. This case illustrates the importance of a high index of suspicion for atrial myxoma, prompt diagnosis with imaging, and early surgical consultation for resection to prevent potentially devastating complications.