CA6 Multiple right atrial myxomas with a patent foramen ovale. (Cardiology).

CA6 MULTIPLE RIGHT ATRIAL MYXOMAS WITH A PATENT FORAMEN OVALE. Ralph Stafford, Jr., MD, Mukta Panda, MD, and William Blalock, MD. Department of Medicine, University of Tennessee, Chattanooga.

Primary cardiac tumors are rare with a frequency of 0.001% to 0.28% in postmortem series. Myxomas comprise nearly 50% of 311 histologically benign tumors, with only 18% occurring in the right atrium, We present a rare case of multiple right atrial myxomas in a patient with a patent foramen ovale presenting with multiple paradoxical emboli. A 43-year-old woman presented with a 7-day history of sudden onset of slurred speech and right-sided weakness. She denied headache or visual changes. initially, she was seen at another facility and diagnosed with Bell's palsy. Cranial CT was normal. Two days later, the patient was seen by her PCP. Repeat cranial CT showed left CVA, and she was started on dipyridamole. Outpatient MRI/MRA showed acute 3 x 5 cm infarction of left posterior frontal lobe, anterior temporal, and insula. This was consistent with thromboembolic occlusion of M2 or M3 branch of the left MCA. Carotid ultrasound was negative. Transthoracic echo showed a large highly mobile serpiginous mass in the right atrium. The mass entered from IVC and advanced to the tricuspid valve with prolapse of the valve leaflets with no evidence of shunt. Neurologic examination revealed an alert and oriented woman with normal tone, right facial weakness, 4/5 strength, and 3+ reflexes on the right. Laboratory data were unremarkable. The patient was admitted to the ICU and taken to the OR. The findings at surgery were two pedunculated masses in the right atrium, one at the IVC and right atrial junction, one on the free atrial wall, and a large patent foramen ovale. Postoperatively, the patient was hypotensive and hypoxic with diminished pulses in both lower extremities (LE) and right upper extremity. Doppler US showed right LE DVT IVC filter was placed to prevent any further PE. The patient continued to deteriorate on three pressers and died soon after. Pathology revealed 2 bulky, gray, irregular, firm, and partially, friable 3 x 2 x 2 cm and 2.5 x 1 x 1 cm masses with a total weight of 7.4 g, suggestive of atrial myxomas. Myxomas account for nearly 50% of all histologically benign tumors and usually originate from the fossa ovalis. They are mostly found in 30- to 60-year-old women. Systemic manifestations are weight loss, fatigue, fever, and distant emboli. Examination may reveal signs of low cardiac output and systemic venous hypertension. Cyanosis may be episodic and vary with the patient's position. Laboratory data may reveal anemia; elevated ES, IL6, IgG; leukocytosis; thrombocytopenia; and polycythemia from tumor production of erythropoeitin. The EGG may be normal or reveal RA enlargement, low voltage or right-axis deviation. Chest x-ray can show enlargement of the RA and mild to moderate cardiomegaly. Echocardiogram provides excellent images of the RA, but TEE more clearly defines site of attachment. Large prolapsing atrial septal aneurysm may mimic myxoma. Embolization is more common from myxomas with irregular, papillary, frondlike surfaces. Forty percent to 50% of LA myxomas embolize to the CNS and to arteries in the kidneys and extremities. Myocardial infarction due to coronary artery embolization can be the first manifestation of cardiac myxomas. Surgery is the only acceptable treatment, but it does have a high risk of embolization. Recurrences are rare but usually occur within 48 months.