Abstract 14216: Acute Heart Failure and Mitral Stenosis in a Patient With Spindle Cell Sarcoma of the Mitral Valve

Abstract

A 27-year-old male with no past medical history presented with a productive cough, intermittent hemoptysis, dyspnea, orthopnea, and lower extremity edema. He underwent an echocardiogram that was significant for a large 6.2 cm x 3.5 cm left atrial mass moving into the left ventricle across the mitral valve concerning for a myxoma. Other pertinent findings were an ejection fraction of 50-55%, severe mitral stenosis with a mean mitral valve gradient of 23, severely reduced right heart function, and severe tricuspid regurgitation. Cardiovascular surgery was consulted for evaluation of atrial mass. He underwent left atrial mass resection. The left atrial mass was attached to the mitral valve, so the mitral valve was repaired with Onyx mechanical valve. After the surgery, there was a significant improvement in right heart function, mitral valve gradient, and velocities were within the expected range. Pathology from atrial mass back with high-grade spindle cell sarcoma so Oncology was consulted. Further studies were negative for metastatic spread. He was started on adjuvant chemotherapy and currently, he is undergoing his fourth cycle. Follow-up TEE six months later noted a left atrial mass located in or where the left atrial appendage should be approximately that was 2.5cm x 1.5cm concerning for recurrence of his sarcoma. This case demonstrates a rare primary cardiac tumor, spindle cell sarcoma, which is generally aggressive and therefore has a poor prognosis. The clinical symptoms are more related to the location of the sarcoma than the histopathology. Depending on location, cardiac sarcomas generally present with heart failure as in this patient. About 25% of cardiac tumors are malignant with the majority of those being sarcomas. Initial evaluation of primary cardiac sarcomas should include echocardiography. The primary treatment is surgical resection of the mass with adjuvant chemotherapy, however, there is a high rate of recurrence and metastatic spread.