Leiomyosarcomasare extremely rare primary cardiac tumors. Wereport a rapidly growing primary leiomyosarcoma of the right ventricle, which obstructed the right ventricular outflow tract within one monthafter symptomonset in a 68-year-old man. Two-dimensionalechocardiography was useful in diagnosing the extent and progression of the tumor. The tumor was surgically resected on an emergency basis, and the right ventricle and pulmonary artery were successfully reconstructed. Recurrence of the tumor on the right ventricle was observed, and the patient was overcomeby sudden dyspnea and died three monthsafter surgery.

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