Malignant tumors originating from the heart are extremely rare. Here, we report a case of severe right ventricular outflow tract (RVOT) stenosis in a 67 year-old woman caused by a massive intimal sarcoma that required venous-arterial extracorporeal membrane oxygenation to support systemic circulation. Surgical resection and RVOT reconstruction with tricuspid and pulmonary valve replacement were performed.
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