Intravenous leiomyomatosis of the uterus is a rare neoplasm characterized by nodular masses of benign smooth muscles with intraluminal growth to the inferior vena cava and, in some cases, to the heart. It may cause abdominal and cardiovascular symptoms and is a serious risk of death when it reaches the tricuspid valve. Surgery is the best treatment and must be applied as soon as possible using cardiopulmonary bypass. The authors report a new case that had cardiac involvement and was successfully resected. The symptoms, imaging diagnosis, pathological and histopathological findings, tumors that mismatched the intravenous leiomyomatosis (IVL), and the use of circulatory arrest and deep hypothermia are discussed. A review of the literature is included.
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