Abstract: Objective: To summarize the diagnosis and surgical treatment of intravenous leiomyomatosis with right intracardiac extension in 5 patients. Methods:From January 2001 to January 2009, 5 female patients diagnosed with intravenous leiomyomatosis with intracardiac extension underwent surgical treatment using circulatory bypass, with single-staged surgical resection in 3 patients and two-staged surgical resection in 2 patients. The total circulatory arrest time in the 2 patients was 29 and 43 minutes. Physical examination revealed severe congestive failure with signs of hepatomegaly, ascites or lower extremity edema in 2 patients. A pelvic mass was present in 3 cases. Four patients had a history of laparotomies due to uterine leiomyoma or gonadal teratoma, with an interval of 15to 36months between the performance of lapratomy and the removal of the IVL. Imaging findings showed intracaval and intracardiac masses in all 5 patients, originating from the left gonadal vein in1 patient, right gonadal vein in1 patient, the left internal iliac vein in2 patients and the right internal iliac vein in 1 patient. Prior to surgery, the 2 patients with tumors originating from the gonadal veins received the placement of ureteral double J stents into the ipsilateral ureters. Results: Of the 5 patients, 2 patients underwent successful two-stage surgical removal of IVL and3 patients had complete one-stage surgical treatment, with 2 patients under deep hypothermic circulatory arrest. The total circulatory arrest time was 29 and 43 minutes in the 2 patients. All patients were followed up for 1~72months after surgery. All patients had uneventful recoveries and no deaths occurred. Four patients were doing well with no signs of recurrence. A pelvic mass was found by ultrasound in the fifth patient at3 months after surgery. This patient had experienced no symptoms and no intravenous mass in spite of rejecting any treatment. Three patients received hormone therapy. No obstruction occurred in the 3 patients with inferior cava ventomy. Postoperative NYHA classification was Ⅰin 3 cases and Ⅱin 2 cases. Conclusion:Leiomyomatosis should be suspected among patients with neoplasm in the right atrium and a history of hysteromyoma. Successful therapy for intravenous leiomyomatosis is dependent on open heart surgical excision of the tumor and laparotomy including hysterectomy, salpingo-oophorectomy and removal of residual or recurrent pelvic mass.