Intraventricular smooth muscle disease is a rare neoplastic disease. The tumor originates in the uterus or in the venous wall of the pelvis outside the uterus. The tumor may protrude into the venous channels of the uterus or pelvis, extend and expand to the inferior vena cava and right atrium via the iliac or ovarian veins, or even enter the right ventricle and pulmonary artery via the tricuspid valve, resulting in syncope or sudden death due to severe circulatory disturbance. The clinical treatment is mainly surgical, and surgical resection is effective. To date, there are about 100 cases of cardiac involvement reported in the literature [NO6], and five cases of intraventricular smooth muscle tumors extending into the right heart cavity were admitted to our hospital. The diagnosis and treatment of this disease are discussed by synthesizing the literature reports and our experience. Data and methods General information Five patients with intraventricular smooth muscle tumor with cardiac involvement were admitted to Peking Union Medical College Hospital from January 2001 to January 2009. 5 cases were female, age 41-49 years; weight 60-80 kg. 3 cases had NYHA class III cardiac function and 2 cases had class II cardiac function. 5 patients had varying degrees of panic, chest tightness, and syncope, and the duration of the disease ranged from 4 days to 11 months. The duration of the disease ranged from 4 days to 11 months. There were 2 cases of bilateral lower limb edema and 2 cases of hepatomegaly. Four cases had a history of hysterectomy for uterine fibroids and one case had a history of left ovarian teratoma removal; three cases had pelvic masses. One case originated from the left and one case from the right genital vein, two cases from the left internal iliac vein, and one case from the right internal iliac vein. two cases had preoperative ureteral DJ tube implantation. Surgical technique: The first stage surgery is a complete resection of the uterus, adnexa, pelvic residual recurrent tumors, and intracardiac and intraventricular smooth muscle tumors in one stage. It is important to fully expose the inferior vena cava of the posterior hepatic segment by first dissecting the free femoral vein for extracorporeal circulation cannulation and then dissecting the free inferior vena cava, hepatic vein, renal vein, ovarian vein and iliac vein through an anterior median thoracoabdominal incision. Extracorporeal circulation is established by venous cannulation through the superior vena cava, and femoral vein or right atrium, and arterial cannulation through the ascending aorta. Vena cava and intracardiac mass resection or extraction, and vascular or valve repair or replacement are then performed without the heart beating, or in arrest, or with deep hypothermic arrest of extracorporeal circulation. Additional bilateral adnexal, hysterectomy and pelvic recurrent masses after or before extracorporeal circulation. In stage 2 surgery, resection or extraction of the inferior vena cava and intracardiac masses is performed under extracorporeal circulation. Four weeks later, elective resection of the uterus, adnexa, and pelvic remnant or recurrent smooth muscle tumors was then performed in the conventional transabdominal setting. Two patients underwent first-stage tumor resection under cardiac arrest and second-stage abdominal surgery, while one patient underwent emergency extracorporeal circulation and removal of the tumor from the right atrium under parallel circulation because the tumor was dislodged into the right heart after simultaneous abdominal tumor resection. The average extracorporeal circulation was (185 ± 30) min. 12,30 min of downtime and 12 h-15 d of postoperative ventilator assistance. no brain injury, phrenic nerve palsy ureter, or abdominal organ injury in the whole group. 1 to 8 years of postoperative follow-up, 4 cases had improved cardiac function to class I and 1 case to class II, and the quality of life was significantly improved. In one case, a recurrent pelvic tumor was detected by ultrasound 3 months after surgery, and the patient was advised to continue taking the medication, but he refused. After 5 years of follow-up, no significant abnormalities were found in the right atrium and iliac vessels, and the pelvic mass did not increase significantly. The patient has no conscious symptoms and is still being followed up. There was no other tumor recurrence. One case was revascularized without vascular graft. Discussion: Imaging can help in the early diagnosis of IVL. The diagnosis can be made by a combination of abdominal B-ultrasound and cardiac ultrasound, enhanced CT, MRI examinations, and can provide sufficient information for surgical design. MRI is generally considered to be a better adjunctive examination. MRI is superior to CT because it can not only clarify the extent of the lesion, but also show the characteristics of the tumor in the blood vessels, whether there are adhesions with the canal wall and the site of adhesions; it also has a guiding significance for determining the scope and method of surgery. However, due to the low cost of CT and its popularity in hospitals, CT is more commonly used in clinical practice. We believe that CT can effectively and conveniently monitor the recurrence and growth of postoperative tumors. The condition of abdominal tumor can be clarified by abdominal ultrasound. Only a few cases still require inferior vena cava and right atrial angiography. The disease needs to be differentiated from mucinous tumors, Buga syndrome, thrombosis in the inferior vena cava, as well as from uterine smooth muscle sarcoma, renal cancer and hepatocellular carcinoma extending into the inferior vena cava. Surgical resection of tumors can not only eliminate symptoms but also prevent tumor recurrence [N10-5,6], and surgical radical treatment can be done in one stage or in two stages. Lam reported that approximately 70.6% of IVL was completely resected, of which 60.4% were treated with staged surgery and the remaining 39.6% with one-stage surgery [3-1]. In recent years, with improved understanding of the pathological anatomy of the disease and advances in extracorporeal circulation techniques, an increasing number of patients are undergoing staged surgery for radical treatment, which avoids complications such as tumor embolism, tumor development and hemodynamic disturbances during the pre- and postoperative interval, as well as the risk of a second surgery. The presence of estrogen receptors in the tissue of intravascular smooth muscle tumors can contribute to tumor growth and recurrence by the action of estrogen in the body, thus also affecting their prognosis. The relevant literature reports some therapeutic value for patients with incompletely resected tumors [3-10]. In conclusion, intraventricular smooth muscle tumor is a rare benign tumor, and ILV can lead to sudden death once it extends into the heart chambers, and timely surgery is the best treatment. Thorough preoperative examination and preparation is the key to surgical eradication of the disease, while the diagnosis and treatment of the disease requires multidisciplinary collaboration to accomplish. Postoperative adjuvant treatment with anti-estrogen is necessary to control tumor recurrence and inhibit tumor growth.