Intravenous leiomyomatosis with extension to the heart: echocardiographic features: a case report

A 40-year-old female was admitted to the hospital for an operation with a presumptive diagnosis of right atrial myxoma. She had had a hysterectomy for leiomyoma 8 months earlier and has since experienced progressive dyspnea on exertion, pedal edema, and two syncope episodes in the past 2 months. Cardiac murmur was detected and two-dimensional echocardiography was arranged. A mobile right atrial mass was discovered, which was thought to be a myxoma. The patient subsequently received open heart surgery. Histologic examination of the resected mass confirmed intravenous leiomyomatosis. Magnetic resonance imaging performed on the eighth postoperative day revealed an intravascular mass from the right common iliac vein to the upper abdominal inferior vena cava. The patient underwent a second operation one and half months after the first operation, with resection of the tumor mass in the inferior vena cava and resection of multiple uterine myoma. She recovered well. Repeat magnetic resonance imaging 6 months following the second operation showed that the inferior vena cava and right atrium were clear of tumor.     

Surgery of hepatoma with intracavitary cardiac extension

Summary A case of primary liver carcinoma with intracavitary cardiac extension is presented. A 36-year-old female was admitted to our surgical clinic with dyspnea and generalized edema. Echocardiography and superior vena cavography demonstrated a large filling defect in the right atrium. After a diagnosis of acute cardiac failure due to an intracardiac tumor, the patient was operated upon immediately. A right atriotomy exposed a large yellow mass within the right atrium, which was not adherent to the atrial wall. The mass was in continuity with similar material in the inferior vena cava and right hepatic vein. With a suspicion of hepatic malignancy, the atrial tumor was removed, and debulking of the mass in the inferior vena cava and right hepatic vein was performed. A postoperative histological examination of the tumor showed hepatocellular carcinoma. Her postoperative course was uneventful, and she was discharged from the hospital.Intracardiac extension of hepatoma is rarely encountered. In this clinical setting, long-term survival cannot be anticipated from any surgery, but palliative clearing of the atrium and inferior vena cava may be of value in preventing cardiac arrest causing sudden death.     

Budd-Chiari syndrome as a late complication following closure of an atrial septal defect

A case of a Budd-Chiari syndrome in a 19-year-old female patient is reported who had undergone surgical closure of a secundum atrial septal defect 13 years before. 8 months before the development of the Budd-Chiari syndrome she started to take oral contraceptives. The clinical picture of the Budd-Chiari syndrome developed within several days. The inferior vena cava did not fill with contrast dye when an angiography was performed using the right vena iliaca approach. The contrast dye disappeared through collateral veins (vena azygos, vena hemiazygos). After 4 days of treatment with systemic streptokinase she underwent open-heart surgery. The orifice of the inferior vena cava was occluded to a diameter of 6 mm. No thrombi were found. The lesion was corrected with two patches, one in the right atrium and the other in the inferior vena cava. This case report demonstrates that a Budd-Chiari syndrome is a possible late complication after closure of an atrial septal defect which should be treated by surgery.     

Intracardiac extension of intravenous leiomyoma

Intravenous leiomyoma with intracaval and intracardiac extension is a rare benign neoplasm affecting women. We report a case in which an intravenous leiomyoma originated from the right iliac vein and extended through the inferior vena cava into the right atrium. The correct diagnosis was made intraoperatively, after resection of the intracardiac extension of the tumor, which was 28 cm in length. At the 2nd stage of the operation, a right common iliac venotomy enabled the removal of all residual tumor from the inferior vena cava.     

Intravascular leiomyomatosis: report of a case and review of the literature

So far, only 13 cases with extension of an intravascular leiomyoma into the heart have been reported. We present the second known case of leiomyomatosis with extension of the tumor through the tricuspid valve into the right ventricle, which was first removed successfully. Ten years after an abdominal hysterectomy for a myomatous uterus, a 53-year old woman presented with a intracavitary mass in the right-sided heart chambers which could be followed retrogradely into the inferior vena cava and right pelvic veins, representing an intravasal extension from the pelvic leiomyoma. The clinical picture, the two-stage operative management, and the postoperative course are reviewed and the literature on the subject is presented.     

Two-dimensional echocardiographic abnormalities of right atrial metastatic tumors in hepatoma

We describe two patients suffering from hepatoma who presented with right atrial metastatic tumors as a result of invasion of the inferior vena cava and extension into the right atrium. Two-dimensional echocardiographic studies detected the right atrial tumor during life in both patients and the invasion of the inferior vena cava in one patient.     

Contrast echocardiography of right atrial mass due to hepatocellular carcinoma

We describe one patient suffering from hepatocellular carcinoma who presented with a right atrial metastatic tumour as a result of invasion of the inferior vena cava and extension into the right atrium. Two-dimensional echocardiography disclosed the right atrium tumour and SonoVue contrast agent echocardiography was employed to assess the local extension of the mass and to exclude an important obstruction due to a mass in the right ventricular inflow. Surgical management in the presence of metastatic right atrial tumour thrombus is described in the literature with poor results and this type of treatment should be reserved only for selected cases in which acute and severe cardiovascular distress due to obstruction of right ventricular inflow is present.     

Bilateral compartment syndrome as a result of inferior vena cava filter thrombosis

A 54-year-old man with an inferior vena cava filter in situ presented to the emergency department (ED) by emergency medical services, with acute onset of severe abdominal, lower back, and leg pain. He had fallen from a ladder 3 days before admission. An abdominal computed tomography scan revealed a large retroperitoneal hematoma and evidence of occlusive thrombus in the inferior vena cava, extending beyond the inferior vena cava filter. The occluded inferior vena cava filter caused increased venous pressures and compartment syndrome in the lower extremities. Measurement of compartment pressures in the ED revealed increased pressures exceeding 60 mm Hg in both calves and 75 mm Hg in the thighs. The patient underwent bilateral fasciotomies of the lower extremities within 3 hours. Postoperatively, he developed extensive tissue necrosis and gangrene, requiring bilateral above-the-knee amputations, and acute renal failure associated with severe rhabdomyolysis, requiring hemodialysis. This case highlights the importance of prompt recognition and treatment of inferior vena cava filter thrombosis.     

Intravenous Leiomyomatosis with Intracardiac Extension: Echocardiographic Study and Literature Review

Uterine leiomyomatosis is a common disease in women; however, intravenous leiomyomatosis with intracaval and intracardiac tumor extension is rare. We sought to analyze the clinical and echocardiographic features of intracardiac leiomyomatosis.From January 2003 through July 2012, 7 women (age range, 24–59 yr) underwent surgical resection of histopathologically diagnosed intracardiac leiomyomas at our hospital. Most of the patients had histories of hysterectomy or uterine leiomyoma. We retrospectively analyzed their preoperative echocardiograms. We found that the tumors had no stalks, did not adhere to the wall of the right side of the heart, were highly mobile, and moved back and forth in the right atrium near the tricuspid orifice. All tumors originated from the inferior vena cava and had borders well demarcated from that structure''s wall. Most of the masses extended into the inferior vena cava and right atrium through the right internal and common iliac veins. Computed tomograms revealed pelvic tumors and contiguous filling defects in 6 patients.When echocardiograms reveal a right-sided cardiac mass that originates from the inferior vena cava, particularly in women who have a history of hysterectomy or uterine leiomyoma, intracardiac leiomyomatosis should be suspected. If the mass has no stalk and freely moves within the inferior vena cava and right-sided cardiac chambers without attachment to the endothelial surface or endocardium, intracardiac leiomyomatosis should be diagnosed. We discuss our findings and briefly review the relevant medical literature.     

Kidney tumour mimicking cardiac mass

The discovery of a mass in the right atrium obliges the clinician to perform a broad differential diagnosis between a primary cardiac tumour (with myxoma being the most frequent), invasion of an extracardiac tumour, vegetations on the tricuspid valve, and an atrial thrombus. We describe the case of a patient who was admitted to our service with a diagnosis of suspected myxoma based on the chance transthoracic echocardiographic discovery of a right atrial mass. A transesophageal echocardiogram showed the process to be extracardiac, and magnetic resonance imaging showed it to originate at the renal level extending via the inferior vena cava to the right atrium. Tumour extension with thrombosis of the vena cava is a relatively frequent complication of renal carcinoma, but only exceptionally does it reach the right atrium. It is also exceptional that this was a chance finding in an asymptomatic patient.     

Leiomyosarcoma of the inferior vena cava--a very rare case report.

The authors present a case report of a patient with abdominal pain that began 6 months before hospital admission. Ambulatory abdominal echography and computed tomography (CT) revealed partial thrombosis of the inferior vena cava (IVC) with right atrial extension. During hospitalization, magnetic resonance imaging (MRI) revealed aspects suggesting a tumoral lesion of the right atrium, rather than a thrombus, with tumoral extension to the IVC. The echocardiogram showed images suggesting a right atrial tumor. Transesophageal echocardiography confirmed the diagnosis. During surgery, an IVC tumor was found invading the right atrium, which histopathology confirmed as a leiomyosarcoma. The authors present this case because this type of tumor is rare (21 cases worldwide at this anatomic site), it is difficult to diagnose, and its management has not been adequately described. The authors review the literature relevant to this case.     

Aortic and vena caval reconstruction with retroperitoneal lymph node dissection for metastatic germ cell tumor

A 49-year-old man who had a huge testicular tumor with retroperitoneal lymph node metastasis and bilateral multiple pulmonary metastases was referred to our hospital. Firstly orchiectomy was done obtaining the pathological diagnosis of mixed type germ cell tumor. After cisplatin-based chemotherapy, he underwent resection of the retroperitoneal lymph node involving the abdominal aorta and the inferior vena cava. Both great vessels were resected with the tumor and reconstructed with prosthetic grafts. Two months after the laparotomy, 12 metastatic nodules in the left lung were resected. Seven months later, he furthermore underwent resection of 4 metastatic nodules in the right lung. Microscopically, all resected metastatic tumors were diagnosed to be mature teratoma without viable malignant cells. The patient remains well 30 months after the first operation. Follow-up CT scan demonstrates patency of aortic and vena caval bypass grafts without local recurrence or distant metastasis.     

Massive intrahepatic haemorrhage responsible for an inferior vena cava syndrome: an exceptional complication of hepatocellular carcinoma

Intralesional massive haemorrhage responsible for inferior vena cava syndrome is a very rare complication of hepatocellular carcinoma. A 36-year-old man with no past medical history was referred to hospital for abdominal pain with clinical and biological signs of internal bleeding. An abdominal CT scan showed a huge right liver mass, with regular margins and a fluid content, indicating a large intratumoral haemorrhage complicating hepatocellular carcinoma. Due to haemodynamic stability and the signs of inferior vena cava compression conservative management was chosen prior to surgical treatment of the tumour. Three months later, the huge liver mass remained unchanged but the inferior vena cava syndrome had decreased and the patient's condition improved. After evaluation of liver function and the extent of the tumour, a surgical procedure was performed via a subcostal incision with midline extension and sternotomy to control the intrapericardial inferior vena cava and perform a veno-venous bypass with the technique used for orthotopic liver transplantation. Then, right hepatectomy (segments V to VIII) was performed with an anterior approach. The postoperative course was uneventful. This two-step strategy allowed successful surgery for this rare complication of a hepatocellular carcinoma on a normal liver and could be recommended for the management of any unruptured intratumoral haemorrhages.     

Screw-in atrial lead in a sick sinus syndrome patient with anomalous inferior vena cava.

Anomalous inferior vena cava without intracardiac anomaly is an unusual condition. Herein, we report a 48-year-old female with left-sided inferior vena cava and azygous continuation. accompanied by sick sinus syndrome. This anomaly resulted in difficulty in implanting a traditional hook-on atrial lead. Atrial lead dislodgment occurred repeatedly soon after implanting the pacemaker because of an anomalous zygous vein draining into the superior vena cava, making a giant connection with the right atrium, thus eliminating the space of the atrial appendage for lead lodgment. Finally, we attempted to utilize a screw-in atrial lead in this patient and she is currently doing well. We therefore suggest that a screw-in atrial lead should be taken into account for such patients in order to obtain a stable fixation.     

Comparison of central venous and inferior vena caval pressures

Inferior vena caval pressures were measured in 60 patients undergoing cardiac catheterization and compared with central venous pressure from within the right atrium. Mean pressures within the abdominal inferior vena cava were essentially the same as mean right atrial pressure, suggesting that the inferior vena cava provides a useful safe alternative for measuring central venous pressure.     

Echocardiographic study of an intravenous leiomyoma: case report and review of the literature

Intravenous leiomyoma is a rare tumor of smooth muscle with invasion to veined channels that affects women at reproductive age. The case of a 45-year-old woman is described, with history of oophorectomy and hysterectomy, in addition to abdominal surgery due to mesenteric tumor. Several months later, the patient developed syncope and dyspnea secondary to pulmonary embolism. Echocardiographic study reported a mass in right side of heart proceding from inferior vena cava. The patient underwent tumor surgical resection from left iliac vein and histologic study concluded intravascular leiomyomatosis.     

Hepatic autotransplantation with ex situ neoplasm extirpation and vena cava replacement

The treatment of a 64-year-old man with a retrohepatic neoplasm deemed not accessible by conventional in situ surgical techniques is presented to illustrate the potential benefit offered by techniques adapted from liver transplantation and vascular surgery. A computed tomography scan performed for uncharacteristic abdominal discomfort revealed a hepatic or retrohepatic tumor compressing the inferior vena cava. Biopsies were interpreted as probably leiomyoma or malignant schwannoma. The liver with neoplasm and retrohepatic inferior vena cava was removed en bloc and taken to the back table where the neoplasm invading the inferior vena cava wall was removed together with the inferior vena cava. The inferior vena cava was then replaced by a 22-mm polytetrafluoroethylene graft and the 3 hepatic veins were reconstructed with anastomoses to this graft. The liver was then autotransplanted by standard transplantation technique. The postoperative course was uneventful and the patient is in good health more than 2 years after surgery.     

Sonospirometry: a new method for noninvasive estimation of mean right atrial pressure based on two-dimensional echographic measurements of the inferior vena cava during measured inspiration

A noninvasive method of estimating mean right atrial pressure would be useful in evaluating hemodynamics and calculating pulmonary pressures by Doppler echocardiography. An electronic pressure gauge was built and tested for measurement of inspiratory pressures during two-dimensional echocardiography to quantitate the diameter of the inferior vena cava. Thirty-one studies were made in 27 alert, informed, consenting patients with an in-place pulmonary artery catheter having right atrial ports. Inferior vena cava diameter was measured in successive 10 mm segments distal to the right atrial-inferior vena cava junction on images obtained while the patient suspended breathing at full inspiration and during each 4 mm Hg increment of a calibrated inspiratory maneuver. Results show that the segment between 5 and 30 mm distal to the right atrial-inferior vena cava junction was the region most responsive to increasing inspiratory pressure. In this segment, the inspiratory pressure required to decrease the inferior vena cava diameter to greater than or equal to 85% of the difference between its maximal (suspended full inspiration) and minimal (over the entire inspiratory maneuver) values was similar or equal to the mean right atrial pressure (measured from the pulmonary artery catheter) (r = 0.87, SEE = 2.9 mm Hg). Minimal inferior vena cava diameter was directly related to mean right atrial pressure (r = 0.56); the minimal to maximal inferior vena cava diameter ratio was inversely related to mean right atrial pressure (r = -0.57). Maximal inferior vena cava diameter and the absolute (measured) amount of inferior vena cava diameter decrease correlated weakly with mean right atrial pressure.(ABSTRACT TRUNCATED AT 250 WORDS)     

Recurrent intracardiac leiomyomatosis

An intravenous leiomyoma, a histologically benign smooth muscle tumour, arises from either a uterine myoma or the walls of a uterine vessel, with extension into veins. The present report describes echocardiographic features of an intravenous leiomyoma that spread into the right-sided cardiac chambers in a middle-aged woman who had undergone a hysterectomy two years earlier. Echocardiographic features included an elongated mobile mass extending from the inferior vena cava and multiple masses in the right atrium and right ventricle. Intracardiac leiomyomatosis should be considered in women who present with a cardiac mass in the right-sided chambers.     

静脉内平滑肌瘤病延伸至右侧心腔的诊断和外科治疗

目的探讨静脉内平滑肌瘤延伸至右侧心腔的诊断与外科治疗。方法2001年1月至2006年5月我院收治静脉内平滑肌瘤延伸至右侧心腔患者2例,均分期手术。首先行常规全麻中低温体外循环下心脏肿物切除术,然后二期切除子宫、附件及子宫病变组织,进行盆腔清扫术。结果无围术期并发症,2例患者随访至今情况良好。结论提高对多发性子宫肌瘤或右心房肿物的警惕性,一期直视下心脏内肿物切除术,二期切除子宫病变组织和盆腔清扫术,可获满意的效果。