Wilms' tumour propagated through the inferior vena cava into the right heart cavities

A report is made of a rare case of Wilms' tumour which simulated clinically a syndrome of `right heart failure', as found in some cases of Ebstein's disease. The clinical, radiological, and electrocardiographic study led to the suspicion of this type of malformation. The cine-angiographic study revealed two important facts: the impossibility of approaching the inferior vena cava with opaque material injected into the azygos vein and a filling defect of the right atrium. The post-mortem study revealed the presence of a Wilms' tumour of the right kidney which extended into the inferior vena cava and into the right atrium without producing metastases. This seems to be the first case of this peculiar course of a Wilm's tumour which has grown into the right atrium, resulting in this bizarre clinical picture.     

Right intra-atrial extension of hydatid cyst mimicking cardiac thrombosis. Apropos of a case]

The authors describe an unusual case of hydatid cyst inserted in the inferior vena cava and extending into the right atrium. The transoesoesophageal echocardiographic appearances were similar to those of a thrombus: the tumour was very mobile, echogenic, polylobular with a cord-like pedicle in the inferior vena cava. The pathological examination revealed a ruptured hydatid cyst. The mass and its insertion were not visible on CT scan or cavography. Transoesophageal echocardiography would therefore seem to be a very useful diagnostic method for tumours arising in the inferior vena cava and extending into the right atrium.     

Metastatic Wilms' tumor causing acute hepatic-vein occlusion (Budd-Chiari syndrome)

We describe a case of Wilms' tumor in a 6-yr-old girl which extended from the right kidney and completely obstructed the inferior vena cava and hepatic veins to the level of the right atrium, producing an acute Budd-Chiari syndrome. After an initial course of chemotherapy, the primary tumor was excised and, using cardiopulmonary bypass, the tumor thrombus was removed from the inferior vena cava and hepatic veins. The Budd-Chiari syndrome resolved and after triple-drug chemotherapy and radiotherapy under the National Wilms' Tumor Study-2 protocol the child has remained disease-free for 5 yr.     

Secondary tumors of the right heart. Echocardiographic aspects. Apropos of 6 cases in the adult

Secondary cardiac tumours are rare but but are now more frequently diagnosed by echocardiography. We report 6 cases of intracardiac metastases affecting the right heart which were diagnosed by 2D echocardiography. In 3 cases, a very mobile, oval-shaped tumour was visualised within the right atrium prolapsing into the tricuspid orifice in diastole like a myxoma but associated in 2 cases with signs of invasion of the inferior vena cava. Two other non-mobile tumours were observed causing massive invasion of the right atrium and the last case was of an infiltrating tumour of the right ventricle resulting in pulmonary infundibular obstruction. In the light of our experience and a review of the literature, it is difficult to distinguish secondary tumours of the right atrium from myxomas especially when the tumours are mobile and when it is impossible to visualise a pedicle inserted on the interatrial septum or tumoral invasion of the inferior vena cava. At the ventricular level, the diagnostic signs differ according to whether there is tumoral invasion of the cavity or infiltration of the muscular wall. These cases illustrate the value of 2D echocardiography in the diagnosis of intracardiac metastases, sometimes even in the absence of clinical signs.     

Budd-Chiari syndrome due to pacemaker-induced thrombosis

Abstract We report a unique case of Budd-Chiari syndrome caused by pacemaker leads-induced thrombosis. A 34 year old female patient was subjected to a permanent pacemaker insertion because of refractory paroxysmal supraventricular tachycardia attacks related to Wolff-Parkinson-White syndrome. Three years later, another pacemaker was re-implanted because of its dislodgement. Four episodes of skin infections at the implantation site were noted thereafter. The patient developed symptoms of abdominal pain and ascites 5 years after the second pacemaker implantation. Ultrasonography and computerized tomography of the abdomen revealed hepatomegaly with ascites and dilated inferior vena cava. An echocardiogram displayed thrombus formation in the superior vena cava, the right atrium and the inlet of the inferior vena cava into the right atrium. Inferior and superior venacavogram confirmed the above findings. With the impression that Budd-Chiari syndrome was caused by pacemaker-induced thrombus, we removed the pacemaker first and thoracotomy with thrombectomy was then performed. The clinical symptoms resolved after the operation. To our knowledge, this is the first case reported in the literature and this observation supported the thrombosis theory for membranous obstruction of inferior vena cava.     

Atrial septal defect and cyanosis. Apropos of 6 cases related to abnormal drainage of the inferior vena cava into the left atrium

The authors report six cases of atrial septal defect (ASD) associated with abnormal drainage of the inferior vena cava into the left atrium responsible for right-to-left shunting, without pulmonary hypertension. The abnormal drainage could be due either to an anatomical malposition of the inferior vena cava opening into the left atrium, or to an abnormal blood flow from this vein, normally located through a low ASD, under the influence of anatomical, mechanical and haemodynamic factors. Clinically, all patients presented with light cyanosis and with the usual signs of ASD. None of them had elevated pulmonary pressure. The lesion, suggested by clinical findings, was diagnosed either at angiography, which in four cases demonstrated an abnormal pulmonary venous return, or at colour-coded doppler echocardiogram, or at surgery. In every case, surgical correction consisted of closure of the often low-sited ADS by a patch which diverted the inferior vena cava into the right atrium and the abnormal venous return towards the left atrium. The short--and long-term results of surgery were excellent. The authors review the literature concerning this unusual association of ASD with an abnormal drainage of the inferior vena cava into the left atrium.     

Intrahepatic cholangiocarcinoma presenting as the Budd-Chiari syndrome: a case report and literature review.

Intrahepatic cholangiocarcinoma, an increasingly recognized primary tumour of the liver, is associated with a very poor prognosis. A patient with this tumour who presented with Budd-Chiari syndrome (the first to the authors' knowledge in Western literature and only the third patient overall) secondary to extensive thrombosis in his inferior vena cava extending from the right atrium down to his iliac vessels is described. Neither curative nor palliative intervention was deemed to be an option in this patient, who deteriorated rapidly while on anti-coagulants. Postmortem examination confirmed the radiological findings, and histological analysis revealed characteristic appearances of this tumour within the biliary tree and invasion into the inferior vena cava. Furthermore, biliary dysplasia, which can be a precursor to this cancer, was also noted within some of the bile ducts.     

Anomalous High Insertion of the Inferior Vena Cava into the Right Atrium: A Case Report with Review of Literature

Objective. Anomalous high insertion of the inferior vena cava into the right atrium is probably the most infrequent anomaly among the anomalies of the inferior vena cava. Patients. We present a case of anomalous high insertion of the inferior vena cava into the posterior wall of the right atrium in a 20‐year‐old asymptomatic man. Conclusions. This anomaly has unique characteristic because of presenting with an isolated anomaly different from earlier reported cases.     

Intravenous leiomyomatosis: A rare cause of intracardiac mass

Intravenous leiomyomatosis is an unusual clinical condition characterized by histologically benign smooth muscle lesions extending from the uterus into pelvic and systemic veins and, more rarely, into the right cardiac chambers. We report the case of a 45-year-old woman who presented with a three-week history of dyspnea on exertion, shortness of breath and fatigue. Echocardiography showed a large mobile mass in the right atrium prolapsing into the right ventricle and extending to the inferior vena cava. A computed tomography scan revealed a large mass extending from the right atrium to the inferior vena cava and through the systemic veins as far as the popliteal veins. A presumptive diagnosis of large thrombus was made; the correct diagnosis of intravenous leiomyomatosis with intracardiac involvement was obtained only after surgical resection and histologic examination.     

Asymptomatic membranous obstruction of the inferior vena cava forming intrahepatic collateral pathways

Intrahepatic and/or extrahepatic collateral pathways result from the membranous obstruction of the inferior vena cava. These collaterals are usually insufficient to prevent Budd-Chiari syndrome. We reprot an unusual case of asymptomatic membranous obstruction of the inferior vena cava in which marked intrahepatic collateral pathways were formed. Although the inferior vena cava terminated above the orifice of the right hepatic vein, the middle and left hepatic veins were patent above the membrane, without narrowing. Blood from the inferior vena cava drained into the right atrium via the intrahepatic collaterals between the right and middle hepatic veins without resistance.     

Inferior Vena Caval and Right Atrial Thrombosis Complicating an Amebic Hepatic Abscess

We describe a 50-yr-old black laborer who presented with right lower chest pain, weight loss, and pedal edema. Ultrasonography and computed tomograms showed a large abscess cavity in the right lobe of the liver which extended very close to the inferior vena cava. The lumen of the adjacent inferior vena cava was partially occluded by thrombus, which could be traced up into the cavity of the right atrium. The hepatic veins were normally patent. Sterile blood-stained pus was aspirated from the abscess. Antibodies against Entamoeba histolytica were present in high titer in the patient's serum. Although propagation of hepatocellular carcinoma into the inferior vena cava and even up into the right atrium is well recognized, inferior vena caval thrombosis extending up into the right atrium has not hitherto been reported as a complication of amebic hepatic abscess.     

Hepatocellular carcinoma invading the right atrium: clinical, echographic and angiographic study

The authors report the case of a 54 year old woman suffering from hepatocellular carcinoma with tumor growth into right hepatic vein, inferior vena cava and right atrium. On cardiac examination, a pansystolic bruit and a diastolic rumble were audible at the tricuspid focus. Diagnosis was confirmed by inferior vena cavography and two-dimensional echocardiography, which demonstrated a large mobile mass in the right atrium moving to and fro through the tricuspid valve. This case report emphasizes the value of routine cardiac examination during the course of hepatocellular carcinoma.     

Meandering right pulmonary vein to the left atrium and inferior vena cava: the first case with associated anomalies

We report a case of a healthy, asymptomatic 6-year-old boy in whom an anomalous right pulmonary vein was noted to drain into both the inferior vena cava and left atrium in association with findings consistent with scimitar syndrome. The anomalous pulmonary vein took a very circuitous route through the lungs before draining into the left atrium, a condition previously termed "meandering pulmonary vein." To aid in the diagnosis, cardiovascular magnetic resonance imaging and magnetic resonance angiography were used to delineate this complex course and the connection of the anomalous pulmonary vein. To our knowledge, this is the 1st reported case of a meandering pulmonary vein with dual drainage to the inferior vena cava and left atrium in association with other anomalies.     

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.     

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.     

Repeat sternotomy and hypothermic circulatory arrest for resection of renal cell carcinoma with tumour thrombus extension into the right atrium

Surgical resection remains the mainstay of treatment for advanced non-metastatic renal cell carcinoma (RCC) with tumour thrombus extending into the inferior vena cava (IVC). There is extensive literature regarding the resection of subdiaphragmatic tumour thrombus however there are fewer reports of resection of tumour thrombus that has extended into the right atrium. We report the successful resection of a right renal cell carcinoma with tumour thrombus extending into the right atrium in a patient who had undergone previous coronary artery bypass grafting.     

内支架置入联合FOLFOX-4方案治疗肝癌并下腔静脉及右心房癌栓6例

目的:探讨血管内支架置入联合FOLFOX-4方案系统化疗治疗肝癌合并下腔静脉及右心房癌栓的疗效.方法:回顾性分析行血管内支架置入联合FOLFOX-4方案系统化疗治疗6例原发性肝癌并下腔静脉癌栓及右心房癌栓患者,记录并发症发生情况及疗效,随访生存期.结果:6例患者均一次性成功置入血管支架,下腔静脉平均压力下降16mmH2O,患者下腔静脉梗阻症状均明显改善.6例患者均可客观评价化疗疗效,以右心房癌栓为靶病灶,PR5例,SD1例.4例患者血清AFP下降超过50%.至2011-10随访结束时,接受治疗患者仍存活2例,中位总存活时间(overall survival,OS)为8.2mo,中位肿瘤进展时间(tumor progression time,TTP)为5mo.全部患者未见因化疗不良反应终止化疗,其中骨髓抑制(白细胞减少,Ⅰ-Ⅱ度)3例,消化系反应(恶心、呕吐、纳差,Ⅰ-Ⅱ度)5例,神经毒性(神经感觉异常Ⅰ-Ⅱ度)1例,乏力3例,发热1例.结论:对于肝癌并下腔静脉及右心房癌栓患者,血管内支架置入能即刻改善下腔静脉梗阻症状,联合FOLFOX-4方案系统化疗,使生存期延长.     

Cardiac myxoma arising from the inferior vena cava

A cardiac myxoma that arises from the inferior vena cava (an extremely rare finding) may interfere with caval cannulation. In such cases venous cannulation for cardiopulmonary bypass has been performed directly into the inferior vena cava or through the femoral vein. We present a case in which routine cannulation through the right atrium proved safe and gave good exposure for complete resection of the tumoral mass.