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.     

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。     

Surgical management of a uterine leiomyoma extending through the inferior vena cava into the right heart

 We present a case of a 38-year-old woman with intravenous leiomyomatosis originating from the uterus involving the right ventricle, right atrium, inferior vena cava, and iliac veins. Tumors in the heart and the inferior vena cava were removed using cardiopulmonary bypass and circulatory arrest; 10 days after the cardiac operation, hysteroadnexectomy was performed. Received: April 26, 2002 / Accepted: August 13, 2002 Correspondence to M. Gaudino     

Leiomyosarcoma of the inferior vena cava extending into the right atrium

A 54-year-old female patient presented with a progressive and deteriorating dyspnea at the slightest exertion in particular during the past few days before presentation. Transthoracic echocardiography revealed a large space-occupying lesion in the right atrium extending into the inferior vena cava (IVC). Abdominal magnetic resonance aortography showed an elongated space-occupying lesion in the IVC with a significant portion of the tumor and almost completely filling the right atrium accompanied by an infiltration of the hepatic and renal veins. A pronounced tumor infiltration of the IVC at the level of the liver was confirmed intraoperatively and immunohistochemical analysis showed a moderate to poorly differentiated leiomyosarcoma. The extended tumor was successfully removed by a complex operation of the thorax and abdomen but the procedure was accompanied by severe bleeding. A few hours following the procedure the patient died due to a further episode of irreversible intra-abdominal hemorrhage.     

Extension of the inferior vena cava into the posteroinferior right atrium

BACKGROUND: The inferior vena cava (IVC) is obliquely connected to the right atrium (RA), and often a low-voltage area is observed in the posteroinferior RA. OBJECTIVES: The purpose of this study was investigate the size of the IVC extension into RA and its anatomic background. METHODS: We investigated 30 human hearts [11 men and 19 women; mean age 79 +/- 10 years; 7 cardiac deaths (group A) and 23 noncardiac deaths (group B)]. After obtaining macroscopic measurements around the RA-IVC junction, serial sections were cut and examined histologically. We defined a horizontal baseline at the level of the cavotricuspid isthmus and measured (1) the length of IVC extension, which was defined as the distance from the baseline to the top of the RA-IVC junction, and (2) the width of the RA-IVC junction at the baseline level. RESULTS: The top of the RA-IVC junction was always located in the posteroinferior RA. The mean length of the IVC extension was 17.6 +/- 6.6 mm, and the mean width was 29.6 +/- 7.5 mm. The IVC extension was wider in group A than in group B (35.8 +/- 9.0 mm vs 27.5 +/- 5.9 mm; P = .0277). The right and left borders of the RA-IVC junction corresponded to the reflection of the pericardium. Histologic examination showed no myocardium in the IVC extension. CONCLUSION: The IVC extension into the posteroinferior RA always exists and varies in size. Because this area lacks myocardium, it is important to consider when analyzing catheter mapping from this area.     

Membranous obstruction of the inferior vena cava and invasion of the inferior vena cava by tumour in hepatocellular carcinoma

A 28 yr old Zulu presented with a painful swelling in the right hypochondrium and severe swelling of the legs of short duration. The serum alpha-fetoprotein concentration was over 2 X 10(5) ng/ml and imaging showed a large hepatic mass-lesion. Radionuclide venography revealed no flow through the inferior vena cava but flow through a large collateral vessel. Contrast venography showed the upper portion of the inferior vena cava to be occluded: large collateral vessels arose from the lower vena cava and the iliac veins. The histological features were those of longstanding hepatic venous outflow obstruction with irregular centrizonal and portal fibrosis: severe acute centrizonal congestion was not seen. This combination of findings indicates the presence of both membranous obstruction of the inferior vena cava, a rare developmental abnormality which predisposes to hepatocellular carcinoma formation, and invasion by the tumour of the inferior vena cava via the hepatic veins, an uncommon complication of hepatocellular carcinoma.     

Disintegration of the 'waterfall phenomenon' in the inferior vena cava due to right heart failure

Abstract The concept of the waterfall phenomenon in Zone 2 in the pulmonary vasculature is well known from West's lung model. It is believed that the flow through this zone is determined by the pressure difference between the pulmonary artery and alveoli, and the left atrial pressure is not transmissible to the alveolar capillaries. However, it is impossible to see whether alveolar capillaries are really displaying the waterfall phenomenon or not. In this review, the interrelation between the flow and geometry of the alveolar capillaries in the waterfall phenomenon is analyzed based on physiological studies using a model system and isolated lung lobe experiments. Further, extending the concept to the analysis of ventilatory changes of the inferior vena cava (IVC) configuration, it is ascertained that the waterfall phenomenon normally occurs in the IVC during inspiration just before it enters the thorax and the waterfall phenomenon in the IVC disintegrates with elevation of the central venous pressure. Because these configurations of the IVC in normal and abnormal conditions are visible with ultrasonography, the technique is very useful as a noninvasive approach to diagnose right heart failure.     

Bilateral inferior venae cava combined with the persistent left superior vena cava and hemiazygos continuation of left inferior vena cava with drainage into right atrium: A case report

The persistent left superior vena cava (PLSVC) is a common venous abnormality. However, malformation of the bilateral inferior venae cava (IVC) is extremely rare, with an incidence rate of .3%. IVC malformation is associated most frequently with heart defects and isomerism and often has a poor prognosis. We presented a case of vascular malformations in the fetus of bilateral caval veins with the interruption of the left-sided venous return with hemiazygos continuation in presence of a right-sided inferior caval vein. Also noted were the PLSVC and a dilated right heart with a widened pulmonary trunk. In this case, there were no heart defects or chromosomal abnormalities, and the newborn postpartum was in a good condition.     

Bilateral superior vena cava with right superior vena cava draining into left atrium

Anomalies of systemic venous return are extremely heterogeneous congenital malformations with variable ranges from completely normal physiology to severe forms of right to left shunting requiring surgical treatment. Anomalous drainage of a right-sided superior vena cava (SVC) to the left atrium (LA) is one of the rarest variants of systemic venous return anomalies, characterized by right-to-left shunt physiology and cyanosis. Here we report a 2 years old girl presented with cyanosis which was observed shortly after birth by her parents but not further investigated. She is otherwise active girl and with normal growth and development. Her clinical examination was unremarkable apart from mild clubbing of the fingers and low oxygen saturation of 88–90% in room air. Her ECG and chest X-ray were unremarkable. Echocardiography showed bilateral SVC connected by a small innominate vein. The right SVC drains directly into the LA while the left SVC drains into the right atrium (RA) via a dilated coronary sinus. There is a small superior sinus venosus type atrial septum defect (ASD) with left to right shunt. Also, there is partial anomalous pulmonary venous return with right upper and right middle pulmonary veins draining directly into the right SVC, which is connected to LA. The right lower pulmonary vein and left pulmonary veins drain directly to LA. The rest of her echocardiography demonstrated normal heart structures and function. This patient was referred for surgical correction, including baffling of the right SVC to the RA and closure of the ASD. We describe this case to highlight the importance of recognizing this rare anomalous systemic venous connection as one of the very rare causes of cyanosis in the pediatric age group as well as at older age.     

Filtering through the data on retrievable inferior vena cava filters

• Technical success rates with implantation and retrieval of retrievable inferior vena cava filters are high
• Inferior vena cava filters are being used for a wide range of indications
• Systems should be put in place to ensure prompt and effective retrieval of inferior vena cava filters once these are no longer needed