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.     

Echocardiography as a first diagnostic step in kidney tumours

Intracardiac tumours are usually found after clinical symptoms lead to a positive imaging study, or as an incidental finding of imaging study, usually echocardiography. Cardiac tumours range from non-neoplastic lesions to high grade malignancies. The majority of primary cardiac tumours are myxomas (in 75% cases) or sarcomas (about 10% cases). In this paper we present cases of 2 patients with right atrial tumour, extending from renal carcinoma, invading renal vein and inferior vena cava into right atrium. Two different therapeutic strategies were undertaken in those patients.     

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.     

Renal cell carcinoma presenting as right atrial mass

Approximately 190 new cases of renal cell carcinoma infiltrate into the inferior vena cava and right atrium every year. Echocardiography often plays a major role in establishing the diagnosis and in guiding surgical therapy. This report describes a patient who presented with shortness of breath but had no classic signs or symptoms of renal cell carcinoma other than chronic anemia. Transthoracic two-dimensional echocardiography and transesophageal echocardiography established the diagnosis of a right atrial mass extending from the inferior vena cava that was later discovered to be renal cell carcinoma.     

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.     

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.     

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.     

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.     

Intracardiac extension of intravenous leiomyomatosis in a pregnant woman: A case report and review of the literature

Intravenous leiomyomatosis is an uncommon clinical entity characterized by the growth of a benign, smooth muscle tumour within the venous system. Intracardiac extension of this tumour is rare, and approximately 35 cases have been reported in the literature. The second case of massive intracardiac extension of intravenous leiomyomatosis is reported in a pregnant patient diagnosed after the unusual presentation of seizure activity. Tumours were successfully removed from the right internal iliac vein, inferior vena cava, and right atrium and ventricle in a single-stage operation using cardiopulmonary bypass and circulatory arrest. Concomitant total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed. Nine months after diagnosis, the patient was evaluated for recurrent disease. A review of the literature from 1994 to 1998 is presented.     

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.     

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.     

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.     

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.     

Transesophageal Echocardiographic Findings in Primary Leiomyoma of Inferior Vena Cava

We describe for the first time the usefulness of transesophageal echocardiography for the identification of a primary leiomyoma of the inferior vena cava, which originated near its junction with the right atrium. A portion of the tumor was initially visualized in the right atrium during a transthoracic echocardiographic study, but its attachment in the inferior vena cava was evident only by transesophageal echocardiography using the transgastric approach. (ECHOCARDIOGRAPHY, Volume 10, November 1993)     

ANATOMICAL AND ECHOCARDIOGRAPHY CORRELATIONS IN THE NORMAL HEART

Knowledge of the normal intracardiac structures and their variations is essential for interpretation of two-dimensional echocardiography. The anatomical features seen in the standard echocardiography views have been demonstrated in normal heart specimens.
Anatomical variants such as a prominent Eustachian valve of the inferior vena cava in the right atrium and fibromuscular strands or 'heart strings' traversing the left ventricular cavity are frequently visualised on the two-dimensional echocardiograms.     

Use of contrast echocardiography in diagnosis of anomalous connection of right superior vena cava to left atrium.

A 4-month-old infant with cyanosis but without other abnormal cardiac findings is presented in whom the diagnosis of anomalous systemic venous connection to the left atrium was made by contrast echocardiography. The diagnosis was later confirmed by cardiac catheterisation and selective cineangiography. When saline was injected into a vein on the dorsum of each hand while echocardiographically recording the cardiac structures, the left atrium, left ventricle, and aorta were opacified without visualisation of the right ventricle. Similar study with injection into the right foot produced opacification of the right ventricle without visualisation of the left-sided structures. These data suggested normal drainage of the inferior vena cava with anomalous connection of the superior vena cava to the left atrium. A review of the previously reported cases of anomalous connection of the right superior vena cava to the left atrium is presented together with the possible embryological origin of this anomaly.     

Right atrial hemangioma presenting as pericardial tamponade. A case report

Intracardiac haemangioma is a very rare benign primary cardiac tumour. A 40-year-old patient, with no notable history, presented with chest pain, accentuated by deep inspiration, associated with NYHA stage III dyspnoea. Physical examination revealed signs of right heart failure and electrocardiogram showed low voltage with diffuse repolarization disorders. Chez x-ray showed a cardiothoracic index CTI > 0.70, and enlarged right margins. A large pericardial effusion with a tumour mass filling the right atrial cavity was discovered on transthoracic echocardiography. Chest computed tomography demonstrated the tissue origin of the tumour and suggested extension to the inferior vena cava and hepatic veins. The patient was referred to a surgeon for tumour resection. Histological examination revealed intracardiac capillary haemangioma. Five month postoperative follow-up did not reveal any abnormalities.     

Detection of atrial septal defect with left-to-right shunt by inferior vena cava contrast echocardiography.

Contrast echocardiography was used before cardiac catheterisation in 37 patients with atrial septal defect and a left-to-right shunt and in 18 patients with a raised right atrial and ventricular pressure to assess the contrast echo effect in the inferior vena cava. Using two dimensional contrast apical echocardiography we found a negative contrast echo effect within the right atrium in many but not all patients with atrial defect. Contrast echoes entering the inferior vena cava during presystole or early to mid-diastole were detected in patients with heart disease causing raised right atrial and ventricular pressures and also in all patients with atrial septal defect. No contrast echo effect in the inferior vena cava was detected in 10 normal subjects. The sensitivity of this contrast pattern in the inferior vena cava in diagnosing atrial septal defect was 100%. When other conditions causing raised right atrial pressure were excluded, the specificity and predictive accuracy were 100% for both. The presystolic contrast echo effect in the inferior vena cava, semiquantitatively graded, correlated with the size of the shunt determined by oximetry. In 20 patients re-examined after the surgical correction of the atrial septal defect, no presystolic contrast echo effect was detected in the inferior vena cava. Contrast echocardiography of the inferior vena cava is a valuable and reliable method for diagnosing atrial septal defect with left-to-right shunt.     

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.     

Drainage of the inferior vena cava to the left atrium

Drainage of the inferior vena cava to the left atrium is an extremely unusual congenital heart disease. We describe a 54-year-old woman, in whom the diagnosis was suggested by transthoracic echocardiography, and then confirmed by a transesophageal exam and magnetic resonance imaging, which also revealed an associated secundum atrial septal defect. Surgical management involved reconstruction of the interatrial septum to include the inferior vena cava in the right atrium. The few previously reported cases in the literature are reviewed.