Right-left shunt caused by sarcoma of the right atrium

A 70 year old woman was admitted for right ventricular failure and cyanosis of recent onset. Echocardiography showed a very large, homogenous, immobile, smooth-contoured mass filling the right atrium. Right atrial pressures were raised but the other intracardiac pressures were normal at catheterisation. Right heart angiography confirmed the voluminous right atrial mass and dilatation of the hepatic veins and showed early opacification of the left heart chambers. A right-to-left shunt was confirmed by oximetry which showed significant desaturation of the blood in the left atrium and ventricle (saturation 78% in the left ventricle). The tumour was also documented by a thoracic CT scan. At surgery, a very large, malignant right atrial tumour was resected which histological examination showed to be an angiosarcoma. The interatrial septum seemed to be intact: there was no true atrial septal defect but a persistent foramen ovale was found. After resection of the tumour the right atrium was reconstructed. The initial postoperative period was uncomplicated but the patient died nine months later of metastatic disease particularly affecting the liver and brain. The clinical presentation of malignant cardiac tumour is very variable but a right-to-left interatrial shunt through a patent foramen ovale has only been reported previously in 2 cases of primary malignant tumours (a rhabdomyosarcoma and an angiosarcoma) and in 1 case of a secondary cardiac metastasis.     

Solitary tumour metastasis: a rare cause of right ventricular outflow tract obstruction and sudden death

The clinical features of a patient presenting with recent breathlessnessindicated right ventricular outflow tract obstruction, whileechocardiography suggested the presence of tumour in the rightventricle. Before further investigations could be instituted,she died suddenly. Autopsy revealed a haemopericardium secondaryto rupture of the right atrium while the right ventricle wasfound to contain a tumour arising from most of its endocardialsurface. Histology showed this to be a poorly differentiatedmucous secreting carcinoma similar in appearance to her coloniccarcinoma excised 8 years previously. There was no other evidenceof metastatic disease.     

Echocardiographic pattern of right atrial tumour motion.

Propagation of Wilm's tumour to the right atrium was diagnosed by echocardiography. The tumour prolapsed into the right ventricle during each atrial systole. The presence of the tumour and its motion were confirmed by cardiac catheterization, cineangiography, and surgical exploration. Successful resection of the tumour was accomplished. Echocardiography was helpful in detecting the presence of the right atrial tumour and accurately reflected the pattern of its atrioventricular motion.     

Delayed post-cardiac-surgery tamponade producing localized right atrial compression

A patient with dyspnea and lethargy 19 days after aortic valve replacement was admitted to the hospital with physical signs suggesting cardiac tamponade. Initial echocardiogram was unhelpful, but Swan-Ganz catheterizatlon showed a gradient in diastole between the right atrium and ventricle. Anglography and repeat echocardlography then showed a mass compressing the right atrium, which was confirmed at surgery.     

Echocardiographic pattern of right atrial tumour motion.

Propagation of Wilm's tumour to the right atrium was diagnosed by echocardiography. The tumour prolapsed into the right ventricle during each atrial systole. The presence of the tumour and its motion were confirmed by cardiac catheterization, cineangiography, and surgical exploration. Successful resection of the tumour was accomplished. Echocardiography was helpful in detecting the presence of the right atrial tumour and accurately reflected the pattern of its atrioventricular motion.     

Angiographic demonstration of blood supply of right atrial myxoma.

A 53-year-old woman underwent cardiac catheterization for assessment of coronary arterial disease. An unexpected finding of a gradient between right atrium and right ventricle led to the discovery of an unsuspected right atrial myxoma. The diagnosis was established by the presence of a filling defect in the right atrial angiocardiogram and by the demonstration of "tumour vessels" by selective right coronary angiography. Selective coronary angiography can visualize the blood supply to an intracardiac tumour and thus confirm the diagnosis preoperatively.     

Cavernous hemangioma of the right atrium

Cardiac hemangioma is a rare primary benign tumour, localised in the right atrium in 23% of cases. In a 60-year-old patient, who complained of remote chest discomfort and recent exertional palpitation, a right atrial mass was discovered by magnetic resonance imaging and echocardiography. A selective operation was performed and the tumour was resected. Histology revealed it to be a cavernous hemangioma. Due to the potential risks associated with cardiac hemangioma, surgical resection and postoperative follow-up are recommended.     

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.     

An incidental calcified right atrial mass

A 58-year-old man presented to his family physician with a mild cough. A routine chest x-ray revealed a 4 cm 'ring opacity' in the right atrium. A transthoracic echocardiogram revealed a 41 mm x 33 mm mass in the right atrium. The patient underwent urgent cardiac surgery, where a firm to hard 4 cm x 3 cm mass was excised. Pathology revealed an old, largely infarcted and calcified right atrial myxoma. A review of the published literature shows that this case is the first to demonstrate an almost completely infarcted atrial myxoma with only a few clusters of viable cells identified histologically.     

Delayed postoperative cardiac tamponade mimicking severe tricuspid valve stenosis

A patient developed acute, severe hemodynamic deterioration five days after an aortic valve replacement. Cardiac catheterization revealed a markedly elevated right atrial pressure but a normal right ventricular end-diastolic pressure. Angiography revealed an extrinsic mass causing compression of the right atrium and the tricuspid anulus. A large clot overlying the right atrium and ventricle was found at emergency surgery. Postoperative cardiac tamponade may result in an atypical hemodynamic presentation when there is selective compression of one chamber or of a valve anulus.     

Persistence of the right venous sinus valve simulating a right atrial myxoma in a case of total anomalous pulmonary venous return. Echocardiographic, anatomo-clinical and surgical aspects

A case of persistence of the right venous sinus valve that on echocardiographic examination simulated right atrial myxoma is reported in a patient with total abnormal pulmonary venous return in the coronary sinus. Echocardiography showed a mobile, pedunculated mass present in systole in the right atrium that shifted to diastole in the right ventricle, highly suggestive of right atrial myxoma. The right ventricle also showed a volume overload and a space without echoes behind the left atrium. A membrane was encountered in the right atrium at surgery. This was removed and the venous return corrected.     

A case of right atrial myxoma--the availability of transesophageal echocardiography in the detection of right atrial myxoma

A 72-year-old woman had experienced palpitation and fatigue during exertion for two months and was referred to our hospital from her nearby hospital. On physical examination, a systolic murmur was heard in the left fourth intercostal space. A chest X-ray film showed cardiac enlargement (CTR 64%). An ECG showed elevated P waves in leads II, III. Transthoracic echocardiography revealed a large oval heterogeneous mass in the right atrium. Transesophageal echocardiography (TEE) revealed the right atrial mass clearly, which was attached to the atrial septum with a short wide stalk. This mass prolapsed from the right atrium into the right ventricle in diastole. And there were some cysts in the homogeneous high-echoic lesion. The chest CT and MRI also showed the mass in the right atrium. However, these images were not clear. Surgical excision of the mass was undertaken. A solid mass measuring 75 x 50 x 45 mm was attached to the fossa ovalis with a wide short stalk. There were several cysts in the mass. Pathological examination showed myxomatous tissue. In this case, TEE was the most valuable means for evaluation of the right atrial mass.     

Arrhythmogenic right ventricular cardiomyopathy and atrial right-to-left shunt

A 47-year-old woman diagnosed with arrhythmogenic right ventricular cardiomyopathy, was studied at our institution with palpitations, progressive dyspnoea and cyanosis over a 3-year period. Her haemoglobin saturation was 76.1%. The transthoracic echocardiogram showed that both right atrium and ventricle were dilated, with diffuse akinetic and hypokinetic areas. Radionuclide scintigraphy, angiography and magnetic resonance imaging showed dilated and severe dysfunction of the right ventricle, with depressed ejection fraction (0.16), and bulging at the level of the right ventricular anterior wall areas. Doppler examination revealed a right-to-left interatrial shunt through a patent foramen ovale. Contrast transoesophageal echocardiography confirmed the right-to-left shunting. Considering the clinical symptoms were severe and refractory to medical treatment, she was referred for cardiac transplantation. Unfortunately the patient died suddenly a few weeks later.     

Right atrial thrombosis as a complication of arrhythmogenic right ventricular cardiomyopathy

A 65-year-old woman was admitted to our hospital due to palpitation. Electrocardiogram (ECG) showed ventricular tachycardia originating from the right ventricle, and transthoracic echocardiography revealed dilatations of the right atrium and ventricle. The diagnosis of arrhythmogenic right ventricular cardiomyopathy was made. Eleven months later, echocardiography revealed a solid thrombus (36x32 mm) attached to the free wall of the right atrium, and it was surgically resected. Four months after the operation, a solid thrombus (48x30 mm) appeared again at the same site despite anticoagulant treatment. The patient died of both left and right heart failure 33 months after the operation.     

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 carcinoma extending to the inferior vena cava and the right atrium. A case report

A case of a 66-year-old male hospitalised due to heart failure is presented. Echocardiography showed an abnormal structure in the right atrium resembling myxoma or thrombus. Abdominal ultrasonography revealed a right renal tumor. Finally, magnetic resonance imaging showed that the abnormal structure in the right atrium was a neoplasmatic plug continuously extending from renal carcinoma. The renal tumor and it's metastatic plug were successfully removed during surgery.     

Primary cardiac angiosarcoma with right coronary-to-right atrium fistula

Primary tumours of the heart are rare. About 25% of all cardiac tumours are malignant and the most common of these is the angiosarcoma. We present a 61-year-old male with a right atrial angiosarcoma that was detected on coronary angiography. The tumour showed marked vascularity and a right coronary-to-right atrium fistula, and the patient underwent surgical resection. Pathological examination of the tumour was consistent with a cardiac angiosacoma and the diagnosis was also confirmed by immuno-histochemistry. He consequently underwent chemotherapy, however the patient died 60 days after the surgery.     

Criss-cross heart with straddling right atrioventricular valve

Angiocardiographic appearance of a criss-cross heart with straddling tricuspid valve has been presented. Atrial situs was normal and the right atrium was connected to the morphological right ventricle situated superiorly and to the left of the left ventricle. The left atrium was connected to the morphological left ventricle situated inferiorly and to the right of the right ventricle. The interventricular septum was horizontal in position. Both great arteries arose from the right ventricle with the anterior aorta. The case was concluded as a criss-cross heart with concordant atrioventricular (A-V) connection and double outlet right ventricle (DORV). Straddling of the right A-V valve was recognized in another institution by two-dimensional (2-D) echocardiography and confirmed by right atrial angiography in our institution. The value of combined investigation with selective four-chamber angiography, especially including right atrial angiography and 2-D echocardiography in the diagnosis of such complex cardiac anomalies has been stressed.     

Primary cardiac lymphoma causing right atrial occlusion

Primary cardiac lymphomas (PCL) are extremely rare, and diffuse large B-cell lymphoma is a highly aggressive subtype. We report a case that was initially diagnosed as chronic right heart dysfunction. Detailed investigations revealed a large lobulated tumour occluding the right atrium, infiltrating the inter-atrial septum, the roof of the left atrium, and the aortic root. Despite adequate surgical debulking and initial successful tricuspid annuloplasty, the patient succumbed to multi-organ failure. Pathological analysis of the resected tumour confirmed a diffuse large B-cell lymphoma with a proliferation rate of 100%. What is unique about this case is the size of this rare cardiac tumour, which we believe to be one of the largest described in the literature for a purely intra-cardiac PCL, its aggressive growth rate, and the relatively mild symptomatology until a late stage of the disease.     

Primary angiosarcoma of the right atrium detected by magnetic resonance imaging.

A 20-year-old woman with a primary angiosarcoma of the right atrium is reported. The patient had a cardiorespiratory arrest due to cardiac tamponade with bloody pericardial effusion. Magnetic resonance imaging (MRI) revealed a tumor, which was corroborated by selective coronary angiography. Open-heart surgery was performed. The tumor relapsed however, and she died four months after operation. The tumor was undetectable by echocardiography, but MRI demonstrated a heterogeneous mass with focal areas of high- and low-signal intensity in the right atrium, suggesting that MRI may allow characterization of cardiac tumors.