Right atrial spleen

A large cardiac tumour occupying most of the right atrium and the right ventricle and causing inflow obstruction to the right heart was confirmed by cross sectional echocardiography in a 41 year old man. After surgical resection histological examination showed that the atrial tumour had the characteristics of splenic tissue. Possible mechanisms for the development of such a tumour include an origin analogous to that of an accessory spleen or the implantation and subsequent growth of lymphoid tissue in a pre-existing superior vena caval or high right atrial angioma.     

Cardiac granulocytic sarcoma diagnosed by intracardiac echocardiography-guided biopsy

A 52-year-old man presented with clinical and echocardiographic signs of cardiac tamponade. A transthoracic echocardiogram revealed a large right atrial mass that obstructed the superior vena cava flow. Cardiac magnetic resonance imaging and computed tomography demonstrated extracardiac tumour invasion of the free atrial wall extending to the right pulmonary hilus. Intracardiac echocardiography-guided biopsy of the tumour revealed the tissue diagnosis-granulocytic sarcoma of the heart. The patient was effectively treated with radiotherapy, chemotherapy and allogeneic haematopoietic stem cell transplantation. He has remained free of the disease for 12 months after treatment.     

Demonstration of the inner structure of a right atrial myxoma by transoesophageal echocardiography

We report a case of a large right atrial myxoma. With the useof transoesophagal echocardiography, several cysts were idntifieldwithin the tumour and necrotic tissue was differentiated frommyxomatous tissue according to its echo-level. Transoesophagealechocardiography was very useful for the precise diagnosis ofcardiac tumour and demonstration of the inner structure.     

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.     

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.     

Angiosarcoma of the heart. Unusual presentation and survival after treatment

A 49 year old man presented with severe cyanosis and dyspnoea on exercise. Clinical examination together with echocardiography, cardiac catheterisation, and angiography showed a balloting tumour in the right atrium, intermittently occluding the tricuspid ostium, and an atrial right to left shunt. At operation a pedunculated vascular tumour was found with a broad base which was embedded in the atrial wall and continued into the interventricular septum. Histological examination showed angiosarcomatous features and signs of a less than radical excision. The patient, who made an uneventful recovery, was given postoperative radiotherapy. After 36 months there are no signs of recurrence or metastasis.     

Angiosarcoma of the heart. Unusual presentation and survival after treatment.

A 49 year old man presented with severe cyanosis and dyspnoea on exercise. Clinical examination together with echocardiography, cardiac catheterisation, and angiography showed a balloting tumour in the right atrium, intermittently occluding the tricuspid ostium, and an atrial right to left shunt. At operation a pedunculated vascular tumour was found with a broad base which was embedded in the atrial wall and continued into the interventricular septum. Histological examination showed angiosarcomatous features and signs of a less than radical excision. The patient, who made an uneventful recovery, was given postoperative radiotherapy. After 36 months there are no signs of recurrence or metastasis.     

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.     

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.     

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.     

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.     

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.     

Surgical experience with intracardiac myxomas

Eighteen patients underwent surgery for intracardiac myxoma (16 left atrial and 2 right atrial) during the last 10 years. Seventeen patients had tumour stalk attached to the oval fossa. The myxoma was excised along with a cuff of the atrial septum, which was reconstructed using a Dacron patch in 15 patients and by direct suture in 2 patients. In the remaining case the myxoma was attached to the left atrial wall and adjacent atrioventricular junction. There was only one early death in a patient who underwent a concomitant lobectomy for lung abscess and one late death due to a noncardiac cause. During the follow-up period of 3-96 months (average 36 months) all the survivors were in New York Heart Association Class I. Scanning electron microscopy of tumour tissue was done in 8 cases. The morphological findings did not help in categorizing the tumours into any pathological subgroups. Postoperative cardiac catheterization done in 3 patients (30-50 months postoperatively) showed return of haemodynamics to normal. Echocardiographic studies done postoperatively have not revealed recurrence of tumour in any patient. Surgical excision of myxomas is possible with very gratifying long-term results.     

Clinical presentation and non-invasive diagnosis of right heart masses.

Right-sided cardiac masses do not have a uniform clinical presentation. Successful diagnosis is dependent on a high index of suspicion and appropriate echocardiographic examination. Five cases of right sided intracardiac masses have been detected during the period that echocardiography has been routinely available to us--two of right atrial myxoma, one of right atrial thrombus, and two of right ventricular tumour. M-mode echocardiography identified four of the five cases. Two-dimensional echocardiography was necessary to establish the non-invasive diagnosis in the fifth case. Echocardiography should be used in any patient in whom a right sided mass is suspected, or in those patients presenting with signs of tricuspid regurgitation, tricuspid stenosis, pulmonary stenosis, cyanotic heart disease, progressive right heart failure, constrictive pericarditis, or pulmonary emboli without obvious source.     

Right atrial rhabdomyoma acting as the substrate for Wolff-Parkinson-White syndrome in a 3-month-old infant

We report a 3-month-old infant girl who presented with supraventricular tachycardia, who was detected to have a large right atrial rhabdomyoma and evidence of Wolff-Parkinson-White (WPW) syndrome with an accessory pathway corresponding to the position of the tumour. In view of the resistant cardiac arrhythmia and large size of the tumour, the infant underwent surgical excision of the tumour. The evidence of WPW syndrome disappeared from the surface electrocardiogram immediately upon removal of the tumour. A second resolving tumour in the interventricular septum was also detected during the investigation. Although there are no other stigmata of tuberous sclerosis at present, our infant requires close follow-up. Right atrial rhabdomyomas are rare and to our knowledge this is the first reported instance where we could document clearly that the tumour itself formed the substrate for the WPW syndrome.     

Novel electrophysiologic parameter of dispersion of atrial repolarization: comparison of different atrial pacing methods

INTRODUCTION: Heterogeneity of ventricular repolarization plays a major role in reentrant tachyarrhythmias in cardiac tissue. However, the role of atrial repolarization added activation time (AT) to refractoriness in atrial vulnerability has not been investigated in detail. METHODS AND RESULTS: The study population consisted of 34 patients: 18 with atrial fibrillation (AF) and 16 without AF (control group). The effective refractory periods (ERPs) in the right atrial appendage, low lateral right atrium, high right septum, and distal coronary sinus, and ATs from P wave onset to each electrogram during sinus rhythm and right atrial appendage, low lateral right atrial, high right septal, distal coronary sinus, and biatrial pacing were measured. Atrial recovery time, defined as the sum of AT and ERP, and its dispersions during sinus rhythm, right atrial appendage, low lateral right atrial, high right septal, distal coronary sinus, and biatrial pacing were calculated. Both ERP dispersion and atrial recovery time dispersion during sinus rhythm were significantly greater in the AF group than in the control group. Atrial recovery time dispersion during distal coronary sinus, high right septal, or biatrial pacing was significantly smaller than that during right atrial appendage or low lateral right atrial pacing in each group. In particular, atrial recovery time dispersion during distal coronary sinus pacing was the smallest of the five pacing methods in the AF group. P wave duration during biatrial or high right septal pacing was significantly shorter than during right atrial appendage, low lateral right atrial, or distal coronary sinus pacing in each group. CONCLUSION: Atrial recovery time dispersion is suitable as an electrophysiologic parameter of atrial vulnerability. Distal coronary sinus pacing may prevent AF by increasing homogeneity of atrial repolarization, whereas biatrial and high right septal pacing contribute not only homogeneity of atrial repolarization but also improvement of atrial depolarization.     

Elevated atrial natriuretic peptide level in giant right atrium complicating Marfan's syndrome

We report a rare case of giant right atrial dilatation in a patient with Marfan's syndrome complicated by severe tricuspid regurgitation. Interestingly, the right atrial pressure was normal, but the atrial natriuretic peptide level was markedly elevated. We suggest that the underlying connective tissue abnormality had allowed extensive atrial distension which by causing an increased intramural tension according to Laplace's law stimulated atrial natriuretic peptide secretion.     

Myxoid leiomyosarcoma of the left atrium: a rare malignancy of the heart and its comparison with atrial myxoma

Primary tumours of the heart are rare. The majority of these tumours are benign, with myxomas located in the left atrium being the most common form. Almost all malignant tumours are sarcomas and occur preferentially in the right side of the heart. An exception to this rule is leiomyosarcoma, a rare form of primary cardiac sarcoma that occurs predominantly in the left atrium, as does cardiac myxoma. The case of a 53-year-old woman who presented with symptoms of mitral valve stenosis and pulmonary hypertension is reported. Cardiac catheterization, angiography and echocardiography revealed a left atrial mass that was interpreted as atrial myxoma. At the time of operation, the myxoid appearance of the tumour mass further supported this assumption. The tumour, including a wide rim of atrial septum, was removed with cautery. Histopathological examination unexpectedly showed that the tumour was not an atrial myxoma but rather a myxoid variant of a primary leiomyosarcoma. Immunohistochemistry and electron microscopy confirmed the diagnosis. Local radiotherapy was considered but deemed contraindicated in view of the longstanding pulmonary hypertension. Two months after excision, a repeat echocardiogram indicated recurrence of tumour in the left atrium, and the patient died a few days later. The preferential left atrial location and the frequently myxoid appearance of primary leiomyosarcomas of the heart make it particularly difficult to differentiate them preoperatively from atrial myxomas. The authors recommend resection of all atrial myxoid tumours with a wide (at least 1 cm) margin, combined with intraoperative frozen section diagnosis, because complete surgical resection appears to correlate with prolonged survival in the few reported cases of atrial leiomyosarcomas. In cases of incomplete initial resection or local recurrence in the absence of metastatic disease, heart transplantation may be a valid option in appropriately selected patients.     

Right atrial myxoma: echocardiographic appearance

A 67-year-old asymptomatic male was admitted for evaluationof his arterial hypertension. The routine echocardiographicstudy revealed a large tumour in the dilated right atrium. Themass appeared to arise from the posterior wall of the rightatrium. After infusion of a contrast agent, the mass appearedto fill with the contrast agent, ruling out the possibilityof the mass being a clot. Transesophageal study revealed a round mass arising from theposterior right atrial wall just adjacent to the extrusion ofthe superior vena cava. The patient subsequently underwent cardiacsurgery and a cardiac tumour was excised that proved to be acardiac myxoma. In this case we present echocardiographic imagesand the macro- and microscopic view of the right atrial myxoma.     

Increased levels of beta-human atrial natriuretic peptide-like immunoreactivity in chronically overloaded atrial tissue

The levels and molecular form of atrial natriuretic peptide-like immunoreactivity (ANP-LI) in human atrial tissue were investigated. The levels of right atrial ANP-LI were significantly higher in mitral disease than in other cardiac or noncardiac diseases. The increased ANP-LI was mainly accounted for by an increase in beta-human ANP-LI (beta-hANP-LI). Both total ANP-LI and beta-hANP-LI levels were associated with the presence of atrial fibrillation and with increased atrial pressure. Plasma beta-hANP-LI levels were also increased in mitral disease. These results suggest that human atrium with hemodynamic overloads is characterized by increased tissue levels of ANP and by a shift to the beta-hANP form.