Atrial septal hematogenous cyst with calcification mimicking myxoma: A rare case

A 61-year-old female presented with right atrial mass during physical examination. Contrast-enhanced left heart echocardiography revealed a mass with the size of 32*23 mm in the right atrium, attached to the atrial septum; there was a certain degree of activity and deformation. MRI showed a mass of about 35*22 mm in the right atrium adjacent to the atrial septum, which was diagnosed with right atrial myxoma. Intraoperative TEE showed that the mass was located in the atrial septum close to the inferior vena cava and spontaneous echo contrast with hyperechoic images within the mass. The lesion was resected under cardiopulmonary bypass. Pathological examination revealed that the filling defect was an atrial septal hematogenous cyst with calcification.     

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.     

Left atrial myxoma with aortic regurgitation.

A 59-year-old woman with a left atrial myxoma was shown to have moderate aortic regurgitation. At operation for removal of the myxoma it was found to be attached to the atrial septum close to the aortic root. It is suggested that the aortic regurgitation was caused by distortion of the aortic root caused by traction upon it from the myxoma as it moved downward into the left ventricle in diastole. Observations are made on the echocardiographic findings which helped in the diagnosis of the left atrial myxoma.     

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.     

Two dimensional echocardiographic detection of intraatrial masses

With two dimensional echocardiography, a left atrial mass was detected in 19 patients. Of these, 10 patients with rheumatic mitral stenosis had a left atrial thrombus. The distinctive two dimensional echocardiographic features of left atrial thrombus included a mass of irregular nonmobile laminated echoes within an enlarged atrial cavity, usually with a broad base of attachment to the posterior left atrial wall. Seven patients had a left atrial myxoma. Usually, the myxoma appeared as a mottled ovoid, sharply demarcated mobile mass attached to the interatrial septum. One patient had a right atrial angiosarcoma that appeared as a nonmobile mass extending from the inferior vena caval-right atrial junction into the right atrial cavity. One patient had a left atrial leiomyosarcoma producing a highly mobile mass attached to the lateral wall of the left atrium. M mode echocardiography detected six of the seven myxomas, one thrombus and neither of the other tumors. Thus, two dimensional echocardiography appears to be the technique of choice in the detection, localization and differentiation of intraatrial masses.     

Infected papillary fibroelastoma attached to the atrial septum.

A 61-year-old woman had intermittent fever of 2 months' duration following a dental extraction. On admission, her body temperature was 39.2 degrees C. A mid-systolic murmur was heard at the apex on ausculation. A 2-dimensional echocardiogram revealed a mobile, heavy stick-like mass with vegetation (5.0 x 1.5 cm) attached to the left atrial septum. Multiple blood cultures grew Streptococcus constellatus. On diagnosis of an infected left atrial myxoma, antibiotics were administered daily and 4 weeks later, the left atrial tumor was resected. The tumor was 5.3cm long, 1.5cm in diameter at the inter-atrial wall and had vegetation on the free edge. On microscopic examination, colonies of Gram-positive cocci were found in the thrombus, on the papillary fibroelastoma. After treatment with antibiotics for a further 4 weeks, the patient was discharged. This is the first report of infected papillary fibroelastoma.     

Inverted Left Atrial Appendage Bonded to Atrial Septum

Inverted left atrial appendage (LAA) is a rare surgical complication. Our patient, a boy aged 2 years, was diagnosed with a partial atrioventricular defect with mild regurgitation of the left atrioventricular valve and a large primum atrial septal defect. Direct postoperative transesophageal echocardiography revealed a new left atrial mass attached to the atrial septum, without left ventricle inflow obstruction. Out of concern about the nature of this mass, we chose surgical direct examination. Intraoperatively, we diagnosed it as an inverted LAA accidentally attached to the atrial septum suture line. Awareness of this condition can avoid unnecessary diagnostic and therapeutic procedures.     

Echocardiographic appearances of right atrial thrombus

A 17-year-old male presented with a four-day history of increasing shortness of breath and right basal pleuritic chest pain. Physical examination revealed central and peripheral cyanosis, sinus tachycardia, elevation of central venous pressure, hypotension, and added third heart sound. Echocardiography (M-mode and two-dimensional) showed a highly mobile mass in the right atrium apparently attached to the interatrial septum. A diagnosis of right atrial myxoma with pulmonary embolization was made and urgent surgery advised. At operation a string of thrombus in the right atrium (presumed from peripheral venous site) occluding a patent foramen ovale was found. The usefulness of echocardiography in diagnosing atrial thrombus and in differentiating clot from tumor is discussed.     

A case of cardiac myxoma associated with congenital oculocutaneous albinism

This case is a 43 year-old-woman of left atrial myxoma associated with oculocutaneous albinism, who was admitted to our hospital because of nocturnal cough everyday. Electrocardiogram showed normal sinus rhythm and complete right bundle branch block. Chest X-ray disclosed slight enlargement of left atrium. Echocardiography revealed the left atrial pedunculated tumor, arising from atrial septum, prolapsing into left ventricle during diastole, which was coincided with lucent tumor by levophase of pulmonary arteriography. Feeder artery of this tumor was visualized by right coronary arteriography. Cardiac catheterization represented normal pressure of right-sided heart. Thus the diagnosis of left atrial myxoma was established. At the operation, radical excision of the base of tumor and septum and patch closure was undergone. The gross morphology and microscopic findings were consistent with myxoma.     

A patient with rheumatic mitral stenosis and an atrial myxoma.

A 34-year-old woman with exertional dyspnoea and clinical diagnosis of mitral stenosis underwent echocardiographic evaluation. Moderate to severe rheumatic mitral stenosis was confirmed but an unsuspected mass lesion in the dilated left atrium attached to inter-atrial septum in fossa ovalis region was found. Although the attachment was in favour of myxoma, it was difficult to differentiate with certainty between left atrial myxoma and thrombus. Warfarin was prescribed but after 1 year the mass lesion remained unchanged. Both the severity of the mitral stenosis and the presence of the mass lesion led to our decision to proceed with surgery. Pathology showed typical histology of a myxoma.     

Enfarte agudo do miocárdio como forma de apresentação de mixoma gigante da aurícula esquerda

Myxomas are the most common type of benign cardiac tumor. The most frequent clinical presentations are symptoms resulting from atrioventricular valve obstruction or systemic embolization. Coronary embolization is a rare, although real and potentially fatal, complication of cardiac myxomas. We present a case report and review of the literature on this disease association. A 57-year-old woman was admitted to our coronary care unit with a diagnosis of non-ST elevation acute myocardial infarction. Transthoracic echocardiography showed a large left atrial mass attached to the interatrial septum, coral-like and with a friable appearance, suggestive of myxoma. Coronary angiography revealed no significant lesions and the patient underwent surgical excision of the mass, which histological study showed to be compatible with myxoma. The postoperative period was uneventful and the patient is doing well, with no recurrence of myxoma.     

Right atrial myxoma in a patient with antiphospholipid antibodies

A 28 year-old asymptomatic male with previously detected elevation of antiphospholipid antibodies (aPL) had a right atrial mass diagnosed by transthoracic echocardiography. Although the morphological characteristics were highly suggestive of myxoma, due to the presence of aPL elevation it had to be differentiated from atrial thrombus. The patient underwent surgery and a 3 x 4 cm mass with its large base attached to the interatrial septum was removed. Histopathological examination confirmed that it was a myxoma. On follow-up, the patient was asymptomatic and antiphospholipid antibodies became undetectable.     

Long-term survival of quiescent left atrial myxoma in an elderly patient

Left atrial myxoma, if left untreated, is inexorably progressive and usually fatal. The classical management of this disease is prompt surgical removal. However, there may be exceptions to this traditional dogma. The authors report an 85-year-old man who presented with features of chronic obstructive airway disease and congestive heart failure 15 years previously. Auscultation of the heart showed a pansystolic murmur. Two-dimensional echocardiography revealed a nonobstructive, calcified, and well circumscribed mass in the left atrium, with a pedicle attached to the interatrial septum. Because the patient refused high-risk cardiac surgery related to his comorbidity, he was managed medically with a bronchodilator, diuretics, and digoxin. There have been no features of embolism or intracardiac obstruction, and serial echocardiography demonstrates no disease progression over this long period of time. This case illustrates that calcified left atrial myxoma may exhibit a quiescent phase. Tumor calcification, slow growth potential, and the absence of intracardiac obstruction may correlate with a better outcome in the elderly patient.     

Left atrial myxoma presenting with acute myocardial infarction--a case report

A 38-year-old man presented with an acute anterior myocardial infarction (MI) and was subjected to thrombolysis. Echocardiography and cardiac catheterization revealed a mass in the left atrium that was considered to be a myxoma. The coronary arteries appeared normal on angiography. The left atrial mass was removed surgically without complication. Histopathologic examination confirmed the diagnosis of the myxoma. Coronary embolism secondary to the myxoma was thought to be the cause of the MI.     

Two-dimensional echocardiographic diagnosis of left atrial myxoma

Two patients with left atrial myxomas detected with one-dimensional echocardiographic studies were evaluated before and after surgery with real-time phased-array two-dimensional echocardiographic studies. This latter technique provided relatively quantitative information regarding the size, shape, and mobility of the tumor and its effect on cardiac function. In case 1, the two-dimensional echocardiogram showed a relatively immobile tumor measuring 3 x 4.5 cm in diameter in the left atrium only. At surgery a 2 x 4.5-cm myxoma that was attached to the left atrium by a broad-based short stalk was removed. In case 2, the left atrial mass appeared to be 3 x 4 cm in diameter, with much movement during the cardiac cycle. At surgery a 3 x 4-cm left atrial myxoma that was attached to the interatrial septum by a long stalk was removed. In both cases, masses in other cardiac chambers were excluded, the mitral valves were normal, and left ventricular function was normal, all of which were confirmed at surgery and by postoperative echocardiograms. In one patient the information obtained by two-dimensional echocardiographic studies was believed to be sufficient to preempt the need for cardiac catheterization. These cases illutstrate that this new noninvasive technique may provide sufficient quantitative preoperative detail in patients with left atrial tumors to obivate the risk and expense of caridac catheterization.     

Mitral valve thrombus attached to the intact mitral valve associated with distal embolism

A 61-year-old man was referred for cardiac investigation because embolism was suspected to be the cause of the sudden onset of severe pain in his right leg after a surgical procedure. The electrocardiogram revealed no atrial fibrillation. Transthoracic echocardiography demonstrated a tumor-like echo at the anterior mitral leaflet, and transesophageal echocardiography documented a mass 13x9mm in size, attached by a stalk to the left atrial side of the anterior mitral leaflet. The other parts of the mitral valve appeared to be intact. At emergency surgery, the mass was located in the center of the left atrial side of the anterior mitral leaflet. It mimicked a myxoma and had a stalk arising from the anterior leaflet. After resection of the mitral valve mass, catheter thrombo-embolectomy was performed and several long pieces of fresh thrombus were removed. On histological examination, the mass consisted of fresh thrombus tissue. No cellular component or myxoma tissue was documented. The distal embolus also consisted of fresh thrombus tissue. This is the first case of a thrombus of the intact mitral valve without atrial fibrillation.     

New diagnosis of left atrial myxoma in a 93-year-old woman

A 93-year-old woman with a history of hypertension was noted to have a mass in the left atrium prolapsing partially through the mitral valve on a routine surface echocardiogram in 2002. A transesophageal echocardiogram was then performed revealing an irregular mass with an appearance of protruding fronds of tissue. The echogenicity of the mass was inhomogeneous with some areas of punctate calcification, and the mass was attached to the interatrial septum. The echocardiographic appearance of the mass was almost pathognomic of atrial myxoma, and this is the oldest patient yet to have been diagnosed with a probable myxoma. The patient did not want surgical removal of the mass. She is still asymptomatic at the age of 95 years.     

Complete detachment of cardiac myxoma causing aortic saddle embolization and cerebral infarction

A 30-year-old previously healthy male was brought to our emergency room with loss of consciousness and convulsion. Physical examination showed that the femoral arterial pulses were absent. Two-dimensional transthoracic and transesophageal echocardiography showed no evidence of intracardiac thrombus or tumor or of aortic dissection. Brain magnetic resonance imaging study showed cerebral infarction involving anterior and bilateral hemispheres. He died of multiple organ failure after admission. Autopsy showed aortic saddle embolization caused by a myxomatous-like material. Histological examination of the embolic material confirmed the diagnosis of myxoma. There was a stalk on the left atrial septum which might possibly represent the site of attachment of the myxoma. This case suggested that a cardiac myxoma completely detached from the left atrial septum and caused aortic saddle embolization.     

Mitral Valve Vegetations in Bacterial Endocarditis Resembling Left Atrial Myxoma

Summary: A patient with Staphylococcus aureus endocarditis is reported whose echocardiogram closely resembled that found in left atrial myxoma. Because of severe mitral incompetence, the valve was replaced using cardiopulmonary bypass and at operation a large 3 cm friable vegetation was found attached to the posterior leaflet of the mitral valve. Histological examination showed changes typical of bacterial endocarditis with no evidence of myxoma.     

Abscess of the mitral annulus mimicking left atrial tumour - case report

A case of a 70-year-old female with hypertension, atrial fibrillation and angina pectoris, admitted to the hospital due to echocardiographically detected left atrial tumour, is presented. Differential diagnosis included thrombus, myxoma, infectious or neoplastic tumour. The patients underwent surgery. Histopathological examination revealed the presence of an abscess in the left atrium. This report underlines the difficulties in the diagnosis of cardiac tumours.