Primary left atrial angiosarcoma mimicking severe mitral valve stenosis

Abstract

Primary cardiac tumours are quite rare and most of these tumours are benign. In this report, a patient presented with heart failure symptoms attributable to severe mitral valve stenosis. Echocardiography showed a dense left atrial mass causing functional mitral valve obstruction. The morphological and intraoperative presentation was highly suggestive of a myxoma but histopathological examination found a primary pedunculated cardiac angiosarcoma. The role of two dimensional and transoesophageal echocardiography in the assessment of cardiac masses and tumours is discussed.A 63 year old woman was referred to our emergency department with subacute onset of dyspnoea and a 2-3 week history of repeated presyncope and exercise intolerance. Known co-morbidities were chronic obstructive pulmonary disease and obstructive sleep apnoea with nasal chronic positive airway pressure treatment at night. No severe cardiovascular diseases were known. On admission, physical examination showed an obese patient (body mass index 31.2 kg/m 2 ) with orthopnoea at rest (New York Heart Association function class IV) and a heart rate of 110 beats/ min. Cardiac auscultation showed a diastolic murmur with the maximum point at the fifth intercostal space. Bipulmonary basal rales were present. The ECG showed sinus tachycardia of 110 beats/min with an intermediate heart axis and a slightly broadened bimodal P wave. A transoesophageal echocardiogram showed good systolic function (ejection fraction 70%) and an echo dense homogeneous left atrial mass (21.2 6 18.2 mm) originating from the anterior leaflet of the mitral valve (fig 1). During each diastole, the mass protruded into the left ventricular inflow causing severe functional mitral stenosis. Subsequent transthoracic echocardiography showed turbulent flow across the mitral valve and a mean gradient of 29 mm Hg (fig 2) consistent with severe functional obstruction. From continuous Doppler recordings of the tricuspid valve, a systolic pulmonary artery pressure of 80 mm Hg was estimated. During cardiac catheterisation, relevant stenoses of the epicardial coronary arteries were ruled out. Severe pulmonary hypertension was confirmed with a pulmonary

Primary Cardiac Angiosarcoma of Left Atrium

“…In a literature review through PUBMED from 1980 to 2008, more than 250 cases of cardiac angiosarcoma were found; the tumors mostly arose from the right atrium with different extensions and clinical presentations. In the last two decades, only eight cases of this malignant tumor originating from LA were reported in the English literature 1‐8 . This present report describes the ninth case of primary cardiac angiosarcoma arising from an extremely unusual location.…”

Cardiac Hemangioma of the Left Atrial Appendag: A Case Report and Discussion

“…In the last two decades, only eight cases of this malignant tumor originating from LA were reported in the English literature. [1][2][3][4][5][6][7][8] This present report describes the ninth case of primary cardiac angiosarcoma arising from an extremely unusual location.…”

Primary left atrial angiosarcoma determining severe mitral valve stenosis

“…In the vast majority of cases these tumours are benign [1,2]; among malignant histotypes, angiosarcoma is the most common, representing 15-35% of all cardiac tumours [2,3].…”

“…Moreover, to the best of our knowledge, no previous case of left-sided angiosarcoma has been reported in patients already undergoing cardiac operations [1,7,8].…”

“…In the vast majority of cases these tumours are benign [1,2]; among malignant histotypes, angiosarcoma is the most common, representing 15-35% of all cardiac tumours [2,3].Primary cardiac angiosarcomas usually arise from the right atrium or the pericardium; they typically occur in the third to fifth decade of life and are two to three times more frequent in males.We report a case of primary angiosarcoma arising from the left atrium in a female patient who had already undergone mitral valve repair.Key words: atrium; cardiac tumours; mitral valve; adult; surgery; emergency …”