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.

Figure 3

Pulmonary artery pressure recording during right heart catheterisation. Note a peak systolic pulmonary artery pressure of nearly 80 mm Hg and a mean pulmonary artery pressure of 55 mm Hg (solid line).

Figure 4

Intraoperative situs with view through the intra-atrial septum (transseptal access). The arrow marks the roughly 2 cm large tumour originating at the segments A3/P3 of the MV. (1) marks the cordae tendineae of the anterior leaflet of the MV.

Figure 5

(A) Haematoxylin and eosin stain (5×) displaying a tumour consisting of irregular neoplastic vascular channels surrounded by atypical spindle shaped and epitheloid tumour cells. (B) Immunohistochemical staining (Mib-1) showing moderate mitotic activity of the tumour cells (100×).

Figure 1

Transoesophageal echocardiogram in the mid oesophageal long axis view with the image plane oriented at 120°. Note an echo dense tumour (TM) in the left atrium (LA) originating from the anterior mitral leaflet protruding into the left ventricular (LV) inflow tract. AO, aorta; RV, right ventricle.

Figure 2

Transthoracic echocardiogram continuous wave Doppler across the mitral valve (MV) with a peak velocity (VP) of 3.58 m/s and a mean diastolic gradient (MPG) of 29 mm Hg between the LA and the LV. The valve area was not calculated according to the pressure half time method because of the high heart rate (HR) and poor definition of the diastolic deceleration slope in the Doppler pressure tracing. bpm, beats/min; PPG, peak pressure gradient; VTI, velocity time integral.