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J Thorac Cardiovasc Surg 2007;133:251-252
© 2007 The American Association for Thoracic Surgery

Brief Communication

A rare type of right atrial tumor in a 66-year-old woman

Alexander Lauten, MD*, Justus T. Strauch, MD, Jens Wippermann, MD, Thorsten Wahlers, MD

Department of Cardiothoracic Surgery, University Hospital Cologne, Cologne, Germany.

Received for publication August 25, 2006; accepted for publication August 31, 2006.

* Address for reprints: Alexander Lauten, MD, Department of Cardiothoracic Surgery, University Hospital of Cologne, Kerpener Str. 62, 50924 Cologne, Germany. (Email: alexander.lauten{at}uk-koeln.de).

Solid cardiac neoplasms are rare; however, depending on their location, they can be associated with serious complications. Fibroelastomas are endocardium-derived tumors that can develop in any chamber of the heart. A valvular position is known, with an appearance most often on the aortic valve and equal distribution on semilunar and atrioventricular valves. Located at the right side of the heart, the tumor generally remains asymptomatic. When sufficient in size, it might interfere with valvular function and hemodynamics and lead to secondary pulmonary embolization.

Clinical Summary

A 66-year-old woman was referred to us by the cardiology department with a recently diagnosed right atrial mass. The tumor had been revealed during a screening echocardiography before an elective orthopedic operation. A magnetic resonance imaging study showed late enhancement of the structure measuring 2.8 x 2.5 cm with a broad base at the right atrial free wall and, although rarely located in the right heart, highly suspicious of an atrial myxoma (Figure 1). In the presence of chronic atrial fibrillation, thrombus formation was considered as a differential diagnosis. Over a period of 3 months, a marked progression in size was noted in repeated studies, and the patient was referred to us for urgent operative removal of the tumor. On admission, physical examination of the cardiopulmonary system was normal, apart from arterial hypertension. A dual-chamber pacemaker had been inserted 7 years earlier for atrioventricular block.


Figure 1
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  		Figure 1. Preoperative magnetic resonance image showing an enhanced solid tumor originating from a broad base at the anterior right atrial wall. On the basis of morphologic criteria, atrial myxoma seems most likely.

 
Removal of the tumor was performed through a standard right atriotomy after bicaval cannulation for cardiopulmonary bypass. Intraoperatively, the tumor presented as a gelatinous and fibrous conglomerate attached by a small base to the anterior atrial free wall just above the tricuspid annulus, causing first-degree insufficiency of the valve (Figure 2). Removal of the tumor, including the basic connection to the heart, was uneventful, and after closure of the atriotomy, the patient was easily weaned from cardiopulmonary bypass. After an uncomplicated postoperative course, she was discharged home 7 days after the operation on an oral anticoagulation regimen. Histologic evaluation of the intraoperative specimen revealed a papillary fibroelastoma of the right atrial endocardium.


Figure 2
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  		Figure 2. Intraoperative specimen and hematoxylin and eosin–stained histologic gross section. The tumor presents with numerous avascular papillary fronds covered by a single-layer endothelium.

 
Discussion

Solid masses of the right atrium are rarely found incidentally by using routine echocardiography at an early stage before causing clinical symptoms. Because diagnostic investigations are usually limited to imaging studies, a precise primary diagnosis is difficult to make. The presence of a right atrial mass is highly suspicious of a thrombus, either passing from a deep venous compartment to the pulmonary circulation or caused by pacemaker leads.1Go Differentiation between thrombus formation and tumors arising from cardiac structures is possible on the basis of morphologic criteria, such as verification of a stalk base, or on the basis of proof-of-enhancement phenomena in magnetic resonance imaging studies. These criteria, however, are unsafe, and final diagnosis of the type and benign or malignant status of the tumor can only be made by means of histologic evaluation.

In consideration of a differential diagnosis, the atrial myxoma is the most likely of a variety of cardiac tumors. Although typically located in the left atrium, myxomas have also been found in the right atrium in a substantial number of patients.2Go After myxoma and lipoma, papillary fibroelastomas represent the third most common type of benign cardiac tumors, accounting for less than 10% of primary cardiac tumors.3Go Although these tumors can arise from any endocardial surface, they most often originate from the valvular endocardium, with almost equal frequencies on semilunar and atrioventricular valves.4Go

Conclusions

Papillary fibroelastomas of the right atrium are extremely rare; only 3 cases have been reported in the literature. Because the tumor is benign, treatment requirements are based on its potential for obstructive effects and embolic events.

References

  1. Chartier L, Bera J, Delomez M, Asseman P, Beregi JP, Bauchart JJ, et al. Free floating thormbi in the right atrial heart: diagnosis, management and prognostic indexes in 38 consecutive patients. Circulation 1999;99:2779-2783.[Abstract/Free Full Text]
  2. Reynen K. Cardiac myxomas. N Engl J Med 1995;333:1610-1617.[Free Full Text]
  3. Howard RA, Aldea GS, Shapira OM, Kasznica JM, Davidoff R. Papillary fibroelastoma: increasing recognition of a surgical disease. Ann Thorac Surg 1999;68:1881-1885.[Abstract/Free Full Text]
  4. Edwards FH, Hale D, Cohen A, Thompson L, Pezzella AT, Virmani R. Primary cardiac valve tumors. Ann Thorac Surg 1991;52:1127-1131.[Abstract]



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