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Circulation. 2004;110:e451-e452
doi: 10.1161/01.CIR.0000146801.57023.65
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(Circulation. 2004;110:e451-e452.)
© 2004 American Heart Association, Inc.


Images in Cardiovascular Medicine

Right Atrial Primary Cardiac Lymphoma Presenting With Stroke

Johannes Binder, MD; Stefan Pfleger, MD; Stefan Schwarz, MD

From the Department of Neurology (J.B., S.S.) and the Department of Medicine I (Cardiology, Angiology, and Pneumology) (S.P.), Universitätsklinikum Mannheim, University of Heidelberg, Germany.

Correspondence to Dr Johannes Binder, Department of Neurology, Universitätsklinikum Mannheim, Theodor Kutzer Ufer 1-3, D-68135 Mannheim, Germany. Email j.binder{at}neuro.ma.uni-heidelberg.de

An immunocompetent 79-year-old man presented with acute dysarthria and hemiparesis on the left side. The cranial CT revealed the typical findings of a middle cerebral artery territory infarction of embolic origin, likely from previously unknown nonvalvular atrial fibrillation. The results of the duplex ultrasound of the carotid arteries were normal. Transesophageal echocardiography, however, showed a right atrial tumor, which suggested either thrombus or myxoma (Figure 1A, Movie I). No evidence of a thrombus or spontaneous echo contrast was observed in the left atrium; the mean left atrial appendage peak emptying velocity was 0.35 m/s. Moreover, few microbubbles appeared in the left atrium, although only after >3 heart cycles (Figure 1B, Movie II). In combination with the findings from contrast transcranial Doppler ultrasound, which showed 3 bubbles within 20 seconds, some evidence of right-to-left shunting was noted, although the formal echocardiographic criteria of a patent foramen ovale were not fulfilled. The thoracic CT showed a right atrial tumor extending into the superior vena cava (VCS), as well as pericardial and right-sided pleural effusions (Figure 2). After surgical removal of the tumor the histological examination surprisingly revealed a primary cardiac non-Hodgkin lymphoma (PCL). The immunohistochemical classification showed a diffuse large B cell lymphoma with high immunoglobulin M expression. An extensive diagnostic work-up yielded no evidence of an extracardial lymphoma manifestation. The patient refused all therapy and died 6 weeks later at home.



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Figure 1. Transesophageal echocardiography demonstrates PCL in right atrium extending into VCS. (A) RA indicates right atrium; VCI, inferior vena cava (see Movie I). (B) Contrast transesophageal echocardiography yields moderate right-to-left shunting of microbubbles only in late phase after opacification of right atrium. LA indicates left atrium; AO, aorta (see Movie II).



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Figure 2. Thoracic CT shows primary cardiac lymphoma in right atrium invading myocardium (white arrows), as well as pericardial (thin gray arrow) and pleural effusions (thick gray arrow).

In our patient, the PCL, which is per se a rare entity, presents the unique origin of stroke. Two different pathophysiological mechanisms are conceivable: The dilatation of the right atrium may have caused atrial fibrillation predisposing to cardioembolism. Alternatively, although rather unlikely, increased thrombogenicity resulting from turbulent flow and the large surface of the tumor in the right atrium may have facilitated a paradoxical embolism either through a small patent foramen ovale or a pulmonary shunt.

Footnotes

The online-only Data Supplement, which contains Movies I and II, is available with this article at http://www.circulationaha.org.


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Circulation 2004 110: 2551. [Extract] [Full Text]



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