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Case Reports
. 2024 Mar 29;2(3):544-547.
doi: 10.1016/j.atssr.2024.03.003. eCollection 2024 Sep.

Cavoatrial Bypass for Cardiac Complications From Rosai-Dorfman Disease

Quynh Nguyen  1 , Rachel Bourke  2   3 , Richard W Gilbert  3   4 , Graziano Oldani  3   4 , Jong Moo Kim  1   3 , Andrew Lytle  5 , Richard C Cook  1   3 , Hilary P Grocott  2   3 , Joel Price  1   3
Affiliations
Case Reports

Cavoatrial Bypass for Cardiac Complications From Rosai-Dorfman Disease

Quynh Nguyen et al. Ann Thorac Surg Short Rep. .

Abstract

Rosai-Dorfman disease (RDD) is a nonmalignant disease of histiocyte proliferation. RDD usually presents with painless cervical lymphadenopathy, although extranodal involvement can occur. Cardiac involvement was reported in <0.1% of cases. We present a case of cardiac RDD with obstruction at the inferior vena cava-right atrial junction.

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Figures

Figure 1
Figure 1
Representative computed tomographic images. (A) Soft-tissue thickening (arrows) of the left atrial (LA) wall between the right superior and right inferior pulmonary vein ostia. (B) Soft-tissue thickening (arrows) of the LA wall involving the interatrial septum (∗) (RA, right atrium). (C) Right superior pulmonary vein (∗) stenosis (arrows). (D) Right inferior pulmonary vein (∗) stenosis (arrows).
Figure 2
Figure 2
(A) Interatrial septum mass pathology. Upper left: Biopsy specimen of the mass shows infiltration of the myocardium by an atypical dense lymphoplasmacytic and histiocytic inflammatory infiltrate (hematoxylin and eosin, original magnification ×40). Upper right: Higher magnification view of the infiltrate features enlarged histiocytes with abundant pale cytoplasm showing emperipolesis (circles) by lymphocytes (hematoxylin and eosin, original magnification ×200). Bottom: Immunohistochemistry shows coexpression of cluster of differentiation (CD) 68 (left) and CD163 (center) with S100 (right) in the histiocytes (all original magnification ×100). Immunostains for CD1a and activin A receptor like type 1 (ALK1) were negative (not shown). (B) Intraoperative photograph demonstrates a 12-mm polytetrafluoroethylene vascular ring graft (W. L. Gore & Associates, Inc) traversing the diaphragm from the right atrium to the retrohepatic inferior vena cava.
Figure 3
Figure 3
Intraoperative transesophageal echocardiograms before the cavoatrial bypass procedure show a large mass extending from the left atrium (LA) and interatrial septum (IAS), with displacement of the right atrium (RA) and obstruction at the junction of the RA and inferior vena cava (IVC). (SVC = superior vena cava.)
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References

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