A previously healthy 49-year-old man presented with an 8-week history of progressive dyspnea on exertion, nonproductive cough, and chills. Physical examination results and laboratory findings suggested right-sided heart failure. A transthoracic echocardiogram (TTE) revealed a large multilobular mass extending from the right atrium through the tricuspid valve (TV) into the right ventricle (Fig. 1). Color-flow Doppler findings at the level of the TV were consistent with functional stenosis (Fig. 2A). The mean gradient across the TV was 9 mmHg at a heart rate of 85 beats/min (Fig. 2B). The uptake of echocardiographic contrast medium enabled us to see the mass further penetrate the myocardial free wall into the pericardium, suggesting neovascularity. Histologic analysis of a biopsy specimen revealed large atypical cells consistent with lymphoma (Fig. 3). A TTE obtained 12 days after chemotherapy began revealed tumor shrinkage (Fig. 4A). The mean gradient had decreased to 3 mmHg, and the patient's symptoms had improved. Four months after chemotherapy had begun, scans obtained with use of positron-emission tomography/computed tomography showed further tumor shrinkage, from 13.55 × 10.3 to 5.8 × 2.8 cm. Eighteen months after chemotherapy initiation, a TTE showed near-resolution of the tumor (Fig. 4B).

Fig. 1

Transthoracic echocardiogram shows a large heterogeneous mass occupying the right atrium and right ventricle, with obstruction at the level of the tricuspid valve.

Fig. 1

Transthoracic echocardiogram shows a large heterogeneous mass occupying the right atrium and right ventricle, with obstruction at the level of the tricuspid valve.

Fig. 2

A) Transthoracic echocardiogram (color-flow Doppler mode) at the level of the tricuspid valve shows functional stenosis. B) Continuous-flow Doppler mode reveals a peak gradient of 14 mmHg and a mean gradient of 9 mmHg.

Fig. 2

A) Transthoracic echocardiogram (color-flow Doppler mode) at the level of the tricuspid valve shows functional stenosis. B) Continuous-flow Doppler mode reveals a peak gradient of 14 mmHg and a mean gradient of 9 mmHg.

Fig. 3

Photomicrograph from a biopsy specimen of the mass shows infiltration of cardiac tissue by large atypical cells (H & E, orig. ×100).

Fig. 3

Photomicrograph from a biopsy specimen of the mass shows infiltration of cardiac tissue by large atypical cells (H & E, orig. ×100).

Fig. 4

Transthoracic echocardiograms. A) Twelve days after chemotherapy began, the mass was substantially smaller; B) 18 months later, it had almost disappeared.

Fig. 4

Transthoracic echocardiograms. A) Twelve days after chemotherapy began, the mass was substantially smaller; B) 18 months later, it had almost disappeared.

Comment

Primary cardiac lymphoma is a rare malignant tumor that typically affects the right atrium.1–3  Presenting symptoms include dyspnea, chest pain, arrhythmias, congestive heart failure, and constitutional disorders.2  Our patient's primary cardiac lymphoma is one of the largest reported in the medical literature.4 

Early detection is crucial for better outcomes.5  Rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy is the mainstay of medical therapy.6  Overall, the response rate to therapy was 84%, and 59% of those tumors resolved.2 

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Author notes

Section Editor: Raymond F. Stainback, MD

From: Department of Cardiovascular Diseases (Drs. Gilliland, Harless, Qamruddin, Shah, and Taylor), John Ochsner Heart and Vascular Institute; and Department of Pathology (Dr. Huang), Ochsner Medical Centre and Ochsner Clinical School/The University of Queensland School of Medicine; New Orleans, Louisiana 70121