Journal of Cardiothoracic and Vascular Anesthesia
Volume 26, Issue 1 , Pages 172-174, February 2012

Dizziness in a Patient With a Right Atrial Mass

  • Zafar Iqbal, MD
    • ,
  • Steven Sivils, DO
    • ,
  • Paul S. Pagel, MD, PhD

      Affiliations

    • Corresponding Author InformationAddress reprint requests to Paul S. Pagel, MD, PhD, Clement J. Zablocki Veterans Affairs Medical Center, Anesthesia Service, 5000 W National Avenue, Milwaukee, WI 53295

published online 20 September 2010.

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Article Outline

Key words:  cardiac tumor , primary cardiac lymphoma , sinoatrial node dysfunction , near-syncope , third-degree heart block

 

A 63-YEAR-OLD, 93-kg, 170-cm man with an extensive history of cardiovascular disease presented to the cardiology clinic in the authors' institution complaining of “feeling lightheaded and dizzy.” The patient stated that his symptoms initially began 3 months before this admission. His dizziness occurred as often as once per minute, was often accompanied by dyspnea, and caused him to awaken several times per night. The patient further reported that he could feel his heart “pause.” He denied syncope, chest pain, and other constitutional complaints including fever, night sweats, and weight loss. The past medical history was notable for coronary artery and peripheral vascular disease. The patient had suffered 3 myocardial infarctions; a left anterior descending coronary artery stent was implanted after the most recent of these events. Global left ventricular (LV) hypokinesis and LV systolic dysfunction (ejection fraction of 30%) were present on a previous transthoracic echocardiogram, consistent with the diagnosis of ischemic cardiomyopathy. Several previous hospital admissions for congestive heart failure also were described. The patient was treated chronically with carvedilol, lisinopril, furosemide, and warfarin. An electrocardiogram was performed in the clinic that showed a 10-second pause. Carvedilol immediately was discontinued, the patient was admitted to a telemetry unit, and several additional pauses in cardiac electrical activity ranging between 5 and 10 seconds in duration were noted. Transesophageal echocardiography was performed as part of the evaluation (Fig 1 and Video 1 [supplementary video is available online]). What is the diagnosis?

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Diagnosis: Primary Cardiac Lymphoma of the Right Atrium Involving the Sinoatrial Node 

A midesophageal transesophageal echocardiographic 4-chamber image showed the presence of a large, polypoid mass in the right atrium (Fig 1) that did not prolapse into the tricuspid valve inlet or create functional tricuspid stenosis or regurgitation. The initial differential diagnosis included right atrial thrombus and myxoma, but the latter diagnosis was favored because the patient was chronically receiving warfarin. A magnetic resonance imaging study confirmed the presence of a large, lobulated, nonenhancing 5.9 × 4.3 × 4.0 cm mass within the right atrium (Fig 2). The mass did not have a discernable stalk but instead appeared to be a broad-based structure that was either adherent to or continuous with the right atrial wall. The patient was taken to the operating room for debulking of the mass. Inspection of the heart after the pericardium was opened indicated that most of the superior aspects of the right atrium, including the superior vena cava–right atrial junction and the sinoatrial node, were thickened markedly by tumor infiltration originating from the interior of the right atrial chamber. A small pink-tinged nodule at the junction of the pulmonary artery and the aorta was excised. Frozen-section analysis of the nodule revealed an atypical lymphoid infiltrate that was highly suspicious for a lymphoproliferative disorder. Other sites of pericardial tumor implantation also were identified. The majority of the tumor mass was excised through an atriotomy. Atrial and ventricular pacing electrodes were implanted for temporary pacing. The patient separated from cardiopulmonary bypass using inotropic support and was transported to the intensive care unit in stable condition. The pericardial and right atrial tumor biopsies were identified as small B-cell CD20+ non-Hodgkin lymphocytic lymphoma (Fig 3). No evidence of lymphoma or lymphocytic leukemia was observed on microscopic examination of a subsequent bone marrow aspirate and biopsy. A positron emission tomography scan failed to show suspicious lymphadenopathy. A dual-chamber permanent pacemaker was implanted 3 days after surgery for the definitive treatment of the bradyarrhythmia. The remainder of the hospital course was uneventful, and the patient was discharged on the 8th postoperative day. He is scheduled to undergo R-CHOP (rituximab [a monoclonal antibody directed against the B-cell CD20 protein] plus cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy1, 2 for the treatment of the primary cardiac lymphoma.

  • View full-size image.
  • Fig 3. 

    A photomicrograph showing hematoxylin-eosin stain of the right atrial myocardium with extensive infiltration of lymphocytic lymphoma cells. (Color version of figure is available online.)

Primary cardiac lymphoma is defined as a lymphoma in which the bulk of the tumor mass is localized within the heart or pericardium.3 Unlike the relatively frequent (10%-20%) occurrence of cardiac involvement in patients with disseminated Hodgkin or non-Hodgkin lymphoma,4 primary cardiac lymphoma is unusual,5 comprising approximately 0.5% of extranodal lymphomas and 1% to 2% of primary cardiac tumors.6, 7 The incidence of primary cardiac lymphoma may be greater in the presence of immunosuppression, resulting from HIV infection or organ transplantation.8 The relative rarity of primary cardiac lymphoma, the often nonspecific nature of the clinical presentation, and the frequently advanced stage when the diagnosis is established contribute to a notoriously poor prognosis.9 Transesophageal echocardiography has been shown to be an especially valuable method to identify primary cardiac lymphoma.10 As observed in the current patient, primary cardiac lymphoma is most common among men in the 6th or 7th decade of life5, 11 and most often presents in the right atrium.12 The clinical manifestations of primary cardiac lymphoma are diverse and vary considerably depending on the areas of the heart involved. Pericardial effusion, congestive heart failure, and atrial-ventricular conduction abnormalities are especially common clinical manifestations of primary cardiac lymphoma, whereas constitutional signs and symptoms of lymphoma (eg, fever, weight loss, night sweats, and peripheral adenopathy) occur less often.7 Caval compression syndromes,8 embolic stroke,13 and pulmonary embolism14 also have been reported. In a recent review of primary cardiac lymphoma, Miguel and Bestetti7 reported that 19% (6/32) of patients with the disease initially presented with intermittent or sustained third-degree heart block as a result of tumor infiltration into the conduction system. The current patient was similar to many of these previous cases15, 16, 17, 18, 19 because the first manifestation of his disease was intermittent complete heart block concomitant with dizziness and near-syncope. Most of these patients were treated using surgical debulking and adjuvant R-CHOP chemotherapy with or without autologous blood stem cell transplantation.20 This approach has been shown to substantially improve survival.7

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Supplementary data 

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Video 1. A midesophageal 4-chamber transesophageal echocardiographic image showing a large polypoid mass in the right atrium.

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References 

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  18. Motto A , Ballo P , Zito D , et al.  Primary cardiac lymphoma presenting as sick sinus syndrome . J Clin Oncol . 2008;26:6003–6005
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  20. Nonami A , Takenaka K , Kamezaki K , et al.  Successful treatment of primary cardiac lymphoma by rituximab-CHOP and high-dose chemotheraphy with autologous peripheral blood stem cell transplantation . Int J Hematol . 2007;85:264–266

 Paul S. Pagel, MD, PhDSection Editor

PII: S1053-0770(10)00311-3

doi:10.1053/j.jvca.2010.07.014

Journal of Cardiothoracic and Vascular Anesthesia
Volume 26, Issue 1 , Pages 172-174, February 2012

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