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Tex Heart Inst J. 2003; 30(1): 62–64.
PMCID: PMC152839

Occult Cardiac Lymphoma Presenting with Cardiac Tamponade

Abstract

Subxiphoid pericardiostomy is the procedure of choice for treatment of a pericardial effusion with tamponade. We report a case in which this procedure not only failed to reveal the presence of an occult malignancy, but also resulted in a recurrent symptomatic effusion. (Tex Heart Inst J 2003;30:62–4)

Key words: Cardiac tamponade/diagnosis/etiology/surgery, catheters, indwelling, heart neoplasms/complications, lymphoma/pathology, pericardial effusion/diagnosis/etiology/surgery, pericardial window techniques, pericardiectomy, pericardium/surgery, postoperative complications/etiology, recurrence, reoperation

Although a variety of surgical techniques to treat pericardial effusion with tamponade have been described, the pericardial window—more appropriately termed the subxiphoid pericardiostomy—remains the procedure of choice. 1 This operation, originally described by Baron Larrey in 1829, 2 was initially believed to accomplish complete and permanent drainage of the pericardium, and to provide adequate samples for cytologic and microbiologic examination. Although the former assertion regarding the mechanism of success that underlies the procedure has been refuted, 3 the usefulness of the pathology specimens obtained is generally considered to be great. 4,5

Herein, we report a case in which a pericardial window failed to reveal the presence of an occult cardiac malignancy, which resulted in both a delay in diagnosis and a symptomatic recurrence of the effusion, requiring definitive therapy.

Case Report

In November 2000, an 83-year-old Filipino man presented at our institution with progressive dyspnea and fatigue. These problems had first been noted several weeks before admission and were associated with a dry, nonproductive cough. In the last few days before presentation, the shortness of breath was noted to be worse when the patient was in the supine position, and he had resorted to sleeping in an upright position.

Review of the patient's medical history revealed no relevant findings. He had not experienced any recent weight loss, fevers, chills, or night sweats. He did not smoke, nor did he routinely consume alcohol. He had returned to the Philippines 4 years earlier, at which time a relative in that country had recently been diagnosed with tuberculosis. The patient had received a bacille Calmette-Guérin (BCG) vaccination as an adolescent.

On examination, the patient was comfortable in his bed and was afebrile, with a blood pressure of 120/80 mmHg and a regular pulse rate of 80 beats/min. He was thin but not cachectic. The jugular veins were distended to a height of 14 cm above the sternal angle while the patient was lying 45° above supine. Auscultation of the chest revealed clear lungs and muffled, distant heart sounds, with a friction rub. No lymphadenopathy was noted in the axillae, neck, or groins. The abdomen was scaphoid and without organomegaly.

A chest radiograph showed a greatly enlarged cardiac silhouette. A transthoracic echocardiogram revealed a large, homogeneous pericardial effusion with right atrial collapse, signifying early tamponade. No abnormalities of the heart, great vessels, or pericardium were noted. Results of a complete blood count, serum chemistry panel, and liver function studies were found to be within normal limits. A mycobacterial antigen skin test resulted in a mild skin reaction.

In order to provide treatment and to discover the cause of this effusion, we performed a subxiphoid pericardiostomy, with the patient under local anesthesia and intravenous sedation. In the operating room, we removed a 3- × 3-cm portion of the anterior pericardium, after which 500 cc of initially clear fluid was drained. Samples of the fluid and pericardium were sent for cytology and pathologic examination, respectively, and for culture. The last 50 cc of pericardial fluid was tinged with blood.

The patient recovered rapidly. As is usually noted, his blood pressure increased immediately in the operating room. The evening after the operation, the patient was free of dyspnea and was able to sleep in the supine position. The next day, the pericardial drain was removed, and the patient was discharged from the hospital. His cough resolved.

Two weeks after discharge, the patient began to develop the same symptoms and physical findings. A repeat transthoracic echocardiogram revealed a recurrence of the pericardial effusion. A heterogeneous area was noted, posterolateral to the left atrium within the pericardial sac (Fig. 1). The patient was returned to the operating room and underwent a sternotomy and an anterior pericardiectomy. An intraoperative transesophageal echocardiogram revealed a mass that was either invading or originating from within the right atrium (Fig. 2). Upon entering the chest, we found the previously placed pericardial window to be widely patent with communication to the peritoneal cavity. The pericardium was filled with bloody fluid, and a large portion of the heart was encased by a granular, friable mass that covered the superior and anterior surfaces of both atria, the root of the pulmonary artery, and the right pulmonary veins. The mass also extended over the apex of the right ventricle, onto the posterior surface of the heart to the level of the coronary sinus. Fluid was again sent to the laboratory for cytologic examination, and several biopsies were taken, including an inconclusive frozen section. We then performed an anterior pericardiectomy.

figure 14FF1
Fig. 1 Transthoracic echocardiogram of the patient upon his admission for recurrence of symptoms. Note: The area labeled Tumor was initially interpreted as clot.
figure 14FF2
Fig. 2 Intraoperative transesophageal echocardiogram. The tumor extends into the right atrial chamber (RA invasion) as indicated by the arrow.

The patient again had an uneventful recovery and went home from the hospital on the 5th postoperative day. He was asymptomatic at that time. Microscopic examination of the tissue samples revealed infiltration of the epicardium by malignant B-cell lymphoma. Immunohistochemistry revealed weak staining for leukocyte common antigen, and strong staining for L26 B cell marker in the malignant population. The patient was referred to our oncology service for subsequent care.

Discussion

Subxiphoid pericardiostomy has been shown to be effective for the treatment of chronic pericardial effusion and useful in the diagnosis of pericardial malignancy. In this case, however, the procedure failed on both accounts.

Pericardial effusion recurs in approximately 1% to 8% of patients after subxiphoid pericardiostomy. 1,3,6 In cases of malignancy, the results are slightly, but not statistically, different, with recurrences in nearly 20% of patients in some series. 6,7 Malignancy may result in pericardial effusion by the obstruction of lymphatic drainage: a process that may defeat the drainage mechanism provided by a subxiphoid window. The case of our patient demonstrates the failure of an adequately performed pericardial window, initially created for what was thought to be benign disease. The immediate recurrence of the effusion heightened our suspicion of an occult malignancy.

Another possible mechanism of pericardiostomy failure may be related to the postoperative management of the patient. Sugimoto and colleagues, 3 in an autopsy review of successful subxiphoid windows in patients with malignant pericardial effusions, described a symphysis between the pericardium and the epicardium, which resulted in the obliteration of the pericardial space. On the basis of their findings and clinical correlation, those authors recommended tube drainage of the pericardial space until this fusion process could begin in earnest. 3 In our patient, drainage ceased within 24 hours of the operation, and the drainage catheter was removed.

Obstruction and malposition of drainage catheters present frequent challenges in surgery. In this patient, malposition may have resulted in a spuriously low postoperative drainage, leading to early catheter removal. In general, another possible cause of inadequate symphysis formation could be a slow but persistent production of pericardial fluid, creating an insignificant daily volume by normal standards but enough to re-form a large effusion over time. Either situation warrants a reevaluation of the routine by which pericardial catheters are removed after pericardiostomy.

Review of the medical literature indicates that symptomatic pericardial effusion, in patients with cancer, is most often the result of malignancy. 8 In the general population, malignancy is one of the top causes of pericardial effusion, responsible for one third to one half of cases. 1 The presence of a sanguine or serosanguineous effusion should raise the suspicion of malignancy. However, lymphoma and mesothelioma often produce false-negative results on cytologic examination. 4 In addition, if the neoplasm involves only the visceral pericardium, a biopsy of the parietal tissue will be nondiagnostic. In our patient, both of these factors led to the initial failure to yield a diagnosis.

The most important factor leading to the success of subxiphoid pericardiostomy as a diagnostic procedure is probably the level of suspicion on the part of the physician. In most patients in whom malignant pericardial effusion occurs, a primary tumor is already known to be present. Malignancies presenting with primary cardiac or pericardial involvement are extremely rare: 1 study examining 789 cases of pericardial tumors noted no primary cases. 4 The route of metastasis of most tumors to the pericardial space is well described. Retrograde lymphatic dissemination of tumor cells from involved mediastinal lymph nodes is thought to be the primary route of carcinomatous metastasis to the heart. In contrast, the hematogenous route has been proposed as the most common mechanism by which lymphomas, leukemias, sarcomas, and melanomas metastasize to the heart. Lymphomas have been reported to constitute over 9% of the total metastases by malignant neoplasms to the heart, and up to 20% of patients with lymphoma are found, at necropsy, to have cardiac involvement. 9 In our patient, no adenopathy or other clinical suggestion of lymphoma was noted; therefore, we had little reason to suspect malignancy.

Several points of interest are illustrated by this case. First, the possibility of malignancy must be considered any time that pericardial effusion occurs in the absence of uremia. Along with this, any bloody fluid drained from a pericardiostomy should heighten the suspicion of malignancy. Failure of pericardial windows may be related to the premature removal of drainage catheters; therefore, when drainage ceases abruptly or prematurely, a test to confirm a dry pericardial sac may be useful to prevent recurrence of effusion. Finally, this case documents the rare occurrence of a B cell lymphoma with the heart as the sole site of involvement on presentation.

Footnotes

Address for reprints: R.L. Quigley, MD, PhD, Division of Cardiothoracic Surgery, Albert Einstein Medical Center, 5501 Old York Road, HB 3, Philadelphia, PA 19141

E-mail: ude.nietsnie@ryelgiuq

References

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