Transvenous biopsy diagnosis of cardiac lymphoma in an AIDS patient

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118

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2

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42 1

syndrome (AIDS). This report documentsan AIDS patient who presented with a pericardial effusion and a cardiac lymphoma that wassubsequentlydiagnosedby tranvenous biopsy. A 40-year-old human immunodeficiency virus positive (HIV+) homosexual male with a history of lymphocytic interstitial pneumonitis wasadmitted with fever, dyspnea, and pleuritic chest pain. Physical examination revealed an acutely ill dyspneic man with a pulserate of 120beats/min. The initial blood pressure was normal but pulsus paradoxus wassubsequentlypresent. The jugular venouspressure was elevated and heart tones were soft but murmurs were absent. The chest percussion revealed dull bases. Auscultation revealed clear upper zones.The abdomenwas distended and was mildly tender. There was no lymphadenopathy. The chest x-ray film revealed a large cardiac silhouette and bilateral pleural effusions.The electrocardiogram showeda sinustachycardia with right axis deviation, REFERENCES low voltage, and nonspecific T wave changes. 1. YoshikawaJ, SabahI, YanagiharaK, Owaki T, Kato H, A two-dimensionalechocardiogramdemonstrateda large TanemotoK. Cross-sectional echocardiographic diagnosis of largeleft atria1tumorandextracardiactumorcompressing the pericardial effusion and evidence of both right atria1 and left atrium. Am J Cardiol1978;42:853-7. right ventricular collapse,consistent with cardiac tampon2. PercyRF,ConettaDA, Miller AB. Esophageal compression of ade. Unexpectedly, one half of the right atrium was occuthe heartpresentingasan extracardiacmasson echocardiopied by a massthat appearedto arise from the interatrial graphy.Chest198$85:826-B. septum (Fig. 1). Clinical improvement occurred after 500 3. BaermanJM, HoganL, SwirynS.Diaphragmatic herniaproducingsymptomsand signsof a left atria1mass.AM HEART J cc of chylous fluid wasremoved from the pericardial space. 1988;116:198-200. This wasrepeated on severaloccasions.Stains and culture 4. Feignenbaum H. Echocardiography. 4th ed.Philadelphia:Lea of the pericardial fluid were negative for bacteria and my& Febiger,1986:592. 5. Khan TA, ShraggeBW, CrispinJS, Linda JF. Esophageal cobacteria. Malignant cells were not found. A subsequent motility in the elderly.Am J DigestDis 1977;22:104$54.6. D&rich H, NicodP. Hoit B. DaltonN. SahnD. Evaluationof Bj8rk-Shilky prostheticvalGesby reai-timetwo-dimensional Doppler echocardiographic flow mapping.AM HEART J

masscompressedthe left atrium and was not an artifact due to far-field imaging and poor lateral resolution.7 Luminal pressuremay have beensufficient to compressthe left atrium, since motor activity may increase pressure throughout the esophagusif the lumen is not ob1iterated.s In addition, the hydrostatic pressureexerted by a column of fluid may contribute to the increasein luminal pressure.2 Thus changesin lower esophagealtone and intermittent esophagealemptying may have resultedin dynamic changes in luminal pressuresufficient to compressand decompress the left atrium. Our caseillustrates that functional aswell asstructural disordersof the esophagusshould be considered in the differential diagnosisof extracardiac masses that compressthe left atrium. It also highlights the superiority of CT scanning over two-dimensional echocardiography for the characterization of extracardiac masses.

1988;115:133-8.

7. NishimuraRA, Tajik AJ, Schattenberg TT, SewardJB. Diaphragmaticherniamimickingan atria1mass:a two-dimensionalechocardiographic pitfall. J AmCo11 Cardiol1988;5:9925. 8. VantrappenG, Janssens J, HellemansJ, Coremans G. Achalasia,diffuseesophageal spasm, andrelatedmotility disorders. Gastroenterology 1979;76:450-7.

Transvenous biopsy diagnosis lymphoma in an AIDS patient

of cardiac

J. Daniel Andress, MD, Louis B. Polish, MD, Donald M. Clark, MD, and Kenneth F. Hossack,MD. Denver,

Colo.

Primary cardiac lymphoma is a rare occurrence; however, several autopsy reportslm3document primary lymphomas occurring in patients with acquired immunodeficiency From the Departments of Medicine and Pathology, Denver General Hospital. Reprint requests: Kenneth F. Hossack, MD, Chief of Cardiology, Denver General Hospital, 777 Bannock St., Denver, CO 80204.

1. This is a frame from the two-dimensionalechocardiographic study in the parasternal short-axis view. The atria1 massis attached to the interatrial septum. A, Aorta; E, effusion; RA, right atrium; RV, right ventricle. Fig.

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Brief Communications

American

August 1989 Heart Journal

Fig. 2. Photomicrograph of the right ventricular endomyocardial biopsy showinginterstitial lymphocytic infiltrate and intact intervening myocardial fibers. Neoplastic cells were not present. (Hematoxylin-eosin stain; original magnification X400.)

3. Photomicrograph of the right atria1massbiopsy showinglymphoma with predominantly intermediate non-cloven cells. There is tumor cell necrosis,occasionalmitoses,and in the top right-hand corner a necrotic myocardial fiber. Also present are bland lymphocytes and in the top center a histiocyte. (Hematoxylin-eosin stain; original magnification X400.) Fig.

two-dimensionalechocardiogramindicated a right ventricular dilation and hypokinesis, while the left ventricle was of normal size and had normal contractility. Diagnostic work-up included thoracocentesis, pleural biopsy, and bone marrow biopsy, all of which were negative for neoplasm.An abdominalcomputed tomography (CT) scanwas normal. Using a femoral vein approach, a right ventricular endomyocardial biopsy and a biopsy of the right atria1masswere performed with a Cordis cardiac biopsy sheath and forceps

(Cordis Corp., Miami, Fla.). Fluoroscopy was used to aid the biopsy of the right atrial mass.Histopathology of the right ventricular biopsy specimen showed an interstitial lymphocytic infiltration consistent with myocarditis, but was negative for neoplastic cells (Fig. 2). Biopsies of the right atria1massrevealeda non-Hodgkin’s lymphoma of an intermediate cell type (Fig. 3). There wasevidence of myocardial cell necrosisand infiltration by the tumor. Chemotherapy with cyclophosphamide, doxorubicin hydrochloride, vincristine, and prednisone was begun and the

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patient was discharged to his home. A follow-up echocardiogram 1 month later showed a definite decrease in the size of the mass. The patient died at home 3 months later, having declined further courses of chemotherapy. Request for autopsy was declined by his family. Primary lymphoma of the heart is exceedingly rare. The association of AIDS and non-Hodgkin’s lymphoma has recently been documented in a study of 90 patients with AIDS who developed lymphoma. Only one patient had cardiac involvement and that was limited to the pericardium.4 The most frequent cardiac involvement with lymphoma appears to be pericardial involvement. Intracavitary masses are the least common. In the majority of cases, the diagnosis of primary cardiac lymphoma in patients with AIDS has been made at autopsy.1-3 In some cases the echocardiogram showed wall motion abnormalities, and at subsequent autopsy these areas were found to be infiltrated with lymphoma. In such cases and in patients in whom an intracavitary mass is present, transvenous biopsy may be a method of making the diagnosis. Although lymphoma was thought to be the most likely diagnosis prior to biopsy, other possibilities included a benign tumor thrombus or an infected thrombus with an unusual organism. Transvenous biopsy was useful in obtaining tissue for diagnosis and avoided the need for surgical exploration. The survival of patients with primary cardiac lymphoma is poor. In this particular case there was partial resolution of the tumor following one dose of chemotherapy. A diagnosis of primary cardiac lymphoma cannot be made conclusively in this patient. Lymphocytic interstitial pneumonitis is regarded by some as part of the lymphoma spectrum. Although the clinical studies failed to demonstrate extracardiac involvement, an autopsy was not done to confirm this. This and other reports indicate that cardiac involvement including lymphoma, Kaposi’s sarcoma,5 and myocarditi@ with or without clinical evidence of cardiomyopathy is becoming an increasing problem in patients with AIDS.

REFERENCES 1.

2. 3. 4.

5. 6.

Gurner J, Brynes RK, Ghan WC, Bordsong G, Hertzler G. Primary non-Hodgkin’s lymphoma of the heart in two patients with acquired immunodeficiency syndrome. Arch Path01 Lab Med 198’7;111:254. Balasubramanyam A, Waxman M, Kazal HL, Lee MJ. Malignant lymphoma of the heart in acquired immune deficiency syndrome. Chest 1986;90:243. Gill PS. Chandraratna PAN. Mever PR. Levine AM. Malianant lymphoma: cardiac involvement at’initial presentation. J Clin Oncol 1987;5:216. Ziegler JL, Beckstead JA, Volberding PA, et al. Non-Hodgkin’s lymphoma in 90 homosexual men. Relation to generalized lvmphadenopathy and the acquired immunodeficiency syndrome. N Engl J-Med 1984;311:565. Silver MA. Macher AM. Reichert CM. Cardiac involvement in acquired immune deficiency_ syndrome (AIDS). Am J Cardiol _ 1984;53:983. Cohen JS, Anderson DW, Virmani R, et al. Congestive cardiomyopathy in association with the acquired immunodeficiency syndrome. N Engl J Med 1986;315:628. I

423

Surgical repair of atrial septal defect in an 8g-year-old man: Progressive shunt due to concomitant aortic stenosis Robert E. Pryor, MD, Lisa Giannetto, MD, and Thomas M. Bashore, MD. Durham, N.C.

Adult patients with atria1 septal defect (ASD) may remain asymptomatic for many years, and survival beyond the ninth

decade

has been reported.l,

2 Few reports

exist of

ASD closure in patients over the age of 80, however. We present the oldest patient in whom successfulASD repair has been reported. A previously healthy 89-year-old man was referred for evaluation of congestive heart failure (CHF) and aortic stenosis. A heart murmur had been noted

at age 21, but he was asymptomatic until age87, when he developed dyspneawhile deer hunting. In December 1986 he presentedwith classIV CHF and atria1 flutter. The patient was placed on a regimen

of furosemide,

digoxin,

and

quinidine, and wasreferred to our institution. Physical examination revealeda thin white man who was mildly dyspneic at rest. Blood pressurewas110/70mm Hg, and pulse was 72 beats/min and regular. Jugular venous pulsations were 10 cm above the sternal angle with a prominent V wave. Lungs were clear. Cardiac examination showeda normal Si and a soft Az with normal splitting of Ss. A II/VI high-pitched crescendo-decrescendomurmur washeard at the right upper sternal border and radiated to the carotids. A III/VI medium-pitched holosystolic murmur was heard at the left lower sternal border. There was l+ pedal edema. The remainder of the examination was unremarkable. Chest x-ray films demonstrated right-sided

cardiomegalyand enlargementof the proximal pulmonary arteries, felt to be consistent

with pulmonary

hypertension.

Echocardiogramwith Doppler and microcavitation studies revealed mitral and tricuspid insufficiency, aortic stenosis, and bidirectional shunting at the atria1 level. At cardiac catheterization, the coronary arteries were normal and ejection fraction was 47 % . There was mild mitral regurgitation, moderate aortic stenosis (calculated aortic valve area 0.8 cm2), and severe tricuspid insufficiency. A sinus venosus ASD was found with a net left-to-right shunt of 3.O:l. Pulmonary artery pressurewas51/14 mm Hg (mean 29 mm Hg) with a pulmonary wedge pressure of 12 mm Hg.

At surgery, the ASD wasclosedwith a pericardial patch and a Kay tricuspid

annuloplasty*

was performed.

The patient

tolerated the procedure well and was discharged on the tenth postoperative day. Two years later, he is asymptomatic and has resumed his annual hunting trips.

From

the Department

of Medicine,

Reprint requests: Thomas Box 3012, Duke University *Kay JH, et al. Surgical 1965;162:53-8.

Duke

University

Medical

Center.

M. Bashore, MD, Cardiovascular Laboratory, Medical Center, Durham, NC 27710. treatment

of tricuspid

insufficiency.

Ann

Surg