254 Sherman et al. Jury 1993

American Heart Journal

Ordinarily, surgical intervention would be indicated for persistent bacteremia despite high-dose, appropriate anti­biotic therapy. Because the family refused surgery, this case may illustrate the echocardiographic natural history of acute bacterial endocarditis. Although the specificity of TEE and T T E for the diagnosis of valvular vegetations is similar, TEE is much more sensitive than TTE. In a study by Erbel et al.,^° the sensitivity of TEE compared with TTE was 100% versus 64%. Large vegetations (>10 mm) were visualized equally by using both techniques. For moderate sized (5 to 10 mm) and small (<5 mm) vegeta­tions, however, T T E detected only 69% and 25% of these lesions, respectively. TEE also allows more accurate deter­mination of the size of vegetations, which may have prog­nostic relevance, particularly with regard to their embolic potential.^^ TEE is also superior to TTE in the detection of myocardial abscess complicating endocarditis. In one prospective study, the sensitivity of TEE for detection of an abscess was 87% compared with only 28.3% for TTE.^^ Those patients noted to have an abscess had a higher in-hospital mortality than those without an abscess, which demonstrates the prognostic importance and the diagnos­tic utility of TEE. TEE has also been shown to be more sensitive than T T E for the detection of valve perforation, aneurysm,^^ and delineation of the exact location of vege­tations. This is particularly true when the vegetations are found in atypical locations, including valvular anuli,^'* or other nonvalvular endocardial surfaces.^^

In our patient, although a TV vegetation was seen on the first TTE, no left-sided valvular lesions were visible despite evidence of systemic embolization. The first TEE clearly demonstrated a small MV vegetation. Before the second and third TEEs, again only the TV vegetation was clearly visible on the transthoracic studies, despite the obvious progression of the valvular lesions as demonstrated by TEE. The use of biplane TEE in the evaluation of the RVOT and the PV is clearly demonstrated here. Although a mass was seen in the RVOT on the second TEE, only in the vertical plane could the origin and true size of the veg­etation be appreciated. The vertical plane allows a longi­tudinal view of the RVOT, PV, main pulmonary artery, and proximal peripheral pulmonary arteries. In addition, the vertical plane provides a longitudinal view of the AV and ascending aorta and may allow discrimination of small mobile masses on the AV not visualized in the standard horizontal plane, which may yield only a cross sectional view of the valve.

REFERENCES

1. Dillon JC, Feigenbaum H, Konecke LL, Davis RH, Chang S. Echocardiographic manifestations of valvular vegetations. AM HEART J 1973;86:698-704.

2. Schelbert HR, Muller OF. Detection of fungal vegetations in­volving a Starr-Edwards mitral prosthesis by means of ultra­sound. J Vase Surg 1972;6:20-5.

3. Fisher EA, Stahl JA, Budd JH, Goldman ME. Transesoph­ageal echocardiography: procedures and clinical application. J Am Coll Cardiol 1991;18:1333-48.

4. Dressier FA, Roberts WC. Infective endocarditis in opiate ad­dicts: analysis of 80 cases studied at necropsy. Am J Cardiol 1989;63:1240-57.

5. Robinson MJ, Greenberg JJ, Korn M, Rywlin AM. Infective endocarditis at autopsy: 1965-1969. Am J Med 1972;52:492-8.

6. Chopra P, Subramaniam R. Bhatia ML. Infective endocardi-tis-an autopsy study. Indian J Med Res 1980;72:258-68.

7. Lam D, Emilson B, Rapaport E. Four-valve endocarditis v*?ith associated right ventricular mural vegetations. AM HEART J 1988;115:189-92.

8. Hobbs RD, Downing SE, Andriole VT. Four-valve polymicro­bial endocarditis caused by Pseudomonas aeroginosa and Serratia marcescens. Am J Med 1982;72:164-8.

9. Fairer W. Four-valve endocarditis caused by Corynebacte-rium CDC Group II. South Med J 1987;80:923-5.

10. Erbel R, Rohmann S, Drexler M, Mohr-Kahaly S, Gerharz CD, Iversen S, Oelert H, Meyer J. Improved diagnostic value of echocardiography in patients with infective endocarditis by transesophageal approach: a prospective study. Bur Heart J 1988;9:43-53.

11. Mugge A, Daniel WG, Frank G, Lichtlen PR. Echocardiogra­phy in infective endocarditis: reassessment of prognostic im­plications of vegetation size determined by the transthoracic and the transesophageal approach. J Am Coll Cardiol 1989; 14:631-8.

12. Daniel WG, Mugge A, Martin RP, Lindert 0, Hausmann D, Nonnast-Daniel B, Lass J, Lichtlen PR. Improvement in the diagnosis of abscesses associated with endocarditis by transe­sophageal echocardiography. N Engl J Med 1991;324:795-800.

13. Cziner DG, Rosenzweig BP, Katz ES, Keller AM, Daniel WG, Kronzon I. Transesophageal vs transthoracic echocardiogra­phy for diagnosing mitral valve perforation. Am J Cardiol 1992;69:1495-7.

14. Tunick PA, Freedberg RS, Schrem SS, Kronzon I. Unusual mitral annular vegetation diagnosed by transesophageal ech­ocardiography. AM HEART J 1990;120:444-6.

15. Schwinger ME, Tunick PA, Freedberg RS, Kronzon I. Vege­tations on endocardial surfaces struck by regurgitant jets: di­agnosis by transesophageal echocardiography. AM HEART J 1990;119:1212-5.

Right atrial angiosarcoma causing a coronary artery fistula: Diagnosis by transesophageal echocardiography

David Sherman, MD,^ Craig Smith, MD,*' Charles Marboe, MD,"̂ Ralph Mosca, MD,'' Judah Weinberger, MD.-^ Marco Di Tullio, MD,^ and Shunichi Homma, MD'' New York, N.Y.

Coronary artery fistula (CAP) is a rare abnormality seen in 0.08% to 0.3% of patients undergoing angiography. The vast majority of coronary artery fistulas are congenital; trauma accounts for most of the acquired cases.^ CAP has never before been associated with a malignant tumor. We present the case of a coronary artery fistula caused by a malignant right atrial tumor diagnosed with the use of transesophageal echocardiography (TEE).

From the "Divisions of Cardiology and '^Pathology and the ""Department of Surgery, College of Physicians & Surgeons, Columbia University. Reprint requests: Shunichi Homma, MD, Columbia University, Echocardi­ography - P H 9-Center, 622 West 168th St., New York, NY 10032. AM HEART J 1993;126:254-256

Copyright ® 1993 by Mosby-Yeax Book, Inc. 0002-8703/93/$1.00 + .10 4 / 4 / 4 6 4 0 6

Volume 126, Number 1

American Heart Journal Sherman et al. 255

Fig. 1. Transesophageal four-chamber view showed 2.5 cm X 3.0 cm mass in right atrium (RA, arrow). Area of continuous flow was demonstrated within this mass (between arrow heads). LA, Left atrium; RV, right ventricle; LV, left ventricle.

A 43-year-old man was admitted to our institution with a 2-week history of a nonproductive cough, pleuritic chest pain, low-grade fever, and a new continuous cardiac mur­mur. At admission he was well developed and well nour­ished, with a regular heart rate of 82 beats/min and a blood pressure of 130/70 mm Hg. He had jugular venous disten­sion, his lungs were clear, and his cardiac examination showed a normal Si and S2, a right ventricular heave, and a continuous murmur heard over the right sternal border. Admission electrocardiogram showed a normal axis and a Rsr ' pattern in lead Vi. On transthoracic, two-dimensional echocardiography and color flow Doppler, the right atrium and right ventricle were enlarged, but no other abnormal­ities were noted. The patient subsequently underwent TEE, which showed a 2.5 cm X 3.0 cm mass in the right atrium adjacent to the tricuspid valve ring. A continuous flow was seen on color Doppler from this area into the right atrium consistent with a right coronary artery fistula (Pig. 1). Cardiac catheterization was then performed. The right atrial pressure was 4 mm Hg, right ventricular pressure was 32/8 mm Hg, and the pulmonary arterial pressure was 28/10 mm Hg. There was a 6% to 8% step-up in oxygen saturations from the superior vena cava to the right atrium. On coronary angiography the right coronary artery was oc­cluded proximally, with dye emptying directly into the right atrium. The distal right coronary artery was collater­alized from the left circumflex artery. On aortic root injec­tion no aortic regurgitation was noted, and the dye enter­ing the right coronary appeared in the right atrium. (Fig. 2). Subsequently the heart was explored through a median sternotomy. The pericardium was densely adherent to the right atrium, which was filled with soft, pink, friable ma­terial, mostly in the trabecular portion of the atrium. There appeared to be multiple sites of drainage in the right

Fig. 2. Aortogram in right anterior oblique projection re­veals regurgitation of contrast from aorta into mass over­lying right atrium (arrow).

atrium. The right coronary was opened just past the acute margin, and a segment of saphenous vein was sewn end to end. Microscopic examination of the surgical specimen showed that the atrium was infiltrated by a neoplasm which formed numerous interanastomosing vascular spaces. His­tologic and immunohistochemical staining results were consistent with an epithelioid angiosarcoma. Postopera­tively, chest and abdominal computed tomography showed

256 Sommariva et al. July 1993

American Heart Journal

multiple bilateral pulmonary nodules and a liver lesion. These findings were thought to represent metastatic dis­ease. The patient was discharged for chemotherapy as an outpatient; however, he died several months after dis­charge.

In our case there were two rare findings: a coronary ar­tery fistula £ind a malignant cardiac tumor both occurred in the same patient. There is a previous report of a fistula associated with a left atrial myxoma, which has a tendency to neovascularize.^ However, to our knowledge ours is the first report of a coronary artery fistula resulting from a malignant tumor. Our case has several instructive points. One is that TEE may be quite useful in delineating struc­tures in the right atrium that are not noted on transthoracic study. Two previous case reports described the use of TEE in delineating a coroneiry artery fistula.^' * In our patient the transthoracic study was normal, and the fistula and its as­sociated mass were only detected by using TEE with color flow Doppler. The second point is that in the differential of an acquired coronary artery fistula, the possibility of an invasive cardiac tumor must now be considered.

The most common type of primary malignant cardiac tumor is an angiosarcoma. Glancy et al.^ reviewed 40 patients with angiosarcoma of the heart and noted that the most common complaints were dyspnea, chest pain, fevers, and systemic venous distention. The latter symptom was a result of almost all of the tumors being located on the right side, usually in the right atrium. Although the heart was considered the primary site of tumor in all 40 patients, in 26 of them extracardiac tumor was also present. However, as in our patient, the largest mass of tumor was found in the heart, and in all of the patients the lung, liver, and brain were the most common sites of extracardiac involvement. In patients without any evidence of metastases, complete surgical resection has been attempted, with the median survival in one series improving to 24 months.^ In patients with evidence of metastases, an Adriamycin-based chemo­therapy regimen or radiation therapy have been used; however, the long-term survival is poor. Although our pa­tient's fistula was caused by an invasive tumor, the distal portion of the right coronary artery was well collateralized. Thus the left ventricular wall motion was normal. In con­clusion, an invasive cardiac tumor needs to be considered in a patient with a coronary artery fistula. TEE may be in­valuable in localizing the mass and assessing for associated abnormeil flow.

REFERENCES

1. Sapin P, Frantz E, Jain A, Nichols TC, Dehmer GJ. Coronary artery fistula: an abnormality affecting all age groups. Medi­cine 1990;69(2):101-13.

2. Jorens PG, Van den Heuvel PA, Van Cauwelaert PA, Parizel GA. Myxoma with a left to left shunt and fistula. Chest 1989;96(4):945-6.

3. Kuo C, Chiang C, Fang B, Lee C, Hsu T, Lee Y, Chang C. Cor­onary artery fistula: diagnosis by transesophageal two-dimen­sional and Doppler echocardiography. AM HEART J 1992; 123:218-20.

4. Calafiore PA, Raymond R, Schiavone WA, Rosenkranz ER. Precise evaluation of a complex coronary arteriovenous fis­

tula: the utility of transesophageal color Doppler. J Am Soc Echo 1989;2:337-41.

5. Glancy DL, Morales JB, Roberts WC. Angiosarcoma of the heart. Am J Cardiol 1968;21:413-9.

6. Putnam JB, Sweeney MS, Colon R, Lanza LA, Frazier OH, Cooley DA. Primary cardiac sarcomas. Ann Thorac Surg 1991;51:906-10.

Right atrial myxoma with atypical features of syndrome myxoma

Luigi Sommariva, MD, Angelo Auricchio, MD, Patrizio Polisca, MD, Alfonso Penta de Peppo, MD, and Luigi Chiariello, MD Rome, Italy

"Syndrome myxoma" is usually associated with familial occurrence of cardiac myxoma and concomitant systemic manifestations including pigmented skin lesions, fibroli-pomas, noncardiac myxoid tumors or neurofibromas, and endocrine neoplasm.'^ This syndrome is observed more fre­quently in young women, and generally the cardiac myx­oma arises from the left or right atrial septum. We describe a middle-aged male patient with right-heart failure in whom two-dimensional echocardiography showed a large mass arising "atypically" from the right atrium-inferior vena cava (RA-IVC) junction. The pathologic findings demonstrated a myxoma. In addition, the patient had as­sociated adrenocortical and hepatic masses, cutaneous fi-brolipomas, and blue nevi with no familial occurrence of any abnormality. This unusual clinical presentation is dis­cussed.

A 58-year-old man was admitted with a 2-month history of increasing effort dyspnea associated with signs of right-sided heart failure and a recent episode (10 days previ­ously) of pulmonary embolism. Before the onset of symp­toms, he had generally been well. However, at examination the patient's overall physical condition was severely com­promized, with orthopnea, turgidity of the jugular veins, moderate hepatomegaly, and minimal leg edema; he also had diffuse cutaneous fibrolipomas (diameter 0.5 to 1 cm) and blue nevi. Electrocardiography showed sinus tachy­cardia (110 beats/min) with no other abnormalities. Two-dimensional echocardiography revealed a large atrial mass (length, 8 cm; width 4 cm) with a small pedunculus arising from the RA-IVC junction; the mass across the tricuspid valve had systolic motion that reached the apex of the right ventricle (Figs. 1 and 2). Computed tomography showed

From the Department of Cardiac Surgery, University of Rome "Tor Verga-ta," European Hospital. Reprint requests: Luigi Sommariva, MD, Department of Cardiac Surgery, University of Rome "Tor Vergata," European Hospital, Via Portuense 694, 1-00149 Roma, Italy. AM HEART J 1993;126:256-258

Copyright ® 1993 by Mosby-Year Book, Inc. 0002-8703/93/$ 1.00 + .10 4/4/46410