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pericardial effusions by Schiller and Botvinick,5 but those authors did not discuss regional anterior tamponade, nor did they mention paradoxical RV wall motion. To our knowledge, paradoxical motion of the RV anterior wall has not been described before. We speculate that high pressure in the anterior loculated effusion produces maximum com- pression of the RV anterior wall in diastole; in systole, an RV pressure rise with ventricular contraction partly over- comes deformation of this chamber by the effusion and re- sults in systolic anterior motion of the RV anterior wall.

Early diastolic inward (posterior) motion of the RV an- terior wall is well recognized as a useful sign of tamponade in patients with circumcardiac (nonloculated) pericardial effusions.6 In that situation, RV anterior wall motion is normal and the M-mode contour is quite different from the pattern encountered in our case. We believe that the echocardiographic detection of a large anterior space com- pressing the right ventricle, with paradoxical systolic mo- tion of the RV anterior wall in a patient recently subjected

to cardiac surgery, may signify cardiac tamponade. Timely diagnosis of this unusual but serious complication and prompt relief of cardiac compression by the hematoma, could be life-saving.

REFERENCES

1.

2.

3.

4.

5.

6.

D’Cruz IA, Kensey K, Campbell C, Replogle R, Jain M. Two- dimensional echocardiography in cardiac tamponade oc- curring after cardiac surgerv. J Am Co11 Cardiol 1985:5: 1250-i

- ”

D’Cruz IA, Kleinman D. Extracardiac causes of paradoxical motion of the left ventricular wall. AM HEART J 1988;115: 473-5. Steele RL, Perez JE. Left ventricular diastolic collapse pro- voking cardiac tamponade. Echocardiography 1986;3:149- 50. Chuttani K, Pandian NG, Mohanty PK, Rosenfield K, Schwartz SL, Udelson JE, Simonetti J, Kusay BS, Caldeira ME. Left ventricular diastolic collapse: an echocardiographic sign of regional cardiac tamponade. Circulation 1991;83:1999- 2006. Schiller NB, Botvinick EH. Right ventricular compression as a sign of cardiac tamponade. Circulation 1977;56:774-9. Armstrong WF, Schilt RF, Helper DJ, Dillon JC, Feigenbaum H. Diastolic collapse of the right ventricle with cardiac tam- ponade. Circulation 1982;65:1491-6.

Malignant mesenchymoma as a primary cardiac tumor

Patrice A. McKenney, MD, Krzysztof Moroz, MD, Christian C. Haudenschild, MD, Richard J. Shemin, MD, and Ravin Davidoff, MB, BCh. Boston, Muss.

From the Evans Memorial Department of Clinical Research and the Department of Cardiothoracic Surgery, Boston University Medical Center. Reprint requests: Patrice A. McKenney, MD, Division of Cardiology, Uni- versity Hospital, 88 E. Newton St., Boston, MA 02118. 414135474

Brief Communications 1071

Malignant mesenchymomas by definition are composed of two or more cellular types that would ordinarily derive from primitive mesenchyme.l They grow rapidly, recur frequently, metastasize, and can be found in a wide variety of locations. Most, occur in the extremities, but extremely rarely malignant, mesenchymomas appear as primary car- diac tumors. Inasmuch as the clinical and echocardio- graphic features are not well described, this tumor may be confused with the more common benign atria1 myxoma. We report the first case of primary cardiac malignant mesen- chymoma to be imaged by transesophageal echocardi- ography and review the world literature on this disease en- tity.

A 4%year-old woman initially had transient left-sided numbness and weakness. Over the next 3 months she had progressive respiratory distress. Echocardiography re- vealed a large left atria1 mass. At surgical resection, the mass was gelatinous, broad based, and infiltrated exten- sively into the posterior wall of the left atrium. Initial his- tologic findings were consistent with a diagnosis of myx- oma. Two months postoperatively she had recurrent symp- toms. Review of the original pathologic findings revealed leiomyosarcoma. On transfer to our institution, results of physical examination were notable for respiratory distress, bibasilar rales, and a grade 3/6 mitral regurgitant murmur. Transthoracic echocardiography documented a recurrent left atria1 mass, with a mean mitral gradient of 24 mm Hg and moderate mitral regurgitation on Doppler examina- tion. Transesophageal echocardiography showed a large mass with variable echogenicity filling the left atrium and prolapsing through the mitral valve (Fig. l), with extension into the pulmonary veins. Results of surgical exploration confirmed that the tumor was grossly unresectable and did extend into the pulmonary veins. Palliative excision of the mass and the entire posterior left atrium was performed. The atrium was reconstructed with a bovine pericardial patch sized as large as possible given the likelihood of re- current obstruction. Results of metastatic evaluation were normal. The patient received combination chemotherapy, and there was no evidence of recurrence 6 months later. The pathologic findings at that time showed multiple cell lines of differentiation consistent with malignant mesen- chymoma. The predominant component was fibrosarco- matous, comprising approximately 60 % of the tumor mass. Well-demarcated areas of chondrosarcomatous differenti- ation (Fig. 2) and foci of osteogenic sarcoma (Fig. 3) were also identified. Rhabdomyosarcoma was found within the most myxomatous parts, often adjacent to areas of exten- sive necrosis (Fig. 4). Storiform and pleomorphic areas of malignant fibrous histiocytoma (Fig. 5) were distinct from the leiomyosarcomatous component and showed a charac- teristic fascicular arrangement with “cigar-shaped” nuclei. There were also areas of angiosarcomatous differentiation, which merged with more anaplastic and undifferentiated foci. The immunocytochemical stains and electron micro- scopic findings confirmed the mesenchymal nature of this tumor and its pleomorphic sarcomatous differentiation.

Malignant mesenchymomas are composed of malignant cells differentiating into two or more cell lines. The origin of the tumor is uncertain, but it may arise from congenitally

1072 Brief Communication-s April 1992

American Hearl Journal

Fig. 1. Transesophageal echocardiogram in standard four-chamber view showing malignant mesenchy- moma filling left atrium and prolapsing through mitral valve. LA, Left atrium; LV, left ventricle; MV, mi- tral valve; RA, right atrium; RV, right ventricle.

Table I. Summary of reported cases of primary cardiac malignant mesenchymoma

Reference Year Sex

Hagstrom 1961 F Stemmermann et a1.4 1965 F Sterns et a1.5 1966 F Muir and Seah6 1966 F Mori et a1.7 1971 M Nagamine et a1.s 1975 F Sasaki et al.’ 1977 F Sasaki et al.’ 1977 M Hoshino et aLlo 1981 F

Frandsen et a1.l’ 1981 F

Ceretto et a1.12 1981 M

Tanaka et a1.13 1982 F

Nonumura et al.‘* 1988 M

Present case 1992 F

Age fyr)

59 26 26 50 39 34 48 27 27

39 60

46

61

48

site of origin

Pulmonary trunk Left atrium Atria1 septum Left ventricle Left atrium Atria1 septum Left atrium Left atrium Left atrium

Atria1 septum Pulmonary trunk,

RVOT Left atrium

Pulmonary trunk Left atrium

Treatment

0 0 0 0

0 Surgery Surgery Surgery,

chemotherapy 0

Surgery

Surgery, chemotherapy, RT

0 Surgery,

chemotherapy

Survival (mo)

43 48 12 10 16

7 15

8 21

5

35

26 Alive

RT, Radiation therapy; RVOT, right ventricular outflow tract

misplaced mesenchymal cells giving rise to dysontogenetic other sites of origin including the atria1 septum, right ven- growths1 or from a primitive precursor mesenchymal cell tricular outflow tract, pulmonary trunk, and left ventricle. with incomplete, immature, or deviated differentiation.2 The usual presenting symptoms are dyspnea and fatigue. These tumors can occur in many locations but are rarely Metastases are frequent, particularly to the lung (43%). seen as primary cardiac tumors. All known reports of pri- Treatment regimens have included surgery, chemotherapy, mary cardiac malignant mesenchymomas are summarized and radiation therapy. Survival ranges from 5 to 48 months in Table I.3-14 Age at presentation ranged from 26 to 61 after the onset of symptoms, with death usually the result years. Ten of the 14 patients were women, suggesting that of local obstruction and refractory congestive heart failure. this tumor may be more likely to develop in women. The Diagnosis is usually made at autopsy, but the tumor has left atrium is the most frequent site of involvement, with been detected by angiography in four patients and by

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Number 4, Part 1 Brief Communications 1073

Fig. 2. Chondrosarcomatous differentiation. Malignant cartilage (left) adjacent to anaplastic tumor cells (right). (Hematoxylin-eosin stain; x170.)

Fig. 3. Osteosarcomatous differentiation. Malignant osteoblasts surrounded by focally calcified osteoid (dark areas). (Hematoxylin-eosin stain; x170.)

transthoracic echocardiography in three. In our patient transesophageal echocardiography demonstrated that the tumor was not homogeneous, possibly because of varying consistencies of the multiple different cell lines. The tumor filled the left atrium and was broadly attached to the pos- terior wall with involvement of the pulmonary veins. Pro- lapse through the mitral valve created both obstruction to inflow and regurgitation.

In summary, malignant mesenchymomas may originate from primitive fibrous tissue, with differentiation into multiple mesenchymal patterns. They rarely are seen as primary cardiac tumors, but when they are they are most often found in the left atrium of young women and may be

confused with the more common myxoma. Echocardio- graphically, suspicion of a malignancy should be high in the presence of rapid tumor growth, absence of a stalk, com- bined intramural and intracavitary location, and extension into the pulmonary veins. Transesophageal echocardi- ography visualizes the tumor well and is useful for assess- ment of the extent of the tumor preoperatively. Patholog- ically, areas of hypercellularity with pleomorphism, mito- ses, necrosis, or extensive vascularity are present on light microscopy. Evaluation of multiple areas with light and electron microscopy, as well as immunocytochemistry, is necessary to confirm the diagnosis. Although this tumor can metastasize, death is usually the result of local exten-

1074 Brief Communications April 1992

American Heart Journal

Fig. 4. Rhabdomyosarcomatous differentiation. Myxomatous area with hyperchromatic, round, and strap-shaped malignant rhabdomyoblasts (arrows). (Hematoxylin-eosin stain; X170.1

Fig. 5. Differentiation into malignant fibrous histiocytoma. Pleomorphic, storiform arrangement with malignant giant cells. (Hematoxylin-eosin stain; ~170.)

sion and obstruction. The prognosis is poor in symptomatic persons, but earlier recognition coupled with aggressive management may improve survival.

REFERENCES

1. Stout AP. Mesenchymoma, the mixed tumor of mesenchymal derivatives. Ann Surg 1948;127:278-90.

2. Klima M, Smith M, Spjut HJ, Root EN. Malignant mesen- chymoma; case report with electron microscopic study. Can- cer 1975;36:1086-94.

3. Hagstrom L. Malignant mesenchymoma in the pulmonary ar- tery and right ventricle. Acta Path01 Microbial Stand 1961; 51:87-94.

4. Stemmermann GN, Kim PM, Berk ME. Malignant mesen- chymoma of the heart. Lancet 1965;85:213-7.

5. Sterns LP, Eliot RS, Varco RL, Edwards JE. Intracavitary cardiac neoplasms. Br Heart J 1966;28:75-83.

6. Muir CS, Seah CS. Primary chondrosarcomatous mesenchy- moma of the mitral valve. Thorax 1966;21:254-62.

7. Mori Y, Sakatani K, Sakamoto T, Miyazawa K. Primary car- diac sarcomas [in Japanese]. Nihon Univ Med J 1971;30:709- 17.

8. Nagamine Y, Sasai K, Sasaki K. Malignant mesenchymoma of the heart. Acta Path01 Jpn 1975;25:241-9.

9. Sasaki S, Young TL, Redington JV, Mendez M, Zubiate P, Kay JH. Primary intracavitary cardiac tumors. J Cardiovasc Surg 1977;18:15-21.

10. Hoshino Y, Fujiwara T, Karino K, Nishimura S, Kamada K, Watanabe N, Yamada 0. Inaoka M, Ohno T. Komatsu S, To- moyori T. Primary malignant mesenchymoma of the heart [in Japanese]. J Jpn Assoc Thorac Surg 1981;29:1076-85.

11. Frandsen NE, Andersen G, Nielsen JR. Malignant mesenchy- moma of the heart presenting as mitral stenosis. Acta Med Stand 1981;209:235-7.

12. Ceretto WJ, Miller ML, Shea PM, Gregory CW, Vieweg WVR.

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Malignant mesenchymoma obstructing the right ventricular outflow tract. AM HEART J 1981;101:114-5.

13. Tanaka T, Bunai Y, Nishikawa A, Kawai T, Mori H, Taka- hashi M. Malignant mesenchymoma of the heart. Acta Path01 Jpn 1982;32:851-9.

14. Nonumura A, Kurmaya H, Naoko K, Nakanuma Y, Ohta G, Terahata S, Matsubara F, Matsuda T, Asaka T, Nishino T. Primary pulmonary artery sarcoma. Acta Pathol Jpn 1988; 38:883-96.

Double-outlet left ventricle: Two-dimensional echocardiographic diagnosis

Bruno Marino, MD, and Maurizio Bevilacqua, MD. Rome, Italy

From the Department of Pediatric Cardiology, Bambino Gesu’ Hospital.

Reprint requests: Bruno Marina, MD, Dipartimento Medico-Chirurgico di Cardiologia Pediatrica. Ospedale Bambino Gesu’, Piazza Sant’Onofrio, 4, 00165 Rome, Italy.

4/4/35405

Double-outlet left ventricle (DOLV) is the rarest type of ventriculoarterial connection. The existence of this mal- formation has been questioned on the basis of embryologic findings,l but numerous studies over the past 20 years have described the anatomic,2-4 angiocardiographic,5,6 and sur- gical aspects7-g of this anomaly. Although many anatomic classifications have been attempted,3-6 the essential fea- tures of this malformation remain the exclusive or preva- lent connection of the great arteries with the left ventricle, the spatial relationships of the vessels, and the position of the ventricular septal defect (VSD). To our knowledge there is only one abstract in the literature concerning the echocardiographic diagnosis of DOLV.‘O We describe the two-dimensional echocardiographic aspects of two addi- tional cases of DOLV.

Case No. 1. A 4-month-old female infant weighing 4.2 kg was admitted to our unit with a history of respiratory in- fections and failure to thrive. On clinical examination she had congestive heart failure, minimal cyanosis, and a sys- tolic murmur. The chest x-ray showed cardiomegaly and increased pulmonary vascular markings, and the ECG re- vealed biventricular hypertrophy. Results of two-dimen- sional echocardiography showed situs solitus, levocardia,

Fig. 1. A, Case 1. Two-dimensional echocardiographic study. Subcostal long-axis view showing leftward aorta (A) arising from left ventricle (LV) surrounded by muscular infundibulum. RV, Right ventricle. 6, Case 1. Two-dimensional echocardiographic study. More posterior view showing rightward pulmonary ar- tery (P) arising from left ventricle (LV) and subpulmonary VSD. LA, Left atrium; RV, right ventricle. C, Case 1. Angiocardiography in anterioposterior view showing both great arteries arising from left ventricle (Lb’). Aorta (A) is left sided. P, Pulmonary artery. D, Case 1. Angiocardiography in lateral view showing both great arteries arising from left ventricle (LV). Aorta (A) is anterior. P, Pulmonary artery.