A c t a Med Scand 206: 123-126, 1979

Massive Embolization of Cardiac Myxoma A Cuse Report

Gunnar Tornvall and Christian O h From the Medical Department, Subbatsberg’s Hospital, and the Thoracic Surgical Clinic,

Karolinska Hospital. Stockholm, Sweden

ABSTRACT. A case of left atrial myxoma with massive embolization in a 20-year-old woman is described. The initial clinieal picture was confusing, but repeated examinations, including enzyme determinations and arteriographies, disclosed the presence of multiple arterial emboli. At embolectomy, histological investigation of embolic masses showed a picture indicating cardiac myxoma. This diagnosis was confirmed by pulmonary angiography and the tumour was removed. Postischaemic oedema of the legs necessitated decompression operations. The peripheral circulation was restored and the final result was excellent. Some aspects of the clinical presentation of this uncommon disease are discussed. Key words: cardiac myxoma, arterial embolization, postischaemic oedema, fasciotomy. Acta Med Scand 206: 123. 1979.

Intracardiac myxoma is a rare tumour that occasionally may cause dramatic and confusing symptoms. W e have recently experienced a case of left atrial myxoma in a young woman presenting with leg cramps and subsequently offering some unusual problems regarding both diagnosis and treatment.

CASE REPOR’T A previously healthy 20-year-old woman was suddenly taken ill with bilateral leg cramps while swimming. As the cramps were quite severe and persistent, she was brought to hospital without delay. On admission to Sabbatsberg’s Hospital on the evening ofJuly 25th, 1976, she complained of painful muscular cramps in both legs and coldness in her left foot. On examination, however, the dorsal arterial pulsations were palpable on both sides and the temperature of the feet did not differ. Physical examination of the heart was normal and the BP was 100/90 mmHg. Laboratory investigation revealed low levels of serum sodium (129 mmol/l) and serum potassium (2.8 mmol/l). As the patient had been standing in the sun all d a y - o n e of the hottest during that summer-a salt depletion syndrome (“miner’s cramps”) was suspected. She was ac-

cordingly treated with salt and fluid replacement; but in spite of normalization of the electrolyte values the painful cramps persisted and she required repeated doses of sedatives and analgesics. During the following hours her condition deteriorated. She complained of abdominal cramps, nausea and vomiting. An abdominal radiographic examination was negative. Six hours after admission, red urine was noted and was shown by laboratory analysis to be caused by myoglobin. Furthermore, CPK and liver enzymes (ASAT and ALAT) were greatly elevated. A repeat examination of the legs now disclosed that the circulation in the left leg had deteriorated and that the pulse of the left femoral artery could no longer be palpated. An arteriography was immediately performed, showing a total occlusion of the left external iliac artery and some smaller contrast defects in other vessels (Fig. 1). The patient was referred to the Thoracic Surgical Clinic on the evening of July 26 for further treatment. On admission she still complained of pain in both legs. The muscular cramps were less pronounced. The left leg was pale and cold below the middle of the thigh and she was not able to move the lower leg. The right foot was also somewhat cold but no severe circulatory disturbance was present on that side. Chest X-ray and ECG were normal. An acute embolectomy was immediately performed and, through an incision in the left femoral artery, large amounts of red-yellowish thrombus could be extracted both proximally and peripherally with the aid of a Fogarty catheter. The specimen was sent for microscopic investigation. After completion of the embolectomy, a blood flow of 300 ml/min was measured in the femoral artery and the foot immediately became red and warm. Some hours after operation the left leg started to swell, due to a diffuse oedema in the previously ischaemic musculature. Eight hours after the embolectomy the lower leg was so intensively swollen that the venous circulation began to be compromised. To prevent further deterioration of the circulation and, in order to decompress the calf muscles that were in immediate danger of pressure necroses, a fasciotomy operation had to be performed. The oedematous muscles literally bulged out through the incision, leaving a 15 cm diastasis between the skin edges. In the meantime the right foot had begun to be ischaemic and an attempt was made to perform an embolectomy on the right leg. Even though the embolectomy catheter could be passed all the way down to the foot, no Acto Mrd Scund 206

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G. Tornvull und C . Olin without problems (Fig. 2). Except for postoperative bleeding, which necessitated re-operation, the postoperative course was uneventful and the patient made a surprisingly rapid recovery. The kidney function returned to normal within a week. Rehabilitation was for natural reasons hampered by the large open wounds on both lower legs. A skin transplantation was suggested by the plastic surgeons, but had to be postponed due to a wound infection. The swelling of the calf muscles had in the meantime diminished and after a weeks daily application of wound tape, the incisions after the fasciotomy could be closed by direct sutures on both sides, leaving a very satisfactory cosmetic result. A more active mobilization program could now be started and after about a month the patient was able to walk again. At a follow-up examination in Jan. 1977 she could walk without problems and had resumed work. The left leg was slightly slimmer than the right and some reduction of muscular strength was evident. Circulation was normal in both legs.

Fig. 1 . Angiogram showing complete occlusion of left external iliac artery.

embolic material could be retrieved. A new arteriography was therefore performed on the right leg and showed a subtotal occlusion of the popliteal artery immediately above the knee. A repeat embolectomy, this time through the right popliteal artery, was performed and an embolus that was partly attached to the vessel wall could be extracted with the aid of a Fogarty catheter. In addition, some fresh thrombotic material could be removed from the distal arteries of the leg. The right foot immediately became warm and distant pulses could be palpated. Following the operation, however, the same thing happened as on the left side, namely the right lower leg became so swollen due to the postischaemic oedema that a fasciotomy procedure had to be carried out on this side as well. At this stage the general condition of the patient was markedly affected. Due to renal damage by myoglobin precipitation and to the repeated operations and blood transfusions, the kidneys started to fail. Serum creatinine wa above 600 mmol/l. Fluid accumulated in the lungs, but the condition could be controlled with intermittent respirator treatment and large doses of furosemide. The result of the microscopic examination of the emboli was available on the sixth day after the first embolectomy. A strong suspicion of cells originating from a cardiac myxoma was at hand. This finding motivated an angiocardiographic examination of the heart. A pulmonary angiogram was performed and a 4 x 6 cm large contrast defect was seen in the left atrium, suggesting the presence of a pedunculated myxoma. On the night after the investigation the patient experienced a sudden thrust of pain in the right hypocondrium, suggesting an embolus to the liver. It wa5 decided not to wait with removal of the myxoma. On the next day, Aug. 4, the operation was performed through a median sternotomy and with the aid of extracomoreal circulation and temporarv cardiac arrest. The friable myxoma together with its attachment on the base of the interatrial septum could be removed (in one piece) .

COMMENTS Peripheral arterial embolism in a previously healthy young individual is a rare occurrence. Among conditions reported to cause arterial emboli in young people may be mentioned bacterial endocarditis, mitral valve disease with atrial fibrillation and certain uncommon coagulation disorders. None of these conditions could be demonstrated in our patient. She had no history of fever, cardiac symptoms or coagulation problems. The heart was normal on auscultation, ECG and chest X-ray. The clue to the

I

Fig. 2. The removed myxoma with its attachment to the interatrial septum.

M~ssiiieeniboliztrtion of cardiac rnyxomci diagnosis was obtained through microscopic examination of the embolic material from the legs. When the massive nature of the embolization was disclosed, atrial myxoma was considered a possibility, although her age and the absence of cardiac symptoms spoke against this diagnosis. The initial clinical picture, with bilateral severe leg cramps, was most confusing. The pronounced muscular cramps can be explained by the fact that the embolization took place during exercise (swimming) and that the response of the musculature to sudden ischaemia is probably different during exercise and at rest. The electrolyte disturbance that was at hand on admission may also have contributed to the altered reaction. The gradual deterioration of the arterial circulation is explained by secondary thrombosis distal to the emboli. The muscular ischaemia became finally so pronounced as to cause release of myoglobin and enzymes. Approximately 40% of patients with left atrial myxoma have peripheral embolization of tumour fragments (4). Almost all organs have been reported to be the site of emboli (9, 17). Cerebral emboli are frequent, and fatal outcome has been reported (3). Most patients with left atrial myxoma have cardiac symptoms due t o obstruction of the mitral orifice. The myxoma usually originates from the lower part of the interatrial septum and is pedunculated. When the myxoma has reached a certain size, it will obstruct the mitral valve during diastole and will in that way simulate mitral stenosis (6). Frequently, the tumour is not detected until an operation for a suspected mitral stenosis is undertaken ( 1 I , 13). Constitutional symptoms such as fever, malaise, weight loss and high ESR are reported to occur in about 90% of the cases, but these findings are unspecific and difficult t o interpret ( 5 ) . Echocardiography is of great value in the diagnosis of cardiac myxoma. At the time in question this diagnostic aid was not available af our institutions. It should also be born in mind that cases have been described in which the myxoma has been small and located in the left ventricle and might thus have been overlooked a t an echocardiographic examination focused on the atria (1). Therefore, an angiographic examination is usually necessary and the easiest way is to perform a pulmonary angiogram, where the myxoma will appear as a mobile contrast defect when the contrast medium has reached the left atrium. In our patient the postischaemic oedema was so

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severe that a bilateral fasciotomy had t o be performed. Had this not been done, further muscular damage would probably have taken place. Fortunately, the muscular swelling soon diminished, rendering skin transplantation unnecessary, and a n excellent cosmetic result was obtained after direct wound closure. The patient is now back in her previous occupation as a swimming teacher and has only a slight reduction of the muscular strength in her left leg. Atrial myxoma is generally considered to be a benign tumour, although the tendency to obstruct the mitral valve and to embolize makes it clinically malign. Recurrence is very rare and is probably due t o incomplete removal of the tumour (16). Follow-up examination including echocardiography is, however, warranted.

REFERENCES 1. Bjork, V. 0. & Bjork, L.: Left ventricular myxoma. Thorax 6 : 534, 1965. 2. Crafoord, C: Panel discussion on late results of mitral commisurotomy. In: International Symposium in Cardiovascular Surgery. Studies in physiology, diagnosis and techniques (ed. C. R. Lamb), p. 202, Saunders, Philadelphia 1955. 3. Floderus, S. & Hedenstedt, S.: Endocardomyxom, tvh fall med emboli. Lakartidningen 52: 3931, 1968. 4. Goodwin, J. F.: Diagnosis of the left atrial myxoma. Lancet 1:464, 1%3. 5 . - The spectrum of cardiac tumors. Am J Cardiol 21:307, 1968. 6. Hedfors, E. & Mogensen, L.: Atrial myxoma. 12 cases operated in Stockholm 1954-1973. Eur J Cardiol 2/1: 101, 1974. 7. Khan, M. S. R. & Mujahed, M. A.: Atrial myxoma producing a saddle embolus in a child. Thorax 25: 634, 1970. 8. Koikkalainen, K., Kostiainen, S. & Luosto, R.: Left atrial myxoma revealed by femoral embolectomy. A case report and review of the literature. Scand J Thorac Cardiovasc Surg 11: 33, 1977. 9. Kulkarni, M., Jessiman, J. M. & French, S.: Entire left atrial myxoma presenting as a saddle embolus. Thorax 24: 629, 1969. 10. Larmi, T. K. J., Karkola, P., Kairaluoma, M. J. & Takkunen, J.: Surgical treatment of cardiac myxoma. A case report. Ann Chir Gynaecol66: 18, 1977. 11. Marpole, D. G. F., Kloster, F. E., Bristow, J. D. & Griswold, H. E.: Atrial myxoma, a continuing diag-

nostic challenge. Am J Cardiol23: 597, 1969. 12. Mundth, E. D., Wheeler, E. 0.. Moses, J. M. & Austen, W. G.: Clinical aspects of left atrial myxoma. Report of a case simulating subacute bacterial endocarditis and review of five cases treated surgically. Ann Thorac Surg 5 : 1968.

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13. Neilson, G. H.: Experiences with left atrial myxoma. Aust Ann Med 17: 38, 1968. 14. Peters, M. N., Hall, R. J., Cooky, D. A,, Leachman, R. D. & Carcia, E.: The clinical syndrome of atrial myxoma. JAMA 5 : 695, 1974. 15. Prichard, P. W.: Tumours of the heart. Review of

subject and report of one hundred and fifty cases. Arch Pathol 51:98, 1951.

16. Read, R. C., White, H. J . , Murphy, M. L., Williams, D., Sun, C. N . & Flanagan, W. H.: Malignant potentiality of left atrial myxoma. .I Thorac Cardiovasc Surg 68: 857, 1974. 17. Silverman, J., Olwin, J. S. & Greatinges, J . S.: Cardi-

ac myxomas with systemic embolization: Review of literature and report of a case. Circulation 26:99. 1962.

Massive embolization of cardiac myxoma. A case report.

A c t a Med Scand 206: 123-126, 1979 Massive Embolization of Cardiac Myxoma A Cuse Report Gunnar Tornvall and Christian O h From the Medical Departm...
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