Left atrial myxoma mimicking vasculitis: echocardiographic diagnosis RUSSELL R.C. BUCHANAN,* MD; JOHN A. CAIRN5,t MD, FRCP[C], FACC; GUNNAR KRAAG,. MD, FRCP[C]; JOHN G. RoBINsoN,§ MD, FRCP[C], FACC The clinical course of left atrial myxoma is characterized by symptoms resulting from obstructive, embolic or "constitutional" effects of the tumour.13 The symptoms of obstructive origin are the most common, are generally dyspnea, pulmonary edema or syncope, and may simulate those of rheumatic valve disease. The auscultatory findings, which are often variable, may mimic those of mitral stenosis or regurgitation. A diastolic tumour "plop" and accentuation, broadening or splitting of the first heart sound may be present. The emboli are generally large and most commonly cerebral, although they may be peripheral, in which case embolectomy may show the underlying condition.4 Constitutional symptoms are seen in about half the patients and comprise weight loss, lowgrade fever, myalgia and arthralgia. Laboratory investigations may reveal an increased erythrocyte sedimentation rate, leukocytosis, elevated serum globulin concentrations and mild anemia. The diagnosis of left atrial myxoma is generally made in a patient who has some combination of obstructive, embolic and constitutional manifestations of the tumour. In no patient previously described has the diagnosis been made during life in the absence of obstructive symptoms From the divisions of cardiology and rheumatology, McMaster University Medical Centre, Hamilton *Fellow in rheumatology, McMaster University tDirector, intensive care and coronary care units, McMaster University Medical Centre, and assistant professor of medicine, McMaster University .Assistant professor of medicine, McMaster University §Director of regional noninvasive cardiologic laboratories and associate professor of medicine, McMaster University Reprint requests to: Dr. John A. Cairns, Division of cardiology, McMaster University Medical Centre, 1200 Main St. W, Hamilton, Ont. L8S 4J9

or large emboli. We describe a patient in whom an eventual echocardiographic diagnosis of left atrial myxoma was made although the initial clinical features - prominent constitutional symptoms and episodic painful pruritic lesions peripherally without symptoms of obstructive phenomena or large emboli - suggested a vasculitis. Case report In a 42-year-old woman a "flulike" illness developed consisting of fatigue, fever without chills, and nausea and vomiting, but without sore throat or lymphadenopathy. Acetylsalicylic acid was prescribed, and within 2 weeks there appeared the first of a number of crops of painful, markedly pruritic, erythematous lesions that were predominantly deep within the pulp of her fingers but also in the palms of her hands and did not involve the skin surface. The second crop occurred prior to resolution of the initial lesions. This sequence was recurrent, giving a picture of groups of lesions in various stages of resolution. These episodes were associated with stiffness and diffuse swelling of the fingers. The woman continued to feel markedly fatigued. Generalized myalgia developed and her appetite decreased. By 8 weeks after the onset of her symptoms she had lost almost 2 kg in weight and was then referred to our rheumatology clinic because the clinical syndrome suggested a connective tissue disorder. At the time of referral the woman's temperature was 37 0C, heart rate 100 beats/mm and blood pressure 120/80 mm Hg. She had multiple tender, erythematous, macular lesions, each approximately 5 to 8 mm in diameter and located deep within the pulp of her fingers. The fingers were diffusely swollen and

1540 CMA JOURNAL/JUNE 23, 1979/VOL. 120

the grip strength was moderately decreased on both sides. There were no lesions in the feet and there was no evidence of joint inflammation. There were no petechial, splinter or conjunctival hemorrhages, and the fundi appeared normal. Auscultation revealed a normal first heart sound, a physiologically split second sound, no third or fourth sound, and no murmurs with the woman lying down, sitting or standing. There was no jugular venous distension or hepatojugular reflux. The hemoglobin concentration was 11.7 g/dl, the leukocyte count 8.3 x 10./l, the platelet count 350 x 1 0./l and the erythrocyte sedimentation rate 88 mm/h. Tests for antinuclear antibody. rheumatoid factor, cryoglobulins and hepatitis B antigen were negative; a test for Creactive protein was strongly positive. Protein electrophoresis and immunoelectrophoresis gave normal results. The serum concentrations of the C3 and C4 components of complement were 120 (normal 50 to 110) mg/dl and 32 (normal 15 to 42) mg/dl respectively. The antistreptolysin 0 titre was less than 160 Todd units. The serum iron concentration was 33 (normal 70 to 190) mg/dl and the serum total iron binding capacity 289 (normal 250 to 440) mg/dl. The serum creatinine and electrolyte concentrations and the blood urea nitrogen concentration were normal. An electrocardiogram showed sinus tachycardia (heart rate 104 beats/mm) and T-wave flattening in leads V5 and V6. A chest roentgenogram was normal. The risk of biopsy of the lesions deep in the finger pulps was thought not to be warranted in view of the small possibility of diagnostic findings. A biopsy of one of the superficial lesions showed only nonspecific inflammation. Recurrence of crops of lesions in

the fingers and palms, combined with stiffness of the fingers, myalgia and lethargy, persisted. Three months after the onset of symptoms the woman reported a 4-day episode of blurred central vision in the left eye that had spontaneously resolved before she consulted a physician. At no time had she experienced dyspnea, orthopnea, chest discomfort, dizziness or syncope. Repeated auscultation by her family doctor and by four internists and three residents during the 3 months had always yielded normal findings. Nevertheless, it was considered possible that an atypical presentation of subacute bacterial endocarditis or left atrial myxoma rather than a connective tissue disorder might explain the clinical course, and the woman was

FIG. 1-Preoperative M-mode echocardiogram. With transducer directed through mitral valve, dense columns of echos are seen between mitral leaflets in diastole. As transducer is swept up into left atrium, dense columns of echos are seen in left atrium during both systole and diastole, and, as the transducer is angled higher, in systole only.

admitted to hospital so that serial blood cultures and echocardiography could be performed and fever documented. The cultures were negative and she was afebrile, but the echocardiogram was abnormal, with dense clouds of echos in the lower atrium in both systole and diastole, and columns of extraneous echos during diastole between the anterior and posterior mitral leaflets, diagnostic of left atrial myxoma (Fig. 1). On one occasion, a week after the echocardiographic diagnosis was made, the consulting cardiologist reported slightly accentuated splitting of the first heart sound and a faint intermittent third sound. Subsequent auscultation by this cardiologist and the echocardiographer revealed no abnormalities. Angiography demonstrated a spherical mass attached by a stalk above the posterior leaflet of the mitral valve and inferior to the pulmonary veins; it occupied half the atrial volume during systole and moved well down into the left ventricle during diastole (Fig. 2). At operation a gelatinous mass 5 x 3.3 x 2.4 cm was found attached by a stalk to the left atrial septum in the region of the inferior right pulmonary vein. The stalk was resected at its atrial septal attachment. Gross and histologic study confirmed myxomatous tissue. Within 4 months the hemoglobin concentration had risen to 14.3 g/dl, the erythrocyte sedimentation rate had fallen to 12 mm/h, the test for

C-reactive protein had become negative, and the woman's malaise and fatigue had completely resolved. Her heart rate gradually fell to 76 beats! mm over the next several months. A postoperative echocardiogram was normal (Fig. 3). Discussion Left atrial myxoma was first diagnosed angiographically in 195 1,. and the first excision was done in 1954.. However, up to 1964, only 60 intracardiac myxomas had been removed surgically.7 Since then numerous cases have been reported,1'3" with good surgical results. The diagnosis of left atrial myxoma remains difficult to make. When it is made ante mortem, almost invariably there are obstructive symptoms, and frequently serious embolic episodes have occurred.1 Constitutional manifestations are present in at least half the cases, and occasionally they are the only abnormalities for some time prior to the occurrence of life-threatening or fatal obstructive or serious embolic complications.1 The echocardlographic manifestations of left atrial myxoma are specific9 and should allow early noninvasive assessment of patients with suspected left atrial myxoma. The patient we have described had had no obstructive symptoms or gross embolic episodes, and repeated auscultation had revealed no abnormalities. Echocardiography was done as part of the differential diagnostic work-up because, although the findings suggested vascu-

'V

.,.A.J1AAJ

FIG. 2-Angiograms of left atrium and ventricle. Left atrium and ventricle have been opacified by pulmonary artery injection (catheter has slipped back into right ventricle) in right anterior oblique position. Large, round filling defect occupies half of left atrium in systole (left). In diastole (right) filling defect has moved down into left ventricle, and left atrium is fully opacifled.

FIG. 3-Postoperative M-mode echocardiogram. With transducer swept in opposite direction from that in Fig. 1, no extraneous echos are seen in left atrium or between leaflets of mitral valve.

CMA JOURNAL/JUNE 23, 1979/VOL 120 1541

The Royal College of Physicians and Surgeons of Canada

Examinations The examinations of the Royal College are held in September of each year. Candidates wishing to sit for the examinations should note the following: 1. Every candidate for admission to the examinations must submit an application for assessment of training. 2. Candidates in training in Canada should apply for preliminary assessment of training at least one year before the date on which they expect to sit for the examinations, that is to say not later than September 1st of the preceding year. Candidates who have had training outside of Canada should submit their initial application for assessment at least eighteen months before they expect to sit for the examinations, that is by March 1st of the preceding year. Only candidates whose assessment of credentials is complete will be accepted to sit for the examinations. 3. Candidates who desire to sit for an examination, having complied with the above requirement of preliminary assessment of training, must notify the College in writing of their intent before February 1st of the year of the examination. Upon receipt of this notice of intent, the evaluation of the candidate's performance during training will be added to the previously completed assessment of credentials. Each candidate will then receive notification as to eligibility together with an application form for admission to the examination which he will complete and return. 4. The following documents may be obtained from the College office: (a) Application forms for assessment ottraining. (bi General Information booklet on training requirements and examinations. (c) Specific requirements for training and regulations relating to the examinations of each specialty. Requests should indicate the specialty or specialties of interest to the applicant. (d) Listing of specialty training programmes in Canada accredited by the College. 5. Address all enquiries to: Division of Training and Evaluation ROYAL COLLEGE OF PHYSICIANS AND SURGEONS OF CANADA 74 Stanley Avenue Ottawa, Ontario KIM 1 P4 Tel.: (613) 746-8177

litis or subacute bacterial endocarditis without cardiac findings, it was thought essential to rule out a left atrial myxoma with an atypical presentation. Even after the echocardiographic diagnosis was made, on only one occasion was a rather subtle auscultatory abnormality noted, and auscultation was of no diagnostic help. Diagnosis of left atrial myxoma at this stage of the disease, prior to the appearance of obstructive symptoms or serious embolic episodes, has not, to our knowledge, been reported previously. The origin of the constitutional manifestations of left atrial myxoma is uncertain. Among the possibilities that have been considered are hemorrhage and degeneration within the tumour,10 microembolism to muscles1 and immunologic response to release of tumour fragments." Currey, Mathews and Robinson12 detected antiheart-muscle antibodies in a patient with a right atrial myxoma; the antibody titre gradually diminished following excision of the tumour. There have been several reported cases of central retinal artery occlusion in patients with a left atrial myxoma."'14 In each case serious embolic events were noted; the central retinal artery occlusion was only one component of more extensive neurologic damage. Our patient had only a transient central field defect in the left eye, and no fundal lesion was observable when she was examined 4 days later. Microemboli may have accounted for the visual impairment and the skin manifestations in our patient. Amaurosis fugax is a recognized symptom of giant cell arteritis, and central retinal arteritis was also considered possible in our patient, although, to our knowledge, it has not been described in other forms of vasculitis. This case illustrates the value of noninvasive assessment of the left atrium by echocardiography in a patient with findings suggestive of vasculitis or subacute bacterial endocarditis in the absence of cardiac findings. The echocardiographic diagnosis of left atrial myxoma in other patients at this early stage of their disease may lead to an opera-

1542 CMA JOURNAL/JUNE 23, 1979/VOL. 120

tion before the occurrence of obstructive or serious embolic complications. We acknowledge the expertise of the following people in the preparation of this clinical study: Mrs. Helen Burley (echocardiography), Dr. Harold 0. Stolberg (angiography), Dr. John Gunstensen (cardiac surgery), Mr. Gordon Cotton (photography) and Miss Phyllis Galliani (typing). This work was supported by Ontario Heart Foundation grant 15-18 (to Dr. Cairns).

References 1. GOODWIN JF: Diagnosis of left atrial

myxoma. Lancet 1: 464, 1963 2. SELZER A, SAKAI FJ, POPPER RW:

Protean clinical manifestations of primary tumors of the heart. Am J Med 52: 9, 1972 3. NASSER WK, DAvIs RH, DILLON JC,

et al: Atrial myxoma. 1. Clinical and pathologic features in nine cases. Am Heart J 83: 694, 1972 4. GREENWOOD WF: Profile of atrial myxoma. Am J Cardiol 21: 367, 1968 5. GOLDBERG HP, GLENN F, DOTTER

CT, et al: Myxoma of the left atrium: diagnosis made during life with operative and postmortem findings. Circulation 6: 762, 1952 6. CRAFOORD C: Panel discussion on late results of mitral commissurotomy, in

international Symposium on Cardiovascular Surgery: Studies in Physiology, Diagnosis and Techniques. Proceedings of the Symposium held at Henry Ford Hospital, Detroit, Michigan, March 195S, LAM CR (ed), Saunders, Philadelphia, 1955, p 202 7. NEWMAN HA, CORDELL AR, PRITCHARD RW: Intracardiac myxomas: literature review and report of six

cases, one successfully treated. Am Surg 32: 219, 1966 8. SUNG RJ, GHAHRAMANI AR, MALLON SM, et al: Hemodynamic features of prolapsing and nonprolapsing left

atrial myxoma. Circulation 51: 342, 1975 9. FEIGENBAUM H: Cardiac tumors, in

Echocardiography, 2nd ed, Lea & Febiger, Philadelphia, 1976, p 447 10. LEKISCH K: Myxoma of the left atrium: report of a case. Ann intern

Med 46: 982, 1957 11. Ross JH, BECHAR M: Myxoma of the heart: report based on four cases. Am J Cardiol 3: 823, 1959 12. CURREY HLF, MATHEWS JA, ROBINSON J: Right atrial myxoma mimicking a rheumatic disorder. Br Med J

1: 547, 1967 13. JAMPOL LM, WONG AS, ALBERT DM:

Atrial myxoma and central retinal artery occlusion. Am J Ophthalmol

75: 242, 1973 14. ANDERSON JD, LUBOW M: Atrial myxoma as a source of retinal em-

bolization. Am J Ophthalmol 76: 769, 1973

Left atrial myxoma mimicking vasculitis: echocardiographic diagnosis.

Left atrial myxoma mimicking vasculitis: echocardiographic diagnosis RUSSELL R.C. BUCHANAN,* MD; JOHN A. CAIRN5,t MD, FRCP[C], FACC; GUNNAR KRAAG,. MD...
1MB Sizes 0 Downloads 0 Views