Primary atrial tumor

Primary Atria1 Tumor* Evaluation

of Clinical Findings in Ten Cases and Review of the Literature

ROBERT P. WIGHT, JR., MARVIN M. MCCALL Atlanta,

P

CASE MATERIAL In reviewing records of six Atlanta hospitalst we found 10 cases of primary atria1 tumor. In 7 cases, signs and symptoms were related to the tumors; in 2 the tumors were incidental findings at autopsy; 1 patient has a calcified posterior left atria1 wall mass on x-ray examination, unchanged for 13 years. The 7 sy-mptomatic cases are reviewed here,: 3 of which were seen by one or more of the authors. REVIEW OF THE LITERATURE tumors

constituted

the

majority

M.D.

Georgia

primary cardiac tumors reported by Prichard.’ Myxomas and sarcomas were the most common pathologic types; hamartoma and angioma also were cited. The pathology and symptomatology of atria1 tumors as compiled from the literature are summarized below: Myxoma: This is the most frequently encountered pathologic type constituting about 50 per cent of primary tumors of the heart.‘,“’ Seventy-five per cent are located in the left atrium. They are solitary tumors, and 80 per cent are described as polypoid rather than sessile.’ ,“,r2 The range in age of patients with myxomas was 3 months to 68 years with the majority between 30 and 60 years of age. Sex distribution was equa1.i The exact origin of the myxoma is controversial, but it probably represents a neoplastic process.‘.13-r5 Clinically, myxomas have most often been misdiagnosed as mitral stenosis on the basis of classic physical signs.‘~4~11~16-40 There are, however, 2 reported cases of proved coincident rheumatic mitral stenosis and atria1 myxoma.41 ,42 The clinically significant problem of atria1 myxoma is blockade of blood flow through the mitral valve due to ball-valve action of the tumor, which may result in svncope or shock.37~40~43-j3 Embolic phenomena may occur, secondary to tumor fragmentation or to bacterial infection of the tumor surface l.R,13,19.28.34,38.44.54-60 Myxomas may cause congestive heart failure, refractory to the Opening snaps usual therapy. 31.37r46.47,58,61-66 and murmurs changing in character with change in position have been recorded.1gf28 ,31~67-6y Sudden death is not uncommon.54*70-7’2 Sarcomas: Prichard’ lists 113 cases of primary. cardiac sarcoma reported before 1951. In

RIMARY atria1 tumors are now included among surgically correctible cardiac abnormalities. Prichard,’ in an extensive review of the literature in 1951, recorded the infrequency of antemortem diagnosis of these tumors ; many unsuspected atria1 tumors have been found with mitral valve surgery and with the increasing use of angiocardiography.“-y The frequency of sudden death in patients with symptomatic atria1 tumors and the unfavorable prognosis associated with incorrect preoperative diagnosis prompted this review. We attempted to determine which clinical and laboratory findings should raise the suspicion of atria1 tumor.

Atria1

M.D. and NANETTE KASS WENGER,

of

t Grady Memorial Hospital, Emory University Hospital, Georgia Baptist Hospital, St. Joseph’s Infirmary, Crawford W. Long Hospital, Veterans Administration Hospital. $ We arc indebted to Drs. Samuel 0. Poole, Edward R. Dorney and Osler A. Abbott for Case 1, to Drs. Jeff Richardson and William A. Hopkins for Case 2, to Drs. B. Woodfin Cobbs and Osler A. Abbott for Case 3, and to Dr. J. Willis Hurst for his help in collecting the casts of atria1 tumor.

* From the Department of Medicine, Emory University School of Medicine and the Medical Service, Grady Memorial Hospital, Atlanta, Ga. 790

THE AMERICAN JOURNALOF CARDIOLOGY

Primary

Atria1

contrast to myxomas, one half of these tumors arose from the right side of the heart, equally distributed between atrium and ventricle. Sarcomas are usually infiltrative or sessile, but 20 per cent have polypoid projections into the chambers.’ Approximately one third of these tumors have distant metastases.1s73 Prichard found prior to 1951, 5 reports of cardiac sarcoma diagnosed ante mortem with no record of antemortem diagnosis of myxoma. The sudden appearance of A-V dissociation in a patient with a muscle biopsy showing rhabdomyosarcoma suggested the diagnosis of cardiac sarcoma to Barnes, Beaver and Sne11.74 In Shelburne’s’” case, recurrent sanguineous pericardial fluid aided the correct antemortem diagnosis in a patient with sarcoma. Intractable congestive heart failure without obvious cause was the only clinical clue in many cases of sarcoma.76a77 A common presenting problem in right atria1 sarcoma is vena caval obstruction.ls*rg Chest pain was occasionally reported.*O Atria1 arrhythmias-persistent sinus tachycardia, paroxysmal atria1 tachycardia, atria1 flutter or fibrillation -bizarre P waves and electrocardiographic changes of right atria1 or right ventricular hypertrophy, have been observedP3 Roentgenologic examination may- reveal atria1 dilatation or calcification.76~81 Angioma,

Rhabdomyoma,

Fibroma,

Hamartoma:

Most angiomas occur in the right atrium near the fossa ovalis, and may cause complete heart block.’ The etiology of rhabdomyomas is obscure ;* ,Ro-*3 they are frequently associated with tuberous sclerosis;83 Kidders2 reports an 80 per cent mortality by age 5. The exact pathologic classification of cardiac fibromas and hamartomas is controversial, and accurate statistics regarding these tumors are not available.rzs’ Surgery: In 1942 BeckE4 suggested removal of atria1 myxomas by closed section or by open cardiotomy employing bypass. The first successful removal of an intra-atria1 myxoma was performed by Crafoord85 in April 1954. Differding et al.s6 cite 43 reports of successful surgical removal of atria1 myxomas and tabulate the technics and results for 38 of the cases. We have found 4 additional reports of successful surgery for primary atria1 tumor.54~87-sg Although atria1 tumors have ‘been removed by the closed surgical approach,w-92 most of the successful surgery has been done under hypothermia or with extracorporeal circulation5zs6 Most surgeons recommend closure and a second JUNE

1963

Tumor

791

procedure under extracorporeal circulation should an atria1 tumor be encountered during closed surgery for mitral stenosis.36 393,s4 Open heart surgery with inflow stasis and hypothermia or extracorporeal circulation is advised (1) because myxomas are extremely friable and the frequency of embolization is increased by closed surgical manipulation and (2) because inspection and complete removal may be a lengthy process and entail prolonged anoxia with a closed surgical technic.s5-‘03

CASE REPORTS CASE 1. [W. J. 113-875 (EUH)]. This 38 year old white man reported dyspnea, orthopnea and paroxysmal nocturnal dyspnea of two months’ duration. He had two episodes of pulmonary infarction or pneumonia or both during the seven months preceding admission. On physical examination he appeared tachypneic, with a respiratory rate of 40/min. The blood pressure was 94/72 mm. Hg in the supine position; the heart rate was 108/min.; and he was afebrile. The neck veins were distended with the patient at a 60’ angle, but showed no abnormal pulsations. Rales were heard at the left lung base posteriorly. The apex impulse was unremarkable, and there was no right ventricular impulse. The first heart sound at the apex was accentuated ; there was a questionable opening snap. A grade 1, low pitched diastolic murmur was heard at the lower left sternal border. The liver edge was palpable three fingerbreadths below the right costal margin. The arm blood pressures at various positions were: upright, 90/70 mm. Hg; supine, 70/50; left lateral decubitus, 70/50; and right lateral decubitus, 54/48. In this last position the patient became anxious ,and agitated with a feeling of impending death. serum electrolytes and Hemogram, urinalysis, serum proteins were within normal limits. The electrocardiogram showed prominent P waves and right ventricular hypertrophy. X-ray examination revealed left atria1 and right ventricular enlargement and pulmonary congestion. Poor left atria1 filling was demonstrated by angiocardiography. The preoperative diagnosis was mitral stenosis with suspicion of atria1 tumor because of the angiocardiographic findings and the positional blood pressure changes. At operation a sessile tum.or projected into the left atria1 cavity and extended into the pulmonary veins; a small tumor was implanted on the mitral valve. The tumor was removed in 3 pieces, the largest measuring 10.5 by 7.5 by 3 cm. The microscopic diagnosis was sarcoma, with wide variety of cell types. Following right middle and right lower lobe lobectomies, the patient returned to manual labor for

792

Wight, McCall

18 months. The day before his death he was hospitalized for pulmonary edema from which he Death was probably caused by did not recover. blockade of the pulmonary vein by recurrent tumor, but no autopsy could be obtained. [B. G. 33530 (St. Joseph’s Infirmary)]. CASE 2. This 25 year old white man presented with dyspnea on exertion and easy fatigability. He had rheumatic fever 9 years previously. There had been no frank congestive failure nor syncopal episodes. On examination, he appeared in no acute distress. Blood pressure was 105/75 mm. Hg supine, 95/75 upright. The pulse rate of lOO/min. upright fell immediately to 80/min. supine. Neck veins were severely distended with the patient at a 45” angle. No cardiac enlargement was detected. There was a grade 2 presystolic murmur and thrill at the apex. The apical first sound was accentuated. No opening snap was heard. The electrocardiogram indicated right axis deviation, right ventricular hypertrophy and a right ventricular conduction delay. At fluoroscopy, a calcified atria1 mass which moved with each cardiac cycle was noted. Its location was uncertain, but it appeared to be in the right atrium. At open heart surgery a pedunculated myxoma protruded into the right atria1 cavity; it measured 5.5 by 4.0 by 3.6 cm. and was successfully removed. The patient recovered uneventfully. The electrocardiogram remained unchanged except for the P waves which were peaked prior to operation but appeared broadened and notched two years postoperatively. When last seen, three years ago, the patient was asymptomatic; his physician thought he had minimal rheumatic mitral stenosis.

CASE 3. [M. A. 113-591 (EUH)]. This 44 year old white woman presented a three year history of progressive dyspnea and orthopnea. Two years before admission she had a 12 hour episode of loss of memory and severe headache. She had tolerated four pregnancies and abdominal surgery without cardiac symptoms. There was no history of rheumatic fever. On physical examination she appeared in no acute Blood pressure was 104/65 mm, Hg supine, distress. respiratory rate 20/min. and heart rate 96/min. The deep jugular veins were She was afebrile. moderately distended without abnormal pulsations with the patient positioned at a 35’ angle. There were fine rales at both lung bases. The apex impulse was normal, and there was a slight sternal lift. There was physiologic splitting of the second heart sound. A grade 3 presystolic murmur and a grade 1 systolic murmur were heard at the apex. No opening snap was heard. The hemogram, urinalysis and serum electrolytes were within normal limits. The electrocardiogram showed prominent P waves and right ventricular

and Wenger X-ray examination revealed an enhypertrophy. larged left atrium with “calcification in the mitral valve area” and probable right ventricular enlargement. The preoFeratiue diagnosis was mitral stenosis, but at surgery a pedunculated myxoma was found originating from the interatrial septum. Two days later at open heart surgery the tumor, measuring 7.0 by 5.0 by 5.0 cm. and weighing 38 gm., was removed. The patient’s recovery was uneventful. CASE 4. [A. S. 90682 (St. Joseph’s Infirmary)]. This 25 year old white man was admitted to the hospital in shock with a history of fatigue, nausea and weakness for nine days. Two days before admission he fainted while having a bowel movement. On admission he was cyanotic and apprehensive. Cardiac examination was not satisfactory. He died within an hour, and no laboratory studies were obtained. At autopsy the heart weighed 700 gm. and showed biventricular hypertrophy. There was an angiosarcoma of the right atrium with rupture of the right atria1 wall. Death was caused by cardiac tamponade due to hemopericardium. The generalized cardiac hypertrophy and microscopic evidence of pericarditis are unexplained. CASE 5. [B. C. C-84559 (GMH)]. This 51 year old Negro woman presented with generalized edema, dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea. Mitral stenosis was diagnosed on the basis of right ventricular hypertrophy and an apical diastolic rumble. Treatment with digitalis and diuretics resulted in marked diuresis. Congestive heart failure recurred on intensive treatment. Digitalis intoxication occasioned the final admission. On physical examination atria1 fibrillation, fixed splitting of the second sound in the pulmonic area, a right ventricular heave, a grade 2 apical systolic murmur and an inconstant apical diastolic rumble with presystolic accentuation were noted. The liver was palpated 10 cm. below the right costal margin. On the electrocardiogram right axis deviation and right ventricular hypertrophy were indicated. At right and left heart catheterization the cardiac index was 1.7 L./min./Mr. and the left atria1 pressure was 60/27 mm. Hg (mean 38). These findings were considered consistent with mitral stenosis. Examination by angiocardiography was not done. Three days after cardiac catheterization the patient became hypotensive, and pulmonary edema was noted. The hypotension did not respond to vasopressor therapy, and she died within three hours. At autopsy, a 6 by 6 by 6 cm. pedunculated left atria1 myxoma was found. CASE 6. [M. S. W-11832 (GMH)]. This 52 year old white woman was hospitalized in December 1959 after a two year history of increasing dyspnea THE AMERICANJOURNAL OF CARDIOLOGY

Primary Atria1 Tumor and pedal edema. She described several episodes of syncope while standing. On physical examination there were systolic deep jugular pulsations, rales at both lung bases, a right pleural effusion, a sternal lift and an Ss gallop. A systolic murmur, consistent with tricuspid insufficiency, and an intermittent mid-diastolic apical rumble accompanied by a loud mitral first sound were heard. The diagnosis was rheumatic heart disease with mitral stenosis; the heart failure improved on a rigid regimen. The patient was readmitted Jan. 1, 1960, with increased congestive heart failure. Again, a rigid cardiac regimen afforded symptomatic relief, and cardiac catheterization was scheduled. On the fourth hospital day hypotension and pulmonary edema developed, and she did not recover. At autopsy, a pedunculated myxoma filled the left atrium, obstructing flow through the mitral valve. CASE 7. (T. C. C-41272 (GMH). This 45 year old diabetic male Negro with possible rheumatic fever in childhood presented a two year history of congestive heart failure unresponsive to digitalis therapy. On physical examination he was in no ‘apparent distress. The heart rate was 120/min., respirations 18/min., blood pressure 120/80 mm. Hg, and he was afebrile. Moderate distention of the deep jugular veins with no abnormal pulsations was noted with the patient positioned at a 45” angle. The precordium was active with a 2 plus right ventricular impulse. The apex impulse was unremarkable. The second sound at the pulmonic area was prominent with physiologic splitting. The first heart sound at the apex was accentuated. No opening snap was heard. A grade 1 short systolic murmur was heard at the apex. There was a grade 1 apical mid-to-late diastolic murmur, not rumbling in character. The liver was enlarged two to three fingerbreadths below the right costal margin with a positive hepatojugular reflux. There was no peripheral edema. Hemogram, urinalysis, serum electrolytes, blood urea nitrogen and serum proteins were within normal limits. The fasting blood sugar was 153 mg./lOO cc.; total serum bilirubin was 2.5 mg./lOO cc. with Bromsul0.5 mg./lOO cc. “direct” bilirubin. phalein retention was greater than 40 per cent in 45 min. The electrocardiogram showed an RSR pattern in lead V,, prominent P waves, right ventricular hyperThe x-ray examination trophy and digitalis effect. revealed cardiomegaly with enlargement of all chambers. The pulmonary arterial segment and superior vena cava were prominent, and there was pulmonary vascular congestion. No abnormal pulsations nor intracardiac calcifications were seen. At right heart catheterization there was marked elevation of the mean pulmonary artery, right ventricular systolic, right ventricular end-diastolic JUNE 1963

793 TABLE I

Age, Sex and Race of Patients at Time of Diagnosis of Atria1 Tumor

No. of

Age (yr.1

Patients

21-30 31-40 41-50 51-60

2 1 2 2

No. of

Race & Sex

White Negro White Negro

Patients

male male female female

3 1 2 1

and right atria1 pressures, with large A waves but no evidence of tricuspid regurgitation. Total pulmonary resistance was significantly elevated. Attempt at transseptal catheterization of the left atrium was unsuccessful. At operative exploration for mitral stenosis, the recorded pressures were pulmonary artery 108/50 (mean 72) mm. Hg, left atrium 64/15 (mean 38) and left ventricle 108/11. The mitral valve was normal with normal hemodynamics. There was a firm, irregular, sessile mass on the inferior posterior interatrial septum extending through the posteromedial commissure of the mitral valve and partially occluding the mitral valve orifice during atria1 systole. The patient died of shock and congestive heart failure before open heart surgery for the atria1 tumor. Postmortem examination was refused. DISCUSSION

The patients 25 to 52 years preponderance studied (Table Five tumors and two in the

in our series ranged in age from and no significant sex nor racial was noted in the small group I). were located in the left atrium Three right atrium (Table II). TABLE II

Tumor Type, Location and Incidence of Antemortem Diagnosis

Type of Tumor Polypoid lIlyX0tU

Polypoid angiosarcoma Sessile sarcoma Sessile tumor, type unknown

Left Atria1 Tumors Diagnosed (No.) Antemortem 3

1 diagnosed at surgery for supposed mitral steno*,s 2 suspected

.. 1 1

Diagnosed at surgery for supposed mitral stenosis

Right Atria1 Tumors Diagnosed Ante(No.) mortem 1

1

..

1

0

1

.. .

..

Wight,

794 TABLE Clinical

Tumor

Left atria1 polypoid

TABLE

III

Clinical

IV

Features

’ --No

R.3JltS

1 successfrd surgery (preop. diagnosis mitral stenosis) 2 sudden deaths prior to diagnostic angiocardiography in suspected cases

Right atria1 polypoid myxoma

S”ccessf”1

Right atria1 polyp&d angiosarcoma

Admitted in shock. Died of ruptured right atrium and cardiac tamponade

Left atria1 sessile sarcoma

Successful

Left atria1 sessile tumor, unknown

exploratory Died suddenly after cardiotomy before scheduled open heart surgery

type

and Wenger

Course

Type

myxoma

McCall

surgery

surgery

left atria1 tumors were polypoid and two were sessile; both right atria1 tumors were polypoid. Four of the tumors were myxomas, three in the left atrium and one in the right atrium. One polypoid angiosarcoma, one sessile sarcoma and one sessile tumor of unknown type were encountered . Two atria1 tumors were diagnosed ante mortem and two more suspected (Table II) ; the 2 patients with suspected atria1 tumors died suddenly before definitive study (Table III). Two tumors were found during surgery for presumptive mitral stenosis. One patient had subsequent successful surgery; the other died suddenly before the scheduled open heart surgery. The seventh patient died of right atria1 rupture one hour after hospital admission, and the tumor was found at autopsy Surgery was successful in both cases of atria1 tumor diagnosed preoperatively, and in reoperation of one case where the tumor was encountered during surgery for supposed mitral It is important to emphasize that the stenosis. remaining 4 patients died suddenly while in the hospital. One died before scheduled open heart surgery for removal of the tumor, two died while awaiting diagnostic studies and one, the only unsuspected case in this review, presented in extremis and died within the hour. Clinical Features: Patients with atria1 tumor comprise a group of operable cases, liable to sudden death if prompt and accurate diagnosis and treatment are not effected. The most consistent findings in our series were apical diastolic rumble and right ventricular hypertrophy on the electrocardiogram, present in 6 of the 7 patients (Table IV). The seventh patient died too suddenly for adequate evalua-

Present

of PatientsInformation UnavailAbsent able

5

2

0

3

2

2

6

0

1

Apical diastohc rumble loudest in upright position

2

2

3

Positional B.P.

History

Progressive or refractory congestive heart failure Syncope

Physical examin.ttion

Apical diastolic rumble

Electrocardiogram

X-ray ex.m1ination

change

in 1

0

6

Accentuated mitral first sound

4

2

1

Right ventricular hypertrophy

3

3

1

Right ventricular hypertrophy

6

0

1

Prominent

P waves

4

2

1

Enlarged

left atrium

4

2

I

Calcified

tumor

2

4

1

mass

Five patients presented a history of tion. progressive or refractory congestive heart failure. Four had an accentuated first sound at the apex; 4 had prominent P waves on the electrocardiogram ; and 4 had an enlarged left atrium on radiographic examination. Syncope, so commonly emphasized as a diagnostic clue, was present in 3 of our patients and specifically denied by 2. Right ventricular hypertrophy by physical examination was found in only 3 of the 6 patients whose electrocardiograms demonstrated Two patients had a calcified this condition. tumor mass, one in the left atrium and one in the right. Only 2 patients demonstrated positional change in the intensity of the apical diastolic rumble; this murmur was distinctly accentuPositional variaated in the upright position. tion was definitely absent in 2 patients and was not recorded in 3. In the 1 patient evaluated for positional blood pressure change, significant lowering of blood pressure occurred when he was supine, particularly in the right lateral decubitus position. No definite opening snap of the mitral valve was heard in this series, but this has been previously described and attributed to tumor impingement on the anterior mitral leaflet .ro4J”~ Dzzerentiation from Mitral Stenosis: Many prominent features of left atria1 tumor present a mitral stenosis. clinical picture simulating THE

AMERICAN

JOURNAL

OF CARDIOLOGY

Primary Atria1 Tumor Other findings, not seen with mitral stenosis, should immediately lead the physician to suspect atria1 tumor. These include: (1) an apical diastolic murmur heard loudest or heard only in the upright position; (2) syncope, not preceded by exertion; (3) hypotension and tachycardia produced by turning a supine patient to one side or the other, thereby obstructing flow through the mitral valve ; and (4) radiographic demonstration of intracardiac calcium not characteristic of mitral valve calcification. Any of these clues in conjunction with signs of mitral or vena caval obstruction demand atria1 angiocardiography to rule out atria1 tumor. All signs and symptoms associated with atria1 tumor may be seen with an atria1 thrombus which produces ball-valve obstruction of the mitral valve; the distinction between atria1 tumor and atria1 thrombus is not always possible by angiocardiography.

7.

8.

9. 10. 11.

12.

13.

14.

15.

SUMMARY The case records of 10 patients with primary atria1 tumor have been reviewed. In 7 cases the tumor was clinically significant. An erroneous diagnosis of mitral stenosis was frequently made. Useful diagnostic clues for atria1 tumor, differentiating it from mitral stenosis, are (1) a history of syncope ; (2) positional change in an apical diastolic rumble; (3) positional change in blood pressure and heart rate; and (4) intracardiac calcification not characteristic of mitral valve calcification. Accurate diagnosis and prompt therapy are mandatory in this group of patients who are prone to sudden death. REFERENCES 1. PRICHARD, R. W.

Tumors of the heart.

17.

18.

19. 20. 21.

22.

23.

Arch.

Path., 51: 98, 1951. 2. STEINBERG, I., DOTTER, C. T. and GLENN, F. Myxoma of the heart; roentgen diagnosis during life in three cases. Dis. Chest, 24: 509, 1953. 3. SANNERSTEDT,R. et al. Right atria1 myxoma. Report of a case and review of the literature. Am. Heart J., 64: 243, 1962. 4. WITTENSTEIN,G. J., GROW, J. B., HOFFMAN, M. S., GENSINI, G. G. and DENST, J. “Myxoma” of the left atrium simulating pure mitral insufficiency ; report of a case with successful removal. Sqery, 45: 981, 1959. 5. TABER, R. E. and LAM, C. R. Diagnosis and surgical treatment of intracardiac myxoma and rhabdomyoma. J. Thwack & Cardiovas. Surg., 40: 337, 1960. 6. CAMPETI, F. L., MAHONEY, E. B. and Yu, P. N. JUNE 1963

16.

24.

25.

26.

27.

28.

795

Myxoma of the right atrium. Circulatton, 22: 730, 1960. HAIR, T. E., JR., ORGAIN, E. S., SEALY, W. C. and MCINTOSH, H. D. Myxoma of the left atrium; report of two cases and review of the diagnostic methods. Circulation, 22: 759, 1960. BEANLANDS,D. S., ROY, D. L., DOLAN, F. G. and SHANE, S. J. Myxoma of the left atrium. Canad. M. A. J., 83: 715,196O. CAMPEAU,L. and DAVID, P. Myxoma of the heart Canad. M. A. J., 82: 586, 1960. GOLDBERG, H. P. and STEINBERG, I. Primary tumors of the heart. Circulation, 11: 963, 1955. FRIEDBERG, C. K. Diseases of the Heart, ed. 2, p. 1070. Philadelphia, 1956. W. B. Saunders Company. YATER, W. M. Tumors of the heart and pericardium; pathology, symptomatology and report of nine cases. Arch. Znt. Med., 48: b27, 1931. NICHOLS,J. and HENNIGAR,G. A case of bilateral multicentric cardiac myxoma. Arch. Path., 67: 24, 1959. FISHER,E. R. and HELLSTROM,H. R. Evidence in support of the neoplastic nature of cardiac myxoma. Am. Heart J., 60: 630, 1960. KROOPF, S. S. and PETERSON,C. A. Anaplastic myxoma of left atrium. Arch. Znt. Med., 100: 819,1957. HAMILTON-PATERSON,J. L. and CASTLEDEN,L. I. M. Intracardiac turnours. &it. Heart. J., 4: 103,194?. HARRISON,J. W., MCCORMACK,L. J. and ERNSTENE, A. C. Myxoma of the left atrium simulating mitral stenosis. Circulation, 10: 766, 1954. THOMPSON,C. E. and MALHAS, Z. A. Myxoma of left atrium simulating mitral stenosis. Arch. Znt. Med., 95: 614, 1955. Myxoma of the left auricle. HARVEY, J. C. Ann. Znt. Med., 47: 1067, 1957. CRISCITIELLO,M. G. Atria1 myxoma, a diagnostic challenge. Am. Heart J., 62: 841, 1961. JONES,G. P. and JULIAN,D. G. Left atria1 tumour simulating mitral stenosis. hit. M. J., 2: 361, 1955. CROPPER, C. F. J. and WINSTANLEY,D. P. Myxht. M. J., 1: 1513, oma of the auricle. 1955. LIKOFF, W., GECKELER, G. D. and GREGORY, J. E. Functional mitral stenosis produced by an intraatria1 tumor. Am. Heart J., 47: 619, 1954. FISH, R. G., TAKARO, T. and CRYMES, T. Left atria1 pressure pulses in the presence of myxoma. Circulation, 20: 413, 1959. WINTERS, W. L., JR., MARK, G. E., JR. and SOLOFF, L. A. Left atria1 pressure curve in left atria1 myxoma. Arch. Znt. Med., 107: 384, 1961. BELCHER, J. R. Successful removal of a myxoma from the left atrium. Brit. J. Tuberc., 52: 62, 1958. COLL~S,J. L. and SHAH, N. J. Successful removal of a myxoma of the left atrium. Thorax, 14 : 232, 1959. COATES, E. O., JR. and DRAKE, E. H. Myxoma right-to-left of right atrium, with variable shunt; clinical and physiologic observations and report of a case with successful operative removal. New England J. Med., 259: 165, 1958.

796

Wight, McCall and Wenger

29. VAN BLJCHEM, F. S. and EERLAND,L. D. Myxoma cordis, diagnosis established preoperatively; successful removal of the tumour. Dis. Chest, 31: 61, 1957. 30. Buzzr, A. Mitral stenosis versus left atria1 tumor. Am. J. Cardiol., 3: 829, 1959. 31. PAQUET, E. Diagnostic approach to auricular myxomas. Canad. M. A. J., 74: 121,1956. 32. CHIN, E. F. and Ross, D. N. Myxoma of the left atrium : successful surgical removal under hypothermia. &it. M. J., 1: 1447, 1957. 33. DAVIS, F. W., JR. and ANDRUS, E. C. Mitral stenosis in facsimile. New England .J. Med., 251: 297, 1954. 34. BLOCK, W. J., PARKER, R. L. and EDWARDS, J. E. “Myxoma” of the left atrium clinically simulating mitral stenosis: report of case and pathologic studies. Proc. Staff Meet. Mayo Clan., 27: 361,1952. 35. WEINSTEIN, M. S. and ARATO, J. E. Mitral stenosis and insufficiency produced by cardiac “myxoma.” Am. Heart J., 38: 781, 1949. 36. SCANNELL, J. G., BREWSTER, W. R. JR. and BLAND, E. F. Successful removal of a myxoma from the left atrium. New En,&nd J. Med., 254: 601, 1956. 37. ALDRIDCE,H. E. and GREENWOOD,W. F. Myxoma of the left atrium. Brit. Heart J., 22: 189, 1960. 38. HORLICK, L. and MERRIMAN, J. E. Myxoma of the left atrium simulating mitral stenosis with cerebral emboli. Canad. M. A. J., 77: 582, 1957. 39. FIELD, M. H., DONOVAN, M. A. and SIMON, H. Primary tumor of the left auricle simulating mitral stenosis. Am. f&art J., 30: 230, 1945. 40. CENTENO, P. A. Myxofibroma of the left atrium simulating rheumatic heart disease. Pediatrics, 16: 489, 1955. 41. WHITE, P. D. and CASTLEMAN,B. Case records of the Massachusetts General Hospital, Case 43111. New England J. Med., 256: 516, 1957. 42. DESHMUKH,M., NICHOLS,H. T. and GOLDBERG,H. Myxoma of the left atrium simulating restenosis of the mitral valve. Am. Heart .J., 58: 623, 1959. 43. MADONIA, P. F., BOCGIANO, R. and GUBNER, R. Ball-valve syndrome caused by primary cardiac tumor. New York J. Med., 53: 3043, 1953. 44. KENDALL, D. and SYMONDS, B. Epileptiform attacks due to myxoma of right auricle. Brit. Heart J., 14: 139, 1952. 45. JACKSON,A. and GARBER, P. E. Myxoma of the left atrium; report of three cases. Am. Heart J., 55: 591, 1958. 46. KIRKEBY, K. and LAREN, P. Myxoma of the heart. Polypoid tumor of left atrium diagnosed ante mortem. Acta. med. scandinau., 143: 385, 1952. 47. MARTIN, B. F. Clinical aspects of myxoma of left auricle. Ann. Znt. Med., 38: 325, 1953. 48. LYONS, H. A., KELLY, J. J., Jr., NUSBAUM,N. and DENNIS C. Right atria1 myxoma; patient in whom preoperative diagnosis made by angiocardiography ; surgically removed. Am. J. Med., 25: 321, 1258. 49. MARIONS, 0. and ODMAN, P. Electrokymographic observations in myxoma of the left atrium. Acto radio/., 47: 461, 1957. 50. ASHMAN, H., ZAROFF, L. I. and BARONOFSKY,I.

51. 52.

53. 54.

55.

56.

57.

58.

59.

60.

61.

62.

63.

64. 65.

66.

67.

68.

69.

70.

71.

Right atrial myxoma, diagnosis during life; successful surgical removal. Am. J. Med., 28: 487, 1960. STRAUS,R. and MERLISR,R. Primary tumor of the heart. Arch. Path., 39 : 74, 1945. MACGREGOR, G. A., and CULLEN, R. A. The syndrome of fever, anemia, and high sedimentation rate with an atria1 myxoma. Brit. M. J., 2: 991, 1959. MCALLEN, P. M. Myxoma of the left auricle. Brit. M. J., 1 : 932, 1950. ZUIDEMA, G. D., BURKE, J. F., VILLEGAS, A. H. and SCANNELL,J. G. Surgery of atria1 myxoma. New England J. Med., 264: 1016, 1961. GLEASON, I. 0. Primary myxoma of the heart, a case simulating rheumatic and bacterial endocarditis. Cancer, 8: 839, 1955. SKANSE, B., BERG, N. 0. and WESTFELT, L. Atria1 myxoma with Raynaud’s phenomenon as the initial symptom. Acta med. scandinar ., 164: 321, 1959. EDWARDS, A. T. and JOHNSON, W. A case of myxoma of the left atrium with peripheral arterial emboli, Bait. J. Suq., 46: 371, 1959. TOWERS, J. R. and NEWCOMBE,C. P. Myxoma of left auricle with direct pressure tracings. Brit. Heart J., 20: 575, 1958. HOFFMAN, M. Cardiac myxoma simulating subacute endocarditis. Minnesota Med., 24: 585, 1941. BREWIN, T. B. “Myxoma” of the heart; report of a case in which death occurred as a result of detachment of the tumor from its pedicle. Guy’s Ho@. Rej., 100: 278, 1951. HUGHES, S. B. and FAZLULLAH, S. Cardiac failure due to tumour of the left atrium. Brit. Heart J., 16: 326, 1954. GOLDBERG,H. P., GLENN, F., DOTTER, C. T. and STEINBERG, I. Myxoma of the left atrium; diagnosis made during life with operative and post-mortem findings. Circulation, 6 : 762, 1952. FRANKENFELD, R. H., WATERS, C. H. and STEINER, R. C. Bilateral myxomas of the heart. Ann. Znt. Med., 53:827, 1960. LEKISCH,K. Myxoma of the left atrium; report of a case. Ann. Znt. Med., 46 : 982, 1957. PROTHEROE, R. H. Angiomyxoma of the left auricle; report of a case. Postgrad. M. J., 33: 402,1957. MILLS, P. and PHILPOTT, M. Myxoma of heart with neurological signs. Brit. Heart J., 13: 115, 1951. LEFCOE, N. M., BRIEN, F. S. and MANNING,G. W. An opening snap recorded in a case of tumor of 257: the left atrium. New England J. Mrd. 178, 1957. KAUFMAN, G., RUTISHAUSER,W. and HEGGLIN, R. Heart sounds in atria1 tumors. Am. J. Cardiol., 8:350, 1961. %-ROUSE, S. Primary benign tumor of the heart of forty-three years’ duration. Arch. Znt. Med., 62: 401,1938. FAWCETT, R. E. M. and WARD, E. M. Cardiac myxoma : a clinical and pathologic study. Brit. Heart J., 1: 249, 1939. HOUCK, G. H. and BENNETT, G. A. Polypoid fibroma of the left auricle (so-called cardiac THE AMERICANJOURNAL OF CARDIOLOGY

Primary Atria1 Tumor

72. 73. 74.

75.

76.

77.

78.

79.

80.

81.

82. 83.

84.

85.

86.

87. 88.

myxoma) causing a ball-valve action. Am. Heart J., 5: 787, 1929-30. ORR, J. W. Endothelioma (pseudomyxoma) of the heart. J. Path. B Bact., 54: 125, 1942. WHORTON, C. M. Primary malignant tumors of the heart. Cancer, 2: 245, 1949. BARNES, A. R., BEAVER, D. C. and SNELL, A. M. Primary sarcoma of the heart: report of a case with electrocardiographic and pathological studies. Am. Heart J., 9 : 480, 1933-34. SHELBURNE,S. A. Primary tumors of the heart; with special reference to certain features which led to a logical and correct diagnosis before death. Ann. Int. Med., 9: 340, 1935-36. LISA, J. R., HIRSCHHORN, L. and HART, C. A. Tumors of the heart: report of 4 cases and review of the literature. Arch. Znt. Med., 67: 91,194l. MARTIN, W. C., TUOHY, E. L. and WILL, C. Primary tumor of the heart (entrance of the Am. Heart J., 17: 728, pulmonary artery). 1939. BRADLEY, E. B. and MAXWELL, E. S. Primary neoplasms of the heart. J.A.M.A., 91: 1352, 1928. HURST, J. W. and COOPER, H. R. Neoplastic Am, Heart J., 50: 782, diseases of the heart. 1955. CHOISSER, R. M. and RAMSEY, E. M. Angioreticuloendothelioma (Kaposi’s disease) of the heart. Am. J. Path., 15: 155, 1939. Mallory, T. B. et al. Case records of the Massachusetts General Hospital, Case No. 22491. New England J. Med., 215: 1082, 1936. KIDDER, L. -4. Congenital glycogenic tumors of the heart. Arch. Path., 49 : 55, 1950. LANDING, B. H. and BANGLE, R., JR. Familial cardiac glycogen storage disease: report of two cases and discussion of relation to other forms of abnormal glycogen deposition. Bull. Internat. A. M. Museums, 31: 84, 1950. BECK, C. S. An intrapericardial teratoma and a tumor of the heart, both removed operatively. Ann. Surg., 116. 161, 1942. DUBOST, C. In: International Symposium on Cardiovascular Surgery, p. 207, edited by Lam, C. R. Philadelphia, 1955. W. B. Saunders Company. DIFFERDING, J. T., GARDNER, R. E. and ROE, B. B. Intracardiac myxomas with report of two unusual cases and successful surgery. Circulation, 23: 929, 1961. DUGAN, D. J. and WALSTAD, P. M. Left atrial myxoma, surgical cure. Dis. Chest, 38: 429,196O. ADAMS, C. W., COLL~S, H. A., DUMMIT,E. S. and ALLEN, J. H. Intracardiac myxomas and

JUNE 1963

89.

90. 91.

92.

93.

94.

95.

96.

97.

98. 99.

100.

101.

102.

103. 104, 105.

797

thrombi, clinical manifestations, pathology and treatment. Am. J. Cardiol., 7: 176, 1961. LEVINSON,J. P. and KINCAID, 0. W. Myxoma of the right atrium associated with polycythemia: report of successful excision. New Englad J. Med., 264: 1187, 1961. FATTI, L. and REID, F. P. Excision of atria1 myxoma. Brit. M. J., 2: 531, 1958. NICHOLS,H. T. The removal of benign pedunculated intra-atria1 tumors, a closed surgical approach. J. Thoracic Surg., 31: 739, 1956. BAHNSON,H. T. and NEWMAN, E. V. Diagnosis and surgical removal of intracavitary myxoma of Bull. Johns Hopkins Hosp., 93: right atrium. 150, 1953. SOLOMON, M. Interstitial pulmonary fibrosis secondary to pulmonary venous hypertension, report of a case due to myxoma of the left atrium. J.A.M.A., 174: 464, 1960. BARNARD, C. N., SWANEPOEL, A., MCKENZIE, M. B. and PHILLIPS,W. L. Left atrial myxoma, report of a case successfully treated using exBrit. J. Surg., 48: tracorporeal oxygenation. 73, 1960. ELLIS, F. H., JR., MANKIN, H. T. and BURCHELL, successful H. B. Myxoma of the atrium; surgical treatment in 2 cases. M. Clm. North America, 42: 1087, 1958. SCANNELL, J. G. and GRILLO, H. C. Primary a surgical problem. J. tumors of heart: Thoracic Surg., 35: 23, 1958. GERBODE, F., OSBORN, J. J., ROBSON, G. B., Left BRAINBRIDGE, M. and HULTCREN, H. successful removal with the atria1 myxoma; Ann. Surg., aid of extracorporeal circulation. 147: 320,1958. BELLE, M. S. Right atria1 myxoma. Circulation, 19: 910,1959. BAHNSON,H. T., SPENCER,F. C. and ANDRUS,E. C. Diagnosis and treatment of intracavitary myxoAnn. Surg., 145: 915, 1957. mas of the heart. The surgical approach to the left BROCK, R. Guv’s atrium, removal of an atrial myxoma. Hosp. Rep., 105: 382, 1956. MALM, J. R., HENRY, J. B. and DETERLING,R. A., JR. Clinical and pathological study of benign report of successful intracardiac tumors; removal of a myxoma of the left atrium. Czrculation, 18: 754, 1958. COOLEY, D. A., MORRIS, G. C., JR. and ATTAR, S. surgical treatment in four Cardiac myxoma; cases. Arch. Surg., 78: 410, 1959. ROBERTSON,R. Primary cardiac tumours, surgical treatment. Am. J. Surg., 94: 183, 1957. COBBS, B. W., JR. Personal communication. DOCK, W. Personal communication.