Atrial Myxomas: Special Emphasis on Unusual Manifestations

Atrial Myxomas: Special Emphasis on Unusual Manifestations* Panagiotis N. Symbas, M.D., F.C.C.P.;•• Osler A. Abbott, M.D .• F.C.C.P.;t William D. Logan, M.D., F.C.C.P.;•• and Charles R. Hatcher, ]r., M.D., F.C.C.P.••

From January 1959 to January 1969, eight patieuts with atrial myxoma were seen at Emory University and Grady Memorial hospitals. Five patients had symptoms and signs compatible with mitral stenosis, three of whom underweut swgery with an incorrect preoperative diagnosis of mitral steuosis. Two myxomas were found at autopsy, one in a patient who died of acute myocardial infarction. One patient had findings suggestive of mitral stenosis and symptoms of subacute bacterial endocarditis. One patient was seen for recurrent dyspnea and a calcified right atrial mass and one for dyspnea and unexplained cyanosis. Myxoma of the heart is more common than generally reaUzed and its clinical manifestations vary and can mimic other clinical entities. Therefore, a high index of suspicion concerning their diagnosis is needed. Early diagnosis should be established utiUzing angiocardiography and immediate resection should be performed before catastrophic complications occur. The results of the surgical treatment of atrial myxoma are gratifying and long lasting.

Despite increased clinical awareness and improved diagnostic techniques, the diagnosis of atrial myxoma may still appear as a surprise at surgery or autopsy. This is due to the tumor's variable clinical presentations and its tendency to mimic other clinical entities.l-6 The purpose of this communication is ·to present ten years' experience with this cardiac tumor and emphasize certain details of three patients with unusual atrial myxoma manifestations.

women) with atrial myxoma, were seen at Emory University and Grady Memorial hospitals, some of which have been previously reported 7 (Table 1). Six of the tumors were located in the left atrium and two in the right atrium, one of which protruded into the left atrium through an atrial septal defect. Four of the patients had symptoms of impairment of blood flow at the left atrioventricular level and the preoperative diagnosis of mitral stenosis was made, but an unexpected atrial myxoma was found in three of them at the time of thoracotomy for mitral commissurotomy, and in one at autopsy. One patient complained of dyspnea and his chest roentgenogram showed a calcified mass in the right atrium. The diagnosis of atrial tumor, prob: ably myxoma, was made which was confirmed after its successful surgical resection. One patient was admitted with symptoms and electrocardiographic findings indicative of acute myocardial infarction and died shortly after admission. At autopsy, an unexpected left atrial myxoma was found in addition to the acute myocardial infarction. In another instance a patient presented

OBSERVATIONS

During the last ten-year period, January 1959 to January 1969, eight patients (four men and four °From the Joseph B. Whitehead Deparbnent of Surgery, Thoracic and Cardiovascular Surgery Division, Emory University School of Medicine, Atlanta, Georgia. •• Associate Profes.wr of Surgery, Thoracic and Cardiovascular Surgery Division, Emory University School of Medicine, Atlanta, Georgia. tProfessor of Surgery, Thoracic and Cardiovascular Surgery Division, Emory University School of Medicine, Atlanta, Georgia. Supported in part by USPHS grant No. HE05861-02. F!esented at the Thirty-Fifth Annual Meeting of the American College of Chest Physicians, Chicago, Illinois, October 29 to November 2, 1969.

504

505

ATRIAL MYXOMAS

Cardiac Patient Age Sex 65

2

3

4

5

6

7

8

26

SymptoJDB and Signa

Cheat X-ray

M Chest pain Dyapnea M Recllm!nt dyspnea

F Dyapnea Orthopnea Variable murmur 49 M CHF nonvariable murmur 43 F CHF Transient CVA Nonvariable murmur 43 F Chest pain Dyspnea Weight 1011 Fever Malaiae Nonvariable murmur 59 M Dyspnea Orthopnea Nonvariable murmur 49 F Severe cyanoais Dyapnea Nonvariable murmur

Mobile calcified RA IIIIIIB

51

RA - Right atrium. LA =- Left atrium.

Opntm or Autopsy FiDdinga

Catheter-

Angio-

Preoperative

iution

cardiography

DiagnOBia

Motive of Death

No

No

No

No

RAtumor

RAmyxoma

Yea

Mitral

No

stenosis ?LA tumor

Sudden death LA myxoma while awaiting

Opntive Procedure

Myocardial Sudden death from myocardial None infan:tion LA myxoma infan:tion ~on of RAmyxoma

Patient Follow-up Deceaaed

Doing well

None.

mitral

cornmiaaurotomy Sudden death LA myxoma while awaiting Cardiotomy for Deceaaed stenoaia ? regurgitation reoperation mitraliii'Alnolis Cardiotomy for LA myxoma Mitral mitral stenollia Doing wen stenosis ~nof LA myxoma Mitral

Yea

No

No

No

Normal

Yea

Yea

LA tumor

LA myxoma

~on

LA enlargement

No

No

Mitral

LA myxoma

mitral stenollia

of LA myxoma

Cardiotomy for stenoaia

Doing well

Doing well

~nof

LA myxoma Normal

Yea

Yea

RA tumor

RAmyxoma

Resection of RAmyxoma

Doing weU

CHF - Congestive heart failure. CVA - Cerebral vaacular accident.

constitutional symptoms, malaise, fever, weight loss, anemia, in addition to chest pain, dyspnea and the murmur of "mitral stenosis." After prolonged but unsuccessful treatment with massive antibiotics for suspected subacute bacterial endocarditis, atrial myxoma was considered, which was diagnosed by angiocardiography and proved on a subsequent histologic examination of the surgically removed left atrial tumor. Another patient was seen with dyspnea, severe cyanosis and a grade 2/6 systolic murmur. This clinical picture was initially considered to be the result of pulmonary hypertensiori. However, angiocardiography and subsequent microscopic study of the excised right atrial tumor revealed the cause to be atrial myxoma. There was no operative mortality in the five patients who had resection of their atrial myxoma and all are doing well ten months to ten years after surgery. One patient died while awaiting reoperaCHEST, VOL. 59, NO. 5, MAY 1971

tion for resection of .the left atrial myxoma diagnosed at the time of thoracotomy for suspected mitral stenosis. One patient died from myocardial infarction and another while awaiting "closed commissurotomy," and at autopsy an unexpected left atrial myxoma was found in both. Pertinent information from three patients with unusual manifestations is summarized in the following case reports. CASE REPoRTS

CASE 1 In January 1962, a 43-year-old woman had an episode of nocturnal chest pain, dyspnea and nausea and two years later developed arthralgia for the first time. By September 1965 weight loss, recurrent pleuritic chest pain, dyspnea on mild exertion and cough were noted. All of her symptoms were more intense when she was lying on the left side. In January 1966 she became febrile. Two months later she was admitted to the hospital complaining of malaise, weight loss,

506

SYMBAS ET AL after exercise 50/20 mm Hg ( mean 39). The pubnonary artery wedge pressure at rest was 19, v 23 (mean 19 mm Hg) and after exercise mean 30 mm Hg. The angiocardiogram showed a 6lling defect in the left atrium ( Fig 1 ) . Soon thereafter, under total cardiopulmonary bypass, a left atrial myxoma attached to the margin of the fossa ovalis was removed ( Fig 2 ) . On histologic examination the findings of the resected specimen were compatible with myxoma. Subsequent to surgery she has been asymptomatic. CASES

FIGURE 1. Cineangiocardiogram with the radiopaque material injected into the main pubnonary artery. dypsnea, pleuritic chest pain and persistent fever. Her admission temperature was IOI•F and a diastolic rumble with presystolic accentuation and an opening snap were heard. Her chest roentgenogram and electrocardiogram were within normal limits and five blood cultures showed no growth. She was anemic and the sedimentation rate was elevated. The diagnosis of subacute bacterial endocarditis superimposed on an old rheumatic mitral valve disease was made, and she was treated with intensive antibiotic therapy. During the third week of her treatment, a friction rub was heard intermittently. The diagnosis of cardiac myxoma was then entertained. In Aprill966 cardiac catheterization was performed. The resting pulmonary artery pressure was 41/15 mm/Hg (mean 26) and

FIGURE 2. Left atrial myxoma.

A 49-year-old woman was seen as an outpatient in April 1967, because of vague upper abdominal pain. Physical and radiologic examinations were unremarkable. In June 1967 she developed weakness, dyspnea on exertion, hepatomegaly, pretibial edema. Hospital study revealed that the hemogram, urine analysis, serum albumin, total serum bilirubin, electrocardiogram, and chest roentgenogram were within normal limits. The serum alkaline phosphatase was 22.5 King Armstrong units, the serum amylase 223 units, the sulfobromophthalein ( Bromsulphalein ) retention at 45 minutes was 32 percent. The liver scan showed diffuse areas of decreased uptake. Percutaneous liver biopsy demonstrated nonspeci6c fatty in6ltration and exploratory laparotomy, performed to confirm a clinical impression of pancreatic carcinoma, revealed only an enlarged liver. The liver biopsy obtained at the time of laparotomy showed "portal and periportal chronic inflammation with periportal scarring and marked sinusoidal dilation." During this procedure, she suddenly developed intense cyanosis which gradually cleared over the next two days. Following this episode, she did well and in December 1967, she underwent an uneventful total abdominal hysterectomy for vaginal bleeding secondary to uterine fibromyoma. In April 1968, she noted cyanosis of the 6ngemails, ears and tongue and a progressive onset of dyspnea which by November 1968, markedly limited her activity. By this time she developed clubbing of the fingers. The arterial blood p02 was 43 mm Hg, which became 52 mm Hg after the administration of 100 percent oxygen by mask, the Peo:z27 mm Hg and the pH 7.46. In December 1968, she was hospitalized with a tentative diagnosis of "pubnonary hypertension." The only abnormal findings on admission were: symmetrical cyanosis with moderate clubbing of the fingers and toes, mild pitting edema of the ankles, a soft grade 2/6 midsystolic murmur audible along the left sternal border and an enlarged liver 5 em below the right costal margin which was firm, smooth, slightly tender and nonpulsatile. Right heart catheterization was performed, during which the catheter met resistance in the right atrium and could not be advanced from the superior to the inferior vena cava nor vice versa but easily crossed the atrial septum. A right-to-left shunt at the atrial level was demonstrated by indicator dilution curves, which was estimated by oximetry to be 3.2 L/min. Angiocardiography was performed via both superior and inferior vena cavae, demonstrating a large apparently immobile defect within the right atrial chamber and the interatrial shunt (Fig 3). In January 1969, the patient was operated upon. The heart was exposed through a right anterolateral thoracotomy incision and the anterior pericardium was resected and preserved in sterile saline. On gentle palpation of the right atrium, a hard tumor occupying practically the entire chamber was felt. The tumor extended down to the inferior vena cava and

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ATRIAL MYXOMAS

507

FIGURE 3. Angiocardiogram with the radiopaque material injected in the inferior vena cava. A (left) Anteroposterior and B (right) lateral view. precluded cannulation of the inferior vena cava through the right atrium. The inferior vena cava, therefore, was cannulated through the femoral vein and the superior vena cava through the appendage of the right atrium. Following cannulation of the left femoral artery, the patient was placed on cardiopulmonary bypass. The inferior vena cava was dissected off the diaphragm and was occluded with a vascular clamp. The right atrium was opened close to the atrioventricular groove and a gray, greenish tumor was visualized which occupied the right atrial cavity and herniated into the left atrium through an atrial septal defect (Fig 4). The tumor was partially obstructing the orifice of the tricuspid valve. Because of its extensive attachment to the right atrial wall and the interatrial septum, the tumor with the right atrial wall and the interatrial septum were resected. After resection, the left atrium and left ventricle were thoroughly irrigated with saline. The interatrial septum was then recon-

FIGURE 4. Drawing depicting the right atrial tumor.

CHEST, VOL 59, NO. 5, MAY 1971

structed with a Tefton felt patch and the right atrial wall was reconstructed with the preserved excised pericardium ( Fig 5) . Pathologic examination revealed a gelati nons tumor which had the microscopic characteristics of a myxoma. Postoperatively the patient was noted to have a left hemiparesis and to be partially aphasic. These neurological deficits were thought to be the result of tumor or air embolization. The aphasia cleared completely and there has been partial resolution of the hemiparesis. Her cyanosis disappeared and she is currently free of cardiopulmonary symptoms. In January 1970 right heart catheterization and angiocardiography were repeated and showed normal pressures, no evidence of shunt and normal appearing right atrium ( Fig 6). C.uE7 A 59-year-old man, in 1967, noted onset of shortness of breath on exertion which progressed by 1968 to severe dyspnea. By that time he noted paroxysmal nocturnal dyspnea when sleeping on his back or left side but not while lying on his right side, and precordial chest pain occurring especially on exertion. He was then seen by his private physician and was started on digoxin and diuretics without improvement. In February 1969 he was admitted to the hospital at which time a loud S1 and P2, a soft presystolic rumble at the apex and an opening snap at 0.10 to 0.11 seconds at the apex and left sternal border were heard but no diastolic murmur was present. The electrocardiogram and the chest roentgenogram were considered compatible with mitral stenosis ( Fig 7). Two days after admission he was operated upon for closed commissurotomy but when the finger was inserted into the left atrium a tumor was palpated. Cardiopulmonary bypass was instituted and a tumor attached to the margin of the fossa oval is was removed ( Fig 8) . Pathologic examination showed a 5 .5 X 4.5 X 4.0 tumor which had the typical histologic picture of an atrial myxoma. Subsequent to surgery the patient has been asymptomatic.

508

SYMBAS ET AL

Teflon felt

I

5. Drawing depicting the right atrial reconstruction.

FIGURE

DISCUSSION

Myxoma of the heart is the most common of all benign cardiac tumors and predominantly involves the atria, the left one three times more frequently than the right. 8 It may appear either as a semitranslucent, gelatinous sessile, lobulated, or as a round firm, green or pink mass, the latter is almost always attached with a pedicle to the interatrial septum at the margin of the fossa ovalis. Histologically, the bulk of the myxoma consists of an amorphous matrix which is considered to be predominantly an acid mucopolysaccharide related to chondroitin-6-sulfate. 9 The clinical manifestations of the myxoma are variable and are dependent, to a certain extent, on

FIGURE

the location of the tumor. 1o The myxomas can miinic several other clinical entities but basically present themselves in three ways: by embolization, 8 • 11 by obstruction of intracardiac blood 8ow 12 • 13 and by constitutional disturbances.11.14 Virtually any artery in the body may be occluded by myxoma emboli. For this reason, the diagnosis of myxoma should be considered in a patient who presents signs and symptoms due to embolism of no apparent cause, and the embolectomy specimen should always be examined histologically because there are several myxomas diagnosed in this way.6.1~

The majority of patients with atrial myxoma present symptoms of stenosis of the atrioventricular valves and in many of them the diagnosis of left

6. One year postoperative angiocardiogram showing the reconstructed right atrium.

CHEST, VOL. 59, NO. 5, MAY 1971

509

ATRIAL MYXOMAS

FIGURE

7. Preoperative chest roentgenography.

atrial myxoma is made at the time of surgery for a planned closed mitral commissurotomy. Four of the reported cases herein had symptoms of mitral stenosis, in three of which the correct diagnosis was made at surgery. In one of the patients operated upon for mitral stenosis, the resection of the tumor ~as postponed for a later day because then the use of prime solutions other than blood and disposable oxygenators was not practiced. He died suddenly the next day as did an additional patient with similar symptoms while he was awaiting surgery, "mitral commissurotomy." These two cases emphasize the risk of postponing the resection of the

FIGURE

8. Resected left atrial myxoma with its pedicle.

CHEST, VOL 59, NO. 5, MAY 1971

myxoma. Resection of this tumor now is immediately performed if it is found unexpectedly at the time of closed commissurotomy, or as soon as possible if the diagnosis is made preoperatively. The variability of the symptoms and signs with the change in the patient's position and the odd crushing noises, which are the result of the tumor rubbing on the endocardial surface are rarely present, but are the most valuable of the diagnostic physical findings. Fever, anorexia, malaise, weight loss, raised sedimentation rate, anemia and elevation of gamma globulin are the constitutional manifestations of atrial myxoma.11.14.t:6 Some of these patients are admitted and treated for subacute bacterial endocarditis. However, if the patient fails to respond to the proper treatment and blood cultures are persistently negative, as seen in case 1, the possibility of cardiac myxoma should be investigated. The factors responsible for the constitutional manifestations are not clear. The presence of breakdown products released from the myxoma into the bloodstream have been suggested. 1 7 An immune response to fragments of tumor released into the circulation has been suggested as a possible cause of the increase in gamma globulin. IS The possibility that an intracardiac myxoma initiates and maintains an autoimmune state analogous to that seen after cardiotomy or myocardial infarction has also been suggested.• 9 Patients with myxoma of the right atrium and tricuspid valve obstruction may have high hemoglobin instead of anemia and arterial oxygen desat-

510

SYMBAS ET AL

uration (as seen in case 2) due to the right-to-left shunt through a coexisting atrial septal defect or through a dilated foramen ovale. The diagnosis of an intra-atrial mass, myxoma, is established in nearly 100 percent of the cases with angiocardiography.2.2o The radiopaque material is injected into the cavae for right atrial myxoma and into the pulmonary artery for those on the left side. 21 The development of the cardiopulmonary bypass unit has made resection of the atrial myxoma possible and the treatment of this tumor gratifying. Resection of the atrial myxoma under total car.diopulmonary bypass should be performed shortly after the diagnosis is established. The excision of the area of the septum where the atrial myxoma is attached remains a controversial point.16.22 Most authors suggest this is not necessary,16 while others, having seen recurrence of the atrial myxoma at the site of the previously transected tumor pedicle, feel that resection of the cardiac wall at the tumor attachment is necessary. 22 Two of the reported patients herein had resection of their tumor with its pedicle, two had, in addition, resection of the atrial septum where the myxoma was attached and one had resection of the intraatrial septum and right atrial wall in addition to the tumor. None of the resected myxomas recurred in the 13 months to ten years' follow-up period. fu:FERENCES 1 Ashman H, Zaroff L, Baronofsky 1: Right atrial myxoma. Amer J Med 28:487, 1960 2 Cooley DA, Morris GC, Jr, Attar S: Cardiac myxoma. Arch Surg 78:410, 1959 3 Ellis FH, Mankin HT, Burchell HB: Myxoma of the atrium: successful surgical treatment in two cases. Med Clin N Amer 42:1087, 1958 4 Emanuel RW, Lloyd WE: Right atrial myxoma mistaken for constrictive pericarditis. Brit Heart J 24:796, 1962 5 Holswade GR, Nydich I, Steinberg 1: Successful removal

6 7 8 9 10 11 12 13 14 15 16

17 18 19 20 21 22

of right atrial myxoma mistaken for liver and pericardia! metastasis. J Thorac Cardiovasc Surg 52:240, 1966 Willman VL, Symbas PN, Mamiya, RT, et al: Unusual aspects of intracavitary tumors of the heart. Report of two cases. Dis Chest 47:669, 1965 Abbott OA, Warshawski FE, Cobbs BW, Jr: Primary tumors and pseudotumors of the heart. Ann Surg 155:855, 1962 Prichard RW: Tumors of the heart. Arch Path 51:98, 1951 Heath D, MacKinnon J: Pulmonary hypertension due to myxoma of the right atrium. Amer Heart J 68:227, 1964 Goodwin JF, Kay JM, Heath D: Clinical-pathologic conference. Amer Heart J 70:239, 1965 Goodwin JF: Diagnosis of left atrial myxoma. Lancet 1:464, 1963 Goodwin JF, Stanfield CA, Steiner RE, et al: Clinical features of left atrial myxoma. Thorax 17:91, 1962 Barlow J, Fuller D, Denny M: A case of right atrial myxoma. Brit Heart J 24:120, 1962 Vuopio P, Nikkilii E: Hemolytic anemia and thrombocytopenia in a case of left atrial myxoma associated with mitral stenosis. Amer J Cardiol17:585, 1965 Silverman J, Olwin JS, Graettinger JJ: Cardiac myxomas with systemic embolization. Review of the literature and report of a case. Circulation 26:99, 1962 Firor WB, Aldridge HE, Bigelow WG: A follow-up study of three patients after removal of left atrial myxoma five to ten years previously. J Thorac Cardiovasc Surg 51:515, 1966 Lekisch K: Myxoma of the left atrium: report of a case. Ann Intern Med 46:982, 1957 Boss JH, Becher M: Myxoma of the heart: Report based on four cases. Amer J Cardiol3:823, 1959 Currey HLF, Matthews JA, Robinson J: Right atrial myxoma mimicking a rheumatic disorder. Brit Med J 1:547, 1967 Morrissey JF, Campeti FL, Mahoney EB, et al: Right atrial myxoma. Report of two cases and review of the literature. Amer Heart J 66:4, 1963 Steiner RE: Radiologic aspects of cardiac tumors. Amer 1 Cardiol 21:344, 1968 Gerbode F, Kerth WJ, Hill JD: Surgical management of tumors of the heart. Surgery 61:94, 1967

Reprint requests: Dr. Symbas, Department of Surgery, 69 Butler Stret>t, SE, Atlanta 30303

CHEST, VOL. 59, NO. 5, MAY 1971