Acta med. scand. Vol. 186, pp. 165-171, 1969

RIGHT ATRIAL MYXOMA

Jgrgen Fischer Hansen, Kjeld Lyngborg, Mogens Andersen and Alf Wennevold

From the Medical Department B, Rigshospitalet (University Hospital), Copenhagen, Denmark

Abstract. A review of the symptomatology of right atrial myxoma is given, based on 33 cases from the literature and on two cases studied by us. This diagnosis should be considered in any patient with

isolated right heart failure without pulmonary congestion, pulmonary disease, or pulmonary stenosis.

A tricuspid murmur, intermittent cardiac or cerebral symptoms or radiographic demonstration of non-valvular intracardiac calcifications should increase the suspicion.

Selective right atrial angiocardiography is the diagnostic method of choice. The diagnosis cannot be excluded with- out this examination, which always should be performed in any patient with isolated tricuspid valvular disease.

Primary tumors of the heart are rare. Straws and Merliss (30) found a rate of 17 primary tu- mors per million autopsies during the period 1938 to 1942. One half of the tumors reported by Prichard (25) were myxomas. Seventy-five per cent of the myxomas were located in the left atrium and about 25% in the right atrium.

Atrial myxoma is now a curable disease. The first successful operation was performed in 1954 by Crafoord (1 1) under direct vision during cardio- pulmonary bypass. Since then several successful operations have been reported (26).

The diagnosis of right atrial myxoma is accord- ingly important. It can be rendered probable by angiocardiography if only the disease is suspected. Our purpose is thus to give a review of right atrial myxoma with emphasis on the symptoma- tology, based on 33 cases previously published (1-10,12-24,26-29,31-33) and on two additional cases, which we have recently encountered in this department.

CASE REPORTS Case I A 51-year-old woman was admitted in February 1967 for possible mitral heart disease.

There was no history of rheumatic fever. At the age of

29 years she was admitted to a local hospital with symp- toms of embolism of the left lung.

Two years later, at the age of 31 years, she had another pulmonary embolus. Treatment with digitalis was started and thereafter continued.

Ever since the last episode the patient had symptoms consisting of attacks of dyspnea at rest but also to some extent on exertion. She had the feeling of being suffocated and drawn forward during these attacks, but she had never fainted. She had furthermore noticed that she was unable to lie on her left side because of dyspnea.

For the last two years the symptoms progressed, and she was admitted for diagnostic investigation.

Physical examination revealed a slender woman in no distress. There was no dyspnea at rest and no cyanosis. Slight engorgement of the neck veins and venous pulsation with a big a-wave was seen. The liver was palpable 4 cm below the curvature. There was no ascites or peripheral edema, and no pulmonary stasis was found. The blood pressure was 120/80 mm Hg.

At auscultation of the heart a systolic murmur grade 2 (of 6) was heard at the apex and at the left lower part of the sternal border. No diastolic murmur was noticed.

The electrocardiogram showed sinus rhythm with a QRS axis of +110 degrees and peaked P-waves in standard leads I1 and 111.

The roentgenogram of the chest showed moderate car- diomegaly with dense intracardiac calcifications (Figs. 1 and 2).

Laboratory tests. The hemoglobin was 14.6 g per 100 ml, and the sedimentation rate 5 mm/h. Electrophoresis of the serum proteins showed normal distribution. A lung scanning with J 131 albumen was performed and showed reduced blood flow through the left lower lobe.

Hemodynamic investigation. At right heart catheteriza- tion the catheter could only with difficulty be passed through the tricuspid ostium. The pressure in the pulmonary artery was 17/5 mm Hg, in the “wedge” position mean 5 mm Hg, in the right ventricle 17/0 mm Hg, and in the right atrium - 1 mm Hg in mid-diastole. The cardiac index was 1.4 1 per ma only.

A selective angiocardiography and cineangiography with contrast injection into the right atrium was performed. A large mobile mass, a part of which was calcified, was demonstrated in the right atrium.

An operation in extracorporeal circulation was performed, and a yellowish tumor was completely removed from

Acta med. scand. 186

166 J . Fischer Hanseti et a!.

Fig. 1. Antero-posterior roentgenogram of the chest of case 1. Note the calcifications of the right atrial myxoma.

the right atrium. The tumor was pedunculated, being at- tached to the margins of fossa ovalis; it filled the entire atrium and was hourglass-shaped. The part facing the tricuspid ostium was calcified, while the rest of the tumor was of brittle consistency. Histological examination showed myxoma with calcification.

Postoperatively the patient developed arrhythmias mainly consisting of intermittent atrio-ventricular block, atrial flutter and atrial fibrillation. One month after operation sinus rhythm was restored through a DC countershock. At a follow-up examination one year later she still had sinus rhythm; however, she still needed diuretic treatment because of moderate right heart failure, and she had a systolic murmur grade 2 at the left lower sternal bor- der-increasing during inspiration-and pulsation of the neck-veins due to moderate tricuspid insufficiency.

Case 2 A 53-year-old man was transferred from another hospital at the end of March 1967 for diagnostic investigation of possible constriction of the heart.

He had never had rheumatic fever, pleurisy or tuber- culosis and he had previously been in good health.

In August 1966 he was admitted for a few days to the local hospital because of an abnormally deep Q,,, in the electrocardiogram, which was taken incidently during a medical examination for insurance purposes. His only symptom at that time had been one episode of dyspnea on climbing stairs one month previously. Physical examina- tion was normal except for obesity. The roentgenogram of the chest was normal. He was discharged with a diagnosis of old myocardial infarction.

Four months later he developed increasing dyspnea on exertion, a persistent unproductive cough, edema of the legs, and swelling of the abdomen. He was readmitted to the local hospital in March 1967. Hypoalbumenia was found in addition to elevated serum bilirubin and low

Acta med. scand. 186

Fig. 2. Lateral roentgenogram of the chest of the same patient as in Fig. 1. Note the posteriorly situated calcifi- cations.

prothrombin time. The roentgenogram of the chest showed enlargement of the cardiac silhouette; the electrocardio- gram showed marked low voltage, which was the main reason for the suspicion of constriction of the heart.

Physical examination revealed an obese man in no acute distress. There was slight dyspnea at rest and slight cyanosis of the ears, lips and nails. Some engorgement of the neck veins was seen, but no venous pulsation. The liver was palpable about 10 cm below the curvature, and there was marked ascites. There was no peripheral edema. No pulmonary stasis could be detected. The blood pres- sure was 130/80 mm Hg.

Fig. 3. Phonocardiogram from the fourth left intercostal space recorded during inspiration in case 2. Note the long diastolic murmur.

Right atrial myxoma 167

Fig. 4. Phonocardiogram of case 2 during atrial flutter with 4 : 1 block. The diastolic murmur during inspiration is divided into short parts synchronous with the P-waves.

Aitscitltarion of the heart. The heart sounds were very faint, the second heart sound being barely audible. In the fourth left intercostal space at the sternal border a very peculiar murmur was heard; it was a diastolic, super- ficial, scratching medium to high frequency murmur, which was only present during inspiration (grade 2), being completely inaudible during held expiration. This was found when the patient had sinus rhythm, nodal rhythm, or atrial fibrillation (Fig. 3).

During part of his time in hospital the patient had atrial flutter with changing block, usually 4: 1 block. During this rhythm the murmur was present during the whole inspiration as short parts interrupted by brief, si!ent intervals. The short parts of the murmur were syn- chronous with the P-waves of the electrocardiogram rhythm, atrial fibrillation and atrial flutter with usually (Fig. 4).

The electrocardiogram showed a markedly low voltage The roentgenogram of the chest showed a diffusely in all leads (Fig. 5 ) . The rhythm changed-as mentioned- between sinus rhythm with wandering pacemaker, nodal Lnboratory tesrs. The hemoglobin was 14.3 g per 100

~ j ~ . 5. Electrocardiogram of our case 2.

4 : 1 block.

enlarged heart.

Fig. 6. Selec:ive angiocardiography with contrast injection into the right atrium af case 2. Anteroposterior (a) and lateral (b) projection.

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168 J . Fischer Hansen et al.

ml, the sedimentation rate 4 mm/l hour, the serum bili- rubin 3.5 mg per 100 ml, the thymol extinction test 2, the alkaline phosphatase 49 units per 100 ml (normal upper limit 38 units per 100 ml), the lactic acid dehydrogenase 34 units per ml (normal upper limit 24 units per ml) with the elevation being confined to isoenzyme no. 1 (myo- cardial fraction), and the prothrombin percentage was 25. There was slight hypoalbuminemia (4.1 g per 100 ml) and hypergammaglobulinemia (2.0 g per 100 ml),

Hernodynamic investigation. At right heart catheteriza- tion the pressure in the pulmonary artery was 21/14 mm Hg, in the “wedge” position mean 11 mm Hg, and in the inflow tract of the right ventricle 33/7 mm Hg. On with- drawal of the catheter to the right atrium a pressure gradient over the tricuspid valve of 19 mm Hg was found, the mid diastolic pressure in the right atrium being 26 mm. The pressure tracings did not show the characteristic configuration of constriction of the heart. The cardiac index was 1.4 1 per ma, and the arterial oxygen saturation 89 % . At selective angiocardiography and cineangiography with contrast injection into the right atrium a mass was seen extending from the right atrium to the right ventricle impeding the flow through the tricuspid valve and through the outflow tract of the right ventricle (Fig. 6). Intra- cardiac phonocardiography demonstrated a diastolic mur- mur in the inflow tract of the right ventricle.

At open-heart operation in extracorporeal circulation a right atrial tumor of the size of a tennis ball was removed. The tumor had a short, broad stem to the atrial septum close to the foramen ovale, and it extended towards the tricuspid valve, which was normal. The tumor was soft and friable. The histological diagnosis was pseudomyxoma. The patient recovered slowly.

At follow-up examination one year later the patient was asymptomatic with sinus rhythm, and the low voltage had disappeared.

SYMPTOMATOLOGY

Our review of the symptomatology is based on the case histories of 33 patients, which have been published previously with sufficient information for our purpose, and on our two patients presented above. The histological diagnosis was established at autopsy in three cases and at operation in the remaining 32 cases.

There were 15 males and 20 females. The age distribution at the time of the onset of symptoms is seen in Fig. 7. The youngest patient was 7 months old, and the oldest 57 years old.

The various symptoms and signs may be divided into four groups as seen in Table 1.

Of the constantly present symptoms right heart failure was seen in all but two patients. It was ob- viously due to the mechanical obstruction caused by the tumor. Dyspnea was also commonly seen, possibly due to a low cardiac output secondary to

Acta med. scand. 186

Numbw d patients

Age in years

Fig. 7. Age distribution of 35 patients with right atrial myxoma.

the low venous return. The low cardiac output might also explain the cyanosis and the signs of insufficient peripheral circulation.

The intermittent symptoms, which were seen in 22 patients, were probably caused by a sudden increase in the obstruction to the blood flow through the tricuspid ostium, e.g. provoked by the

Table I. Frequency of symptoms and signs in 35 patients with right atrial myxoma

Initial No. of in no. pats. of pats.

Constant Right heart failure Dyspnea during exercise Dyspnea at rest Cyanosis Insufficient peripheral circulation

Leg cramps Gangrene Raynaud’s symptoms Thrombophlebitis

Intermittent Dyspnea

In certain positions Nocturnal Unprovoked

Cerebral symptoms Syncope or dizziness Epileptic seizures

Precordial or epigastric pain Cyanosis Flushing General Fever Weight loss Pigmentation Pulmonary embolism

2 1 1 2

7 2 2

12 1

33 0 2 1 19 12 1 8 1

6 0

11 2 0 0 2

I 0

13 1

13 7 9 0 4 0

7 2 7 1 3 0 6 1

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Table 11. Auscultatory findings in 35 patients with right atrial myxoma

No. of pats.

Diastolic murmur in tricuspid area 24 Systolic murmur in tricuspid area 21 Systolic/diastolic murmur only at the apex 2 Gallop rhythm 1 1 Friction rub 6 Normal findings 2

Total 35

Table 111. Electrocardiographic findings in 35 patients with right atrial myxoma

No. of pats.

Right atrial hypertrophy 19 Right axis deviation 8 Low voltage 18 Right bundle branch block 11 Sinus rhythm 29 Atrial fibrillation 4 Intermittent atrio-ventricular block 3 Entirely normal 2

Total 35

assumption of a new position as the left lateral recumbent position. The cerebral symptoms were often alarming and dominating. The precordial and epigastric pain was often intense but otherwise uncharacteristic.

Of the general symptoms which usually accom- pany tumors, fever and weight-loss were each found in one fifth of the cases.

Pulmonary embolism occurred in six patients, the embolus consisting of either tumor tissue or thrombotic tissue. It was fatal in one case.

The duration of the symptoms until correct diagnosis averaged 3 years, ranging from five days to 36 years. The duration was less than one year in 25% of the cases.

A uscultation As seen in Table I1 the auscultatory findings are mainly those of tricuspid valvular disease. The findings are often transient and may vary with the position of the patient.

The gallop rhythm was most likely due to tumor impaction as suggested by Sannerstedt et al. (26), and the friction rub was probably secondary to

affection of the pericardium, as pericardial exudate was found in six cases.

The diastolic atrial murmur which was inter- rupted and synchronous with the P-waves (Fig. 4) in our case 2 could easily be mistaken for a fric- tion rub.

Electrocardiogram The findings are summarized in Table 111. In most cases a right-sided heart disease was indicated by signs of right atrial hypertrophy (peaked P-waves in 11, 111, aVF or right-sided precordial leads), right axis deviation, or right bundle branch block. As many as half of the patients had unexplained low voltage (Fig. 5).

Roentgenogram of the chest A roentgenogram of the chest was obtained in all patients but one. In 22 cases cardiomegaly was noted. A prominent right heart border was seen in 18 cases. In five patients the heart was normal.

In four patients calcifications within the cardiac silhouette were noticed. Pleural exudate was de- monstrated in four cases. Pulmonary congestion was not noted in any case.

Laboratory tests These were uncharacteristic, in some cases con- sisting of anemia, leucocytosis and elevated sedi- mentation rate.

Right heart catheterization Heart catheterization was performed in 27 pa- tients. In all but three patients a diastolic pressure gradient was measured across the tricuspid valve. In two patients a pressure gradient was found over the pulmonary valve and over the outflow tract of the right ventricle, respectively. One patient had pulmonary hypertension due to recurrent tu- mor embolism.

Cardiac output was measured at rest in 13 pa- tients. Seven patients had either a cardiac output lower than 3.5 1 per min or a cardiac index lower than 2 1 per square meter per min.

A ngiocardiography Selective angiocardiography with contrast injection into the right atrium was performed in 25 cases without complications. In all cases the tumor was demonstrated.

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170 J . Fischer Hansrn et 01.

Table 1V. Initial clinical diagnosis of 35 patients with right atrial rnyxorna

No. of pats.

Pericarditis 8 Unspecific chronic 5 Tuberculous chronic 2 Acute 1

Mitral stenosis 4 Tricuspid stenosis 4 Rheumatic fever 2 Ebstein’s disease 2 Myocardial infarction 2 Carcinoid tumor 2 M yocarditis 1 Bacterial endocarditis (subacute) 1 Peripheral arterial insufficiency, lower extr. 1 Polycythemia Vera I Addison’s disease I Rheumatoid disease (arthritis) 1 “Functional” symptoms 1 No information 4

Total 35

Prognosis and treatment Three patients died without attempts at surgery, the cause being heart failure in two cases and pulmonary embolism in one case. In the remaining 32 cases surgery was performed.

Eight cases were operated on without extracor- poreal circulation, and four died. Twenty-four cases were operated on in extracorporeal circula- tion, with 16 survivors. The usual finding at opera- tion was a pedunculated tumor, by which the inter- mittent symptoms could be explained. Tricuspid insufficiency following operation was reported in five cases.

DISCUSSION

The clinical diagnosis may be difficult, as seen from the listing of the initial diagnosis in the 35 patients in Table IV.

As soon as a myxoma has reached a certain size, it will give rise to symptoms, of which right heart failure is dominant. In the presented material the lack of pulmonary congestion on the roentgeno- gram and the majority of the electrocardiographic findings clearly pointed to the right side of the heart as site of the disease.

Isolated right heart failure is found in pulmonary diseases, pulmonary stenosis, disease of the right ventricle, constrictive pericarditis, tricuspid valve disease and tumor of the right atrium. The first

Acta med. scnnd. 186

possibility is easily ruled out. Auscultation will in the maiority of cases reveal a tricuspid murmur, which in combination with a history of intermittent cardiac or cerebral symptoms should arouse the suspicion of myxoma. Non-valvular calcifications inside the cardiac silhouette point to the same possibility.

Furthermore right heart catheterization, which would be indicated in cases with isolated right heart failure, will usually show an isolated tricuspid stenosis and exclude constrictive pericarditis, pul- monary valve disease and disease of the right ventricle. As isolated tricuspid stenosis is extremely rare,

angiocardiography should be performed in all cases of apparent isolated tricuspid stenosis as well as in cases of isolated right heart failure in which the heart catheterization fails to give a definite diagnosis.

Selective right atrial angiocardiography will demonstrate the tumor. No complication to this examination in cases with right atrial myxomas has been reported.

Removal of the tumor by open heart surgery must be performed.

1.

2.

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8.

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1 1 .

12.

13.

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