Diagnostic features of right ventricular myxoma Stanley N. Snyder, M.D.* D a v i d C. S m i t h , M . D . * * Francis Y. K. Lau, M.D., F.A.C.C.*** A. F r a n k l i n T u r n e r , M . D . , F . A . C . C . * * * *
Los Angeles and San Diego, Calif.
Primary intracardiac tumors are uncommon, and those of ventricular origin are the least frequent. The intent of this communication is t o r e p o r t i m portant diagnostic features noted recently in a patient with a right ventricular myxoma and relate these findings to those previously reported in the literature.
Case report This 36-year-old white woman (V. B.) was referred for cardiac consultation as the result of a heart m u r m u r noted on a preemployment physical examination in June, 1972. She had no cardiovascular symptoms at the time of consultation and specifically denied any shortness of breath, chest pain, edema, cough, or hemoptysis. She was treated successfully with antibiotics for uncomplicated "pneumonia" eight months prior to consultation. On review of her hospital records, a nondescript systolic cardiac m u r m u r was noted 12 years previously. At t h a t time the peripheral pulses were normal and the remainder of the examination was not remarkable. At physical examination the patient was a healthy-appearing female with a blood pressure of 104/70 in both arms and normal pulse, respiration, and temperature. A prominent awave was noted in the jugular venous pulse. There was a mild pectus excavatum and a left parasternal lift. The apical impulse was in the fourth left intercostal space at the midclavicular line. The first heart sound was slightly diminished. The second sound was widely split and varied slightly, but physiologically, with respiration. The pulmonic component From the Los Angeles County-University of Southern California Medical Center, Los Angeles, and the United States Naval Hospital, San Diego. Received for publication" Jan. 3, 1974. Reprint requests to: Dr. Stanley N. Snyder, 9209 Colima Rd., Whittier, Calif. 90605. *Cardiology Fellow, LAC-USC Medical Center, Los Angeles, Calif. **Cardiologist, Lieutenant Commander, MC, USNR, United States Naval Hospital, San Diego, Calif. ***Professor of Medicine and Radiology, LAC-USC Medical Center, Los Angeles, Calif. ****Professor of Radiology and Medicine, LAC-USC Medical Center, Los Angeles, Calif.
24(}
was accentuated and audible at the apex. A Grade I I I / V I systolic ejection murmur was heard at the third and fourth left intercostal spaces and became more prominent with inspiration. A fourth heart sound was present at the left sternal border and was unchanged with the phases of respiration. Complete blood count, serum electrolytes, and cardiac enzymes were normal. The chest roentgenogram (Fig. 1) demonstrated a right-sided aortic arch and pleural thickening at the right lateral base. The electrocardiogram (Fig. 2, A) revealed a mean QRS axis of q 90 degrees in the frontal plane and a Pwave axis with leftward and superior orientation, There was an rSr' in Lead V,, and S-waves in the lateral precordial leads. T-wave abnormalities were noted inferiorly and over the right precordium. The Frank lead vectorcardiogram (Fig. 3, A) demonstrated rather prominent posterior and rightward terminal forces. Right ventricular hypertrophy was suggested on both the electrocardiogram and vectorcardiogram. The phonocardiogram and pulse tracings (Figs. 4, A and 4, B) demonstrated a systolic murmur, wide splitting of the second heart sound with slight respiratory variation, and a prominent a-wave in the venous pulse tracing. A radioisotope pulmonary perfusion scan disclosed right upper and lower lobe filling defects. A 48-hour cardiac scan (Fig. 5, A) utilizing 5 mCi. of 67Ga-citrate showed heavy gallium accumulation in the area of the mediastinum in the region of the sternum and extending to the left and cephalad, corresponding to the location of the main pulmonary artery, as demonstrated by the superimposed outline of the cardiomediastinum by a chest roentgenogram done at the same time. Preoperative cardiac catheterization (Table I and Fig. 6) demonstrated a right ventricnlar pressure of 92/8 and a pulmonary artery pressure of 35/14. Cineangiocardiographic studies (Figs. 7, A and 7, B) demonstrated a large, mobile, lobulated filling defect in the right ventricle extending through the pulmonary valve during systole. The selective pulmonary angiogram (Fig. 8, A) showed absence of some vascular branches in the right upper lobe and, to a lesser degree, in the right lower lobe. At surgery the right atrium, left atrium, and right ventricle were enlarged. The left ventricle appeared normal in size. There was a systolic thrill in the right ventricular outflow tract extending into the pulmonary artery. The right ventricle was opened transversely, revealing a gelatinous, irregular mass filling much of the right ventricle and outflow tract. I t was attached by a narrow stalk to the right ventricular wall at
February, 1976, Vol. 91, No. 2, pp. 240-248
Right ventricular myxoma
Fig. 3, A. Preoperative vectorcardiogram. Prominent posterior and rightward forces in the horizontal plane loop and vertical axis in the frontal plane. Fig. 1. Chest x-ray of the right-sided aortic arch. Upper arrow: impression on tracheal air column. Lower arrow: descending aorta to right of sternum. Note: pleural thickening at right costophrenic angle.
Fig. 2, A. Preoperative electrocardiogram. Tendency toward right axis deviation (q 90 degrees), rSr' in right precordial leads, abnormal T-waves in Leads II, IiI, aVF, and right precordial leads, and ectopic atrial focus.
Fig. 2, B. Postoperative electrocardiogram. Axis is q 60 degrees and T-waves upright in Leads II, III, and aV F.
American Heart Journal
Fig. 3, B. Postoperative vectorcardiogram. More normal terminal forces and frontal plane axis.
the junction of the anterior and posterior leaflets of the tricuspid valve. A 36-gram mass (Fig. 9, A) was removed, including a small portion of the endocardium with the stalk, to insure complete removal of the tumor. Histologically (Figs. 9, B, C, and D), the tumor revealed changes consistent with a myxoma. The patient was discharged after an uneventful recovery and readmitted approximately one m o n t h after surgery for reevaluation. Physical examination was normal, and the chest roentgenogram was unchanged. The electrocardiogram (Fig. 2, B) and the Frank vectorcardiogram (Fig. 3, B) now revealed a mean QRS axis of q 60 degrees. The right precordial rSr' and the T-wave abnormalities persisted, but the T-waves in the diaphragmatic leads were now upright. The phonocardiogram and pulse tracings (Figs. 4, C and D) showed no murmur, a decrease of the splitting interval of the second heart sound,
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S n y d e r et al.
Fig. 4, A and B. Preoperative phonocardiogram and pulse tracings. Long systolic murmur, As - P~ interval 150 msec. A-wave of venous pulse in upper limits of normal. There appears to be an abnormal relationship between P2 and V-wave. A, Paper speed = 100 mm. per second; SM = systolic murmur; E X = expiration; and CP = carotid pulse tracing. B, Paper speed = 100 mm.~ per se~cond and VP = venous pulse. and a normal venous pulse tracing. Cardiac catheterization (Table I) disclosed a right ventricular pressure of 35/5, with no outflow gradient. The right ventricular filling defects were absent (Fig. 7, C). The radioisotope pulmonary Perfusion scan and pulmonary angiograms (Fig. 8, B) were unchanged. Six months postoperatively, a 48-hour cardiac scan (Fig. 5, B), again using 5 mCi. 67Ga-citrate, revealed less gallium uptake in the area where the tumor had been.
Review of 16 cases This is the sixteenth case of right ventricular myxoma in our review of the world literature (Table II). '-15 Except for Catton, Guntheroth, and Reichenbach's7 case, the patients' ages ranged from 16 to 56, most being young adults at the time of diagnosis. There was slight predominance of females. Symptoms or signs antedated diagnosis by as much as 14 years. Only two patients were without complaint. Fever, shortness of breath, chest pain, cyanosis, syncope, seizures, and sudden death have been described. In two cases, pain was exertion~ al, 3"" and exertional syncope was noted in another patient. 1~
242
In one instance, episodes of positional palpitation were caused by ventricular tachycardia. TM The most common physical finding (14 out of 16 p a t i e n t s ) w a s a Grade III to IV/VI systolic murmur along the left sternal border, and of maximal intensity in the second or third intercostal space. Some authors described changes in intensity or location of the murmur from time to time. In one instance, these changes occurred after cardiac catheterization. 11 Some were associated with a palpable thrill, and a peculiar feeling of movement or a "worm-like" thrill has been described. A right ventricular lift was noted occasionally. In our case, and in one other, ~ the pulmonic component of the second sound was separated from the aortic component by an interval of 100 to 130 msec. In most reports, no information was available with regard to the second heart sound. To-and-fro scratches and clicks simulating pericarditis" have been described with right ventricular myxoma, and a diastolic murmur 7 was noted in one patient. Several patients had elevated jugular venous pressure and prominent a-waves. Two patients presented with fever and three patients were noted to be cyanotic. Electrocardiographic abnormalities included right axis devi-
February, 1976, Vol. 91, No. 2
R i g h t ventricular myxoma
Fig. 4, C and D. Postoperative phonocardiogram and pulse tracings. Murmur is now absent, A2 - P~ relationship is normal, A-wave is less prominent, and there is restoration of the normal relationship between P2 and the V-wave peak. C, Paper speed = 100 mm. per second; ECG = Lead II electrocardiogram; Apex PCG = phonocardi0gram, apex; 2 LICS PCG = phonocardiogram, second left interspace parasternal line; CPT = carotid pulse tracingi and DC = dicrotic notch. D, Paper speed = 100 mm. per second; ECG = Lead II electrocardiogram; 4 LICS phonocardiogram, fourth left interspace, parasternal line; and VFT = venous pulse tracing. PCG =
ation (eight patients), the so-called "incomplete right bundle branch block pattern" (five patients), complete right bundle branch block (three patients), right ventricular hypertrophy (five patients), and right atrial enlargement (two patients). Abnormal T-waves in the inferior a n d / o r right precordial leads were also noted (six patients). Three chest roentgenograms were normal, four disclosed enlargement of the main pulmonary artery, and three were virtually diagnostic of intracardiac tumor by the presence of calcification in the area of the right ventricular outflow tract, x"" " ~ Right ventricular enlargement was noted in two patients. Decreased pulmonary vascularity, enlarged main pulmonary artery, gross cardiomegaly, and right atrial enlargement were each noted in one case. Increased pulmonary vascularity was present in a patient who had, in addition, an atrial-septal defect. Our case was normal roentgenographically, except for a right-sided aortic arch and right costophrenic pleural thickening. At cardiac catheterization , significant right ventricle-topulmonary artery gradients were present in most patients. Occasionally, as in our patient, an infundibular chamber was simulated2 .... The right ventricular pressure was over 60 mm. Hg in the majority of patients, with a range of 44 to 140 mm. Hg. Cineangiocardiography was diagnostic with the demon-
A m e r i c a n Heart Journal
stration of filling defects in the right ventricle or its outflow tract. Diagnosis prior to surgery ~, 7:12 was not made in the absence of right ventricular calcification or cineangiocardiography. In one of these cases/~ a coincidental atriaI-septal defect was correctly diagnosed, b u t the tumor went undetected. Eleven out of thirteen patients operated upon survived, one patient having residual symptoms. In two patients, diagnosis was made at autopsy. The tumors were attached by single or multiple pedicles, most frequently to the right ventricular septum or free wail, but other sites of origin included the conus, the chordae, the pulmonary artery, and also the ventricular surface of the tricuspid or pulmonic valves. The tumors were described pathologically as myxoma tissue alone, except for four instances in which additional tissue origins were noted.'. ' 3 ,~
Discussion Fourteen out of Sixteen patients had murmurs at the left sternal border that suggested right ventricular outflow obstruction. The physical findings associated with right ventricular myxoma strongly resemble those of pulmonic valvular
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Snyder et al.
Fig. 5, A. Preoperative cardiac scan, anterior and posterior. Heavy concentration of gallium in the mid-chest area extending leftward and cephalad from right ventricle toward the main pulmonary artery,
Fig. 5, B. Postoperative cardiac scan, anterior and posterior. Considerable decrease in concentration of gallium in the mid-chest region, and the extension toward the pulmonary artery is now absent.
Fig. 6. Preoperative pressure tracing. During cardiac catheterization, with pullback from main, pulmonary artery to infundibulum to right ventricle demonstrating pressure gradients.
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Fig. 7, A. Preoperative cineangiography of right ventricle and outflow tract. Large, mobile filling defect occupying portions of the right ventricle and extending through the pulmonary valve (during systole).
~~
PulmonaryArtery
$~
~
~(vlYXOMA ~ ~ ( ~
.'-~-~Right Ventricle TricuspidValve Catheter
f --
Diaphragm
Fig. 7, B. Diagrammatic illustration of filling defect noted in
Fig. 7A. or infundibular stenosis. T h e dramatic history of effort syncope can occur in all three entities. W i t h regard to age of onset, Catton, G u n t h e r o t h , and Reichenbach's ~ t w o - m o n t h - o l d p a t i e n t suggests t h a t age of onset of s y m p t o m s and signs m a y n o t be helpful. W h e n present, a widely split and a c c e n t u a t e d pulmonic c o m p o n e n t of the second h e a r t sound,
American Heart Journal
Fig.7,C.
Postoperative cineangiogram of right ventricle and outflow tract. Absence of the previously described filling defect.
noted by Sakakibara and co-workers '1 and present authors, m a y be the best differentiating physical finding accompanying the systolic murmur. With pulmonic valvular or infundibular stenosis, wide splitting of the second s o u n d is expected, b u t the pulmonic c o m p o n e n t is not accentuated. T h e delayed and a c c e n t u a t e d pulmonic c o m p o n e n t m a y be due to prolonged right ventricular obstruction or right bundle b r a n c h block. T h e pulmonic valve closure m a y be delayed due to prolapse of the t u m o r t h r o u g h the pulmonic valve. T h e a c c e n t u a t i o n of the pulmonic compon e n t m a y have its origin as a result of t u m o r pulm o n a r y emboli and secondary p u l m o n a r y hypertension. An ejection click was noted in only one of the cases of right ventricular m y x o m a , TM and when present usually suggests pulmonic valvular stenosis r a t h e r t h a n m y x o m a . T h e flow m u r m u r s associated with atrial-septal defect, ventricular-septal defect, and idiopathic dilation of the p u l m o n a r y a r t e r y m a y have similar physical findings as a right ventricular myxoma. Patients with atrial-septal defects usually exhibit f i x e d second-sound splitting seldom greater t h a n 80 msec,, and have chest roentgenograms showing increased flow. T h e ven-
245
Snyder et al.
Fig. 8, B. Pulmonary angiogram approximately five weeks postoperative. Essentially unchanged from preoperative study, with failure to demonstrate improved flow to the right upper lobe of the lung.
Fig. 8, A. Preoperative puhnonary angiogram. Filling defect in right upper lobe of lung, considerable peripheral disorganization, particularly in the right lower lobe and pleural thickening in the right costophrenic angle.
T a b l e I. C a r d i a c c a t h e t e r i z a t i o n
Postoperative (8/72)
Preoperative (6/72) Position Right atrium Right ventricle Right ventricular infundibulum Pulmonary artery Left ventricle Aorta Cardiac output (Fick) Cardiac index
Pressure ~ 92/8 58/7 20 35/14 104/7 77 104 / 62 3.51 L./min. 2.17 L./min./M. 2
I
Pressure
02 Saturation 65 60 65 94 93
[
1-0'
20
35/5 35/5 35/10
~-0 115/65 3.57 L./min. 2.20 L./min./M. 2
02 Saturation 60 55 54 96
*= After angiogTam.
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February, 1976, Vol. 91, No. 2
Right ventricular myxoma
Fig. 9, A. Macroscopictumor tissue.
Fig. 9, D. High-powermicroscopetumor tissue.
Fig. 9, B
and C. Low-powermicroscopetumor tissue.
tricular-septal defects usually have similar chest roentgenographic findings to atrial-septal defects and usually electrocardiographic evidence of left ventricular hypertrophy. Calcification in the region of the right ventricular outflow tract that
American Heart Journal
moves with the cardiac cycle is virtually diagnostic of an intracavitary tumor. ~TGa scanning helped to localize and diagnose the obstructive right ventricular problem. Follow-up scanning should be delayed until two-to-six months after surgery because healing tissue may concentrate gallium similarly to tumor tissue. The presence of a pressure gradient across the right ventricular outflow tract and angiographic identification verifies the diagnosis of right ventricular tumor. The management of this lesion should be early surgery to avoid the possibility of sudden death. The review of the literature reveals that 12 out of 13 patients, including ours, survived their surgery. Prognosis following surgical removal of the tumor is excellent, with no recurrences having been noted in the previously described cases. Other tumors of the right ventricle, such as sarcomas, rhabdomyomas, metastases, and endometriosis may clinically simulate myxomas, but do not have as good a surgical prognosis.
Summary The clinical diagnostic features of right ventricular myxoma are described in a recent patient
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Snyder et al.
Table II
RV to PA gradient (mm. Hg)
Year
Au~o~s)
reported Age }Sex
R V angiogram
Method of diagnosis
Surgery IResult
t Kishimoto and Sakaibori 2 Michaud, et al. 3 MacRae and Galea 4 Ernst, et al.
1959
27
F
Unknown
Not done
Autopsy
No
Died
1960 1961 1962
17 21 56
M M F
35 Unknown 51
Filling defect Not done Not done
Yes No Yes
L&W Died L&W
5 Dong, et al.
1962
55
F
77
Filling defect
Catheterization Autopsy Chest roentgenogram and catheterization Catheterization
Yes
6 Gottsegen, et al. 7 Catton, et al.
1963 1963
16 1/6
M M
Not done Surgery "Deformed valve" Surgery
Yes Yes
8 Doohen, et at. 9 Crummy and Hipona 10 Wada, et al. 11 Sakakibara, et al. 12 Byron and Thompson 13 Morrow, et al. 14 Hubbard and Neil
1964 1964 1965 1965 1966 1966 1971
26 21 16 18 39 21 38
M F M F F M F
92 Unknown (RV = 140) 40 61 60 12 18 48 Unknown
Neurological deficit Died Died
Filling defect Filling defect Filling defect Filling defect Not done Filling defect Not done
Yes Yes Yes Yes Yes Yes No
L&W L&W L&W L&W L&W L&W Died
15 Warembourg, et al.
1971
16
F
Filling defect
Catheterization Catheterization Catheterization Catheterization Surgery Catheterization Chest roentgenogram Catheterization
Yes
L&W
16 Present case
1973
36
F
Filling defect
Catheterization
Yes
L&W
8 (isoproterenol) 57
F = female, M = male, L & W = living and well, PA = pulmonary artery, RV = right ventricle. a n d r e l a t e d t o t h e 15 c a s e s p r e v i o u s l y r e p o r t e d . The presence of a pulmonic systolic ejection murm u r w i t h a d e l a y e d (120 t o 140 m s e c . ) a n d a c c e n tuated pulmonic second sound, or calcification in the region of the right ventricular outflow tract should suggest this lesion. Cardiac catheterizat i o n w i t h a n g i o c a r d i o g r a p h y is d i a g n o s t i c . ~TGa s c a n n i n g m a y a s s i s t in t h e d i a g n o s i s a n d f o l l o w up after surgical removal of the myxoma. Early surgical removal will avoid the possibility of sudden death.
REFERENCES 1. Kishimoto, M., and Sakaibori, S.: Primary tumor of the heart: report of a case with myxofibroma of the right ventricle, AM. HEART J. 57:769, 1959. 2. Michaud, P., Saubier, E., Maret, G., et al.: Exerese sous circulation extracorporelle d'un myxome du ventricule droit, Lyon Chit 55:788, 1960. 3. MacRae, F. R., and Galea, M.: Myxoma of the right ventricle of the heart, Canad. Med. Assoc. J. 85:435, 1961. 4. Ernst, R. W., Siebel, E. K., Ogle, J. C., et al.: Successful removal through the main pulmonary artery of a tumor arising from the ventricular septum: a case report, Ann. Surg. 156:54, 1962. 5. Dong, E., Jr., Hurley, E. J., and Shumway, N. E.: Pri-
248
mary cardiac sarcoma, Am. J. Cardiol. 10:871, 1962. 6. Gottsegen, G., Wessely, J., Array, A., et al.: Right ventricular myxoma simulating pulmonic stenosis, Circulation 27:95, 1963. 7. Catton, R. W., Guntheroth, W. E., and Reichenbach, D. D.: A myxoma of the pulmonary valve causing severe stenosis in infancy, AM. HEART J. 66:248, 1963. 8. Doohen, D. J., Greer, J. W., Diorio, N., et al.: Emergency excision of a myxoma of the right ventricle which was obstructing the right ventricular outflow tract, J. Thorac. Cardiovasc. Surg. 47:342, 1964. 9. Crummy, A. B., Jr., and Hipona, F. A.: The radiographic aspects of right ventricular myxoma, Br. J. Radiol. 37:468, 1964. 10. Wada, J., Ikeda, T., Komatsu, S., et al.: Right ventricular myxoma, Ann. Thorac. Surg. 1:84, 1965. 11. Sakakibara, S., Osawa, M., Konno, S., et a|.: Myxoma of the right ventricle of the heart: report of a case with successful removal and review of the literature, AM. HEART J. 69:382, 1965. 12. Byron, F. X., and Thompson, W. P.: Myxoma of the right ventricle, Ann. Thorac. Surg. 2:424, 1966. 13. Morrow, A. G., Kahler, R. L., and Reis, R. L.: Primary myxoma of the, right ventricle, Am. J. Med. 40:954, 1966. 14. Hubbard, T. F., and Nell, R. N.: Myxoma of the right ventricle, AM. HEART J. 81:548, 1971. 15. Warembourg, H., Ducloux, G., Pauchant, M., et al.: Les myxomes du ventricule droit, Arch. Mal. Coeur 11:1557, 1971.
February, 1976, Vol. 91, No. 2
Los Angeles and San Diego, Calif.
Primary intracardiac tumors are uncommon, and those of ventricular origin are the least frequent. The intent of this communication is t o r e p o r t i m portant diagnostic features noted recently in a patient with a right ventricular myxoma and relate these findings to those previously reported in the literature.
Case report This 36-year-old white woman (V. B.) was referred for cardiac consultation as the result of a heart m u r m u r noted on a preemployment physical examination in June, 1972. She had no cardiovascular symptoms at the time of consultation and specifically denied any shortness of breath, chest pain, edema, cough, or hemoptysis. She was treated successfully with antibiotics for uncomplicated "pneumonia" eight months prior to consultation. On review of her hospital records, a nondescript systolic cardiac m u r m u r was noted 12 years previously. At t h a t time the peripheral pulses were normal and the remainder of the examination was not remarkable. At physical examination the patient was a healthy-appearing female with a blood pressure of 104/70 in both arms and normal pulse, respiration, and temperature. A prominent awave was noted in the jugular venous pulse. There was a mild pectus excavatum and a left parasternal lift. The apical impulse was in the fourth left intercostal space at the midclavicular line. The first heart sound was slightly diminished. The second sound was widely split and varied slightly, but physiologically, with respiration. The pulmonic component From the Los Angeles County-University of Southern California Medical Center, Los Angeles, and the United States Naval Hospital, San Diego. Received for publication" Jan. 3, 1974. Reprint requests to: Dr. Stanley N. Snyder, 9209 Colima Rd., Whittier, Calif. 90605. *Cardiology Fellow, LAC-USC Medical Center, Los Angeles, Calif. **Cardiologist, Lieutenant Commander, MC, USNR, United States Naval Hospital, San Diego, Calif. ***Professor of Medicine and Radiology, LAC-USC Medical Center, Los Angeles, Calif. ****Professor of Radiology and Medicine, LAC-USC Medical Center, Los Angeles, Calif.
24(}
was accentuated and audible at the apex. A Grade I I I / V I systolic ejection murmur was heard at the third and fourth left intercostal spaces and became more prominent with inspiration. A fourth heart sound was present at the left sternal border and was unchanged with the phases of respiration. Complete blood count, serum electrolytes, and cardiac enzymes were normal. The chest roentgenogram (Fig. 1) demonstrated a right-sided aortic arch and pleural thickening at the right lateral base. The electrocardiogram (Fig. 2, A) revealed a mean QRS axis of q 90 degrees in the frontal plane and a Pwave axis with leftward and superior orientation, There was an rSr' in Lead V,, and S-waves in the lateral precordial leads. T-wave abnormalities were noted inferiorly and over the right precordium. The Frank lead vectorcardiogram (Fig. 3, A) demonstrated rather prominent posterior and rightward terminal forces. Right ventricular hypertrophy was suggested on both the electrocardiogram and vectorcardiogram. The phonocardiogram and pulse tracings (Figs. 4, A and 4, B) demonstrated a systolic murmur, wide splitting of the second heart sound with slight respiratory variation, and a prominent a-wave in the venous pulse tracing. A radioisotope pulmonary perfusion scan disclosed right upper and lower lobe filling defects. A 48-hour cardiac scan (Fig. 5, A) utilizing 5 mCi. of 67Ga-citrate showed heavy gallium accumulation in the area of the mediastinum in the region of the sternum and extending to the left and cephalad, corresponding to the location of the main pulmonary artery, as demonstrated by the superimposed outline of the cardiomediastinum by a chest roentgenogram done at the same time. Preoperative cardiac catheterization (Table I and Fig. 6) demonstrated a right ventricnlar pressure of 92/8 and a pulmonary artery pressure of 35/14. Cineangiocardiographic studies (Figs. 7, A and 7, B) demonstrated a large, mobile, lobulated filling defect in the right ventricle extending through the pulmonary valve during systole. The selective pulmonary angiogram (Fig. 8, A) showed absence of some vascular branches in the right upper lobe and, to a lesser degree, in the right lower lobe. At surgery the right atrium, left atrium, and right ventricle were enlarged. The left ventricle appeared normal in size. There was a systolic thrill in the right ventricular outflow tract extending into the pulmonary artery. The right ventricle was opened transversely, revealing a gelatinous, irregular mass filling much of the right ventricle and outflow tract. I t was attached by a narrow stalk to the right ventricular wall at
February, 1976, Vol. 91, No. 2, pp. 240-248
Right ventricular myxoma
Fig. 3, A. Preoperative vectorcardiogram. Prominent posterior and rightward forces in the horizontal plane loop and vertical axis in the frontal plane. Fig. 1. Chest x-ray of the right-sided aortic arch. Upper arrow: impression on tracheal air column. Lower arrow: descending aorta to right of sternum. Note: pleural thickening at right costophrenic angle.
Fig. 2, A. Preoperative electrocardiogram. Tendency toward right axis deviation (q 90 degrees), rSr' in right precordial leads, abnormal T-waves in Leads II, IiI, aVF, and right precordial leads, and ectopic atrial focus.
Fig. 2, B. Postoperative electrocardiogram. Axis is q 60 degrees and T-waves upright in Leads II, III, and aV F.
American Heart Journal
Fig. 3, B. Postoperative vectorcardiogram. More normal terminal forces and frontal plane axis.
the junction of the anterior and posterior leaflets of the tricuspid valve. A 36-gram mass (Fig. 9, A) was removed, including a small portion of the endocardium with the stalk, to insure complete removal of the tumor. Histologically (Figs. 9, B, C, and D), the tumor revealed changes consistent with a myxoma. The patient was discharged after an uneventful recovery and readmitted approximately one m o n t h after surgery for reevaluation. Physical examination was normal, and the chest roentgenogram was unchanged. The electrocardiogram (Fig. 2, B) and the Frank vectorcardiogram (Fig. 3, B) now revealed a mean QRS axis of q 60 degrees. The right precordial rSr' and the T-wave abnormalities persisted, but the T-waves in the diaphragmatic leads were now upright. The phonocardiogram and pulse tracings (Figs. 4, C and D) showed no murmur, a decrease of the splitting interval of the second heart sound,
241
S n y d e r et al.
Fig. 4, A and B. Preoperative phonocardiogram and pulse tracings. Long systolic murmur, As - P~ interval 150 msec. A-wave of venous pulse in upper limits of normal. There appears to be an abnormal relationship between P2 and V-wave. A, Paper speed = 100 mm. per second; SM = systolic murmur; E X = expiration; and CP = carotid pulse tracing. B, Paper speed = 100 mm.~ per se~cond and VP = venous pulse. and a normal venous pulse tracing. Cardiac catheterization (Table I) disclosed a right ventricular pressure of 35/5, with no outflow gradient. The right ventricular filling defects were absent (Fig. 7, C). The radioisotope pulmonary Perfusion scan and pulmonary angiograms (Fig. 8, B) were unchanged. Six months postoperatively, a 48-hour cardiac scan (Fig. 5, B), again using 5 mCi. 67Ga-citrate, revealed less gallium uptake in the area where the tumor had been.
Review of 16 cases This is the sixteenth case of right ventricular myxoma in our review of the world literature (Table II). '-15 Except for Catton, Guntheroth, and Reichenbach's7 case, the patients' ages ranged from 16 to 56, most being young adults at the time of diagnosis. There was slight predominance of females. Symptoms or signs antedated diagnosis by as much as 14 years. Only two patients were without complaint. Fever, shortness of breath, chest pain, cyanosis, syncope, seizures, and sudden death have been described. In two cases, pain was exertion~ al, 3"" and exertional syncope was noted in another patient. 1~
242
In one instance, episodes of positional palpitation were caused by ventricular tachycardia. TM The most common physical finding (14 out of 16 p a t i e n t s ) w a s a Grade III to IV/VI systolic murmur along the left sternal border, and of maximal intensity in the second or third intercostal space. Some authors described changes in intensity or location of the murmur from time to time. In one instance, these changes occurred after cardiac catheterization. 11 Some were associated with a palpable thrill, and a peculiar feeling of movement or a "worm-like" thrill has been described. A right ventricular lift was noted occasionally. In our case, and in one other, ~ the pulmonic component of the second sound was separated from the aortic component by an interval of 100 to 130 msec. In most reports, no information was available with regard to the second heart sound. To-and-fro scratches and clicks simulating pericarditis" have been described with right ventricular myxoma, and a diastolic murmur 7 was noted in one patient. Several patients had elevated jugular venous pressure and prominent a-waves. Two patients presented with fever and three patients were noted to be cyanotic. Electrocardiographic abnormalities included right axis devi-
February, 1976, Vol. 91, No. 2
R i g h t ventricular myxoma
Fig. 4, C and D. Postoperative phonocardiogram and pulse tracings. Murmur is now absent, A2 - P~ relationship is normal, A-wave is less prominent, and there is restoration of the normal relationship between P2 and the V-wave peak. C, Paper speed = 100 mm. per second; ECG = Lead II electrocardiogram; Apex PCG = phonocardi0gram, apex; 2 LICS PCG = phonocardiogram, second left interspace parasternal line; CPT = carotid pulse tracingi and DC = dicrotic notch. D, Paper speed = 100 mm. per second; ECG = Lead II electrocardiogram; 4 LICS phonocardiogram, fourth left interspace, parasternal line; and VFT = venous pulse tracing. PCG =
ation (eight patients), the so-called "incomplete right bundle branch block pattern" (five patients), complete right bundle branch block (three patients), right ventricular hypertrophy (five patients), and right atrial enlargement (two patients). Abnormal T-waves in the inferior a n d / o r right precordial leads were also noted (six patients). Three chest roentgenograms were normal, four disclosed enlargement of the main pulmonary artery, and three were virtually diagnostic of intracardiac tumor by the presence of calcification in the area of the right ventricular outflow tract, x"" " ~ Right ventricular enlargement was noted in two patients. Decreased pulmonary vascularity, enlarged main pulmonary artery, gross cardiomegaly, and right atrial enlargement were each noted in one case. Increased pulmonary vascularity was present in a patient who had, in addition, an atrial-septal defect. Our case was normal roentgenographically, except for a right-sided aortic arch and right costophrenic pleural thickening. At cardiac catheterization , significant right ventricle-topulmonary artery gradients were present in most patients. Occasionally, as in our patient, an infundibular chamber was simulated2 .... The right ventricular pressure was over 60 mm. Hg in the majority of patients, with a range of 44 to 140 mm. Hg. Cineangiocardiography was diagnostic with the demon-
A m e r i c a n Heart Journal
stration of filling defects in the right ventricle or its outflow tract. Diagnosis prior to surgery ~, 7:12 was not made in the absence of right ventricular calcification or cineangiocardiography. In one of these cases/~ a coincidental atriaI-septal defect was correctly diagnosed, b u t the tumor went undetected. Eleven out of thirteen patients operated upon survived, one patient having residual symptoms. In two patients, diagnosis was made at autopsy. The tumors were attached by single or multiple pedicles, most frequently to the right ventricular septum or free wail, but other sites of origin included the conus, the chordae, the pulmonary artery, and also the ventricular surface of the tricuspid or pulmonic valves. The tumors were described pathologically as myxoma tissue alone, except for four instances in which additional tissue origins were noted.'. ' 3 ,~
Discussion Fourteen out of Sixteen patients had murmurs at the left sternal border that suggested right ventricular outflow obstruction. The physical findings associated with right ventricular myxoma strongly resemble those of pulmonic valvular
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Snyder et al.
Fig. 5, A. Preoperative cardiac scan, anterior and posterior. Heavy concentration of gallium in the mid-chest area extending leftward and cephalad from right ventricle toward the main pulmonary artery,
Fig. 5, B. Postoperative cardiac scan, anterior and posterior. Considerable decrease in concentration of gallium in the mid-chest region, and the extension toward the pulmonary artery is now absent.
Fig. 6. Preoperative pressure tracing. During cardiac catheterization, with pullback from main, pulmonary artery to infundibulum to right ventricle demonstrating pressure gradients.
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February, 1976, Vol. 91, No. 2
Right ventricular myxoma
Fig. 7, A. Preoperative cineangiography of right ventricle and outflow tract. Large, mobile filling defect occupying portions of the right ventricle and extending through the pulmonary valve (during systole).
~~
PulmonaryArtery
$~
~
~(vlYXOMA ~ ~ ( ~
.'-~-~Right Ventricle TricuspidValve Catheter
f --
Diaphragm
Fig. 7, B. Diagrammatic illustration of filling defect noted in
Fig. 7A. or infundibular stenosis. T h e dramatic history of effort syncope can occur in all three entities. W i t h regard to age of onset, Catton, G u n t h e r o t h , and Reichenbach's ~ t w o - m o n t h - o l d p a t i e n t suggests t h a t age of onset of s y m p t o m s and signs m a y n o t be helpful. W h e n present, a widely split and a c c e n t u a t e d pulmonic c o m p o n e n t of the second h e a r t sound,
American Heart Journal
Fig.7,C.
Postoperative cineangiogram of right ventricle and outflow tract. Absence of the previously described filling defect.
noted by Sakakibara and co-workers '1 and present authors, m a y be the best differentiating physical finding accompanying the systolic murmur. With pulmonic valvular or infundibular stenosis, wide splitting of the second s o u n d is expected, b u t the pulmonic c o m p o n e n t is not accentuated. T h e delayed and a c c e n t u a t e d pulmonic c o m p o n e n t m a y be due to prolonged right ventricular obstruction or right bundle b r a n c h block. T h e pulmonic valve closure m a y be delayed due to prolapse of the t u m o r t h r o u g h the pulmonic valve. T h e a c c e n t u a t i o n of the pulmonic compon e n t m a y have its origin as a result of t u m o r pulm o n a r y emboli and secondary p u l m o n a r y hypertension. An ejection click was noted in only one of the cases of right ventricular m y x o m a , TM and when present usually suggests pulmonic valvular stenosis r a t h e r t h a n m y x o m a . T h e flow m u r m u r s associated with atrial-septal defect, ventricular-septal defect, and idiopathic dilation of the p u l m o n a r y a r t e r y m a y have similar physical findings as a right ventricular myxoma. Patients with atrial-septal defects usually exhibit f i x e d second-sound splitting seldom greater t h a n 80 msec,, and have chest roentgenograms showing increased flow. T h e ven-
245
Snyder et al.
Fig. 8, B. Pulmonary angiogram approximately five weeks postoperative. Essentially unchanged from preoperative study, with failure to demonstrate improved flow to the right upper lobe of the lung.
Fig. 8, A. Preoperative puhnonary angiogram. Filling defect in right upper lobe of lung, considerable peripheral disorganization, particularly in the right lower lobe and pleural thickening in the right costophrenic angle.
T a b l e I. C a r d i a c c a t h e t e r i z a t i o n
Postoperative (8/72)
Preoperative (6/72) Position Right atrium Right ventricle Right ventricular infundibulum Pulmonary artery Left ventricle Aorta Cardiac output (Fick) Cardiac index
Pressure ~ 92/8 58/7 20 35/14 104/7 77 104 / 62 3.51 L./min. 2.17 L./min./M. 2
I
Pressure
02 Saturation 65 60 65 94 93
[
1-0'
20
35/5 35/5 35/10
~-0 115/65 3.57 L./min. 2.20 L./min./M. 2
02 Saturation 60 55 54 96
*= After angiogTam.
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February, 1976, Vol. 91, No. 2
Right ventricular myxoma
Fig. 9, A. Macroscopictumor tissue.
Fig. 9, D. High-powermicroscopetumor tissue.
Fig. 9, B
and C. Low-powermicroscopetumor tissue.
tricular-septal defects usually have similar chest roentgenographic findings to atrial-septal defects and usually electrocardiographic evidence of left ventricular hypertrophy. Calcification in the region of the right ventricular outflow tract that
American Heart Journal
moves with the cardiac cycle is virtually diagnostic of an intracavitary tumor. ~TGa scanning helped to localize and diagnose the obstructive right ventricular problem. Follow-up scanning should be delayed until two-to-six months after surgery because healing tissue may concentrate gallium similarly to tumor tissue. The presence of a pressure gradient across the right ventricular outflow tract and angiographic identification verifies the diagnosis of right ventricular tumor. The management of this lesion should be early surgery to avoid the possibility of sudden death. The review of the literature reveals that 12 out of 13 patients, including ours, survived their surgery. Prognosis following surgical removal of the tumor is excellent, with no recurrences having been noted in the previously described cases. Other tumors of the right ventricle, such as sarcomas, rhabdomyomas, metastases, and endometriosis may clinically simulate myxomas, but do not have as good a surgical prognosis.
Summary The clinical diagnostic features of right ventricular myxoma are described in a recent patient
247
Snyder et al.
Table II
RV to PA gradient (mm. Hg)
Year
Au~o~s)
reported Age }Sex
R V angiogram
Method of diagnosis
Surgery IResult
t Kishimoto and Sakaibori 2 Michaud, et al. 3 MacRae and Galea 4 Ernst, et al.
1959
27
F
Unknown
Not done
Autopsy
No
Died
1960 1961 1962
17 21 56
M M F
35 Unknown 51
Filling defect Not done Not done
Yes No Yes
L&W Died L&W
5 Dong, et al.
1962
55
F
77
Filling defect
Catheterization Autopsy Chest roentgenogram and catheterization Catheterization
Yes
6 Gottsegen, et al. 7 Catton, et al.
1963 1963
16 1/6
M M
Not done Surgery "Deformed valve" Surgery
Yes Yes
8 Doohen, et at. 9 Crummy and Hipona 10 Wada, et al. 11 Sakakibara, et al. 12 Byron and Thompson 13 Morrow, et al. 14 Hubbard and Neil
1964 1964 1965 1965 1966 1966 1971
26 21 16 18 39 21 38
M F M F F M F
92 Unknown (RV = 140) 40 61 60 12 18 48 Unknown
Neurological deficit Died Died
Filling defect Filling defect Filling defect Filling defect Not done Filling defect Not done
Yes Yes Yes Yes Yes Yes No
L&W L&W L&W L&W L&W L&W Died
15 Warembourg, et al.
1971
16
F
Filling defect
Catheterization Catheterization Catheterization Catheterization Surgery Catheterization Chest roentgenogram Catheterization
Yes
L&W
16 Present case
1973
36
F
Filling defect
Catheterization
Yes
L&W
8 (isoproterenol) 57
F = female, M = male, L & W = living and well, PA = pulmonary artery, RV = right ventricle. a n d r e l a t e d t o t h e 15 c a s e s p r e v i o u s l y r e p o r t e d . The presence of a pulmonic systolic ejection murm u r w i t h a d e l a y e d (120 t o 140 m s e c . ) a n d a c c e n tuated pulmonic second sound, or calcification in the region of the right ventricular outflow tract should suggest this lesion. Cardiac catheterizat i o n w i t h a n g i o c a r d i o g r a p h y is d i a g n o s t i c . ~TGa s c a n n i n g m a y a s s i s t in t h e d i a g n o s i s a n d f o l l o w up after surgical removal of the myxoma. Early surgical removal will avoid the possibility of sudden death.
REFERENCES 1. Kishimoto, M., and Sakaibori, S.: Primary tumor of the heart: report of a case with myxofibroma of the right ventricle, AM. HEART J. 57:769, 1959. 2. Michaud, P., Saubier, E., Maret, G., et al.: Exerese sous circulation extracorporelle d'un myxome du ventricule droit, Lyon Chit 55:788, 1960. 3. MacRae, F. R., and Galea, M.: Myxoma of the right ventricle of the heart, Canad. Med. Assoc. J. 85:435, 1961. 4. Ernst, R. W., Siebel, E. K., Ogle, J. C., et al.: Successful removal through the main pulmonary artery of a tumor arising from the ventricular septum: a case report, Ann. Surg. 156:54, 1962. 5. Dong, E., Jr., Hurley, E. J., and Shumway, N. E.: Pri-
248
mary cardiac sarcoma, Am. J. Cardiol. 10:871, 1962. 6. Gottsegen, G., Wessely, J., Array, A., et al.: Right ventricular myxoma simulating pulmonic stenosis, Circulation 27:95, 1963. 7. Catton, R. W., Guntheroth, W. E., and Reichenbach, D. D.: A myxoma of the pulmonary valve causing severe stenosis in infancy, AM. HEART J. 66:248, 1963. 8. Doohen, D. J., Greer, J. W., Diorio, N., et al.: Emergency excision of a myxoma of the right ventricle which was obstructing the right ventricular outflow tract, J. Thorac. Cardiovasc. Surg. 47:342, 1964. 9. Crummy, A. B., Jr., and Hipona, F. A.: The radiographic aspects of right ventricular myxoma, Br. J. Radiol. 37:468, 1964. 10. Wada, J., Ikeda, T., Komatsu, S., et al.: Right ventricular myxoma, Ann. Thorac. Surg. 1:84, 1965. 11. Sakakibara, S., Osawa, M., Konno, S., et a|.: Myxoma of the right ventricle of the heart: report of a case with successful removal and review of the literature, AM. HEART J. 69:382, 1965. 12. Byron, F. X., and Thompson, W. P.: Myxoma of the right ventricle, Ann. Thorac. Surg. 2:424, 1966. 13. Morrow, A. G., Kahler, R. L., and Reis, R. L.: Primary myxoma of the, right ventricle, Am. J. Med. 40:954, 1966. 14. Hubbard, T. F., and Nell, R. N.: Myxoma of the right ventricle, AM. HEART J. 81:548, 1971. 15. Warembourg, H., Ducloux, G., Pauchant, M., et al.: Les myxomes du ventricule droit, Arch. Mal. Coeur 11:1557, 1971.
February, 1976, Vol. 91, No. 2
