- Email: [email protected]
Right atrial mass with attachment to the atrial septum caused by metastatic renal cell carcinoma
November 1995
1126
S i v a r a m et al.
report is the first description of coronary artery fistulas acquired after operation i n children. I n all three patients v e n t r i c u l a r septal muscle was removed either from the right or left ventricular side of the septum. E x a m i n a t i o n at the site where myocardium was excised demonstrated a jet on Doppler color flow mapping. I n one p a t i e n t two jets were present. I n each case the source of the jet could be traced to septal branches of the left coronary artery. The children did not have clinical signs of a coronary fistula, and the coronary artery itself was not enlarged. This finding suggests t h a t the leak through the fistula was small. However, a future increase of flow through the fistula with enlargem e n t of the communicating coronary a r t e r y cannot be ruled out. Because the patients r e m a i n without symptoms a n d the fistulous communications appear to be very small, we have not considered surgical closure. S a n d h u et al.1 observed spontaneous closure of acquired coronary artery fistulas after endomyocardial biopsy in 3 of 14 patients, a n d other reports have described spontaneous or nearcomplete closure, s-l° F u r t h e r follow-up will be necessary to decide on t r e a t m e n t . I n summary, Doppler color flow m a p p i n g is a very sensitive noninvasive tool to detect flow through very small coronary artery fistulas even in the absence of clinical signs. Acquired coronary artery fistulas after surgery m a y occur more frequently t h a n previously described. A prospective study after surgical resection of myocardium will be necessary to show the frequency of coronary artery fistulas acquired after surgery.
REFERENCES
1. Sandhu JS, Uretsky BF, ZerbeTR, GoldsmithAS, ReddyPS, Kormos RL, Griffith BP, Hardesty RL. Coronary artery fistula in the heart transplant patient: a potentialcomplicationof endomyocardialbiopsy. Circulation1989;79:350-6. 2. Lee RT, Mudge GH, ColucciWS. Coronaryartery fistula al~er mitral valve surgery. AMHEARTJ 1988;115:1128-30. 3. ChenzbraunA, Pinto FJ, MeyerB, StinsonEB, PoppRL. Frequencyof acquiredcoronary-cameralfistulaal~erventricularseptal myectomyin hypertrophic cardiomyopathy.Am J Cardiol 1993;71:1244-6. 4. Barton CW, Snider AR, RosenthalA. Two-dimensionaland Doppler echocardiographic features of lel~ circumflexcoronaryartery to right ventricle fistula: case report and literature review. Pediatr Cardiol 1986;7:167-70. 5. VelvisH, SchmidtKG, SilvermanNH, TurleyK. Diagnosisofcoronary artery fistula by two-dimensionalechocardiography, pulsed Doppler ultrasound and colorflowimaging.J Am Co]]Cardiol 1989;14:968-76. 6. Nishiguchi T, Matsuoka Y, Sennari E, Oklshima T, Suzumiya H, AkimotoK, Takamura K, KawaguchiK, TashiroS, Yamasaki S, Hayakawa K. Congenitalcoronaryartery fistula:diagnosisby two-dimensional Doppler echocardiography. AMHEARTJ 1990;120:1244-8. 7. ZahuEM, SmallhornJF, Egger G, BurrowsPE, RebeccaIM, Freedom RM. Echocardiographicdiagnosisoffistulabetweenthe lei~circumflex coronary artery and the lei~atrium. Pediatr Cardiol 1992;13:178-80. 8. FarookiZQ, NowlenT, Hakimi M, Pinsky WW. Congenitalcoronary artery fistulae:a review of 18 cases with special emphasis on spontaneous closure. Pediatr Cardiol 1993;14:208-13. 9. MahoneyLT, SchiekenRM, Lauer RM. Spontaneousclosure of a coronary artery fistula in childhood.Pediatr Cardiol 1982;2:311-2. 10. GriffithsSP, Ellis K, HordofAJ, Martin E, LevineOR, GersonyWM. Spontaneouscompleteclosure of a congenitalcoronaryartery fistula. J Am Coll Cardiol 1983;2:1169-73.
American Heart Journal
Right atrial mass with attachment to the atrial septum caused by metastatic renal cell carcinoma C h i t t u r A. Sivaram, MD, Terrance Khastgir, MD, Sharon L. S a n e m a n , BSRT, RDCS, and Ronald C. Elkins, MD Oklahoma City, Okla.
I n t r a c a v i t a r y masses in the right a t r i u m (RA) m a y be benign or malignant. Myxoma is the most common benign atrial mass, and a t t a c h m e n t to the atrial septum is seen i n 85% of cases with myxoma. 1, 2 M a l i g n a n t tumors, which m a y be either p r i m a r y or metastatic, are more commonly seen in the right a t r i u m t h a n i n the left. Metastatic RA masses arise from p r i m a r y tumors i n the lung, breast, esophagus, kidney, or testes or from p r i m a r y m e l a n o m a or lymphoma. 2 Metastatic atrial masses are u s u a l l y attached to the atrial wall r a t h e r t h a n the atrial septum. 2 Often atrial masses occur in renal cell carcinoma from transvenous extension of the t u m o r via the renal vein a n d inferior v e n a cava (IVC). An u n u s u a l case of right atrial mass caused by metastatic disease from renal cell carcinoma diagnosed by transesophageal echocardiography (TEE) is described here. The mass showed a t t a c h m e n t to the atrial septum n e a r the coronary sinus, b u t the t u m o r did not extend from the IVC into the RA. A 64-year-old white m a n was hospitalized because of dizziness and n e a r syncope while sitting or standing; there were no premonitory symptoms. These episodes were not associated with palpitation, chest pain, or shortness of breath b u t were often precipitated by change of posture from r e c u m b e n t to Upright position. The patient's medical history was remarkable for r e n a l cell carcinoma diagnosed i n 1990 for which he had undergone left nephrectomy. I n 1991 he had wedge resection of the left lung for metastatic lesion from hypernephroma. Subsequently he received interleukin-2 immunotherapy. He was not currently t a k i n g a n y medication t h a t could have caused his symptoms. On examination, the patient's lying blood pressure a n d pulse were 105/60 m m Hg a n d 80 beats/min, respectively, which changed to 84/60 m m Hg a n d 60 beats/min, respectively, when the p a t i e n t sat upright. Skin turgor was normal, and mucous m e m b r a n e s were moist. Cardiovascular examination showed n o t h i n g r e m a r k a b l e a n d no signs of congestive h e a r t failure. His hematocrit, s e r u m electrolyte, renal function, and u r i n a r y electrolyte levels were w i t h i n normal
From the Divisions of Cardiology and Cardiothoracic Surgery, University of Oklahoma Health Sciences Center. Reprint requests: Chittur A. Sivaram, MD, OU Health Sciences Center, 920 Stanton L. Young (5SP300), Oklahoma City, OK 73104-5020. AM HEARTJ 1995;130:1126-7. Copyright © 1995 by Mosby-Year Book, Inc. 0002-8703/95/$5.00 + 0 4/4/66526
Volume 130, Number 5 American Heart Journal
Fig. 1. TEE in t r a n s v e r s e p l a n e shows t u m o r m a s s in right a t r i u m with a t t a c h m e n t to a t r i a l septum. RV, Right ventricle; LA, left atrium; T, tumor.
Fig. 2. TEE shows t u m o r m a s s in right a t r i u m with no involvement of inferior vena cava. T, Tumor. range. Transthoracic echocardiographic e x a m i n a t i o n during t h e work-up for n e a r syncope showed a small pericardial effusion a n d a m a s s in t h e RA close to the tricuspid valve. The tricuspid inflow Doppler velocities were increased at 0.9 m/sec, suggesting obstruction to diastolic filling. Subsequently the p a t i e n t u n d e r w e n t TEE for better delineation of this RA mass. TEE revealed t h a t a large 6 x 5 cm lobulated m a s s in the RA was a t t a c h e d to the lower a t r i a l s e p t u m adjacent to coronary sinus (Fig. 1). No prolapse of the m a s s into the tricuspid orifice was seen even though t h e r e was significant encroachment of RA cavity by the mass. The proximal IVC did not reveal a n y m a s s extending from below (Fig. 2). The p a t i e n t u n d e r w e n t t u m o r resection u n d e r c a r d i o p u l m 0 n a r y bypass. The m a s s was resected from the lower a t r i a l s e p t u m n e a r the ostium of the coronary sinus, a n d the a t r i a l s e p t u m was r e p a i r e d
Sivaram et al.
1127
with a pericardial patch without difficulty. Histopathologic e x a m i n a t i o n confirmed r e n a l cell carcinoma of the clearcell type. The postoperative period was uneventful, and the p a t i e n t h a d no recurrence of dizziness and n e a r syncope during the follow-up examination. The u n u s u a l features of the p r e s e n t case of RA m a s s h y p e r n e p h r o m a are the a t t a c h m e n t of the m e t a s t a t i c m a s s to the a t r i a l septum, the absence of t u m o r extension into the RA from below via the IVC, and n e a r syncope. H y p e r n e p h r o m a extends to the IVC in 5% to 10% of patients, a n d propagation m a y r e s u l t in RA mass. 3 M e t a s t a t i c disease within the RA in hypern e p h r o m a is almost i n v a r i a b l y the result of intravenous extension of viable t u m o r m a s s and benign t h r o m b u s via the IVC. 4 Absence o f v e n a caval involvement in RA metastatic m a s s e s caused by h y p e r n e p h r o m a h a s been occasionally described. Gindea et al. 5 described a similar example discovered by magnetic resonance imaging. Right a t r i a l m e t a s t a t i c lesions can often be asymptomatic; however, p u l m o n a r y emboli, i n t e r m i t t e n t tricuspid valve obstruction, right-sided congestive h e a r t failure, and a t r i a l a r r h y t h m i a s have been reported. 4 Fogel et al. 6 reported a case of m e t a s t a t i c carcinoma to the left a t r i u m t h a t manifested as a p r i m a r y cause of syncope. They postulated t h a t obstruction to the left a t r i a l outflow, as well as bradycardia, resulted in an a b r u p t decrease in cardiac output t h a t lead to m a r k e d hypotension and syncope. It is likely t h a t with u p r i g h t posture the large m a s s in this p a t i e n t could prolapse into the tricuspid orifice, causing significant drop in cardiac o u t p u t t h a t would lead to n e a r syncope. Atrial m a s s e s with a t t a c h m e n t to the a t r i a l s e p t u m most often r e p r e s e n t myxoma. M a l i g n a n t m a s s e s are more likely to have a t t a c h m e n t to the a t r i a l wall. B r y a n t and Vuckovic 7 have described an autopsy in which a t u m o r nodule was seen a t t a c h e d to the a t r i a l s e p t u m in addition to another RA m a s s as a result of IVC extension in a p a t i e n t with hypernephroma. To our knowledge, a case of m e t a s t a t i c RA m a s s a t t a c h e d to the a t r i a l s e p t u m without t u m o r extension from below v i a IVC has not previously been published in h y p e r n e p h r o m a .
REFERENCES
1. Wold LE, Lie JT. Cardiac myxomas: a clinicopathologic profile. Am J Pathol 1980;101:219-40. 2. Panidis IP, Kotler MN, Mintz GS, Ross J. Clinical and echocardiographic features of right atrial masses. AM HEARTJ 1984;107:745-58. 3. Shahier DM, Libertiao JA, Zinman LN, Leonardi HK, Eyre RC. Resection of cavoatrial renal cell carcinoma employing total circulatory arrest. Arch Surg 1990;125:727-32. 4. Bissada NK, Finkbeiner AE, Williams GD, Weiss JB. Successful extraction of intracardiac tumor thrombus of renal carcinoma. J Urol 1977;118:474-5. 5. Gindea AJ, Benjamin G, Naidich DP, Freedberg RS, McCauley D, Kronzon I. Unusual cardiac metastasis in hypernephroma: the complementary role of echocardiography and magnetic resonance imaging. AM HEARTJ 1988;116:1359-61. 6. Fogel RI, Balady GJ, Klein MD, KhorasanJ AR. Metastatic renal cell carcinoma. An unusual cause of syncope. Chest 1990;98:481-2. 7. Bryant J, Vuckovic G. Metastatic tumors of the endocardiam. Arch Pathol Lab Med 1978;102:206-8.
1126
S i v a r a m et al.
report is the first description of coronary artery fistulas acquired after operation i n children. I n all three patients v e n t r i c u l a r septal muscle was removed either from the right or left ventricular side of the septum. E x a m i n a t i o n at the site where myocardium was excised demonstrated a jet on Doppler color flow mapping. I n one p a t i e n t two jets were present. I n each case the source of the jet could be traced to septal branches of the left coronary artery. The children did not have clinical signs of a coronary fistula, and the coronary artery itself was not enlarged. This finding suggests t h a t the leak through the fistula was small. However, a future increase of flow through the fistula with enlargem e n t of the communicating coronary a r t e r y cannot be ruled out. Because the patients r e m a i n without symptoms a n d the fistulous communications appear to be very small, we have not considered surgical closure. S a n d h u et al.1 observed spontaneous closure of acquired coronary artery fistulas after endomyocardial biopsy in 3 of 14 patients, a n d other reports have described spontaneous or nearcomplete closure, s-l° F u r t h e r follow-up will be necessary to decide on t r e a t m e n t . I n summary, Doppler color flow m a p p i n g is a very sensitive noninvasive tool to detect flow through very small coronary artery fistulas even in the absence of clinical signs. Acquired coronary artery fistulas after surgery m a y occur more frequently t h a n previously described. A prospective study after surgical resection of myocardium will be necessary to show the frequency of coronary artery fistulas acquired after surgery.
REFERENCES
1. Sandhu JS, Uretsky BF, ZerbeTR, GoldsmithAS, ReddyPS, Kormos RL, Griffith BP, Hardesty RL. Coronary artery fistula in the heart transplant patient: a potentialcomplicationof endomyocardialbiopsy. Circulation1989;79:350-6. 2. Lee RT, Mudge GH, ColucciWS. Coronaryartery fistula al~er mitral valve surgery. AMHEARTJ 1988;115:1128-30. 3. ChenzbraunA, Pinto FJ, MeyerB, StinsonEB, PoppRL. Frequencyof acquiredcoronary-cameralfistulaal~erventricularseptal myectomyin hypertrophic cardiomyopathy.Am J Cardiol 1993;71:1244-6. 4. Barton CW, Snider AR, RosenthalA. Two-dimensionaland Doppler echocardiographic features of lel~ circumflexcoronaryartery to right ventricle fistula: case report and literature review. Pediatr Cardiol 1986;7:167-70. 5. VelvisH, SchmidtKG, SilvermanNH, TurleyK. Diagnosisofcoronary artery fistula by two-dimensionalechocardiography, pulsed Doppler ultrasound and colorflowimaging.J Am Co]]Cardiol 1989;14:968-76. 6. Nishiguchi T, Matsuoka Y, Sennari E, Oklshima T, Suzumiya H, AkimotoK, Takamura K, KawaguchiK, TashiroS, Yamasaki S, Hayakawa K. Congenitalcoronaryartery fistula:diagnosisby two-dimensional Doppler echocardiography. AMHEARTJ 1990;120:1244-8. 7. ZahuEM, SmallhornJF, Egger G, BurrowsPE, RebeccaIM, Freedom RM. Echocardiographicdiagnosisoffistulabetweenthe lei~circumflex coronary artery and the lei~atrium. Pediatr Cardiol 1992;13:178-80. 8. FarookiZQ, NowlenT, Hakimi M, Pinsky WW. Congenitalcoronary artery fistulae:a review of 18 cases with special emphasis on spontaneous closure. Pediatr Cardiol 1993;14:208-13. 9. MahoneyLT, SchiekenRM, Lauer RM. Spontaneousclosure of a coronary artery fistula in childhood.Pediatr Cardiol 1982;2:311-2. 10. GriffithsSP, Ellis K, HordofAJ, Martin E, LevineOR, GersonyWM. Spontaneouscompleteclosure of a congenitalcoronaryartery fistula. J Am Coll Cardiol 1983;2:1169-73.
American Heart Journal
Right atrial mass with attachment to the atrial septum caused by metastatic renal cell carcinoma C h i t t u r A. Sivaram, MD, Terrance Khastgir, MD, Sharon L. S a n e m a n , BSRT, RDCS, and Ronald C. Elkins, MD Oklahoma City, Okla.
I n t r a c a v i t a r y masses in the right a t r i u m (RA) m a y be benign or malignant. Myxoma is the most common benign atrial mass, and a t t a c h m e n t to the atrial septum is seen i n 85% of cases with myxoma. 1, 2 M a l i g n a n t tumors, which m a y be either p r i m a r y or metastatic, are more commonly seen in the right a t r i u m t h a n i n the left. Metastatic RA masses arise from p r i m a r y tumors i n the lung, breast, esophagus, kidney, or testes or from p r i m a r y m e l a n o m a or lymphoma. 2 Metastatic atrial masses are u s u a l l y attached to the atrial wall r a t h e r t h a n the atrial septum. 2 Often atrial masses occur in renal cell carcinoma from transvenous extension of the t u m o r via the renal vein a n d inferior v e n a cava (IVC). An u n u s u a l case of right atrial mass caused by metastatic disease from renal cell carcinoma diagnosed by transesophageal echocardiography (TEE) is described here. The mass showed a t t a c h m e n t to the atrial septum n e a r the coronary sinus, b u t the t u m o r did not extend from the IVC into the RA. A 64-year-old white m a n was hospitalized because of dizziness and n e a r syncope while sitting or standing; there were no premonitory symptoms. These episodes were not associated with palpitation, chest pain, or shortness of breath b u t were often precipitated by change of posture from r e c u m b e n t to Upright position. The patient's medical history was remarkable for r e n a l cell carcinoma diagnosed i n 1990 for which he had undergone left nephrectomy. I n 1991 he had wedge resection of the left lung for metastatic lesion from hypernephroma. Subsequently he received interleukin-2 immunotherapy. He was not currently t a k i n g a n y medication t h a t could have caused his symptoms. On examination, the patient's lying blood pressure a n d pulse were 105/60 m m Hg a n d 80 beats/min, respectively, which changed to 84/60 m m Hg a n d 60 beats/min, respectively, when the p a t i e n t sat upright. Skin turgor was normal, and mucous m e m b r a n e s were moist. Cardiovascular examination showed n o t h i n g r e m a r k a b l e a n d no signs of congestive h e a r t failure. His hematocrit, s e r u m electrolyte, renal function, and u r i n a r y electrolyte levels were w i t h i n normal
From the Divisions of Cardiology and Cardiothoracic Surgery, University of Oklahoma Health Sciences Center. Reprint requests: Chittur A. Sivaram, MD, OU Health Sciences Center, 920 Stanton L. Young (5SP300), Oklahoma City, OK 73104-5020. AM HEARTJ 1995;130:1126-7. Copyright © 1995 by Mosby-Year Book, Inc. 0002-8703/95/$5.00 + 0 4/4/66526
Volume 130, Number 5 American Heart Journal
Fig. 1. TEE in t r a n s v e r s e p l a n e shows t u m o r m a s s in right a t r i u m with a t t a c h m e n t to a t r i a l septum. RV, Right ventricle; LA, left atrium; T, tumor.
Fig. 2. TEE shows t u m o r m a s s in right a t r i u m with no involvement of inferior vena cava. T, Tumor. range. Transthoracic echocardiographic e x a m i n a t i o n during t h e work-up for n e a r syncope showed a small pericardial effusion a n d a m a s s in t h e RA close to the tricuspid valve. The tricuspid inflow Doppler velocities were increased at 0.9 m/sec, suggesting obstruction to diastolic filling. Subsequently the p a t i e n t u n d e r w e n t TEE for better delineation of this RA mass. TEE revealed t h a t a large 6 x 5 cm lobulated m a s s in the RA was a t t a c h e d to the lower a t r i a l s e p t u m adjacent to coronary sinus (Fig. 1). No prolapse of the m a s s into the tricuspid orifice was seen even though t h e r e was significant encroachment of RA cavity by the mass. The proximal IVC did not reveal a n y m a s s extending from below (Fig. 2). The p a t i e n t u n d e r w e n t t u m o r resection u n d e r c a r d i o p u l m 0 n a r y bypass. The m a s s was resected from the lower a t r i a l s e p t u m n e a r the ostium of the coronary sinus, a n d the a t r i a l s e p t u m was r e p a i r e d
Sivaram et al.
1127
with a pericardial patch without difficulty. Histopathologic e x a m i n a t i o n confirmed r e n a l cell carcinoma of the clearcell type. The postoperative period was uneventful, and the p a t i e n t h a d no recurrence of dizziness and n e a r syncope during the follow-up examination. The u n u s u a l features of the p r e s e n t case of RA m a s s h y p e r n e p h r o m a are the a t t a c h m e n t of the m e t a s t a t i c m a s s to the a t r i a l septum, the absence of t u m o r extension into the RA from below via the IVC, and n e a r syncope. H y p e r n e p h r o m a extends to the IVC in 5% to 10% of patients, a n d propagation m a y r e s u l t in RA mass. 3 M e t a s t a t i c disease within the RA in hypern e p h r o m a is almost i n v a r i a b l y the result of intravenous extension of viable t u m o r m a s s and benign t h r o m b u s via the IVC. 4 Absence o f v e n a caval involvement in RA metastatic m a s s e s caused by h y p e r n e p h r o m a h a s been occasionally described. Gindea et al. 5 described a similar example discovered by magnetic resonance imaging. Right a t r i a l m e t a s t a t i c lesions can often be asymptomatic; however, p u l m o n a r y emboli, i n t e r m i t t e n t tricuspid valve obstruction, right-sided congestive h e a r t failure, and a t r i a l a r r h y t h m i a s have been reported. 4 Fogel et al. 6 reported a case of m e t a s t a t i c carcinoma to the left a t r i u m t h a t manifested as a p r i m a r y cause of syncope. They postulated t h a t obstruction to the left a t r i a l outflow, as well as bradycardia, resulted in an a b r u p t decrease in cardiac output t h a t lead to m a r k e d hypotension and syncope. It is likely t h a t with u p r i g h t posture the large m a s s in this p a t i e n t could prolapse into the tricuspid orifice, causing significant drop in cardiac o u t p u t t h a t would lead to n e a r syncope. Atrial m a s s e s with a t t a c h m e n t to the a t r i a l s e p t u m most often r e p r e s e n t myxoma. M a l i g n a n t m a s s e s are more likely to have a t t a c h m e n t to the a t r i a l wall. B r y a n t and Vuckovic 7 have described an autopsy in which a t u m o r nodule was seen a t t a c h e d to the a t r i a l s e p t u m in addition to another RA m a s s as a result of IVC extension in a p a t i e n t with hypernephroma. To our knowledge, a case of m e t a s t a t i c RA m a s s a t t a c h e d to the a t r i a l s e p t u m without t u m o r extension from below v i a IVC has not previously been published in h y p e r n e p h r o m a .
REFERENCES
1. Wold LE, Lie JT. Cardiac myxomas: a clinicopathologic profile. Am J Pathol 1980;101:219-40. 2. Panidis IP, Kotler MN, Mintz GS, Ross J. Clinical and echocardiographic features of right atrial masses. AM HEARTJ 1984;107:745-58. 3. Shahier DM, Libertiao JA, Zinman LN, Leonardi HK, Eyre RC. Resection of cavoatrial renal cell carcinoma employing total circulatory arrest. Arch Surg 1990;125:727-32. 4. Bissada NK, Finkbeiner AE, Williams GD, Weiss JB. Successful extraction of intracardiac tumor thrombus of renal carcinoma. J Urol 1977;118:474-5. 5. Gindea AJ, Benjamin G, Naidich DP, Freedberg RS, McCauley D, Kronzon I. Unusual cardiac metastasis in hypernephroma: the complementary role of echocardiography and magnetic resonance imaging. AM HEARTJ 1988;116:1359-61. 6. Fogel RI, Balady GJ, Klein MD, KhorasanJ AR. Metastatic renal cell carcinoma. An unusual cause of syncope. Chest 1990;98:481-2. 7. Bryant J, Vuckovic G. Metastatic tumors of the endocardiam. Arch Pathol Lab Med 1978;102:206-8.