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Right-Sided Cardiac Tumors Detected by Transesophageal Echocardiography and Its Usefulness in Differentiating the Benign From the Malignant Ones
Right-Sided Cardiac Tumors Detected by Transesophageal Echocardiography and Its Usefulness in Differentiating the Benign from the Malignant Ones Mary Lynch, MD, Stephen D. Clements, MD, Jack S. Shanewise, Cyeng C. Chen, MD, and Randolph P. Martin, MD
MD,
Eighteen patients (3 men and 15 women; mean age 63 years) with right-sided tumors were evaluated by both transthoracic and transesophageal echocardiography from 1989 to 1996. The indications for echocardiographic studies included evaluation for a presumed mass and further evaluation of ventricular function and valvular function. Fifteen patients had right atrial tumors. These included 5 hypernephromas, 4 myxomas, 2 angiosarcomas, 1 lipoma, 1 cavernous hemangioma, 1 hepatoma, and 1 chondrosarcoma. Three patients had right ventricular (RV) tumors: 1 metastatic olfactory neuroblastoma, a leiomyosarcoma, a chondrosarcoma, and a fourth patient had infiltration of the RV free wall of
unknown etiology. Biopsy of either right atrial or RV masses was performed with transesophageal echocardiographic guidance in 2 patients, and allowed histologic diagnosis before surgical resection. These findings indicate that tumors are more often found in the right atrium than in the right ventricle, and females predominate. Most tumors arising within the right atrium are benign, whereas those extending into the right atrium from outside are malignant. RV tumors are rarely encountered; when present, they are likely to be malignant. Q1997 by Excerpta Medica, Inc. (Am J Cardiol 1997;79:781–784)
he diagnosis of intracardiac tumors has been greatly facilitated by the advent of echocardiogT raphy. Initially, transthoracic and, more recently,
derwent transesophageal echocardiographic study. A tumor mass was identified as the presence of an abnormal mass in 1 of the right-sided cardiac chambers not due to a variant anatomic structure nor considered to be a thrombotic mass or a device (e.g., catheter or pacemaker). A complete transthoracic echocardiographic study was performed with either a Hewlett-Packard Sonos 1000, 1500, or 2500 system or an Acuson 128 XP/10 system. Patients underwent transesophageal echocardiographic examination with the Hewlett-Packard (5 MHz) biplane or the (5 MHz) omniplane probe or with the Acuson (5 MHz) biplane probe. All patients fasted for at least 4 hours before the procedure. The oropharynx was anesthetized with topical lidocaine spray. Intravenous midazolam was administered for sedation. The right atrium, right ventricle, and pulmonary arteries were evaluated for the presence of any masses and for extension of these masses into the great vessels or other cardiac chambers. Images were recorded on 1/2-inch VHS videotape for later analysis. All patients gave informed witnessed consent for the procedure and no complications were encountered. In patients who underwent transvenous biopsy under transesophageal echocardiographic guidance, a cardiac bioptome was introduced through the right internal jugular vein using the Seldinger technique and advanced to the site of the mass under transesophageal imaging where optimum positioning was done before biopsy samples were taken.
transesophageal echocardiography have proved the imaging modalities of choice in these patients. It has been the policy in this echocardiographic laboratory to perform transesophageal echocardiographic– guided biopsies of right-sided masses in patients whose diagnosis has not been obvious from the transesophageal echocardiographic study. A transvenous biopsy may help guide management and avoid a thoracotomy to establish the diagnosis. This study was designed to (1) evaluate the frequency and pathologic spectrum of tumor masses encountered in the right side; (2) determine the echocardiographic features of these masses to determine if any morphologic features differentiated these tumors from other frequently encountered masses on the right side; and (3) determine the role of transesophageal echocardiographic–guided biopsies in a selected group of these patients.
METHODS The study included all patients found to have right-sided cardiac tumor masses on echocardiographic studies performed at the echocardiographic laboratory at Emory University Hospital from 1989 to March 1996. All patients found to have a tumor mass on a transthoracic echocardiographic study unFrom The Division of Cardiology, Department of Medicine, Emory University Hospital, Atlanta, Georgia. Manuscript received July 24, 1996; revised manuscript received and accepted October 23, 1996. Address for reprints: Mary Lynch, MD, D433 Emory University Hospital, 1364 Clifton Road, N.E., Atlanta, Georgia 30322.
RESULTS Eighteen patients (3 men and 15 women; mean age 63 years) were found to have a right-sided cardiac tumor by transesophageal echocardiographic
Q1997 by Excerpta Medica, Inc.
0002-9149/97/$17.00 PII S0002-9149(96)00868-5
All rights reserved.
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TABLE I Clinical Characteristics Patient Number 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18
Age (yr) & Sex 67 79 70 68 74 54 47 72 21 83 69 80 60 54 71 52 28 82
F F F F F F F F M F F F F M F F M F
Symptoms
TTE/TEE Findings
Biopsy/Surgery
Pathologic Diagnosis
Outcome
Dyspnea / edema Dyspnea Dyspnea Dyspnea Dyspnea Chest pain Pathologic fracture Dyspnea Chest pain Fever / night sweats Dyspnea Dyspnea Dyspnea Dyspnea / edema Dyspnea Abnormal ECG Murmur Dyspnea
Mass / AoV papilloma Mass attached to IAS Mass attached to IAS Mass attached to IAS Free RA wall mass Posterior wall RA mass Large RA mass / PE Large RA mass / PE RA / RV masses Hepatic vein to RA mass IVC to RA mass IVC to RA mass IVC to RA mass IVC to RA mass IVC to RA mass RV mass Large RVOT mass RVOT mass
Surgery Surgery Surgery Surgery Surgery Surgery Refused surgery Biopsy / surgery Biopsy / surgery Surgery Surgery Surgery Surgery Surgery Surgery 0 Biopsy / surgery Surgery
Myxoma Myxoma Myxoma Myxoma Lipoma Hemangioma Angiosarcoma Angiosarcoma Chondrosarcoma Hepatoma Hypernephroma Hypernephroma Hypernephroma Hypernephroma Hypernephroma Fat infiltration Neuroblastoma Leiomyosarcoma
Well Well Well Well Well Well Died Well (to 10 mo) Well Died Well Died – Well – Well Well (9 mo) Well (9 mo)
AoV Å aortic valve; ECG Å electrocardiogram; IAS Å interatrial septum; IVC Å inferior vena cava; RA Å right atrium; RV Å right ventricle; RVOT Å right ventricular outflow tract; – Å no information available.
studies. Table I lists the clinical demographics of the patients included in the study. Fifteen of the tumors were right atrial (RA) in origin or extending into the right atrium from the inferior vena cava. Thirteen of these patients were women. Four patients had right ventricular (RV) masses including 1 patient who had separate RA and RV tumor masses (who is included in both the RA and RV groups). Among the 15 tumors found in the right atrium, 6 were benign (4 myxomas, 1 lipoma, 1 hemangioma), 2 were primary malignant tumors, 6 patients had direct spread from the primary organ via the inferior vena cava directly into the right atrium (5 hypernephromas and 1 hepatoma) and a chondrosarcoma that involved both the right atrium and the right ventricle. Of the 4 myxomas, 3 had typical attachments to the interatrial septum. The fourth was attached to the atrial free wall near the appendage. All were multilobulated, smooth-surfaced structures. Those arising from the interatrial septum were pedunculated and mobile (Figure 1). All of these tumors were identifiable as suggestive of myxomas by their point of origin, texture, and mobility. None was associated with invasion of the atrial wall, caval compression, or a pericardial effusion. All patients underwent uneventful surgical resection based on echocardiographic findings. Histologic examination confirmed the echocardiographic diagnosis. Two patients had nonmyxomatous benign RA tumors. A 74-year-old woman was found to have a large homogenous, smooth, lobulated mass arising from the free wall of the right atrium, just superior to the inferior vena cava. There were no associated satellite lesions or a pericardial effusion. This proved to be a lipoma following resection. A 54-year-old woman had a large homogenous mass arising from the posterosuperior aspect of the right atrium, close 782
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to the superior vena cava and compressing it. This proved to be a benign cavernous hemangioma following resection. Nine patients were found to have malignant RA tumors. Two were primary malignant cardiac tumors and 7 were metastatic to the right atrium. Both primary tumors were angiosarcomas. Both of these patients had large irregular masses arising from the free RA wall and occupying most of the RA cavity. The masses were seen to compress the superior vena cava and RA junction and distort the tricuspid annulus and had associated moderate-sized pericardial effusions (Figure 2). One patient had histologic diagnosis made by transvenous biopsy under transesophageal echocardiographic guidance before successful surgical resection. The second patient had the diagnosis confirmed by bone marrow aspirate; she refused surgery and subsequently died. A 21-year-old man presented with left-sided chest pain and was found to have a metastatic bony deposit in his left-sided sixth
FIGURE 1. A right atrial myxoma is seen attached to the interatrial septum in this longitudinal view. LA Å left atrium; RA Å right atrium.
MARCH 15, 1997
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FIGURE 2. In this transverse view an encapsulated tumor is seen in the free wall of the right atrium (RA), compressing the right atrial cavity and encroaching onto the tricuspid annulus. The tumor is associated with a moderately sized pericardial effusion (PE). The right ventricle (RV), left ventricle (LV), and left atrium (LA) are seen.
rib by chest x-ray. Both transthoracic and transesophageal echocardiographic studies showed 2 tumor masses within his heart, a large RA mass emanating from the inferior vena cava and prolapsing through the tricuspid valve, and a second separate mass arising in the RV outflow tract. This patient underwent successful resection of the masses, which proved to be metastatic chondrosarcoma. Six patients (1 man and 5 women) who were evaluated for dyspnea and/or edema were found to have tumors extending into the RA cavity from the inferior vena cava. One of these masses was seen to extend from a hepatic vein, a hepatoma, which was successfully resected. Five patients were found to have masses extending from the abdominal vena cava into the RA cavity; all proved to be hypernephromas. All masses were seen to extend into the RA cavity, none was seen to invade or adhere to the RA wall (Figure 3). All patients underwent resection of the involved kidney and removal of the tumor from the inferior vena cava and right atrium. Four patients (2 men and 2 women; mean age 46 years) were found to have RV masses. A 28-yearold man with a remote history of an olfactory neuroblastoma was found to have a murmur on routine physical examination. A transthoracic study demonstrated a mass arising from the free wall of the RV outflow tract, causing significant RV outflow tract obstruction (Figure 4). He underwent biopsy under transesophageal echocardiographic guidance and proved to be a metastatic recurrence of his earlier tumor which was successfully resected. Separate RA and RV tumor masses were found in a patient with a chondrosarcoma. The RV mass was in the ouflow tract and prolapsed through the pulmonary valve. Both masses were successfully resected. A leiomyosarcoma was found in the free wall of the RV outflow tract of an 82-year-old woman who presented with dyspnea. An echocardiographic study, performed to evaluate left ventricular function in a 53-year-old
FIGURE 3. A, transverse view of the inferior vena cava (thick arrow) with a tumor mass, a hypernephroma. A hepatic vein is also seen (thin arrow). B, transverse view of the right atrium with a large tumor mass emanating from the inferior vena cava. The right ventricle (RV), left ventricle (LV), and left atrium (LA) are seen.
woman demonstrated fatty-like infiltration of the free RV wall. On transesophageal echocardiographic study, the infiltrative lipomatous mass was noted to extend from the apex of the right ventricle to the tricuspid annulus. Magnetic resonance imaging, obtained to evaluate the mass further, also suggested fatty infiltration. Two patients (1 patient with a RA mass and 1 with a mass in the RV outflow tract) underwent biopsy of the tumor mass under transesophageal echocardiographic guidance. Several tissue samples were taken and a histologic diagnosis was made in both cases. No complications were encountered.
DISCUSSION Over 30,000 studies were performed in this laboratory over the time period reviewed. Right atrial tumors were found in only 15 patients, 9 of which were malignant. In contrast, over the same period, only 4 patients with RV masses suggestive of tumor were identified. One of these was presumed to be an infiltration of the RV free wall, the etiology of which is not known. Thus, right-sided cardiac tumors are MISCELLANEOUS/RIGHT-SIDED CARDIAC TUMORS
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FIGURE 4. In this longitudinal view, a large tumor mass is seen in the free wall of the right ventricle (RV) and right ventricular outflow tract (RVOT), which it almost obliterates. AoV Å aortic valve; LA Å left atrium; RA Å right atrium.
rare, and as found by other investigators, RA tumors predominate over RV tumors.1 – 3 Nine malignant RA tumors were found. These were easily differentiated from the nonmalignant tumors echocardiographically. Six were the result of direct extension into the right atrium via the inferior vena cava from the primary organ, namely the kidney or liver. Two were angiosarcomas and were noted to be irregular masses with invasion of the RA wall, causing anatomic distortion of the right atrium due to compression by the tumor. Both had associated significant pericardial effusions. In contrast, patients with nonmalignant tumors were noted to have smooth regular textured masses. No RA wall invasion was noted to be present, nor any anatomic distortion seen. None was associated with a pericardial effusion. The incidence of malignant tumors was high in our study compared with other reports.1,2 This was due to the high incidence of hypernephromas among
784
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our patients, and may be explained by a referral bias to our institution. These patients generally require complex surgery, a simultaneous thoracotomy and laparotomy, and such patients would be more likely to be referred to a tertiary care center. If tumors extending into the right atrium from the inferior vena cava are excluded, most of the remaining tumors would be benign, reflecting the more commonly encountered pathologic spectrum of RA tumors.1,2,4 Transvenous cardiac biopsy was performed under transesophageal echocardiographic guidance in 2 patients with bulky nonfriable masses (1 with RA and 1 with RV outflow tract masses). This allowed optimal positioning of the bioptome, minimizing the risk of embolization and yielded a histologic diagnosis without having to resort to a thoracotomy. Several tissue samples were obtained in each patient and no complications were encountered. Our study confirms the findings of previous investigators, that right-sided cardiac masses are rare and RA masses are more common than RV masses. RA tumors are predominantly benign if they are not associated with invasion of the RA wall or a pericardial effusion, or arising from the inferior vena cava. RV and pulmonary artery masses are generally rare. Transesophageal echocardiographic–guided transvenous biopsy is a safe and effective method of obtaining tissue for histologic diagnosis in rightsided cardiac masses. 1. McAllister HA, Fenoglio JJ. Tumors of the cardiovascular system. In: Atlas
of Tumor Pathology, Fascicle 15, series 2. Washington DC: Armed Forces Institute of Pathology, 1978. 2. Miralles B, Bracamonte L, Soncul H, Diaz del Castillo R, Akhtar R, Bors V, Pavie A, Gandjbackhch I, Cabrol C. Cardiac tumors: clinical experience and surgical results in 74 patients. Ann Thorac Surg 1991;52:886–895. 3. Molina JE, Edwards JE, Ward HB. Primary cardiac tumors: experience at the University of Minnesota. Thorac Cardiovasc Surg 1990;38:183–191. 4. Obeid AI, Mudamgha AA, Smulyan H. Diagnosis of right atrial mass lesions by transesophageal and transthoracic echocardiography. Chest 1993;103:1447– 1451.
MARCH 15, 1997
EL–AJC (v. 79, no. 6 ’97)
0853
MD,
Eighteen patients (3 men and 15 women; mean age 63 years) with right-sided tumors were evaluated by both transthoracic and transesophageal echocardiography from 1989 to 1996. The indications for echocardiographic studies included evaluation for a presumed mass and further evaluation of ventricular function and valvular function. Fifteen patients had right atrial tumors. These included 5 hypernephromas, 4 myxomas, 2 angiosarcomas, 1 lipoma, 1 cavernous hemangioma, 1 hepatoma, and 1 chondrosarcoma. Three patients had right ventricular (RV) tumors: 1 metastatic olfactory neuroblastoma, a leiomyosarcoma, a chondrosarcoma, and a fourth patient had infiltration of the RV free wall of
unknown etiology. Biopsy of either right atrial or RV masses was performed with transesophageal echocardiographic guidance in 2 patients, and allowed histologic diagnosis before surgical resection. These findings indicate that tumors are more often found in the right atrium than in the right ventricle, and females predominate. Most tumors arising within the right atrium are benign, whereas those extending into the right atrium from outside are malignant. RV tumors are rarely encountered; when present, they are likely to be malignant. Q1997 by Excerpta Medica, Inc. (Am J Cardiol 1997;79:781–784)
he diagnosis of intracardiac tumors has been greatly facilitated by the advent of echocardiogT raphy. Initially, transthoracic and, more recently,
derwent transesophageal echocardiographic study. A tumor mass was identified as the presence of an abnormal mass in 1 of the right-sided cardiac chambers not due to a variant anatomic structure nor considered to be a thrombotic mass or a device (e.g., catheter or pacemaker). A complete transthoracic echocardiographic study was performed with either a Hewlett-Packard Sonos 1000, 1500, or 2500 system or an Acuson 128 XP/10 system. Patients underwent transesophageal echocardiographic examination with the Hewlett-Packard (5 MHz) biplane or the (5 MHz) omniplane probe or with the Acuson (5 MHz) biplane probe. All patients fasted for at least 4 hours before the procedure. The oropharynx was anesthetized with topical lidocaine spray. Intravenous midazolam was administered for sedation. The right atrium, right ventricle, and pulmonary arteries were evaluated for the presence of any masses and for extension of these masses into the great vessels or other cardiac chambers. Images were recorded on 1/2-inch VHS videotape for later analysis. All patients gave informed witnessed consent for the procedure and no complications were encountered. In patients who underwent transvenous biopsy under transesophageal echocardiographic guidance, a cardiac bioptome was introduced through the right internal jugular vein using the Seldinger technique and advanced to the site of the mass under transesophageal imaging where optimum positioning was done before biopsy samples were taken.
transesophageal echocardiography have proved the imaging modalities of choice in these patients. It has been the policy in this echocardiographic laboratory to perform transesophageal echocardiographic– guided biopsies of right-sided masses in patients whose diagnosis has not been obvious from the transesophageal echocardiographic study. A transvenous biopsy may help guide management and avoid a thoracotomy to establish the diagnosis. This study was designed to (1) evaluate the frequency and pathologic spectrum of tumor masses encountered in the right side; (2) determine the echocardiographic features of these masses to determine if any morphologic features differentiated these tumors from other frequently encountered masses on the right side; and (3) determine the role of transesophageal echocardiographic–guided biopsies in a selected group of these patients.
METHODS The study included all patients found to have right-sided cardiac tumor masses on echocardiographic studies performed at the echocardiographic laboratory at Emory University Hospital from 1989 to March 1996. All patients found to have a tumor mass on a transthoracic echocardiographic study unFrom The Division of Cardiology, Department of Medicine, Emory University Hospital, Atlanta, Georgia. Manuscript received July 24, 1996; revised manuscript received and accepted October 23, 1996. Address for reprints: Mary Lynch, MD, D433 Emory University Hospital, 1364 Clifton Road, N.E., Atlanta, Georgia 30322.
RESULTS Eighteen patients (3 men and 15 women; mean age 63 years) were found to have a right-sided cardiac tumor by transesophageal echocardiographic
Q1997 by Excerpta Medica, Inc.
0002-9149/97/$17.00 PII S0002-9149(96)00868-5
All rights reserved.
/ 2w1b 0853 Mp
781
Thursday Feb 13 06:31 PM
EL–AJC (v. 79, no. 6 ’97)
0853
781
TABLE I Clinical Characteristics Patient Number 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18
Age (yr) & Sex 67 79 70 68 74 54 47 72 21 83 69 80 60 54 71 52 28 82
F F F F F F F F M F F F F M F F M F
Symptoms
TTE/TEE Findings
Biopsy/Surgery
Pathologic Diagnosis
Outcome
Dyspnea / edema Dyspnea Dyspnea Dyspnea Dyspnea Chest pain Pathologic fracture Dyspnea Chest pain Fever / night sweats Dyspnea Dyspnea Dyspnea Dyspnea / edema Dyspnea Abnormal ECG Murmur Dyspnea
Mass / AoV papilloma Mass attached to IAS Mass attached to IAS Mass attached to IAS Free RA wall mass Posterior wall RA mass Large RA mass / PE Large RA mass / PE RA / RV masses Hepatic vein to RA mass IVC to RA mass IVC to RA mass IVC to RA mass IVC to RA mass IVC to RA mass RV mass Large RVOT mass RVOT mass
Surgery Surgery Surgery Surgery Surgery Surgery Refused surgery Biopsy / surgery Biopsy / surgery Surgery Surgery Surgery Surgery Surgery Surgery 0 Biopsy / surgery Surgery
Myxoma Myxoma Myxoma Myxoma Lipoma Hemangioma Angiosarcoma Angiosarcoma Chondrosarcoma Hepatoma Hypernephroma Hypernephroma Hypernephroma Hypernephroma Hypernephroma Fat infiltration Neuroblastoma Leiomyosarcoma
Well Well Well Well Well Well Died Well (to 10 mo) Well Died Well Died – Well – Well Well (9 mo) Well (9 mo)
AoV Å aortic valve; ECG Å electrocardiogram; IAS Å interatrial septum; IVC Å inferior vena cava; RA Å right atrium; RV Å right ventricle; RVOT Å right ventricular outflow tract; – Å no information available.
studies. Table I lists the clinical demographics of the patients included in the study. Fifteen of the tumors were right atrial (RA) in origin or extending into the right atrium from the inferior vena cava. Thirteen of these patients were women. Four patients had right ventricular (RV) masses including 1 patient who had separate RA and RV tumor masses (who is included in both the RA and RV groups). Among the 15 tumors found in the right atrium, 6 were benign (4 myxomas, 1 lipoma, 1 hemangioma), 2 were primary malignant tumors, 6 patients had direct spread from the primary organ via the inferior vena cava directly into the right atrium (5 hypernephromas and 1 hepatoma) and a chondrosarcoma that involved both the right atrium and the right ventricle. Of the 4 myxomas, 3 had typical attachments to the interatrial septum. The fourth was attached to the atrial free wall near the appendage. All were multilobulated, smooth-surfaced structures. Those arising from the interatrial septum were pedunculated and mobile (Figure 1). All of these tumors were identifiable as suggestive of myxomas by their point of origin, texture, and mobility. None was associated with invasion of the atrial wall, caval compression, or a pericardial effusion. All patients underwent uneventful surgical resection based on echocardiographic findings. Histologic examination confirmed the echocardiographic diagnosis. Two patients had nonmyxomatous benign RA tumors. A 74-year-old woman was found to have a large homogenous, smooth, lobulated mass arising from the free wall of the right atrium, just superior to the inferior vena cava. There were no associated satellite lesions or a pericardial effusion. This proved to be a lipoma following resection. A 54-year-old woman had a large homogenous mass arising from the posterosuperior aspect of the right atrium, close 782
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to the superior vena cava and compressing it. This proved to be a benign cavernous hemangioma following resection. Nine patients were found to have malignant RA tumors. Two were primary malignant cardiac tumors and 7 were metastatic to the right atrium. Both primary tumors were angiosarcomas. Both of these patients had large irregular masses arising from the free RA wall and occupying most of the RA cavity. The masses were seen to compress the superior vena cava and RA junction and distort the tricuspid annulus and had associated moderate-sized pericardial effusions (Figure 2). One patient had histologic diagnosis made by transvenous biopsy under transesophageal echocardiographic guidance before successful surgical resection. The second patient had the diagnosis confirmed by bone marrow aspirate; she refused surgery and subsequently died. A 21-year-old man presented with left-sided chest pain and was found to have a metastatic bony deposit in his left-sided sixth
FIGURE 1. A right atrial myxoma is seen attached to the interatrial septum in this longitudinal view. LA Å left atrium; RA Å right atrium.
MARCH 15, 1997
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0853
FIGURE 2. In this transverse view an encapsulated tumor is seen in the free wall of the right atrium (RA), compressing the right atrial cavity and encroaching onto the tricuspid annulus. The tumor is associated with a moderately sized pericardial effusion (PE). The right ventricle (RV), left ventricle (LV), and left atrium (LA) are seen.
rib by chest x-ray. Both transthoracic and transesophageal echocardiographic studies showed 2 tumor masses within his heart, a large RA mass emanating from the inferior vena cava and prolapsing through the tricuspid valve, and a second separate mass arising in the RV outflow tract. This patient underwent successful resection of the masses, which proved to be metastatic chondrosarcoma. Six patients (1 man and 5 women) who were evaluated for dyspnea and/or edema were found to have tumors extending into the RA cavity from the inferior vena cava. One of these masses was seen to extend from a hepatic vein, a hepatoma, which was successfully resected. Five patients were found to have masses extending from the abdominal vena cava into the RA cavity; all proved to be hypernephromas. All masses were seen to extend into the RA cavity, none was seen to invade or adhere to the RA wall (Figure 3). All patients underwent resection of the involved kidney and removal of the tumor from the inferior vena cava and right atrium. Four patients (2 men and 2 women; mean age 46 years) were found to have RV masses. A 28-yearold man with a remote history of an olfactory neuroblastoma was found to have a murmur on routine physical examination. A transthoracic study demonstrated a mass arising from the free wall of the RV outflow tract, causing significant RV outflow tract obstruction (Figure 4). He underwent biopsy under transesophageal echocardiographic guidance and proved to be a metastatic recurrence of his earlier tumor which was successfully resected. Separate RA and RV tumor masses were found in a patient with a chondrosarcoma. The RV mass was in the ouflow tract and prolapsed through the pulmonary valve. Both masses were successfully resected. A leiomyosarcoma was found in the free wall of the RV outflow tract of an 82-year-old woman who presented with dyspnea. An echocardiographic study, performed to evaluate left ventricular function in a 53-year-old
FIGURE 3. A, transverse view of the inferior vena cava (thick arrow) with a tumor mass, a hypernephroma. A hepatic vein is also seen (thin arrow). B, transverse view of the right atrium with a large tumor mass emanating from the inferior vena cava. The right ventricle (RV), left ventricle (LV), and left atrium (LA) are seen.
woman demonstrated fatty-like infiltration of the free RV wall. On transesophageal echocardiographic study, the infiltrative lipomatous mass was noted to extend from the apex of the right ventricle to the tricuspid annulus. Magnetic resonance imaging, obtained to evaluate the mass further, also suggested fatty infiltration. Two patients (1 patient with a RA mass and 1 with a mass in the RV outflow tract) underwent biopsy of the tumor mass under transesophageal echocardiographic guidance. Several tissue samples were taken and a histologic diagnosis was made in both cases. No complications were encountered.
DISCUSSION Over 30,000 studies were performed in this laboratory over the time period reviewed. Right atrial tumors were found in only 15 patients, 9 of which were malignant. In contrast, over the same period, only 4 patients with RV masses suggestive of tumor were identified. One of these was presumed to be an infiltration of the RV free wall, the etiology of which is not known. Thus, right-sided cardiac tumors are MISCELLANEOUS/RIGHT-SIDED CARDIAC TUMORS
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FIGURE 4. In this longitudinal view, a large tumor mass is seen in the free wall of the right ventricle (RV) and right ventricular outflow tract (RVOT), which it almost obliterates. AoV Å aortic valve; LA Å left atrium; RA Å right atrium.
rare, and as found by other investigators, RA tumors predominate over RV tumors.1 – 3 Nine malignant RA tumors were found. These were easily differentiated from the nonmalignant tumors echocardiographically. Six were the result of direct extension into the right atrium via the inferior vena cava from the primary organ, namely the kidney or liver. Two were angiosarcomas and were noted to be irregular masses with invasion of the RA wall, causing anatomic distortion of the right atrium due to compression by the tumor. Both had associated significant pericardial effusions. In contrast, patients with nonmalignant tumors were noted to have smooth regular textured masses. No RA wall invasion was noted to be present, nor any anatomic distortion seen. None was associated with a pericardial effusion. The incidence of malignant tumors was high in our study compared with other reports.1,2 This was due to the high incidence of hypernephromas among
784
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our patients, and may be explained by a referral bias to our institution. These patients generally require complex surgery, a simultaneous thoracotomy and laparotomy, and such patients would be more likely to be referred to a tertiary care center. If tumors extending into the right atrium from the inferior vena cava are excluded, most of the remaining tumors would be benign, reflecting the more commonly encountered pathologic spectrum of RA tumors.1,2,4 Transvenous cardiac biopsy was performed under transesophageal echocardiographic guidance in 2 patients with bulky nonfriable masses (1 with RA and 1 with RV outflow tract masses). This allowed optimal positioning of the bioptome, minimizing the risk of embolization and yielded a histologic diagnosis without having to resort to a thoracotomy. Several tissue samples were obtained in each patient and no complications were encountered. Our study confirms the findings of previous investigators, that right-sided cardiac masses are rare and RA masses are more common than RV masses. RA tumors are predominantly benign if they are not associated with invasion of the RA wall or a pericardial effusion, or arising from the inferior vena cava. RV and pulmonary artery masses are generally rare. Transesophageal echocardiographic–guided transvenous biopsy is a safe and effective method of obtaining tissue for histologic diagnosis in rightsided cardiac masses. 1. McAllister HA, Fenoglio JJ. Tumors of the cardiovascular system. In: Atlas
of Tumor Pathology, Fascicle 15, series 2. Washington DC: Armed Forces Institute of Pathology, 1978. 2. Miralles B, Bracamonte L, Soncul H, Diaz del Castillo R, Akhtar R, Bors V, Pavie A, Gandjbackhch I, Cabrol C. Cardiac tumors: clinical experience and surgical results in 74 patients. Ann Thorac Surg 1991;52:886–895. 3. Molina JE, Edwards JE, Ward HB. Primary cardiac tumors: experience at the University of Minnesota. Thorac Cardiovasc Surg 1990;38:183–191. 4. Obeid AI, Mudamgha AA, Smulyan H. Diagnosis of right atrial mass lesions by transesophageal and transthoracic echocardiography. Chest 1993;103:1447– 1451.
MARCH 15, 1997
EL–AJC (v. 79, no. 6 ’97)
0853