Q1997 by Excerpta Medica, Inc. 0002-9149/97/$17.00 781All rights reserved. PII S0002-9149(96)00868-5

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Right-Sided Cardiac Tumors Detected byTransesophageal Echocardiography and

Its Usefulness in Differentiating theBenign from the Malignant OnesMary Lynch, MD, Stephen D. Clements, MD, Jack S. Shanewise, MD,

Cyeng C. Chen, MD, and Randolph P. Martin, MD

Eighteen patients (3 men and 15 women; mean age 63years) with right-sided tumors were evaluated by bothtransthoracic and transesophageal echocardiographyfrom 1989 to 1996. The indications for echocardio-graphic studies included evaluation for a presumedmass and further evaluation of ventricular function andvalvular function. Fifteen patients had right atrial tu-mors. These included 5 hypernephromas, 4 myxomas,2 angiosarcomas, 1 lipoma, 1 cavernous hemangioma,1 hepatoma, and 1 chondrosarcoma. Three patients hadright ventricular (RV) tumors: 1 metastatic olfactory neu-roblastoma, a leiomyosarcoma, a chondrosarcoma, anda fourth patient had infiltration of the RV free wall of

unknown etiology. Biopsy of either right atrial or RVmasses was performed with transesophageal echocar-diographic guidance in 2 patients, and allowed histo-logic diagnosis before surgical resection. These findingsindicate that tumors are more often found in the rightatrium than in the right ventricle, and females predom-inate. Most tumors arising within the right atrium arebenign, whereas those extending into the right atriumfrom outside are malignant. RV tumors are rarely en-countered; when present, they are likely to be malig-nant. Q1997 by Excerpta Medica, Inc.

(Am J Cardiol 1997;79:781–784)

The diagnosis of intracardiac tumors has beengreatly facilitated by the advent of echocardiog-

raphy. Initially, transthoracic and, more recently,transesophageal echocardiography have proved theimaging modalities of choice in these patients. It hasbeen the policy in this echocardiographic laboratoryto perform transesophageal echocardiographic–guided biopsies of right-sided masses in patientswhose diagnosis has not been obvious from thetransesophageal echocardiographic study. A trans-venous biopsy may help guide management andavoid a thoracotomy to establish the diagnosis. Thisstudy was designed to (1) evaluate the frequency andpathologic spectrum of tumor masses encountered inthe right side; (2) determine the echocardiographicfeatures of these masses to determine if any mor-phologic features differentiated these tumors fromother frequently encountered masses on the rightside; and (3) determine the role of transesophagealechocardiographic–guided biopsies in a selectedgroup of these patients.

METHODSThe study included all patients found to have

right-sided cardiac tumor masses on echocardio-graphic studies performed at the echocardiographiclaboratory at Emory University Hospital from 1989to March 1996. All patients found to have a tumormass on a transthoracic echocardiographic study un-

From The Division of Cardiology, Department of Medicine, EmoryUniversity Hospital, Atlanta, Georgia. Manuscript received July 24,1996; revised manuscript received and accepted October 23,1996.

Address for reprints: Mary Lynch, MD, D433 Emory UniversityHospital, 1364 Clifton Road, N.E., Atlanta, Georgia 30322.

derwent transesophageal echocardiographic study. Atumor mass was identified as the presence of an ab-normal mass in 1 of the right-sided cardiac chambersnot due to a variant anatomic structure nor consid-ered to be a thrombotic mass or a device (e.g., cath-eter or pacemaker). A complete transthoracic echo-cardiographic study was performed with either aHewlett-Packard Sonos 1000, 1500, or 2500 systemor an Acuson 128 XP/10 system. Patients under-went transesophageal echocardiographic examina-tion with the Hewlett-Packard (5 MHz) biplane orthe (5 MHz) omniplane probe or with the Acuson (5MHz) biplane probe. All patients fasted for at least4 hours before the procedure. The oropharynx wasanesthetized with topical lidocaine spray. Intrave-nous midazolam was administered for sedation. Theright atrium, right ventricle, and pulmonary arterieswere evaluated for the presence of any masses andfor extension of these masses into the great vesselsor other cardiac chambers. Images were recorded on1/2-inch VHS videotape for later analysis. All pa-tients gave informed witnessed consent for the pro-cedure and no complications were encountered.

In patients who underwent transvenous biopsyunder transesophageal echocardiographic guidance,a cardiac bioptome was introduced through the rightinternal jugular vein using the Seldinger techniqueand advanced to the site of the mass under trans-esophageal imaging where optimum positioning wasdone before biopsy samples were taken.

RESULTSEighteen patients (3 men and 15 women; mean

age 63 years) were found to have a right-sided car-diac tumor by transesophageal echocardiographic

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TABLE I Clinical Characteristics

PatientNumber

Age (yr)& Sex Symptoms TTE/TEE Findings Biopsy/Surgery

PathologicDiagnosis Outcome

1 67 F Dyspnea / edema Mass / AoV papilloma Surgery Myxoma Well2 79 F Dyspnea Mass attached to IAS Surgery Myxoma Well3 70 F Dyspnea Mass attached to IAS Surgery Myxoma Well4 68 F Dyspnea Mass attached to IAS Surgery Myxoma Well5 74 F Dyspnea Free RA wall mass Surgery Lipoma Well6 54 F Chest pain Posterior wall RA mass Surgery Hemangioma Well7 47 F Pathologic fracture Large RA mass / PE Refused surgery Angiosarcoma Died8 72 F Dyspnea Large RA mass / PE Biopsy / surgery Angiosarcoma Well (to 10 mo)9 21 M Chest pain RA / RV masses Biopsy / surgery Chondrosarcoma Well

10 83 F Fever / night sweats Hepatic vein to RA mass Surgery Hepatoma Died11 69 F Dyspnea IVC to RA mass Surgery Hypernephroma Well12 80 F Dyspnea IVC to RA mass Surgery Hypernephroma Died13 60 F Dyspnea IVC to RA mass Surgery Hypernephroma –14 54 M Dyspnea / edema IVC to RA mass Surgery Hypernephroma Well15 71 F Dyspnea IVC to RA mass Surgery Hypernephroma –16 52 F Abnormal ECG RV mass 0 Fat infiltration Well17 28 M Murmur Large RVOT mass Biopsy / surgery Neuroblastoma Well (9 mo)18 82 F Dyspnea RVOT mass Surgery Leiomyosarcoma 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 ventricularoutflow tract; – Å no information available.

FIGURE 1. A right atrial myxoma is seen attached to the inter-atrial septum in this longitudinal view. LA Å left atrium; RA Åright atrium.

studies. Table I lists the clinical demographics of thepatients included in the study.

Fifteen of the tumors were right atrial (RA) inorigin or extending into the right atrium from theinferior vena cava. Thirteen of these patients werewomen. Four patients had right ventricular (RV)masses including 1 patient who had separate RA andRV tumor masses (who is included in both the RAand RV groups).

Among the 15 tumors found in the right atrium,6 were benign (4 myxomas, 1 lipoma, 1 heman-gioma), 2 were primary malignant tumors, 6 patientshad direct spread from the primary organ via the in-ferior vena cava directly into the right atrium (5 hy-pernephromas and 1 hepatoma) and a chondrosar-coma that involved both the right atrium and the rightventricle.

Of the 4 myxomas, 3 had typical attachments tothe interatrial septum. The fourth was attached to theatrial free wall near the appendage. All were multi-lobulated, smooth-surfaced structures. Those arisingfrom the interatrial septum were pedunculated andmobile (Figure 1). All of these tumors were identi-fiable as suggestive of myxomas by their point oforigin, texture, and mobility. None was associatedwith invasion of the atrial wall, caval compression,or a pericardial effusion. All patients underwent un-eventful surgical resection based on echocardio-graphic findings. Histologic examination confirmedthe echocardiographic diagnosis.

Two patients had nonmyxomatous benign RA tu-mors. A 74-year-old woman was found to have alarge homogenous, smooth, lobulated mass arisingfrom the free wall of the right atrium, just superiorto the inferior vena cava. There were no associatedsatellite lesions or a pericardial effusion. This provedto be a lipoma following resection. A 54-year-oldwoman had a large homogenous mass arising fromthe posterosuperior aspect of the right atrium, close

to the superior vena cava and compressing it. Thisproved to be a benign cavernous hemangioma fol-lowing resection.

Nine patients were found to have malignant RAtumors. Two were primary malignant cardiac tumorsand 7 were metastatic to the right atrium. Both pri-mary tumors were angiosarcomas. Both of these pa-tients had large irregular masses arising from the freeRA wall and occupying most of the RA cavity. Themasses were seen to compress the superior vena cavaand RA junction and distort the tricuspid annulus andhad associated moderate-sized pericardial effusions(Figure 2). One patient had histologic diagnosismade by transvenous biopsy under transesophagealechocardiographic guidance before successful sur-gical resection. The second patient had the diagnosisconfirmed by bone marrow aspirate; she refused sur-gery and subsequently died. A 21-year-old man pre-sented with left-sided chest pain and was found tohave a metastatic bony deposit in his left-sided sixth

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FIGURE 2. In this transverse view an encapsulated tumor is seenin the free wall of the right atrium (RA), compressing the rightatrial cavity and encroaching onto the tricuspid annulus. The tu-mor is associated with a moderately sized pericardial effusion(PE). The right ventricle (RV), left ventricle (LV), and left atrium(LA) are seen.

FIGURE 3. A, transverse view of the inferior vena cava (thick ar-row) with a tumor mass, a hypernephroma. A hepatic vein isalso seen (thin arrow). B, transverse view of the right atrium witha large tumor mass emanating from the inferior vena cava. Theright ventricle (RV), left ventricle (LV), and left atrium (LA) areseen.

rib by chest x-ray. Both transthoracic and trans-esophageal echocardiographic studies showed 2 tu-mor masses within his heart, a large RA mass ema-nating from the inferior vena cava and prolapsingthrough the tricuspid valve, and a second separatemass arising in the RV outflow tract. This patientunderwent successful resection of the masses, whichproved to be metastatic chondrosarcoma.

Six patients (1 man and 5 women) who were eval-uated for dyspnea and/or edema were found to havetumors extending into the RA cavity from the infe-rior vena cava. One of these masses was seen to ex-tend from a hepatic vein, a hepatoma, which wassuccessfully resected. Five patients were found tohave masses extending from the abdominal venacava into the RA cavity; all proved to be hyper-nephromas. All masses were seen to extend into theRA cavity, none was seen to invade or adhere to theRA wall (Figure 3). All patients underwent resectionof the involved kidney and removal of the tumorfrom the inferior vena cava and right atrium.

Four patients (2 men and 2 women; mean age 46years) were found to have RV masses. A 28-year-old man with a remote history of an olfactory neu-roblastoma was found to have a murmur on routinephysical examination. A transthoracic study dem-onstrated a mass arising from the free wall of the RVoutflow tract, causing significant RV outflow tractobstruction (Figure 4). He underwent biopsy undertransesophageal echocardiographic guidance andproved to be a metastatic recurrence of his earliertumor which was successfully resected. Separate RAand RV tumor masses were found in a patient witha chondrosarcoma. The RV mass was in the ouflowtract and prolapsed through the pulmonary valve.Both masses were successfully resected. A leiomyo-sarcoma was found in the free wall of the RV outflowtract of an 82-year-old woman who presented withdyspnea. An echocardiographic study, performed toevaluate left ventricular function in a 53-year-old

woman demonstrated fatty-like infiltration of the freeRV wall. On transesophageal echocardiographicstudy, the infiltrative lipomatous mass was noted toextend from the apex of the right ventricle to thetricuspid annulus. Magnetic resonance imaging, ob-tained to evaluate the mass further, also suggestedfatty infiltration.

Two patients (1 patient with a RA mass and 1with a mass in the RV outflow tract) underwent bi-opsy of the tumor mass under transesophageal echo-cardiographic guidance. Several tissue samples weretaken and a histologic diagnosis was made in bothcases. No complications were encountered.

DISCUSSIONOver 30,000 studies were performed in this lab-

oratory over the time period reviewed. Right atrialtumors were found in only 15 patients, 9 of whichwere malignant. In contrast, over the same period,only 4 patients with RV masses suggestive of tumorwere identified. One of these was presumed to be aninfiltration of the RV free wall, the etiology of whichis not known. Thus, right-sided cardiac tumors are

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FIGURE 4. In this longitudinal view, a large tumor mass is seen inthe free wall of the right ventricle (RV) and right ventricular out-flow tract (RVOT), which it almost obliterates. AoV Å aorticvalve; LA Å left atrium; RA Å right atrium.

rare, and as found by other investigators, RA tumorspredominate over RV tumors.1–3

Nine malignant RA tumors were found. Thesewere easily differentiated from the nonmalignant tu-mors echocardiographically. Six were the result ofdirect extension into the right atrium via the inferiorvena cava from the primary organ, namely the kid-ney or liver. Two were angiosarcomas and werenoted to be irregular masses with invasion of the RAwall, causing anatomic distortion of the right atriumdue to compression by the tumor. Both had associ-ated significant pericardial effusions. In contrast, pa-tients with nonmalignant tumors were noted to havesmooth regular textured masses. No RA wall inva-sion was noted to be present, nor any anatomic dis-tortion seen. None was associated with a pericardialeffusion.

The incidence of malignant tumors was high inour study compared with other reports.1,2 This wasdue to the high incidence of hypernephromas among

our patients, and may be explained by a referral biasto our institution. These patients generally requirecomplex surgery, a simultaneous thoracotomy andlaparotomy, and such patients would be more likelyto be referred to a tertiary care center. If tumors ex-tending into the right atrium from the inferior venacava are excluded, most of the remaining tumorswould be benign, reflecting the more commonly en-countered pathologic spectrum of RA tumors.1,2,4

Transvenous cardiac biopsy was performed undertransesophageal echocardiographic guidance in 2 pa-tients with bulky nonfriable masses (1 with RA and1 with RV outflow tract masses). This allowed op-timal positioning of the bioptome, minimizing therisk of embolization and yielded a histologic diag-nosis without having to resort to a thoracotomy. Sev-eral tissue samples were obtained in each patient andno complications were encountered.

Our study confirms the findings of previous in-vestigators, that right-sided cardiac masses are rareand RA masses are more common than RV masses.RA tumors are predominantly benign if they are notassociated with invasion of the RA wall or a peri-cardial effusion, or arising from the inferior venacava. RV and pulmonary artery masses are generallyrare. Transesophageal echocardiographic–guidedtransvenous biopsy is a safe and effective method ofobtaining tissue for histologic diagnosis in right-sided cardiac masses.

1. McAllister HA, Fenoglio JJ. Tumors of the cardiovascular system. In: Atlasof Tumor Pathology, Fascicle 15, series 2. Washington DC: Armed Forces In-stitute 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 andsurgical results in 74 patients. Ann Thorac Surg 1991;52:886–895.3. Molina JE, Edwards JE, Ward HB. Primary cardiac tumors: experience atthe University of Minnesota. Thorac Cardiovasc Surg 1990;38:183–191.4. Obeid AI, Mudamgha AA, Smulyan H. Diagnosis of right atrial mass lesionsby transesophageal and transthoracic echocardiography. Chest 1993;103:1447–1451.