Transesophageal Echocardiographic (TEE) Evaluation of Intracardiac and Pericardial Masses

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TRANSESOPHAGEAL ECHOCARDIOGRAPHY

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TRANSESOPHAGEAL ECHOCARDIOGRAPHIC (TEE) EVALUATION OF INTRACARDIAC AND PERICARDIAL MASSES Jonathan H. Goldman, BSc, MD, MBBS, MRCP, and Elyse Foster, MD

Transesophageal echocardiography (TEE) is extremely useful in the delineation of intracardiac and extracardiac masses. The excellent resolution afforded by TEE and the proximity of the probe to the posterior structures of the heart facilitate diagnosis in this pathologic category. Equally important is the ability to differentiate artifacts and normal anatomic structures from pathologic structures. This article addresses intracardiac tumors and masses that are not covered in other articles and focuses on those artifacts and normal intracardiac anatomy that can mimic a mass. Intracardiac masses on the left side of the heart are discovered most often when a patient is referred for transthoracic echocardiography to evaluate a possible cerebral or systemic embolic event. Right heart masses may be detected following clinical presentation with dyspnea owing to pulmonary embolization. Rarely, the patient is referred for evaluation of an abnormal heart sound (e.g., tumor plop) or a newly recognized murmur (caused by flow obstruction). In addition, a mass may be an incidental finding on transthoracic echocardiography. TEE is indicated when the mass is defined incompletely by surface im-

aging. The sites of attachment and origin of tumor, the presence of associated myocardial infiltration, and the existence of other smaller lesions can be defined better by TEE. This article addresses (1) normal intracardiac structures that may simulate a pathological mass, (2) secondary tumors, and (3) primary tumors. The appearance of valvular vegetations and atrial thrombi is discussed elsewhere in this issue.

NORMAL INTRACARDIAC ANATOMY AND ARTIFACTS

The higher resolution of TEE enhances visualization of normal cardiac anatomy. When the operator is unfamiliar with variations in normal anatomy, erroneous diagnoses can lead to inappropriate medical therapy and even inappropriate referral for surgery. This section describes some of the common variants in normal and diseased states that should be recognized. Identification of normal artifactual variants is becoming increasingly important in the

From the Division of Cardiology, Department of Medicine (JHG, EF); and the Echocardiography Laboratory (EF), University of California, San Francisco, San Francisco, California ~

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CARDIOLOGY CLINICS VOLUME 18 * NUMBER 4 * NOVEMBER 2000

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new era of device closure of atrial septa1 defects. In such cases, precise identification of the borders of the defect is essential. In addition, identification of normal atrial structures is required during TEE studies that attempt to exclude an embolic source in the left atrium (Fig. l), or catheter and pacing wireassociated vegetation in the right atrium. Right Atrium

The Chiari network is an embryologic remnant of the valve of the inferior vena cava, which is variable in its appearance. It is a filamentous structure that may extend from the base of the inferior margin of the inferior vena cava and may prolapse into the orifice of the tricuspid valve. It rarely is associated with obstruction or endocarditis. The eustachian valve is an embryonic remnant that can be identified at the ostium of the inferior vena ~ a v aAnother . ~ ~ embryonic remnant, the thebesian valve, occasionally is seen at the ostium of the coronary sinus. The crista terminalis is a ridge at the superior aspect of the right atrium in the region of the superior vena cava, which is an important landmark in electrophysiology for the location of the sinus node (Fig. 2). Lipomatous hypertrophy of the atrial septum is a relatively common finding, increasing in frequency with age. Sparing of the fossa ovalis produces a characteristic "dumbbell" appearance (Fig. 3). Very

Figure 2. The figure demonstrates normal right atrial anatomy in the longitudinal view. C = crista terminalis; T = trabeculae.

pronounced thickening may occur and, when visualized in off-axis views, may simulate a tumor mass. The trabeculations (so-called "pectinate muscles") within the broad-based right atrial appendage may be very prominent and should not be confused with throm-

Figure 1. Large thrombus within the left atrial appendage in a case of mitral stenosis. T = thrombus; LA = left atrium; LV = left ventricle.

TEE EVALUATION OF INTRACARDIAC AND PERICARDIAL MASSES

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Figure 3. A and 6,Lipomatous hypertrophy of the atrial septum. The “dumbell” appearance of the interatrial septum (6)is characteristic. RA = right atrium; LA = left atrium; Ao = ascending aorta.

bus or tumor (see Fig. 2). These trabeculations ”spill out” of the appendage and extend toward the tricuspid valve. Left Atrium A source of confusion can be the prominent fold of tissue that separates the left upper pulmonary vein and the left atrial appendage. This fold has been nicknamed ”the Coumadin ridge,” because its most proximal portion may have a knoblike appearance that simu-

lates thrombus and leads to the misguided initiation of anticoagulant therapy (Fig. 4). The left atrial appendage may have multiple smaller lobes; more than 50% of people have more than two The tissue separating these crenulations may be confused with tumor or thrombus. Multiplane imaging can help define these separate infoldings. The trabeculations or pectinate muscles are confined to the body of the left atrial appendage. The tip of the left atrial appendage occasionally is seen in cross-section within the transverse sinus and may be mistaken for a pericardial

Figure 4. Normal appearance of ridge of tissue separating left upper pulmonary vein from left atrial appendage. R = ridge; LUPV = left upper pulmonary vein.

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tumor. Reduplications of the interatrial septum are an uncommon variant. Commoner is the finding of an interatrial septal aneurysm. With off-axis imaging, this hypermobile structure may have the appearance of abnormal mass. This finding may have clinical importance because atrial septal aneurysm is associated with patent foramen ovale and clinical cerebral ischemia.’

trabeculations and span the ventricle, tensing in diastole when the ventricle is maximally filled. Ruptured chordae usually are associated with a flail portion of the mitral leaflet and appear in the atrium during systole (Fig. 5). The thickened and fibrotic chordae seen in rheumatic disease may simulate the appearance of a mass. CARDIAC TUMORS

Right Ventricle

The right ventricle is by definition a heavily trabeculated chamber. The moderator band, a normal structure in the inlet portion of the ventricle, is seen well on the four-chamber view, and is one of the defining features of the right ventricle. In the presence of severe right ventricular hypertrophy, this muscular band may be very prominent. The tricuspid valve has septal attachments, and the morphology of the papillary muscles is variable. Left Ventricle

Normally, the left ventricle is smooth walled. In the presence of hypertrophy, the coarse trabeculations that develop may be confused with thrombus. Papillary muscles rarely may be displaced apically. Ectopic chordae or “heart strings” may traverse two

Clinical recognition of secondary and primary tumors often occurs late in the course of the disease because of relatively low index of suspicion. In metastatic disease, establishing a secure diagnosis is not merely an academic exercise, because palliation of the hemodynamic effects of secondary cardiac involvement can be achieved by such interventions as pericardial drainage or surgical debulking along with focused oncologic thera ~ yFurthermore, .~ many primary cardiac tumors, although rare, are now curable with early diagnosis, or can be palliated by operation. A high index of suspicion is essential to make an early diagnosis of cardiac involvement in patients with known neoplasm and in patients with right- or left-sided embolic events. Recent developments in echocardiographic imaging and, specifically TEE, have greatly facilitated noninvasive diagnosis. Hemodynamic assessment in the presence of

Figure 5. Ruptured chord attached to anterior leaflet of rnitral valve. LA = left atrium; ALMV = anterior leaflet of rnitral valve; C = ruptured chord.

TEE EVALUATION OF INTRACARDIAC AND PERICARDIAL MASSES

flow disturbances cardiac tumors also can help dictate therapy. Two-dimensional echocardiography can define tumor anatomy and determine suitability for resection. Those tumors that are suitable for surgical excision can be evaluated perioperatively to guide surgical therapy, and can be re-evaluated postoperatively to detect recurrence. In particular, TEE provides an excellent view of the left atrium and also permits detection of intracardiac masses with higher resolution and sensitivity than transthoracic echocardiography.2 In one study of 17 patients investigated for cardiac tumor,5* transthoracic echocardiography showed 4 false-positives and 2 false-negatives, whereas TEE resulted in only 1 false-positive and no false-negatives. Thus, TEE has superior specificity and sensitivity for diagnosing cardiac tumors. Three-dimensional reconstruction has been performed to enhance the anatomic information obtained from TEE in the context of myxoma.28,49

Secondary Cardiac Tumors Metastatic disease involving the heart is relatively common, and postmortem studies suggest cardiac involvement in up to 5% cases. Although most malignancies can metastasize to the heart, carcinoma of the breast

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and lung, leukemia, lymphoma, and melanoma have the highest incidence of cardiac inv~lvernent.~~ Metastatic disease affects the pericardium more frequently than the heart itself, occurring in up to 15%of patients with malignancies at Consequently, pericardial effusion is the commonest echocardiographic finding in patients with metastatic cardiac disease. Clinical recognition of secondary cardiac involvement indicates diffuse spread of the primary malignancy; isolated cardiac or pericardial involvement is uncommon. Intramyocardial infiltration is suggested by localized changes in echocardiographic density or a regional wall motion abnormality but may be missed if the echodensity of tumor is similar to that of the surrounding myocardium. In this situation, color flow Doppler or newer contrast techniques may reveal abnormal v a s c ~ l a r i t y In . ~ advanced disease, myocardial infiltration may coexist with pericardial disease (Fig. 6). Metastatic intracavitary masses are commoner on the right than the left side of the heart and often occur as a consequence of hematogenous spread, including the inferior vena cava. TEE is useful because it provides excellent visualization of the venous inflow to the heart. Among the tumors that metastasize by this route, renal cell carcinoma (hypernephroma) is commonest. Less common are hepatoma, Wilms’ tumor, and uterine leiomy-

Figure 6. Transthoracic echocardiogram demonstrating a large pericardial effusion with infiltration of the visceral pericardium anterior to the right ventricle with metastatic melanoma (arrow). PE = pericardial effusion. (Courtesy of Mark Highman, MD, San Mateo, CA)

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oma. TEE can guide resection of an abdominal primary tumor that has extended into the right heart by way of the inferior vena cava and monitor for embolization (Fig. 7). Rarely, carcinoma involving the lung (primary or secondary) can invade the pulmonary veins34,48 and consequently reach the left atrium. Direct invasion of the heart by primary mediastinal, pulmonary, or subdiaphragmatic tumors is rare. Secondary tumors also can invade the heart by lymphatic Primary Cardiac Tumors

Metastatic cardiac tumors are far commoner than primary disease, which is reported to

have an incidence of less than 0.3%.9 Approximately three-fourths of primary cardiac tumors are benign pathologically. The lack of malignant potential does not necessarily imply an uncomplicated clinical course.15Even benign cardiac tumors are associated with significant morbidity and mortality because of embolization, valvar obstruction, cavity obstruction, or arrhythmogenic effects.23 In contrast to the more frequent pericardial involvement of secondary tumors, primary tumors are more likely to involve the heart itself, most often occurring in the myocardium and less frequently in the endocardium or valves. In general, primary cardiac neoplasms with ventricular involvement tend to

Figure 7. A, lntraoperative transesophageal echocardiogram (TEE) of a uterine leiomyoma (UL) that has spread to the right heart by direct extension by way of the inferior vena cava. B, After resection of tumor. LA = left atrium; RA = right atrium.

TEE EVALUATION OF INTRACARDIAC AND PERICARDIAL MASSES

be intramural, whereas atrial tumors are often intra~avitary.~~ Intracavitary and pericardial tumors present as echodense structures within the cardiac chambers or pericardial spaces and are easier to detect than intramyocardial metastases. Echocardiography is useful in the diagnosis and response to therapy of primary cardiac tumors. Because these are rare diseases, however, there are few published data on guidelines for the use of echocardiography. Although echocardiography, especially TEE, is very sensitive for detecting abnormal masses, there are few specific echocardiographic features that facilitate diagnosis. One exception is that pulmonary venous involvement can suggest primary extraskeletal osteosarcoma, but histologic examination almost always is required to make a diagnosis. Nevertheless, echocardiography provides important information that guides invasive techniques, including the site of origin of tumor, any evidence of valvular involvement, and pericardial i n ~ o l v e m e n t . ~ ~ Primary Cardiac Malignant Tumors

Primary cardiac malignancy is rare. Such lesions are commoner in the adult than the child, and are seen in the right heart more frequently than the left.41Although most are sarcomas (usually angiosarcoma or rhabdomyosarcoma), extraskeletal osteosarcomas, lymphosarcomas, fibrosarcomas, liposarcomas, and leiomyosarcomas also occur. Most primary cardiac malignancies are intramural and tend to grow rapidly, invading the pericardium and surrounding structures. They are destructive and may cause rupture of the myocardial wall. They metastasize often either by direct invasion of adjacent structures or by hematogenous spread. They are associated with high mortality. Angiosarcoma. This tumor is the commonest primary cardiac sarcoma and occurs more often in men than in women.31The right atrium is involved most often, where the appearance can resemble m y ~ o m a although ,~~ the left atrium also may be involved." The clinical presentation is usually with right heart failure, although nonspecific symptoms can occur in i ~ o l a t i o nTEE . ~ ~ has been used to make the diagnosis and to guide biopsy.19,27, 55 Metastases are common, and, although the prognosis used to be exclusively poor, surgical therapy now can be successfu1.42

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Rhabdomyosarcomas. Both sides of the heart are involved equally, and multiple intracardiac tumors are reported." 30 Patients present with breathlessness, chest pain, or emboli~m.~O Valvular obstruction and stenosis can result from this tumor. Extraskeletal Cardiac Osteosarcomas. These tumors develop near the junctions of the pulmonary veins and can extend from the left atrium into the pulmonary veins. This feature can distinguish them at TEE from left atrial myxoma. Primary Cardiac Lymphosarcoma. These lesions may cause heart failure, outflow or inflow obstruction, conduction abnormalities, 20, 37, 60, 64 Both sides and pericardial effu~ion.'~, of the heart are affected equally, and the tumors may be nodular and multiple or single and large. Primary Benign Tumors Myxomas. Myxomas are the commonest primary cardiac tumors, accounting for approximately 25% of all primary cardiac tumors and 30% to 50% of the histologically benign 41, 47 Myxomas can develop in all cardiac chambers or but three-fourths are reported to occur in the left atrium, with most of the remainder in the right atrium.'" 25, 41, 62 Approximately 90% of atrial myxomas arise from the interatrial septum by a stalk of variable length and mobility, usually on or near the fossa ovalis, yet they can' originate from any wall including the appendage. Approximately 5% of patients have multiple myxomas.41Larger myxomas become pedunculated and remain attached to the septum by the stalk, which ultimately can enable them to prolapse through the mitral annulus during the cardiac cycle. The presence of a stalk is not essential to make the echocardiographic diagnosis, however, because 10% of myxomas are sessile and nonpedunculated.61 Some myxomas are spherical and smooth, whereas others develop villous projections, which may increase embolic potential. Intramural hemorrhage and necrosis are common and cause heterogeneous echo density, but significant calcification is less common.25,59 Atrial myxoma usually is suspected by transthoracic echocardiography, but TEE has advantages because it provides excellent atrial vis~alization.~~ It also has the ability to better localize the stalk and define more clearly the characteristic appearance of cysts

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(that represent hemorrhage) and bone islands, and degree of mobility.z,50, 58, 66 Compromise of mitral and tricuspid valve function may occur and is disclosed by transthoracic Doppler imaging, but TEE can define the mechanism precisely.22, 24 Moreover, TEE may identify smaller, multiple myxomas. The typical appearance of myxoma is that of a spherical mass attached by a stalk to the endocardia1 surface of the fossa ovale, often with fronds, cysts, and a speckled appearance. Myxomas that develop at other sites can be misdiagnosed as other tumors or even thrombi (Fig. 8).36Larger myxomas tend to be mobile, and, therefore, large atrial masses that are immobile are unlikely to be myxomas. Although these echocardiographic features can be strongly suggestive of the diagnosis of myxoma, a final diagnosis requires histologic confirmation from embolized tissue or the surgical specimen following excision. In most cases, patients with left-sided myxomas should be referred for early surgery, especially when the patient has presented with embolic events, or the tumor has a highly mobile frondlike appearance, which suggests high embolic potential. Papillary Fibroelastomas and Lambl's Excrescenses. These tumors are small pedunculated tumors that usually are 1 cm smaller in diameter than that which can occur anywhere in the heart but most often are attached to

cardiac valves.3,45, 57 They have a central core with multiple fronds and can embolize.18,21, 26, They usually are clinically silent and, therefore, often present unexpectedly at echocardiography or at cardiac surgery.8,l3 The typical findings are of small mobile homogeneous echo-dense structures that are attached to valves or chordal structure^.^^ The main differential diagnoses are of vegetations or myxoma. Surgery is recommended for leftsided fibroelastomas, but there is a lack of consensus on the need for surgery when the mass is in the right heart (Fig. 9). Lambl's excrescences are small fibrous strandlike masses attached to any of the valves and occur with aging. They have not been associated clearly with embolic events and should not be confused with vegetations. Cardiac Lipomas. Cardiac lipomas are benign polypoid tumors of varying size, which most frequently are identified in the right atrium and left ventricle and are uncommon on valves, but they have the potential to cause valvar obstruction.16,57 The nature of the attachment also varies: some are pedunculated, whereas others are sessile.58Thus, an echocardiographic diagnosis is problematic because lipomas can mimic myxomas, fibromas, papillary fibroelastomas, and thrombi in their appearance. Other imaging techniques or histology may be required to make the diagnosis.

Figure 8. Transthoracic (A) and transesophageal (B)views of myxoma with atypical site of attachment to the atrial side of the anterior mitral annulus and causing mitral valve obstruction. LA = left atrium; LV = left ventricle.

TEE EVALUATION OF INTRACARDIAC AND PERICARDIAL MASSES

Figure 9. Fibroelastoma of the tricuspid valve. RA atrium; LV = left ventricle; F = fibroelastoma; TV

Rhabdomyomas. Rhabdomyoma is the commonest benign cardiac tumor in children and tends to occur in the ventri~1e.I~ Most patients have multiple tumors, which can be large, obstruct valves, and cause arrhythmia and ventricular arrhythmias.l0 There is a strong association between rhabdomyomas and tuberous sclerosis; consequently, echocardiographic screening is advised for patients with tuberous sclerosis. Rhabdomyomas classically present on two-dimensional echocardiography as multiple intracavitary and intramural masses, which have greater echodensity than adjacent myocardium; however, biopsy may be required to confirm the diagnosis in the case of single masses. Fibromas. Fibromas are the second commonest benign pediatric cardiac tumors and are rare in adults.41These unencapsulated tumors usually are approximately 5 cm in diameter and often are found in the ventricles or in the atrioventricular ring. At echocardiography, the single echo-dense structures with central calcification are seen.46They can appear as areas of isolated hypertrophy within the ventricular wall, occasionally with regional wall motion abn0rmality.6~Being single, they can be useful in the differentiation from rhabdomyomas, which usually are multiple, but the differential diagnosis of apical fibroma should include thrombus, which has similar characteristic^.^^ Other benign tumors include teratoma (which occurs mostly in infants), hemangioma, benign mesothelioma of the atrioven-

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right atrium; LA tricuspid valve.

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tricular node (which may cause heart block), lymphangioma, neurofibroma, Purkinje's cell tumor, and granular cell tumor. Extensive discussion of these entities is beyond the scope of this article. Other Benign Masses Pericardial Cysts

Pericardial cysts tend to be asymptomatic and often present as unexpected findings on a chest radiograph. They usually occur in the right costophrenic angle and are of clinical importance because, although they are associated with low morbidity and mortality, they may be misdiagnosed as primary or metastatic malignant tumors.3940, The cysts rarely cause symptoms and usually have a benign prognosis, although there are exceptions to this rule.7,52 Rarely, rupture of a large cyst may lead to tamponade. The cysts are simple fluid-filled structures that are differentiated echocardiographically from more aggressive tumors by the lack of compression of cardiac structures. Echinococcal Cysts

Rarely, intracardiac or intrapericardial rupture of a cyst can cause anaphylaxis and cardiac tamponade. Echocardiographic detection of multiseptated cysts in the left ventricle or interventricular septum is pathogn~monic.~~

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Figure 10. Atrial mass in a transplant patient. The echo-dense structure associated with atrial septum at the site of anastomosis between donor and host atria, causing right atrial obstruction, was found to be hematoma. H = hernatorna; RV = right ventricle. (See also Color Plate 3, Fig. 18.)

Miscellaneous Masses

Miscellaneous masses may occur as a complication of surgery. An example is shown that represents walled-off hematoma at the site of atrial anastomosis of the recipient and donor hearts in a transplant patient. (Fig. 10; see also Color Plate 3, Fig. 18). SUMMARY

Transesophageal echocardiography has assisted the detection of intracardiac masses and, in certain cases, aided our ability to define the specific type of mass. This technique, when coupled with three-dimensional imaging, may help to define infiltration of the wall. TEE aids in the intraoperative management of cardiac tumors. References 1. Agmon Y, Khandheria BK, Meissner I, et al: Frequency of atrial septa1 aneurysms in patients with cerebral ischemic events. Circulation 99:1942-1944, 1999 2. Alam M, Sun I: Transesophagael echocardiographic evaluation of left atrial mass lesions. J Am SOCEchocardiogr 4:323-330, 1991 3. Almagro UA, Perry LS, Choi H, et al: Papillary fibroelastoma of the heart Report of six cases. Arch Pathol Lab Med 106:318-321, 1982 4. Asanuma T, Tanabe K, Yoshitomi H, et al: Clinical

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Address reprint requests to Elyse Foster, MD Division of Cardiology Department of Medicine Echochardiography Laboratory University of California, San Francisco 505 Parnassus Avenue, M344 San Francisco, CA 941434214 e-mail: fosterQmedicine.ucsf.edu