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REVIEW ARTICLE The Echocardiographic Evaluation of Intracardiac Masses: A Review Priscilla J. Peters, BA, RDCS, FASE, and Sean Reinhardt, MD, Camden, New Jersey Echocardiography is an invaluable procedure for the evaluation of intracardiac masses, and can reliably identify mass location, attachment, shape, size, and mobility, while defining the presence and extent of any consequent hemodynamic de- rangement. With careful attention to mass loca- tion and morphology, and appropriate applica- tion of clinical information, echocardiography can usually distinguish between the 3 principal intracardiac masses: tumor, thrombus, and vege- tation. Transesophageal imaging frequently adds additional important information to the assess- ment of mass lesions and should always be con- sidered when image quality is inadequate or per- tinent clinical questions remain unanswered with surface imaging. This review will focus on pri- mary and metastatic tumors of the heart. (J Am Soc Echocardiogr 2006;19:230-240.) Echocardiography is an invaluable procedure for the evaluation of intracardiac masses, and can reli- ably identify mass location, attachment, shape, size, and mobility, while defining the presence and ex- tent of any consequent hemodynamic derangement. With careful attention to mass location and morphol- ogy, and appropriate application of clinical informa- tion, echocardiography can usually distinguish be- tween the 3 principal intracardiac mass lesions: tumor, thrombus, and vegetation. Transesophageal imaging frequently adds additional important infor- mation to the assessment of masses and should always be considered when image quality is inade- quate or pertinent clinical questions remain unan- swered with surface imaging. This review will focus on primary and metastatic tumors of the heart. Echocardiography provides a dynamic evaluation of intracardiac masses, and can identify and evaluate associated abnormalities. In addition, unlike other imaging modalities, a complete echocardiographic study can be performed at the bedside. However, echocardiographic image quality can be subopti- mal, and ultrasound artifact can occasionally be mistaken for an anatomic mass. In addition, echo- cardiography provides a relatively narrow field of view compared with computed tomography and magnetic resonance imaging. Importantly, current echocardiographic techniques cannot character- ize tissue, although contrast enhancement can demonstrate mass vascularity. Careful identification of a mass lesion throughout the cardiac cycle in more than one imaging plane with appropriate machine settings and transducers (eg, high frequency and short focal length for the evaluation of suggested thrombus at the left ventric- ular [LV] apex) is an important first step in evaluat- ing a suggested mass, and will decrease the likeli- hood of misinterpreting artifact as pathology. A thorough understanding of normal anatomy, of nor- mal variants and embryologic remnants, and of the structural changes seen with certain operative and interventional procedures is crucial and will fur- ther avoid misdiagnosis. Lastly, it is important that pertinent clinical and historic information be available and thoughtfully applied to the final echocardiographic interpretation. NORMAL VARIANTS AND MIMICKERS Numerous normal anatomic variants exist that can easily be confused with primary mass lesions. In the LV, webs and heartstrings, prominent or calcified papillary muscles, and dense mitral annular calcifi- cation 1 can mimic abnormal pathology (note: some LV cords may contain conduction fibers of the left bundle). 2,3 Prominent apical trabeculations, true ventricular noncompaction, and the apical form of hypertrophic cardiomyopathy can also be confused with tumors. 4-7 (Ventricular noncompaction and apical hypertrophic cardiomyopathy are significant and distinct disease processes, not normal variants. Administration of contrast agents can help to distin- guish these important entities from mass lesions.) In the left atrium (LA), beam-width artifact can cause interpretive confusion, as can the suture lines asso- ciated with cardiac transplantation. LA cords are rare but well-recognized findings (at least in the pathol- ogy suite). These cords typically originate at the From the Cooper University Hospital. Reprint requests: Priscilla J. Peters, BA, RDCS, FASE, Heart Station/Echocardiography Laboratory, Cooper University Hospi- tal, One Cooper Plaza, Camden, NJ 08103 (E-mail: peters- [email protected]). 0894-7317/$32.00 Copyright 2006 by the American Society of Echocardiography. doi:10.1016/j.echo.2005.10.015 230

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Page 1: Pi is 089473170500996 x

REVIEW ARTICLE

The Echocardiographic Evaluation ofIntracardiac Masses: A Review

Priscilla J. Peters, BA, RDCS, FASE, and Sean Reinhardt, MD, Camden, New Jersey

Echocardiography is an invaluable procedure forthe evaluation of intracardiac masses, and canreliably identify mass location, attachment, shape,size, and mobility, while defining the presenceand extent of any consequent hemodynamic de-rangement. With careful attention to mass loca-tion and morphology, and appropriate applica-tion of clinical information, echocardiography

can usually distinguish between the 3 principal

doi:10.1016/j.echo.2005.10.015

230

intracardiac masses: tumor, thrombus, and vege-tation. Transesophageal imaging frequently addsadditional important information to the assess-ment of mass lesions and should always be con-sidered when image quality is inadequate or per-tinent clinical questions remain unanswered withsurface imaging. This review will focus on pri-mary and metastatic tumors of the heart. (J Am

Soc Echocardiogr 2006;19:230-240.)

Echocardiography is an invaluable procedure forthe evaluation of intracardiac masses, and can reli-ably identify mass location, attachment, shape, size,and mobility, while defining the presence and ex-tent of any consequent hemodynamic derangement.With careful attention to mass location and morphol-ogy, and appropriate application of clinical informa-tion, echocardiography can usually distinguish be-tween the 3 principal intracardiac mass lesions:tumor, thrombus, and vegetation. Transesophagealimaging frequently adds additional important infor-mation to the assessment of masses and shouldalways be considered when image quality is inade-quate or pertinent clinical questions remain unan-swered with surface imaging. This review will focuson primary and metastatic tumors of the heart.

Echocardiography provides a dynamic evaluationof intracardiac masses, and can identify and evaluateassociated abnormalities. In addition, unlike otherimaging modalities, a complete echocardiographicstudy can be performed at the bedside. However,echocardiographic image quality can be subopti-mal, and ultrasound artifact can occasionally bemistaken for an anatomic mass. In addition, echo-cardiography provides a relatively narrow field ofview compared with computed tomography andmagnetic resonance imaging. Importantly, currentechocardiographic techniques cannot character-ize tissue, although contrast enhancement candemonstrate mass vascularity.

From the Cooper University Hospital.Reprint requests: Priscilla J. Peters, BA, RDCS, FASE, HeartStation/Echocardiography Laboratory, Cooper University Hospi-tal, One Cooper Plaza, Camden, NJ 08103 (E-mail: [email protected]).0894-7317/$32.00Copyright 2006 by the American Society of Echocardiography.

Careful identification of a mass lesion throughoutthe cardiac cycle in more than one imaging planewith appropriate machine settings and transducers(eg, high frequency and short focal length for theevaluation of suggested thrombus at the left ventric-ular [LV] apex) is an important first step in evaluat-ing a suggested mass, and will decrease the likeli-hood of misinterpreting artifact as pathology. Athorough understanding of normal anatomy, of nor-mal variants and embryologic remnants, and of thestructural changes seen with certain operative andinterventional procedures is crucial and will fur-ther avoid misdiagnosis. Lastly, it is important thatpertinent clinical and historic information beavailable and thoughtfully applied to the finalechocardiographic interpretation.

NORMAL VARIANTS AND MIMICKERS

Numerous normal anatomic variants exist that caneasily be confused with primary mass lesions. In theLV, webs and heartstrings, prominent or calcifiedpapillary muscles, and dense mitral annular calcifi-cation1 can mimic abnormal pathology (note: someLV cords may contain conduction fibers of the leftbundle).2,3 Prominent apical trabeculations, trueventricular noncompaction, and the apical form ofhypertrophic cardiomyopathy can also be confusedwith tumors.4-7 (Ventricular noncompaction andapical hypertrophic cardiomyopathy are significantand distinct disease processes, not normal variants.Administration of contrast agents can help to distin-guish these important entities from mass lesions.) Inthe left atrium (LA), beam-width artifact can causeinterpretive confusion, as can the suture lines asso-ciated with cardiac transplantation. LA cords are rarebut well-recognized findings (at least in the pathol-

ogy suite). These cords typically originate at the
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Journal of the American Society of EchocardiographyVolume 19 Number 2 Peters and Reinhardt 231

atrial septum and insert into the atrial surface of themitral leaflets; no clinical significance has ever beendescribed, although there is some speculation thatthese strings may be the cause of vibratory murmurs(these cords should not be confused with trueobstructive cor triatriatum.) The most notable de-scription of an atrial cord came from Goforth8 in1926: a 12-cm cord passed from the right atrium(RA) through the interatrial septum, LA, and LV,looping round the anterior mitral cusp, transfixingthe anterior aortic cusp, and finally inserting into theaorta nearby. An interatrial septal aneurysm can bequite provocative and may appear as a cystic massbulging into either atrium.9-12 A dilated coronarysinus can mimic a LA mass in the parasternal long-axis view, as can a prominent descending aorta fromthe apex. A left arm injection of agitated saline willdefine a persistent left superior vena cava draininginto the coronary sinus, which is the most commonstructural anomaly associated with dilatation of thecoronary sinus. Occasionally multiple lobes of theLA appendage can be visualized with transthoracicimaging and can be confused with mass lesions inthe main pulmonary artery; the transverse sinus canbecome prominent in this position as well when alarge accumulation of pericardial fluid is present.Pectinate muscles in the appendage can mimicthrombus. An inverted LA appendage (typically apostoperative finding) can also be confused withtumor.13,14 A hiatal hernia can impinge on both atriaand can be confirmed after ingestion of carbonatedbeverage.

A prominent moderator band or tricuspid papil-lary muscles can create diagnostic confusion in theright ventricle (RV). A fatty tricuspid annulus can bequite prominent. Monitoring lines and pacemakerwires create significant reverberation artifact (butdo occasionally harbor thrombus). The RA can hosta substantial number of congenital remnants, all ofwhich can create confusion for echocardiographers.The crista terminalis is a dense muscle ridge thatextends between the right sides of the superior andinferior caval orifices and continues cephalad toopen into the RA appendage. It can be quite prom-inent, but is commonly seen in the same imageplane as the superior vena cava, which should be aclue to its identity.15 Well known to echocardio-graphers, the eustachian valve is an incompetentvalve flap of variable fullness and mobility thatguards the orifice of the inferior vena cava (IVC)(interestingly, the superior vena cava has no valve).The Chiari network is a network of both coarse andfine fibers with attachments extending from theregion of the crista terminalis to the eustachian valveor floor of the RA. Although not an unusual echo-cardiographic finding, and generally of no clinicalsignificance, these fibers may become the site of

thrombus formation. Entrapment of a catheter in the

network has been described, requiring thoracotomyfor retrieval; several recent reports describe entan-glement of an atrial septal defect closure device andseptal ablation guidewire in the network.16-18 Thenetwork itself is generally quite mobile, but typicallydoes not prolapse through the tricuspid orifice.

Pectinate muscles can be seen to layer the interiorof the RA appendage and the free wall of the RA and,when prominent, can mimic thrombus. Venousvarices (clumps of veins) of the heart, rare lesions ofunknown incidence and with no distinct or specificechocardiographic characteristics, are surprisinglywell described in the pathology literature.19 Theyalmost always occur in the RA at the posteroinferiorborder of the fossa ovalis. They are clumpy, dilatedvenous channels with irregular borders. Reports ofthe echocardiographic diagnosis of these lesionssuggest they closely resemble myxomas.20 As notedpreviously, a hiatal hernia can mimic a mass lesioncompressing the atria, but the “mass” should opacifywhen the patient drinks a carbonated beverage.Monitoring lines and pacer wires will also be evidentin the RA and can occasionally become coiled,simulating a mass.

Fatty infiltrate of the atrial septum (“lipomatoushypertrophy”) is preferential to the RA. Even thoughit can occasionally be quite prominent (�6 cm) andis a true cardiac mass, it should be distinguishablefrom a tumor by typical location and shape. Fattypically spares the fossa ovalis producing a charac-teristic dumbbell appearance, with infiltrate in thesuperior and inferior portions of the atrial septum,with the preponderance of fat typically in the supe-rior (cephalad) portion of the septum. When theatrial septum is massively infiltrated by fat, theamount of adipose tissue in other parts of the heartis always increased, particularly the RV epicardialsurface. In the series described by Shirani andRoberts,21 hearts with widely distributed, massivedeposits of fat actually floated in water. When thereis diagnostic uncertainty regarding the cause of theinfiltrate in the atrial septum, (as might be true ifthere is concern about metastatic disease), magneticresonance imaging can easily distinguish fat fromthrombus and neoplasm.

Certain valvular structures can be confused withmass lesions as well. The nodules of Arantius andlunulae, and the threadlike Lambl’s excrescences(Figure 1), which are commonly found on the aorticvalve in patients older than 60 years, can be con-fused with vegetations. Redundant supporting appa-ratus and redundant leaflet tissue of the mitral valvemay mimic vegetations as well. Thoughtful applica-tion of clinical information is necessary to distin-guish degenerative processes from infectious mech-anisms. Excess epicardial fat may be confused withtumor contained in the pericardium, as can fibrinous

debris in free pericardial fluid. Atelectatic segments
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Journal of the American Society of Echocardiography232 Peters and Reinhardt February 2006

of lung can be misinterpreted as primary masses inthe pleura.

PRIMARY TUMORS

The first description of an intracardiac mass waspublished in 1559. Before 1960, cardiac tumorswere rarely diagnosed before death. An accuratepremortem diagnosis was not made until 1934,when a primary cardiac sarcoma was identified. Thefirst successful resection of a cardiac myxoma was in1954.22-26 Today, although still rare, cardiac tumorsrepresent an important group of cardiovascular ab-normalities that, at least in the case of myxoma, havethe potential for complete cure with timely diagno-sis and surgical excision.

Primary tumors of the heart and pericardium aredifficult to diagnose because they are so uncommonand because their clinical presentation is so variable.The incidence of primary tumors at autopsy rangesfrom 0.002% to 0.3%.27,28 Approximately 75% ofprimary cardiac tumors are benign. In adults themost common cardiac tumor is the myxoma; inchildren younger than than 15 years, the mostcommon tumor is the rhabdomyoma.28 Rhabdomy-omas are strongly associated with tuberous sclerosis.Of the primary malignant tumors, sarcomas are byfar the most common, including angiosarcoma,rhabdomyosarcoma, and fibrosarcoma. Mesothelio-mas and primary intracardiac lymphomas compriseapproximately 6% of the malignant tumors of theheart.29 Primary malignant cardiac tumors are rare inchildren.

The most common cardiac tumor is the myxoma,accounting for 25% of all cardiac neoplasms and 50%

Figure 1 Transesophageal long-axis view of left ventricularoutflow tract (LVOT) and aortic (AO) valve, demonstrat-ing threadlike Lambl’s excrescence (arrow) prolapsingthrough valve in diastole.

of the benign tumors of the heart in adults (Figure 2).

Approximately 75% of myxomas are found in the LA,with 20% in the RA and the remaining 5% equallydistributed between the LV and RV.29-33 Valvularorigin of these tumors is uncommon. Typicallymyxomas are pedunculated (approximately 10% aresessile) and attached in the region of the fossa ovalis.Depending on the size, the tumor may prolapsethrough the mitral orifice in diastole, occasionallyproducing a tumor “plop” and accounting for thecommon misdiagnosis of mitral stenosis. Myxomasare usually globular and irregular in shape (grapecluster), and appear heterogeneous on echocardiog-raphy, occasionally demonstrating cavitations andprotruding frondlike extensions. Color flow andspectral Doppler are useful to evaluate functionalobstruction to LA emptying. Work by Malekzadehand Roberts34 suggests that myxomas grow on aver-age 1.8 cm or 14 g each year.

Myxomas usually present between the ages of 30and 60 years, and can be sporadic, familial, orcomplex (syndrome myxoma). The sporadic varietyis by far the most common. The familial varietyconstitutes approximately 7% of all myxomas, pre-sents earlier in life, may be part of a syndrome(Carney’s complex, NAME, LAMB), frequently in-cludes multiple tumors in several chambers, and hasa high rate of tumor recurrence.27 If the patient isyoung with multiple tumors, screening of first-de-gree relatives is indicated. The complex variety, alsoknown as Carney’s complex, may include a combi-nation of the following: (1) multiple pigmented skinlesions (lentigines); (2) breast adenomas; (3) skinmyxomas; (4) endocrine overactivity (eg, pituitaryadenomas); and (5) cardiac myxomas, often multi-ple.35,36 Syndrome myxoma is also occasionally re-ferred to as the NAME syndrome (nevi, atrial myxoma,myxoid neurofibroma, ephelides) or the LAMB syn-drome (lentigines, atrial myxoma, and blue nevi).36

Figure 2 Transthoracic apical 4-chamber view demonstrat-ing typical left atrial myxoma (arrow). LV, Left ventricle.

Transesophageal imaging is usually indicated when a

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Journal of the American Society of EchocardiographyVolume 19 Number 2 Peters and Reinhardt 233

myxoma is suggested to characterize the morphol-ogy and extent of the mass, and to exclude thepresence of additional tumors. Surgical removal forall varieties of myxomas is the treatment of choiceand intraoperative transesophageal echocardiogra-phy post-tumor excision is indicated to identify anyresidual tumor fragments. Careful follow-up is alsoindicated in patients with familial or syndromemyxoma as the tumor recurrence rate for thesepatients may be as high as 21%.37

Papillary fibroelastomas are uncommon cardiacneoplasms of unknown prevalence.29 They are gen-erally small (�1 cm), usually single, almost alwaysattached to valve surfaces, and may be pedunculatedwith some mobility (Figures 3 and 4). Recently, Sunet al38 of the Cleveland Clinic published a compre-hensive review of 162 patients with fibroelastomasidentified during a period of 16 years, 141 of whomunderwent echocardiography. Approximately 45%of the tumors were on the aortic valve (24 on theright coronary cusp (RCC), 6 on the left coronarycusp (LCC), and 19 on the non-coronary cusp (NCC)with 40 tumors on the aortic side of the valve. Atotal of 40 tumors were identified on the mitral valve(23 on the anterior and 17 on the posterior leaflet),with 32 on the atrial surface of the leaflets. Thesetumors can also attach to the supporting apparatusof the mitral valve. Typical echocardiographic fea-tures of fibroelastomas were identified, including:(1) the tumor is round, oval, or irregular in appear-ance with well-demarcated borders and a homoge-neous texture; (2) most are small (99% � 20 mm);(3) many have stalks, and those with stalks aremobile; and (4) fibroelastomas may be single ormultiple and are most often associated with cardiacvalve disease, although in no patient in this serieswas the fibroelastoma believed to be responsible forthe valve disease. Although fibroelastomas are often

Figure 3 Transthoracic basal short-axis view demonstrat-ing fibroelastoma of pulmonary valve (arrow). Ao, Aorta;LA, left atrium; PA, pulmonary artery; RVOT, right ven-tricular outflow tract.

diagnosed incidentally, cerebral embolization, an-

gina, sudden death, acute myocardial infarction,pulmonary emboli, and retinal artery emboli relatedto fibroelastomas have been described; in the Cleve-land series, 23 of 26 patients with prospectivelydiagnosed tumors had symptoms that could beattributable to embolization.38-43 Echocardiographyis a reliable means of evaluating the extent andanatomic attachment of these very small tumors, butmany go undetected. In the Cleveland series, 141patients had echocardiograms, and 48 patients hadfibroelastomas confirmed by pathologic examina-tion that were not visible on echocardiographybecause they were less than .2 cm. The Clevelandauthors reasoned that the tumors went unidentifiedbecause: (1) the tumor was masked by an associatedlesion; (2) the tumor was too small; (3) the exami-nation was not done carefully with a significantindex of suspicion; or (4) there were no significantcharacteristics that enabled differentiation of fibro-elastoma from degenerative valve disease. Surgicalexcision may be indicated in patients with large,mobile, left-sided tumors.

Rhabdomyomas are the most common cardiactumor of infants and young children, are commonlymultiple, and occur with equal frequency in the LVand RV.44,45 As previously noted, rhabdomyomashave a strong association with tuberous sclerosis, afamilial neurologic syndrome characterized byhamartomas, epilepsy, mental retardation, and skinlesions. One study has indicated that 80% of patientswith cardiac rhabdomyomas have tuberous sclero-sis, and 60% of patients with tuberous sclerosisyounger than 18 years have cardiac rhabdomyo-mas.45 When associated with mechanical complica-tions such as outflow tract obstruction, surgicalexcision may be indicated. Spontaneous resolutionof these tumors is well described, however, andsurgical intervention is usually not necessary in the

Figure 4 Transthoracic 4-chamber image demonstratingfibroelastoma of tricuspid valve (arrow). RA, Right atrium;RV, right ventricle.

asymptomatic patient.46,47

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Journal of the American Society of Echocardiography234 Peters and Reinhardt February 2006

Fibromas are found primarily in the LV and aretypically intramural.27 They commonly invade theseptum, anterior apex, and free wall, and mayappear as markedly disproportionate, irregular hyper-trophy. These tumors are histologically benign butfrequently have a malignant course, accounting forlethal ventricular dysrhythmias and intractable heartfailure associated with dyspnea and fatigue.48-50 Fibro-mas localized to the apex can be confused withthrombus or true apical hypertrophy, but perhapscan be distinguished by their abnormal texture.Although successful surgical resection is commonnow, these tumors can be extensively infiltrative,and resection may cause further myocardial dysfunc-tion. Transplant has been described as an alterna-tive.49

Lipomas are tumors composed of mature fat cells,and may occur throughout the heart, including thepericardium. Although most are small and of noconsequence, they may be massive (�4 kg) and canoccasionally result in dysrhythmias or conductionabnormalities.51 Most lipomas occur in the subepi-cardium or subpericardium; approximately 25% areintramuscular. The most common sites affected arethe LV, RA, and interatrial septum27 (Figure 5).Intrapericardial lipomas may cause compression ofthe heart and pericardial effusion, or present ascardiac or mediastinal enlargement on chest radio-graph. Surgical excision is indicated when tumorsize and location produce symptomatic complica-tions.

Although lipomas are true neoplasms, the previ-ously mentioned fatty infiltrate of the atrial septum(lipomatous hypertrophy) is a nonencapsulated hy-perplastic accumulation of mature adipose tissuewithin the atrial septum. This fatty infiltrate can bequite large (�7 cm), favors the RA, and is a common

Figure 5 Parasternal long-axis view demonstrating lipoma(arrow) at base of heart. Tumor was confirmed by magneticresonance imaging. Ao, Aorta; LV, left ventricle.

finding in women who are elderly and obese.21 A

variety of atrial dysrhythmias have been attributed tothis infiltrate, but a definite cause-and-effect relation-ship has not been established.21 Echocardiographersshould recognize this lesion by its characteristicdumbbell shape, the result of sparing of the fossaovalis (Figures 6 and 7). Magnetic resonance imagingis reliable at characterization of fat if diagnosticissues remain.

Angiomas, teratomas, and mesotheliomas of theatrioventricular (AV) node, and endocrine tumorsare extremely rare, representing less than 7% of allcardiac tumors.29 Hemangiomas are commonlyfound in the interventricular septum or the AV node,

Figure 6 Four-chamber view by transthoracic imagingdemonstrating right atrial mass lesion in superior portion ofatrial septum (arrow). There is thickening of inferior por-tion of atrial septum as well. LV, Left ventricle.

Figure 7 Transesophageal bicaval view from same patientas in Figure 6, demonstrating massive fatty infiltrate of atrialseptum. Note preponderance of fat involves right atrial(RA) side of interatrial septum. There is sparing of fossaovalis. Because patient had history of melanoma, magneticresonance imaging was done to confirm lesion was fat. LA,Left atrium.

where they can cause complete heart block and

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Journal of the American Society of EchocardiographyVolume 19 Number 2 Peters and Reinhardt 235

sudden death. Angiomas are vascular tumors, andmyocardial contrast echocardiography has beenparticularly useful in determining their vascularnature.52-54 Teratomas are usually found in themediastinum. Only rarely are they intracardiac,and are usually within the pericardial space. Thesetumors most commonly occur in children. Terato-mas have elements of all 3 germ cell layers, andcan have skin, hair, and muscle. Large bloodypericardial effusions causing hemodynamic com-promise are a well-described presentation of thisuncommon tumor.55,56 Fetal echocardiographyhas been useful in identifying mediastinal massescausing cardiac compression, with associated ac-cumulations of pericardial fluid.

About one fourth of all primary cardiac tumors aremalignant, and nearly all are sarcomas.29 Sarcomasmost commonly occur in patients between the agesof 30 and 50 years, are unusual in children, aretypically found in the right heart chambers, andoccur equally in men and women.27 There areseveral unsettling reports of sarcomas developingaround surgically implanted Dacron grafts or pros-thetic valves, both in the heart and in other periph-eral vascular sites.57-60 Apparently, as reported byOppenheimer et al,61 the common denominator oftumor-producing materials is a long-chain polymerstructure, such as Dacron. Cardiac sarcomas arecharacteristically very aggressive tumors and, oncediagnosed, are associated with a precipitous down-hill course. They grow rapidly, and death is usuallythe result of widespread infiltration of the myocar-dium or extensive distant metastasis. Sarcomas maycause right heart failure as a result of obstructionanywhere in the right heart inflow/outflow tract;penetration into the pericardial space and subse-quent pericardial effusion may occur. Dysrhythmiasare common. Cardiac findings are determined bythe location of the tumor. A recent report by Planaet al62 described a patient with angiosarcomacompressing the left upper pulmonary vein andLA appendage, invading the anteroseptal surfaceof the LA with extensive involvement of the RA aswell. This patient presented with invasive hemo-dynamics consistent with mitral stenosis (pulmo-nary wedge-LV gradient) and effusive-constrictivepericarditis. Echo characteristics of sarcoma arenot specific, but, less like a myxoma, sarcomasmay attach at any site in the chamber, and manyare sessile. A heterogeneous mass lesion in theright heart associated with dysrhythmia or con-duction disturbance should include sarcoma inthe differential. Although not commonly consid-ered because these tumors metastasize so aggres-sively, cardiac extirpation, transplantation, andextensive right heart reconstruction have been

performed with modest success.63,64

METASTATIC TUMORS

Whereas primary tumors of the heart are rare,cardiac metastases have been described in up to 20%of patients with malignancies of other organ sys-tems, and are up to 40 times more common thanprimary tumors.29 No malignant tumor preferen-tially metastasizes to the heart, with the possibleexception of malignant melanoma, which involvesthe heart in up to 50% of patients.65 Cardiac metas-tases are encountered typically in patients withwidespread systemic tumor dissemination; even inthis setting, the heart may still be spared tumordeposition because of vigorous cardiac contractilityand rapid coronary blood flow.

Tumors spread to the heart by lymphatic chan-nels, direct/venous extension, and hematogenousspread.66 The tumors that most commonly manifestcardiac metastasis are lung, breast, ovarian, kidney,leukemia, lymphoma, esophageal, and, as noted,melanoma.27,29 Although solid intracardiac metasta-sis from melanoma is well described, most com-monly cardiac extension of melanoma is subclinicaland manifests as “charcoal” heart, with tumor stud-ding the pericardial surface.67 Both benign andmalignant tumors can extend to the heart throughthe IVC. The most commonly reported benign tu-mor with IVC intracardiac extension is intravascularleiomyomatosis of pelvic or uterine origin. Of alltumors that metastasize to the heart by the IVC,renal cell carcinoma (hypernephroma) is the mostcommon, with up to 43% of patients with this tumordemonstrating RA involvement68 (Figures 8 and 9).Wilms’ tumor (common in children), uterine leiomy-osarcoma, and hepatomas may also metastasize tothe heart by the IVC. Their point of origin and

Figure 8 Subcostal view of hypernephroma (*) invadingright atrium (RA) by dilated inferior vena cava (IVC).Tumor is heterogeneous in appearance and abuts superiorborder of IVC.

extension into the IVC usually can distinguish these

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Journal of the American Society of Echocardiography236 Peters and Reinhardt February 2006

metastases from the typical myxoma, and this attach-ment is best imaged in the subcostal plane. Carcino-mas of the lung and breast commonly invade theheart, either by direct extension or by lymphaticchannels, given the proximity of the heart to medi-astinal lymphatic channels27 (Figure 10).

Pericardial metastasis occurs more often thanmyocardial invasion, and manifestations includepericarditis, pericardial effusion with and withouttamponade, and atrial dysrhythmias.27 Pericardialeffusion is overall the most common echocardio-graphic finding in metastatic disease. The effusionmay contain solid material adherent to the visceralor parietal pericardium; these masses may be tumoror may be clotted blood. As is true of every masslesion identified in the heart or pericardium, cyto-logic examination of pericardial fluid is essential, asnot all pericardial effusions in the setting of otherprimary organ malignancy yield malignant cells.Unfortunately, routine cytologic examination of thefluid is associated with a false-negative rate of per-haps as high as 20%.69 When large effusions associ-ated with tamponade and adherent masses are dis-covered, constrictive physiology may be operantafter pericardiocentesis. Recurrent effusions in thissetting are common, and pericardial window may benecessary. Solid pericardial metastasis that extendsinto cardiac chambers can be very aggressive, withthe tumor expanding rapidly and causing significanthemodynamic derangement, including obstructionto cavity emptying and filling. Because of the aggres-sive nature of metastatic tumors, patients with int-racavitary extension are likely to have symptomsdirectly related to the tumor. Chemotherapy andtumor resection or debulking may alleviate symp-toms and prolong survival.

The carcinoid syndrome (flushing, gastrointes-tinal hypermotility with secretory diarrhea, bron-chospasm associated with wheezing, and carci-

Figure 9 Four-chamber view of hypernephroma nearlyfilling dilated right atrium (RA). LV, Left ventricle.

noid heart disease) results from circulating

humoral substances secreted by the tumor.70 Thediarrhea probably results from circulating serotonin,which is secreted in large amounts by carcinoidtumors; the flushing and bronchospasm are relatedto the release of kinin peptides. Patients with pri-mary carcinoid tumors of the ileum are those whodevelop carcinoid syndrome; only those patients inthat group who have liver metastasis develop thedistinctive lesions of the heart. These lesions arealways located on the right side of the heart andoccasionally on the left as well. When the primarycarcinoid tumor is of a pulmonary bronchus, thecarcinoid valvular lesions may be limited to theleft-sided valves. In this setting, the liver may betumor free.71 Carcinoid valve lesions are character-ized by plaquelike, fibrous endocardial thickeningthat causes retraction and fixation of the tricuspidand pulmonary valve leaflets. Tricuspid regurgita-tion (TR) is a nearly universal finding; tricuspidstenosis, pulmonary regurgitation, and pulmonarystenosis may also occur.70 In an extensive review of76 patients with carcinoid syndrome, Pellikka et al72

from the Mayo Clinic identified 5 patients withleft-sided valve involvement. Carcinoid tumor in-volved the lung in one of those 5 patients; in 3, apatent foramen ovale with right-to-left shunting wasidentified with contrast echocardiography. One ofthose 5 patients had neither lung involvement norshunting on contrast echocardiography, but hadbeen symptomatic for 5 years before the diagnosis ofcarcinoid syndrome was made. Carcinoid involve-ment of all 4 valves was identified, and the patientunderwent replacement of 4 valves. These 5 pa-tients all had typical right-sided valvular lesions aswell.

As noted, the principal cardiac finding of carci-

Figure 10 Transesophageal image at level of aortic valve,demonstrating large mass lesion that has eroded into bothatria and originates outside heart. Patient presented withemboli to both upper extremities, and was subsequentlydetermined to have metastatic lung cancer. LA, Left atrium;RA, right atrium.

noid involvement of the heart is TR. The mechanism

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of TR is well described in a review by Roberts.70 Theplaquing occurs nearly entirely on the downstream(ventricular) side of the septal and posterior tricus-pid leaflets. On the anterior tricuspid leaflet thedeposits can occur on both sides, which results inadherence of the leaflet to the underlying muralendocardium and in significant valvular incompe-tence and occasionally some degree of stenosis aswell. The dominant pulmonic valve lesion tends tobe stenosis. Carcinoid is the only condition in whichboth right-sided valves are uniformly involved, andthe lesions are pulmonary stenosis and TR. Thecoexistence of these lesions is particularly unfavor-able because the pulmonic stenosis increases theTR. As noted in the review of Pellikka et al,72 thespectral Doppler display of the TR was characterizedby a fairly low velocity (2.5 M) dagger-shaped pro-file, with early peaking and rapid fall-off, consistentwith rapid equalization of RA and RV pressures. Thetypical valve morphology was of rigid leaflets fixedin a semi-open position. Doming of the tricuspidleaflets was not noted in any patients in this series.Surgical replacement of dysfunctional valves is de-scribed, but the mortality appears to be fairly high(30-day mortality of 35% in one series and 56% inanother).73,74 In addition, carcinoid plaquing onbioprostheses has been observed at autopsy.75

Although carcinoid syndrome with valvular plaquedeposition is the most common cardiac consequenceof carcinoid tumor, solid tumor metastasis may occa-sionally occur. In a series of 11 patients with patho-logically confirmed myocardial metastasis, 9 intra-cavitary tumors were detected by echocardiographyin 6 of the 11 patients.76 The mean tumor size was2.4 cm. Of the 9 tumors, 4 were located in the LVmyocardium, 4 in the RV myocardium, and one inthe interventricular septum. The tumors were ho-mogeneous and none were pedunculated or mobile.

Figure 11 Two-chamber transgastric transesophagealechocardiographic image. LA, Left atrium; LV, left ventri-cle; MV, mitral valve.

Six macroscopic tumors were noted at autopsy, with

a mean size of .35 cm. None of those tumors weredetected by echocardiography, even on retrospec-tive review. Interestingly, although all 11 patientshad carcinoid syndrome, the typical valve lesionswere present in only 8 patients.

Although not true neoplasms, cysts are occasionallyfound within the heart and pericardium. The mostcommon cysts that have described echocardiographiccharacteristics are pericardial cysts, echinococcal (hy-datid) cysts, and blood cysts. Echinococcus is a parasiteendemic to Greece and Turkey, and intracardiaccysts result from the larval stage of the parasite.Intracardiac cysts account for less than 2% of allhydatid disease, and invade the myocardium by thecoronary circulation. They are found in the LV freewall, the interventricular septum, the RV, and thepericardium. Transesophageal imaging is indicatedfor the evaluation of these lesions. They are usuallyseptated and can occasionally calcify. Rupture, dys-rhythmia, and emboli are common and can result indeath. Operation is the treatment of choice.77-81

Pericardial cysts are rounded echolucent structurestypically adjacent to the RA. The diagnosis can bemade on chest radiograph by the identification of arounded mass along the right heart border. Pericar-dial cysts are usually asymptomatic, but can becomequite large and cause compression of the RA and RV,and the surrounding mediastinal structures, includingthe bronchus and esophagus. Operation is indicatedwhen significant symptoms dominate the clinicalpicture.82-85 Blood cysts are presumed congenitalcysts typically found on the lines of closure ofvalvular endocardium (Figures 11 and 12). Theyappear as well-circumscribed masses with thin wallsand an echolucent core. Common findings at infant

Figure 12 Same patient as in Figure 11, in same transgas-tric transesophageal echocardiographic plane, demonstrat-ing well-circumscribed, echolucent structure (arrow) in-volving mitral valve leaflets with extension into supportingapparatus. At operation, lesion was blood cyst. LA, Leftatrium; LV, left ventricle; MV, mitral valve.

autopsy, these cysts are rare in adults, and only a few

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reports of prospective echocardiographic diagnosisare available. There is no consensus as to the origin ofblood cysts, but the cysts may form during valvedevelopment as a result of blood pressed andtrapped in crevices that later seal. Because bloodcysts are so rare, consensus is also lacking concern-ing appropriate treatment. Careful echocardio-graphic monitoring of the cysts for change in sizeand for the assessment of change in cardiac functionmay be appropriate, and operation may be indicatedwhen the cysts are noted to cause cardiac dysfunc-tion.86-89

Because echocardiography is the procedure ofchoice for the evaluation of intracardiac masses,techniques must be meticulously applied and datacautiously interpreted. The mandate for the echo-cardiographer is appropriate knowledge of and care-ful attention to cardiac anatomy, use of multiplescan planes, and sound application of clinical infor-mation.

REFERENCES

1. Harpaz D, Auerbach I, Vered Z, Motro M, Tobar A, Rosen-blatt S. Caseous calcification of the mitral annulus: a ne-glected, unrecognized diagnosis. J Am Soc Echocardiogr2001;14:825-31.

2. Tamborini G, Pepi M, Celeste F, Muratori M, Susini F,Maltagliati A, et al. Incidence and characteristics of left ven-tricular false tendons and trabeculations in the normal andpathologic heart by second harmonic echocardiography. J AmSoc Echocardiogr 2004;17:367-74.

3. Asmussen I, Ornvold K. Strings containing left bundle branchconductive tissue in the left ventricle. Acta Pathol MicrobiolImmunol Scand (A) 1985;93:345-9.

4. Agmon Y, Connolly H, Olson L, Khanderia B, Seward J.Noncompaction of the ventricular myocardium. J Am SocEchocardiogr 1999;12:859-63.

5. Lowery MH, Martel JA, Zambrano JP, Ferreira A, Eco L,Gallagher A. Noncompaction of the ventricular myocardium:the use of contrast-enhanced echocardiography in the diagno-sis. J Am Soc Echocardiogr 2003;16:94-6.

6. Stollberger C, Finsterer J. Left ventricular hypertrabecula-tion/noncompaction. J Am Soc Echocardiogr 2004;17:91-100.

7. Ward RP, Weinert L, Spencer KT, Furlong KT, Bednarz J,DeCara J, et al. Quantitative diagnosis of apical cardiomyop-athy using contrast echocardiography. J Am Soc Echocardiogr2002;15:316-22.

8. Goforth JL. Unique heart anomaly: free fibrous cord passingthrough three heart chambers to the aorta. JAMA 1926;86:1612-3.

9. Mugge A, Daniel WG, Angermann C, Spes C, Khanderia B,Kronzon I, et al. Atrial septal aneurysm in adult patients: amulticenter study using transthoracic and transesophagealechocardiography. Circulation 1995;91:2785-92.

10. Malaterre HR, Cohen F, Kallee K, Deharo JC, Djiane P. Giantinteratrial septal aneurysm mimicking a right atrial tumor. IntJ Card Imaging 1998;14:163-6.

11. Malaterre HR, Kallee K, Deharo JC, Djiane P. Right and leftsided interatrial septal aneurysm mimicking atrial tumor and

stroke. J Am Soc Echocardiogr 1998;11:829-31.

12. Ginon I, Mestrallet C, Barthelet M, Robin J, Andre-Fouet X.A closed interatrial septal aneurysm, filled with blood, mim-icking a tumor in the right atrium. Eur J Echocardiogr 2000;1:289-90.

13. Minich LL, Hawkins JA, Tani LY, Judd VE, McGough EC.Inverted left atrial appendage presenting as an unusual leftatrial mass. J Am Soc Echocardiogr 1995;8:328-30.

14. Toma DM, Stewart RB, Miyake-Hull CY, Otto CM. Invertedleft atrial appendage mimicking a left atrial mass during mitralvalve repair. J Am Soc Echocardiogr 1995;8:557-9.

15. Pharr J, West M, Kusomoto F, Figueredo V. Prominent cristaterminalis appearing as a right atrial mass on transthoracicechocardiogram. J Am Soc Echocardiogr 2002;15:753-5.

16. Goldschlager A, Goldschlager N, Brewster H. Catheter en-trapment in a Chiari network involving an atrial septal defect.Chest 1972;62:345-6.

17. Cooke J, Gelman J, Harper R. Chiari network entanglementand herniation into the left atrium by an atrial septal defectoccluder device. J Am Soc Echocardiogr 1999;12:601-3.

18. Shimoike E, Ueda N, Maruyama T, Kaji Y, Niho Y. Entrap-ment of a guide wire by the Chiari network in a patient withablated idiopathic ventricular tachycardia. J Interv Card Elec-trophysiol 2001;5:219-22.

19. Heggtveit HA. Thrombosed varices of the heart [abstract].Am J Pathol 1966;48:50a.

20. Harrity P, Tazelaar H, Edwards W, Orszulak T, Freeman W.Intracardiac varices of the right atrium: a case report andreview of the literature. Int J Cardiol 1995;48:177-81.

21. Shirani J, Roberts WC. Clinical, electrocardiographic andmorphologic features of massive fatty deposits (“lipomatoushypertrophy”) in the atrial septum. J Am Coll Cardiol 1993;22:226-38.

22. Mahaim I. Les Tumeurs et les Polypes de Coeur. EtudeAnatomo-clinique. Paris: Masson; 1945.

23. Barnes AR, Beaver DC, Snell AM. Primary sarcoma of theheart: report of a case with ECG and pathological studies. AmHeart J 1934;9:480-4.

24. Goldberg HP, Glenn F, Dotter CT, Steinberg I. Myxoma ofthe left atrium: diagnosis made during life with operative andpost-mortem findings. Circulation 1952;6:762-6.

25. Crafoord CL. A case report. In: Lam CR, editor. Proceedingsof international symposium on cardiovascular surgery. Phila-delphia: WB Saunders Co; 1955. p. 202.

26. Effert S, Domanig E. Diagnosis of intra-auricular tumors andlarge thrombi with the aid of ultrasonic echography. DtschMed Wochenschr 1959;84:6-8.

27. Roberts WC. Primary and secondary neoplasms of the heart.Am J Cardiol 1997;80:671-82.

28. Lam KY, Dickens P, Chan ACL. Tumors of the heart: a20-year experience with review of 12,485 consecutive autop-sies. Arch Pathol Med 1993;117:1027-31.

29. Burke A, Virmani R. Atlas of tumor pathology. In: Tumors ofthe heart and great vessels. Third series, fascicle 16. Washing-ton (DC): Armed Forces Institute of Pathology; 1996. p. 231.

30. St John Sutton MG, Mercier LA, Guiliani ER, Lie JT. Atrialmyxoma: a review of clinical experience in 40 patients. MayoClin Proc 1980;55:371-6.

31. Markel ML, Waller BF, Armstrong WF. Cardiac myxoma: areview. Medicine (Baltimore) 1987;66:114-25.

32. Burke AP, Virmani R. Cardiac myxomas: a clinicopathologicstudy. Am J Clin Pathol 1994;100:671-80.

33. Keeling IM, Oberwalder P, Anelli-Monti M, Schuchlenz H,

Demel U, Tilz GP, et al. Cardiac myxomas: 24 years of
Page 10: Pi is 089473170500996 x

Journal of the American Society of EchocardiographyVolume 19 Number 2 Peters and Reinhardt 239

experience in 49 patients. Eur J Cardiothorac Surg2002;22:971-7.

34. Malekzadeh S, Roberts WC. Growth rate of left atrial myx-oma. Am J Cardiol 1989;64:1075-6.

35. Carney JA, Gordon H, Carpenter PC, Shenoy BV, Go VL.The complex of myxomas, spotty pigmentation, and endo-crine overactivity. Medicine (Baltimore) 1985;64:270-83.

36. Mahilmaran A, Seshadri M, Nayar PG, Sudarsana G, AbrahamKA. Familial cardiac myxoma; Carney’s complex. Tex HeartInst J 2003;30:80-2.

37. Van Gelder M, O’Brien DJ, Staples ED, Alexander JA. Famil-ial cardiac myxoma. Ann Thorac Surg 1992;53:419-24.

38. Sun JP, Asher C, Yang XS, Cheng G, Scalia GM, Massed AG,et al. Clinical and echocardiographic characteristics of papil-lary fibroelastomas. Circulation 2001;103:2687-701.

39. Zell DN, Diamond M, Beringer D. Angina and sudden deathresulting from papillary fibroelastoma of the aortic valve. AnnEmerg Med 1985;14:470-3.

40. Kasarskis EJ, O’Connor W, Earle G. Embolic stroke fromcardiac papillary fibroelastoma. Stroke 1988;19:1171-3.

41. Topol EJ, Biern RO, Reitz BA. Cardiac papillary fibroelas-toma and stroke: echocardiographic diagnosis and guide toexcision. Am J Cardiol 1986;80:129-32.

42. Waltenberger J, Thelin S. Papillary fibroelastoma as an un-usual source of repeated pulmonary embolism. Circulation1994;89:24-33.

43. Zamora RL, Adelberg DA, Berger AS, et al. Branch retinalartery occlusion caused by a mitral valve papillary fibroelas-toma. Ophthalmology 1995;119:325-9.

44. Chan HSL, Sonley MJ, Moes CAF, Daneman A, Smith CR,Martin DJ. Primary and secondary tumors of the heart, peri-cardium, and great vessels: a report of 75 cases and review ofthe literature. Cancer 1985;56:825-36.

45. Bass JL, Breningstall GN, Swaiman KF. Echocardiographicincidence of cardiac rhabdomyoma in tuberous sclerosis. Am JCardiol 1985;55:1379-82.

46. Fesslova V, Villa L, Rizzuti T, Mastrangelo M, Mosca F.Natural history and long-term outcome of cardiac rhabdomy-omas detected prenatally. Prenat Diagn 2004;24:241-8.

47. Verhaaren HA, Vanakker O, DeWolf D, Suys B, Francois K,Matthys D. Left ventricular outflow obstruction in rhabdomy-oma of infancy: meta-analysis of the literature. J Pediatr 2003;143:258-63.

48. Cina SJ, Smialek JE, Burke AP, Virmani R, Hutchins GM.Primary cardiac tumors causing sudden death: a review of theliterature. Am J Forensic Med Pathol 1996;17:271-81.

49. Wong JA, Fishbein MC. Cardiac fibroma resulting in fatalventricular arrhythmia. Circulation 2000;101:e168-70.

50. Cho JM, Danielson G, Puga F, Dearani JA, McGregor CGA,Tazelaar HD, et al. Surgical resection of ventricular cardiacfibromas: early and late results. Ann Thorac Surg 2003;76:1929-34.

51. Lang-Lazdunski L, Oroudji M, Pansand Y, Vissuzaine C,Hvass U. Successful resection of giant intrapericardial lipoma.Ann Thorac Surg 1994;58:238-40.

52. Bednarz JE, Spencer KT, Weinert L, Sugeng L, Mor-avi V,Lang RM. Identification of cardiac masses and abnormalblood flow patterns with harmonic power Doppler contrastechocardiography. J Am Soc Echhocardiogr 1999;12:871-5.

53. Kirkpatrick JN, Wong T, Bednarz JE, Spencer KT, Sugeng L,Ward RP, et al. Differential diagnosis of cardiac masses usingcontrast echocardiographic perfusion imaging. J Am Coll

Cardiol 2004;43:1412-9.

54. Lepper W, Shivalkar B, Rinkevich D, Belcik T, Wei K. Assess-ment of the vascularity of a left ventricular mass using myo-cardial contrast echocardiography. J Am Soc Echocardiogr2002;15:1419-22.

55. Tollens M, Grab D, Lang D, Hess J, Oberhoffer R. Pericardialteratomas: prenatal diagnosis and course. Fetal Diagn Ther2003;18:432-6.

56. Tollens T, Casselman F, Devlieger H, Gewillig MH, Vanden-berghe K, Lerut TE, et al. Fetal cardiac tamponade due to anintrapericardial teratomas. Ann Thorac Surg 1998;66:559-60.

57. Okada M, Takeuchi E, Mori Y, Ichihara S, Usui A, Ueda Y. Anautopsy case of angiosarcoma arising around a woven Dacronprosthesis after a Cabrol operation. J Thorac Cardiovasc Surg2004;127:1843-5.

58. Ben-Izhak O, Vlodavsky E, Ofer A, Engel A, Nitecky S,Hoffman A. Epithelioid angiosarcoma associated with a Da-cron vascular graft. Am J Surg Pathol 1999;23:1418-23.

59. Durrleman NM, El-Hamamsy I, Demaria RG, Carrier M,Perrault LP, Albat B. Cardiac lymphoma following mitralvalve replacement. Ann Thorac Surg 2005;79:1040-2.

60. Fyfe BS, Quintana CS, Kaneko M. Aortic sarcoma four yearsafter Dacron graft insertion. Ann Thorac Surg 1994;58:1752-4.

61. Oppenheimer BS, Oppenheimer ET, Stout AP, Willhite M,Danishefski I. The latent period in carcinogenesis by plastics inrats, and its relation to the presarcomatous stage. Cancer1958;11:204.

62. Plana JC, Iskander SS, Ostrowski M, Howell JF, KurrelmeyerK, Raizner AE, et al. Cardiac angiosarcoma: an unusual pre-sentation simulating mitral stenosis and constrictive-effusivepericarditis. J Am Soc Echocardiogr 2003;16:1331-3.

63. Talbot SM, Taub RN, Keohan ML, Edwards N, GalantowiczME, Schulman L. Combined heart and lung transplantationfor unresectable primary cardiac sarcoma. J Thorac CardiovascSurg 2002;124:1145-8.

64. Hoffmeier A, Deiters S, Schmidt C, Tjan TD, Schmid C,Drees G, et al. Radical resection of cardiac sarcoma. ThoracCardiovasc Surg 2004;52:77-81.

65. Mukai K, Shinkai T, Tominaga K, Shomosato Y. The inci-dence of secondary tumors of the heart and pericardium: aten-year study. Jpn N Clin Oncol 1988;18:195-201.

66. Colucci WS, Braunwald E. Primary tumors of the heart. In:Braunwald E, editor. Heart disease: a textbook of cardiovascularmedicine. Philadelphia: WB Saunders; 1980. p. 1501-16.

67. Waller BV, Gottdeiner JS, Virmani R, Roberts WC. The“charcoal heart”: melanoma to the cor. Chest 1980;77:671-6.

68. Almasi GH. Surgery for tumors with cavoatrial extension.Semin Thorac Cardiovasc Surg 2000;12:111-8.

69. Warren WH. Malignancies involving the pericardium. SeminThorac Cardiovasc Surg 2000;12:119-29.

70. Roberts WC. A unique heart disease associated with a uniquecancer: carcinoid heart disease. Am J Cardiol 1997;80:251-6.

71. Kulke M, Mayer RJ. Medical progress: carcinoid tumors.N Engl J Med 1999;340:858-68.

72. Pellikka PA, Tajik AJ, Khandheria BK, Seward JB, CallahanJA, Pitot HC, et al. Carcinoid heart disease: clinical andechocardiographic spectrum in 74 patients. Circulation 1993;87:1188-96.

73. Connolly HM, Nishimura RA, Smith HC, Pellikka PA, Mul-lany CJ, Kvols LK. Outcome of cardiac surgery for carcinoidheart disease. J Am Coll Cardiol 1995;25:410-6.

74. Robiolio PA, Rigolin VH, Harrison JK, Lowe JE, Moore JO,Bashore TM, et al. Predictors of outcome of tricuspid valvereplacement in carcinoid heart disease. Am J Cardiol 1995;75:

485-8.
Page 11: Pi is 089473170500996 x

Journal of the American Society of Echocardiography240 Peters and Reinhardt February 2006

75. Ridker PM, Chartow GM, Karlson EW, Neish AS, Schoen FJ.Bioprosthetic tricuspid valve stenosis associated with extensiveplaque deposition in carcinoid heart disease. Am Heart J1991;121:1835-8.

76. Pandya U, Pellikka P, Enriquez-Sarano M, Edwards WD,Schaff H, Connelly H. Metastatic carcinoid tumor to theheart: echocardiographic-pathologic study of 11 patients.J Am Coll Cardiol 2002;40:1328-32.

77. Ben-Hamda K, Maatouk F, Ben-Farhat M, Betbout F, GamraH, Addad F, et al. Eighteen year experience with Echinococ-cosus of the heart: clinical and echocardiographic features in14 patients. Int J Cardiol 2003;91:145-51.

78. Atilgan D, Kudar H, Tukek T, Ozcan M, Yildirim O, ElmaciTT, et al. Role of transesophageal echocardiography in diag-nosis and management of cardiac hydatid cyst: report of threecases and review of the literature. J Am Soc Echocardiogr2002;15:271-4.

79. Makaryus AN, Hametz C, Mieres J, Kort S, Carneglia J, Man-gion J. Diagnosis of suspected cardiac echinoccosis with negativeserologies: role of transthoracic, transesophageal, and contrastechocardiography. Eur J Echocardiogr 2004;5:223-7.

80. Hassine E, Kraoua S, Marniche K, Bousnina S, Lefi A,Kachboura S, et al. A dead and calcified right ventricularhydatid cyst: the limits of imaging. Presse Med 2003;32:

1802-4.

81. Nisanoglue V, Erdil N, Isik B, Battaloglu B, Alat I. Acuteabdominal aortic embolism caused by rupture of a cardiachydatid cyst. Ann Vasc Surg 2004;18:484-6.

82. Patel J, Park C, Michaels J, Rosen S, Kort S. Pericardial cyst:case reports and a literature review. Echocardiography 2004;21:269-72.

83. Bava GL, Magliani L, Bertoli D, Gorrieri PF, Rimini A,Zaccagnini G, et al. Complicated pericardial cyst: atypicalanatomy and clinical course. Clin Cardiol 1998;21:862-4.

84. Bandeira FC, de Sa VP, Moriguti JC, Rodrigues AJ, Jurca MC,Filho OC, et al. Cardiac tamponade: an unusual complication ofpericardial cyst. J Am Soc Echocardiogr 1996;9:108-12.

85. Antonini-Canterin F, Piazza R, Ascione L, Pavan D, NicolosiGL. Value of transesophageal echocardiography in the diag-nosis of compressive, atypically located pericardial cysts. J AmSoc Echocardiogr 2002;15:192-4.

86. Pelikan HMP, Tsang TSM, Seward JB. Giant blood cyst of themitral valve. J Am Soc Echocardiogr 1999;13:1005-7.

87. Nkomo V, Miller FA. Eustachian valve cyst. J Am Soc Echo-cardiogr 2001;14:1224-6.

88. Sim EKW, Wong ML, Tan KT, Sim SK. Blood cyst of thetricuspid valve. Ann Thorac Surg 1996;61:1012-3.

89. Prasad A, Callahan MJ, Malouf JF. Acquired right atrial bloodcyst: a hitherto unrecognized complication of cardiac opera-

tion. J Am Soc Echocardiogr 2003;16:377-8.