152

Clinical Aspects of Cardiac Tumors H. Tillmanns

Summary

Klinisch e Aspe kte der Ilerztumorcn

Due to surgical advances, cardiac tumors now represent a potentia lly curable form of heart disease . Primar y car diac tumors a re very rar e; secondary, metastatic tum ors are 10-40 times more frequent. The majority (75 %) of prima ry tumors is benign. Cardiac tumors may produce hemodyna mic disturb ances, mechan ical hemolysis, biochemical effects and constitutional symptoms. According to the a natomic location of the tumor , some speci al symptoms and physical findings ca n be grouped with rega rd to perica rdia!, myocard ial, and endocardial involvement. Severa l laboratory dat a (elevated sedimenta lion rate, a nemia, thro mbocytopenia etc.I ma y give additional hints to suggest card iac involvement in neoplastic disease. Among the diagnostic procedures, two-dimensio nal echocardiography has become the most importa nt met hod for noninvasive dete ction of cardiac tumors . The importa nce of invasive procedures has declined by the imp rovement of non-invasive imaging techn iques , but press ure meas ure me nts within the cardio pulmonary syste m, left-sided , pulmona ry angiogra phy as well as corona ry a ngiogra phy give additio nal useful a nd necessar y informatio n prior to surgery. Within the spectrum of differential diagnosis, pa rticula rly mitral, aortic, or tricupid valve disease, bacterial endocarditis, or a utoimmune diseases have to be ruled out. Thus, in the vast majority of cases the correct diagnosis ca n be made. This is crucial for further man agement of the patie nt since the majority of prim a ry ca rdiac tumors can be su ccessfully treated by surge ry.

Herztumoren kdnne n heute groBenteils erfolgreic h chiru rgisch behandelt wer den. was allerdings eine korr ekte Diagnose voraussetzt. Primare Tumoren des ka rdlovaskula ren Syste ms sind sehr selten: kardiale Metastasen von Primar tumoren andere r Organe kommen 1O-40mal hauflger vor. Die Mehrza hl (75'%) der prima ren ka rdialen Tumoren ist gutartig. Die Ha uptma nifestati onen kardialer Tumore n stellen ham odvnam ische Storu ngen , mechanische Ha molyse. biochemische Veran der un gen und unspezifische Allgemeinsymptome dar . Der Lokalisation des Tumors kcnn en spezifische Symptome und Untersuchu ngsbefunde zugeordnet wer den , wie z. B. prakc rdialer Schmerz (Pertkar d). Zeichen der Herzinsuffizienz (Myokard l bzw. lageabhe ngige Synkopen und Herzgerausche oder Embolien IEndoka rdl. Pathologische Labord aten - erhohte Blutk6rp erchensenkungsgeschwindigkeit, Ana mie und Thrombopenie - kdnnen zusatzllch a uf eine kardi ale Beteiligung bei Tumorer kra nkungen hinweisen. Unter den diagnostischen Verfahren hat die zweidime nsiona le Echokar diogra phie die groBte Bedeutung erla ngt; invasive Method en (Pulmonalkapilla rdruckmess ung, Durchlauf-Angiogramm , Koron a r-Angiogramm) konnen zusatzliche, gelegentlich unentbeh rliche Informationen im vo rfeld der chirurgischen Thera pie liefer n. Differentialdiagnostisch sind in erster Linie Mitr al-. Aorte n- und Trikusp idal klappenfehler, bakt erielle Endokar ditis und Autoimmunerkra nkungen (Kollagenosen) a uszuschlieBen. Mit Hilfe der eben genannten Verfa hren kann in der iiberwi egende n Mehrzahl die korrekte Diagnose eines Herztum ors gestellt werden . was fur das weitere thera peutis che Vorgehe n essentlell ist, da die Mehrzahl der prlmaren Herztumoren heute eine r erfolgreichen chiru rgischen Behand lung zugefiihrt warden kan n.

Key w ord s Ca rdiac tumor - Hemodyna mic distur ba nces Mecha nical he molysis - Biochemical effects - Constitutiona l effects - Echocardiograp hy - Ca rdiac catheterizatio n

Introduction Due to surgical adva nces, cardiac tumors now repr esent a potentially curable form of heart disease. The first step, however, is an ea rly, correct diagno sis, an d clinical recognition of cardiac tumo rs is a diagnostic challenge. The tumor s are often misdiagnosed, since they can mimic almost all hem odynami c synd romes and common cardiovascular diseases. This diagnostic problem may be shortl y illustrated by a case report. In October 1987 , a 42 year old male patient was admitted to our clinic with the initial diagnosis of "cons trictive pericarditis". In this pati ent , in 1961 surgical tr eatm ent of aortic coarctation ha d been performed. In 1977 cardiac calcification was detected, and pericardial calcification was suspected . Since summer 1982 the patient experienced

Thorae. eardiovasc. Surgeon 38 (1990) (Special lssuel 152-156 © Georg Thieme Verlag Stuttgart- New York

episodes of dizziness, mostly at night, with maximal dura tion of 1 minut e. Since January 1987 he observed dyspnea. on exertion, increasing to orthopnea during summer, as well as episodes of cold sweat occurring dur ing exercise or at night. The physical examination revea led briefly a low cardiac output state with tachycard ia and low pressure pulse. In addition,jugular venous distention was noted. Aholosystolic murm ur and an early diastolic extra sound were noted over the apex. The electrocardiogra m revealed a sinus ta chycar dia, but did not show any conduction abn or malities. By mean s of echocardiogra phy, a left ventricular tumor (fibroma) was detected; the tumor was attached to the lateral wall of the vent ricle. Chest radiograph revealed extensive calcification in projection on the cardiac silhouette (Fig. 1, upper panel). Left ventricular cinea ngiogra phy showed irregular boundaries of the left ventricular cavity; the aforementioned calcifications extended far into the ventricular myocardium (Fig. I , lower panel). By mean s of

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Med. Univ.-Klinik . Abt. ln n. Med. - Kardiologie, Giessen, FUG

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Fig.2 Gatedthallium-201myocardialscintigram ofthe42-yea r-oldpatient withleftventricularfibroma(systolic image, 30°LAOprojection). After i.v. injection of 2 mCI thallium-20 1,anareaof diminished thallium-20 1uptake can be recognized inthe lateralregion

gated tha llium -201 myocard ial scintigra phy, the extension of the tumor within the cardiac muscle could be demonstrated (Fig. 2); the tha llium-201 scan revealed a lar ge lateral upt ake defect, caused by the tu mor mass invading the lateral wall of the left ventricle. Becau se of the large amount of myocard ium involved, resection of the tumor seem ed not possib le, and cardiac transplantation was performed . The histologic examination

of the tumor tissu e revea led a calcified fibroma invading the lateral wall of the left vent ricular chambe r. Primary tum ors of the heart

cular decompensat ion secondar y to chemothera py; 3) pulmonary involvement from chro nic lung disease (10). The most common cardiovasc ular sym ptoms of cardiac tumors

ar e dyspn ea, syncope, chest pain, palpitations, and conges tive heart failure . The sudden onset of dyspn ea in patients with cance r should alert us to the poss ibility of acute cardiac involvement.

Some special symptoms a nd physical findings of patients with primary or seco ndary cardiac tumors can be

grouped according to the anatomic location of the tumor ; thu s we discern tumors with 1) pericardial involvem ent , 2) myocardial involvement , and 3) endocardial involvem ent

(6,10). Primary tumors of the heart are very rare occurring in

0.002 -0.3% of autopsy specimens (15,1 8, 21,24). Secondary tumo rs (meta stases to the heart) a re 10-40 times more frequent (11, 12,15 , 17); they commonly invade the pericardium and thu s may produce a blood-stained pericardial effusion (11). The major ity, 75 % of primar y cardiac tumors are benign; they occur mor e frequently in women, and the preferential age of onset of symptoms is 30- 60 years (12).

A. Clinical man ifes tatio ns of car diac tumors

What are the man ifestations of card iac tumors? Cardiac tumors may produce 1) hemodynam ic disturbances; 2) mechan ical hemolysis; 3) biochemical effects; 4) constitutional symptoms (6, 10). Hemodynamic disturban ces include 1) a rrhyt hmias; 2) pe ricardial effusion; 3) heart failure (low output syndrome), as was the case in our42 year old pati ent; 4) intracavitary obliterat ion and obstruction; 5) system ic embolism (6). With rega rd to general, constitutional symptoms, gener a lized weakness , weight loss, malaise, easy fatigabil ity, and dyspn ea are symptoms that a re common to man y disease states an d do not give a clue to which organ syste m is involved . These constitutiona l symptoms may be due to a) the und er lying malignant disease; b) ass ociated ca rdiovas-

1) Pericardial involvement

Pericardial tumors may provoke pericarditis or pericardial

effusion resulting io palpitations and dyspnea. Chest pain aggravated by positiona l or res piratory changes suggests the presence of a tumor around the heart; ches t pain cornmonly occurs in patients with cardiac angiosa rcoma seco n-

da ry to myocardial infiltrat ion (3). In pati ents with cancer of other organs, pericar dial friction sound, tachyca rdia, elevated jugular distention, changing electrocar diogram and pulsus paradoxus point at pericar dial involvement. Additional features may be cardiac enlargement (X-ray silhouette) and hepatomegaly. 2) Myocardial involvemen t The spectrum of symptoms of patients with myocard ial tumors is wide. Patients may complain about palpitations,

tachycardia, even Adams-Stokes attacks. The ECG ma y show arrhythmias, primarily atrial fibrillation, an d recurrent supraventricular or ventricular tachycardias . Furthe rmore , the invas ion of the ventricular wall by a tumor sometimes gives rise to ches t pain and the syndrome of myoca rdial infarction with electrocardiographic signs of necrosis .

Conduction abnormalities and av-block, especially in

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Fig. 1 Upper panel: Chest radiographofa 42yearoldpatient with acalcified leftventricular fibroma (300 RAO projection) Lowerpanel:Cineangiogram oftheleft ventricular chamber (300 RAO projection):After injectionof45 ml contrast agent, irregular boundaries of the left ventricularchamber canbedetected,and it isevidentthat the calcified tumormasses extend far beyond the leftventricularcavity and invadetheheart muscle

JI. Tillmanns

Thome. eardiovase. Surgn 38 (1990)

younger pat ients, are noted; angiomas and mesoth eliomas may produce complete heart block and sudden death (1). In the pres ence of extended myocard ial infiltration by the tumor, pat ients may show symptoms of left- or right-sided heart failure, such as dyspnea, peripheral edema, tiredness , and general fatigue (low output syndrome). 3) Endocardial involvement In patients with intracavitary cardiac tumors dizziness ,

nonexertional syncop e, or palpitations related to changes in position are often observed (8). This is probabiy related to tumor obstruction of ventricular outflow or inflow. Myxomas , fibromas , or sarcomas commonly originating in atria may provoke valve obstruction , resu lting in valve regurgita-

tion or stenosis. Right-sided intracavitary tumors may pro voke head ache, visual disturbances, generalized weakne ss, and light-headed spells (7). Left atrial tumors may caus e paroxysmal nocturnal dyspn ea, orthopn ea , acute pulmonary edema, hemoptysis, fatigue, syncope, and sudden deat h (7). The duration of symptoms is commonly short (6). Intracavitary tumors or tumors with valvular neoplastic

B. Lab or atory da ta suggesting cardiac involvement in neoplasti c diseas e

Since 1959 it has been known that left atrial myxomas often product anemia and a high erythrocyte sedim entati on rat e (13). Furth ermore, left atrial myxomas can cause thrombocytopenia , and hypergammaglobulinemia (5). The detection of mechanical hemolysis in patients with heavily calcified aortic valves or in patients with prosth etic valves led to the hypothesis that an emia accompanying left atrial myxoma is also due to mechanical hemolysis (23). The red cells ar e destro yed by the traumatic effect of the tumo r, particularly when this is mobile. Thrombocytopenia which is often observed in patients with left atrial myxoma, is prob ably caused by mechanical thr ombolysis of platelets . By contrast, a raised platelet count, associ ated with polycythemia , can also occur. In patient s with left atria l myxoma, formation of antibodies and hypergammaglobulinemia have been observed (23). Other relevant labor ator y data in patients with cardiac tumors include increas ed 5-hydroxytryptophan and serotonin levels, as well as hypereosinophilia.

implants causing tricuspid and mitral involvement. are usu-

ally associated with patho logical heart murmurs. Murmurs caused by intracavitary tumors typically cha nge with position (5, 20). Leftatri al myxomas may mimic the aus cultatory signs of mitral stenosis; in addition , there may be a systolic murmur of mitral regurgitation. These murmurs are either due to tumor obstruction to valve flow or tumor interference

with valve closure (14). It is common for the signs of mitr al valve disease to be much less impr essive than the degree of pulmonary hypert ension that accompanies them ; this discrepa ncy is useful in suggesting the presence of a myxoma. Tum ors located in the right ventricle may simulate pulmonary steno sis, tricuspid incompet ence, or restrictive car-

diomyopathy (4). In 11 patients with right ventricular tumors, cardiac catheterization data in all patients revealed a grad ient at pulmonary valve or infundibu lar levels, or both , during systole. The tum ors proved to be rh abd omyomas, myxomas, fibrosarcomas. or hamartomas (4). The constitutional symptoms which are so common in patients

Table 1 Diagnosis of cardiactumors 1) Laboratoty studies inclu ding sedimentation rate, red and white blood cell count, and serum proteinelectrophoresis. 2) Electrocard iogram. 3) Chest radiograph. 4) Two-dimensional echocardiogram. 5) Radionuclide stud ies (T 1- 201 scintigraphy, gated blood pool). 6) Computerized tomography. 7) Nuclear magneticresonancetomography. 8) Cardiaccatheterization (pressure measurements, cineangiography, coronaryangiography). 9) Endomyocardial biopsy. 10) Pericardiocentesis(pericardial fluidanalysis). Thediagnostic procedures wh ich have been underlined in this table, yield the largest contributioninestablishing thediagnosisof cardiacneoplasms.

with atrial myxomas , ar e usually abs ent in patients with tumors of the right ventricl e (6). Left ventricular tumor s may mimic findings of aortic stenosis, subaortic stenosis , hyper-

trop hic cardiomyopathy, and endocardial fibroelastosis (9). Intracardiac tumors may give rise to systemic or pul-

monary embolism. The embolism may be due to blood clots which become atta ched to the tum or, or due to fragments of the tumor itself. Tumor fragm ents originating from rightsided tumors such as myxomas, may provoke acute pleuritis

with effusion or pulmonar y hypert ension (in the case of multiple emboli). Systemic emboli from left-sided cardiac tum ors are rather frequent ; they commonly occur with left atrial myxoma (6). The tumors may embolize to the central nervous system, peripheral art eries , viscera, kidney, and rarely to the corona ry arteries. The neurologic syndromes due to systemi c embolization include transient ischemic attacks, major cerebrovascular accidents, especially in younger

patients (with ret arded healing phase), and spin al cord or retinal infar ction (19). Sometimes emboli may be multiple and involve many different organs, thus simulating a generalized vascular diseas e with little, if any, signs of cardiac obstruction or of constitutional featur es (2, 16).

C. Diagnos is of cardiac tumors Diagnostic work-up for cardiac tumors can proceed in a

stepwise fashion , as shown in Table 1. The diagn ostic pr ocedures which have been underlined in this ta ble yield the iargest contribution in esta blishing the diagnosis of cardiac neoplasms. Two-dimension al (and M-mode) echocardiogra phy nowada ys has become the most important method of non-invasive detection of cardiac tumors (Figs. 3 and 4). Either by using tr ansthoracic or transesophageal techniques, sensitivity of detecting cardiac neoplasms is very high. Radionuclide method s, parti cularly gated tha llium201 scintigraphy, may add information regarding the extent of invasion of the myocardium by the tumor (Fig. 2). Computerized tomography, parti cularly fast CT, as well as nuclear magneti c resonan ce tomography are also powerful tools in the non-invasive detection of cardiac tumors .

The import ance of cardiac catheterization (and cineangiogra phy) within the diagn ostic work-up has been diminish ed by the improvement of non-in vasive imaging

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Fig,3 Two-dimensional echoca rdiogram ofa45·year·oldpatientwithaleft atrial myxoma prolapsing into the left ventricular chamber (fourchamber view)

Fig.5 Left-sided pulmonary angiogram delineatingthetumorin the leftatriumduring ventricular systole;the tumor(black) isto be seen withintheleft atrial cavity

techniques , but pressure measurements in cardiac chambers an d pulmonar y arte ries as well as corona ry angiograpby give useful and often necessary additiona l inform ation prior to surge ry. By mean s of left-sided pulmonary angiography. cardiac tumors such as prolapsing left atr ial myxomas can be visualized (Figs. 5 and 6). These prolap sing tumors move from the left ventricle to the left atrium in early systole (Fig. 5)and from the left atrium to the left ventricle in ea rly diastole. thereby causing specific cha nges in left atrial hemodynamics, namely prominent c and v waves accompan ied by a rapid y descent (22). Nonprolapsing tumors remain within the left atrium during the enti re cardiac cycle. impeding flow across the mitr al valve. In these cases. pulmonary we dge pressure measurements show a slow y descent which is indistinguishable from tha t cause d bv mitral valve ste nosis (22).- In the presence of tumors with pericardial involvement, pericardiocentesis and subse quent pericardial fluid analysis may establish the diagnosis of cardiac neoplastic disease.

Fig.4 M-mode echocardiograrn of the45-year-oldpatient withaleftatrial myxoma.Acloudof echoesisseen fillingtheleftventricularchamberduring diastoleand disappearingduringventricularsystole.Thiscorrespondstoprolapse ofthemobile tumorintothe leftventricular chamberduringdiastoleand its return totheleft atrium during systole

Fig.6 Left-sidedpulmonary angiogram delineatingthetumor (myxoma)intheleft atrium during ventricu lar diastole;thetumor (black) now prolapsed into theleft ventricular chamber

D. Differential diagnosis of cardiac tumors

The diITerenti al diagnosis of car diac tumors may be extreme ly difficult. for they are the great imitators in cardiovascular disease. They are capable of counterfeiting almost any hemodynamic syndrome and of pr oducing symptoms or signs that suggest diseases of other systems. thus directing attenti on away from the heart. Thus , differential diagnos is of cardiac tumors includes 1) pericarditi s of other origin. particular ly const rictive pericarditis; 2) coronary artery disease with good or impaired left ventricular function; 3) dilated cardiomyopat hy; 4) congestive heart failure of unkno wn cause (parti cularly right-sided heart failure); 5) pulmonary hyper tension (primary, or seconda ry to repeated emboli from venous sites);

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Thome . cardiovasc. Surgn 38 (1990)

ClinicalAspe cls a/Cardiac Turner s

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6) 7) 8) 9) 10)

restrictive cardiomyopathy: mitral , tricuspid, aortic, or pulmonary valve disease: bacterial endocarditis (6): rheumatic disease: autoimmune diseases, often with renal involvement (particularly connective tissue disorders) (2, 16), Despite these differential diagnostic pitfalls. the constellation of signs and symptoms as described above, togethe r with some laboratory clues and particularly echocardiographic as well as cardiac catheterization findings will enable us to arrive at the correct diagnosis of a cardiac tumor in the vast majority of cases.

II. Tillmanns 12

Lammers. R. J.• and C. M. Bloor: Path ology of cardiac tumours . In: Cancer and the heart. A. S. Kapoor (ed.) Springer. New York 1986.

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Ma c Gregor. G. A.. and R. A. Cullen: The syndrome of fever. anemia.

pp. 1-20

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Dr. H. TUlmanns Med. Univ- Klinik

Abt.lnnere Med. - Kardio logte 0 ·6 300 Giessen

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156

Clinical aspects of cardiac tumors.

Due to surgical advances, cardiac tumors now represent a potentially curable form of heart disease. Primary cardiac tumors are very rare; secondary, m...
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