Tumors of the Heart Experiences at the Giessen University Clinic R. Moosdorj, H. H. Scheid, and F W Hehrl ein


Herztumoren: Erfahrungen der Giellener Universitatsklinik

Of 54 cardiac tumors operated upon in our clinic, 42 were classified as benign a nd only 12 as malign an t. Th e major par t of the beni gn tum ors were myxom a, mainl y located in the left at rium. While sm aller tum ors could be tr eated by local resections, extens ive res ections we re necessary in 14 pati ent s with grea ter tumors followed by reconstructions of th e pulmonary a nd caval vein, mitral and tric uspid valve, a nd major part s of the right and left ven tri cular wa ll. In on e pati ent with a hu ge beni gn myxom a, tum or exposition a nd total re section could only be achieved by an autotran splantation of th e hear t. While mortality after surgical ther ap y of beni gn tumors was only 1.4 % (1142) with in a mean follow-up time of 48 month s, th e prognosi s of malign ant tumo rs is still fatal with a mortality of 50% (6/1 2) with in a mean follow-up time of24 month s, despit e add itional che mothe ra py or ra diati on.

Unte r 54 in un ser er Klinik operierte n Tumo ren des Her zens fand en sich 42 ben igne und 23 maligne Geschwtilste . Der iiberwiegend e Teil benign er Tum oren war en Myxom e, die hauptsachlich im linken Vorhof lokalisiert war en . Wahrend bei kleineren Tum oren lokale Resektion en a usre iche nd war en , mull ten bei insgesamt 14 Pati ent en mit beni gnen und malignen Tumoren ausgiebig Resektion en mit Rekonstruktion von Lungen ven e, Hohlvene, Mitr al- und Tricuspidalklapp e sowie groBere n Anteilen der Vorhofwand e vorge nomme n wer de n . In eine m Faile wurde eine kompl ett e Tum orent fernung erst mit Hilfe eine r Autotr ansplantat ion errnoglicht. Wahrend die Mortalitat mit 2,4% (1/42) in eine m Nachbe oba chtungszeitr aum von 48 Monat en bei den beni gnen Tumoren sehr niedri g lag, ist die Progn ose maligner Tumo re n mit einer Mortalitat von 50% (6/12 ) in eine m Nachbeobac htungszeitraum von 24 Monat en trotz adjuvanter Che mo- und Bestr ahlungsth erapi e weiterhin schlecht.

Keywords Cardiac Tumor - Atri al myxoma - Surgical technique - Autotr an splantati on of the hear t

Cardiac tumors represent a rare diagnosis among the number of pati ents und ergoing cardiac surgery. Depending on their location and extension they can appear und er var ying symptoms which ar e often not even related to the heart (1). Most cardiac tumors are benign and well access able for sur gical th erapy. Among the minority of malignant tumors we hav e to differentiate between prim ar y cardiac tumors , dir ectly originating from different structures of the heart, and secondary cardiac tumors app earing as metastases or as dir ect infiltrations of adja cent mediastinal or pulmonary tumors (2). Thus a precise diagnostic proc edure ha s to pr ecede the final decision for a surgical ther apy, radiation, chemo-the rapy or combin ed approaches (3, 4). Nevertheless the pr ognosis of these tumors is even under joint efforts still poor. Since 1960 we ha ve operated on 54 patients with primary and secondary tumors. The most frequent symptom among these pati ents was mitral valve disease in 25 pati ents , followed by embolic phenom ena (9), dysrh ythmi a

Thorac. cardiovasc. Surgeon 38 (1990) (Special Issue) 208-210 © Georg Thieme Verlag Stuttgart · New York

or arrhythmia (6), pericardial effusion (6), myocardial infarction (5), symptoms of tricuspid valve diseas e (2), and symptoms of pulmonary valve diseas e in one pati ent. The decision-making in this cohort of pati ents has changed during the years . Before 1969 10 pati ents wer e exclusively investigated by cardiac cath eterization. Between 1970 and 1979 the majority of patients with cardiac tumors was diagnosed by echocardiography (11), while only 8 pati ents still underwent catheterization. The clinical introduction of computed tomography caused another shift in the decision-making, so that am ong the remaining 25 pati ents which we operated upon since 1980, 11 wer e only diagnos ed by computed tomography, while echocardiography was perform ed in 10 pati ents and cardiac cath eterization only in 4 pati ents . The majority of the tumors wer e benign myxomas : 64.8 % (35). Moreover we found 3 benign lipoma , 3 fibroma , and one hydatid cyst. Among the 12 malign ant tumo rs wer e 5 primary fibrosarcoma, 3 primary angiosarcoma, 1

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Department of Cardiovascular Surgery, Justus-Liebig-University, Giessen, FRG

Thorac. cardiovasc. Surg eon 38 (1990)

Experiences at th e Giess en Unive rsity Clinic


teratoma , and as secondary tumors 2 malignant thymoma with infiltration of pericardium and cardiac structures and one immunocytoma. Characteristically the majority of the myxomas were located in the left atrium (30), only 5 originated from the right atrial site . Among the benign tumors the right atrium was affected in 8 cases (Table 1). Table 1 Tumors of the Heart: Location of primary and secondary cardiac tumors in 54 patients Type

Atrium right left

Benign (n = 42)

Myxoma' Fibroma Lipoma Hydatid Cyst


Malignant (n = 12)

Fibrosarcoma Angiosarcoma Immunocytoma Thymoma Teratoma



1 1 2 1


Ventricle right left

30 3 2

, multicentric foci (n = 4) biatrial (n = 3) multiple (n ~ 1)

Fig.1 Extensive resection incaseofamalignantmyxoma (Diagram). The tumororiginatedfromtheleftsideof the interatrialseptumand extended into the rightupper pulmonary vein (leftside). Resectionoftheinteratrial septum by aright atrial approachanda partial resectionofthe right upper pulmonaryvein fortotaltumorextraction(right above). Reconstructionof thedefects inthe pulmonaryvein and interatrial septum by twoappropriately sized PTFE-patches (right below)

Surgical therapy was performed und er extracorporeal circulation . Surgical procedures includ ed local resection of most of the benign tumors including the benign myxoma : a right- , left- or biatrial approach was chosen for resection of the tumor including partial excision of the interatrial septum from whi ch the tumor originated. In 14 patients with benign as well as malignant primary or secondary tumors, extensive resections and subs equent plasty reconstructions had to be performed (Table 2). Table 2 Tumors of the Heart: Extensive Resection and plasty reconstruction in cardiactumors rightatrium', vena cava" septum, valve replacement" valve repair (MV2, AV1) septum, rightupperpulmonary vein' septum', posteri or left atrialwall" rightventricular wall" left ventricular wall'" o

4 3 3 1 10 0

PTFE Patch, " St. Jude Prosthesis, ' " Dacron Patch PTFE Prosthesis, 00 Autotransplantati on

This included tumor infiltration or tumor extension into the caval or pulmonary veins with partial resection of the vascular wall and replacement by pat ches or vascular prostheses (Fig. 1). In one patient with a huge myxoma which besid es the interatrial septum had also affected major parts of the post erior left atri al wall, neither tumor exposition nor total resection were possibl e by means of conventional surgical approaches. Thus the heart was totally excised so that an optimal visualization of the tumor extension could be achi eved. After res ection and following plasty reconstruction of the interatrial septum and major parts of the posterior left atrial wall by a PTFE-patch , the excised heart, which had been kept cool in cardioplegic solution, was reimplant ed again (Fig. 2).


Fig 2 Tumor resectionbyautotransplantation(diagram). Huge myxoma with attachment to theinteratrial septum and majorpartsof the posteriorleftatrial wall (A). Afterexcisionofthe heart thetumorcould betotallyresected and the interatrial septumaswell asthe posterior leftatrial wall be reconstructedbya PTFE-patch (B)

The mortality after surgical therapy of benign cardiac tumors was 2.4 % ( /42), within a mean follow-up time of48 months. The mortality after resection of malignant tumors was 50% (6/12) after a mean follow-up time of 24 months. Thr ee pati ents died within the first 30 days after operation, while the remaining thr ee pati ents died of a tumor recurrency despit e adjunctive chemo-therapy or radiation up to one year after surgery (Fig. 3). Only one pati ent survived for now mor e than four years. Patients with cardiac tumors ar e often misdiagnosed becaus e of their var ying symptom s (5). Often rapidly developing symptoms of mitral valve disease ar e th e first indication of a myxoma , and also embolic phenomena, especially in young pati ents , may be a first sign of cardiac tumor, so that pathohistologic investigations should be per-

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Th om e. ea rdiovase. S u rgeon 38 (1 9 90)

R. Mo osdorf, H. H. S ch eld, F. W Hehrlein

Fig. 3 Check-up chest radi ographone yearaftertotal resection ofa malignant myxoma andadditional chemotherapyina twentyyearoldfemale patient(see also Fig. 1). While the heart showednotumorrecurrency, multiple pulmonary metastases can be seen

References 1

M iller, 1. I.. H. T. Mankin. 1. C Broadb ent. E. R. Giulia ni. G. H. Dan ielson: Primary Cardiac Tumors; Surgical Considerations and


Mc/s llis ter, Jr.. H. A.: Tumors of the Heart and pericardium. In: S ilve r. M. S.. ed.: Cardiovascular Pathology, Volume 2. Churchill

Results of Operation. Circulation 45 (1972), Supp!. 1:134-1 38



5 6 7 8

form ed in each of these cases. Methods ofinv estigation have changed during the last 20 year s, so that many patients with cardiac tumors can toda y be diagn osed by non-invasive methods such as computed tomography or echocardiogra phy (4,6) . Nevertheless, cardiac catheterization is still mandatory in a numb er of pati ents , in which accomp anying cardiac lesions or an additional coronary heart disease have to be excluded prior to surgery (7). Developing techniqu es of cardiac sur gery also allow extensive resections in cases of wide spread or multilocular tumor affections. This also includ es th e techniques of autotransplantation in thos e very selected cases , in which otherwis e a sufficient surgi cal exposition and tumor res ection can not be performed (8). Our pat ient with a huge myxoma of the interatrial septum and posterior left atrial wall, in whom we performed an autotransplantation for total tumor res ection and reconstruction of the posterior left atrial wall, is now well and free of symptoms or tumor recurrency 22 months after surg ery. Despit e th ese successful surgi cal pro cedures the prognosis of malignant cardiac tumors is still poor . Even und er a management of sur gical resection, combine d chemotherapy and /or radiation, the mort ality is still very high (9). The prognosis of benign tumors after sur gical resection is much more adventagous and we only lost one out of 42 pati ents by postop erative low-cardiac output and ren al failur e. Early decision-making is of major importance for these pati ents and can help to pr event the development of valvular disturbances, abnormal myocardial load, or embolic lesion s.


Livingstone, New York, Edinburg, London, Melbourn e 1983, pp. 917-943 Bubenh eim er, P.: Echogene Masse in Herzhiihlen - prima rer Herztumor? Herz + Gefaf3 e 3 (1983) 188 -197 Godwin. 1. D.. L. Axel. 1. R. A dams . N. B. Sc hi ller. P. C Simp son . E. W. Gert z : Computed tomograph y: A new method for diagnosing tumors of the heart. Circulation 63 (1981) 448 Sc he id. H. H.. J. Mul ch. F. W. Hehrlein : Myxome des Herzens. Herz/ Kreislauf 4 (1984) 181-1 89 Lee. Y. C . M. Y. Magram: Nonprolapsing Left Atrial Tumor. The MMode Echocardiogra phic Diagnosis. Chest 78 (1980) 332 - 333 S che id. H. H.. F. W. Hehrlein. 1. Kra cht. G. Fra edr ieh : Primate Herztumor en. Med. Welt 33 (1982) 709- 717 S cheid. H. H.. H. W. Ne stle. D. Kling. W. A. S tertmann. U. Langebar te ls, F. W. Hehrlein: Tumorresection of the heart by autotra nsplantation. The Cardiovasc. Surgeon 36 (1988) 40-43 Hehrlein, F. w. . J. Mul ch: Palliativ-chirurgi sche EingrifTe bei malignen Tumoren. Herz und Herzbeute!. In: S chultis . K.. H. Ecke, H. Sc hoe n (Hrsg.): Palliativ-chirurgische EingrifTe bei malignen TumorenoThieme Verlag, Stuttgart 197 3, p. 53

Priv.-Doz. Dr. R. Mo osdorf

Klinik f. Herz- und Gefiif3chirurgie am Zentrum f. Chirurgie der Justus-Liebig Univers ita t Gief3en 6300 GieBen FRG

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Tumors of the heart. Experiences at the Giessen University Clinic.

Of 54 cardiac tumors operated upon in our clinic, 42 were classified as benign and only 12 as malignant. The major part of the benign tumors were myxo...
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