Although the existence of cardiac tumors has been known since the Middle Ages (1), they remain a diagnostic puzzle and a therapeutic challenge. The first successful resection of a cardiac tumor was carried out by Crafoord in 1954, using a heart–lung machine (2), and ensuing advances in cardiosurgical technique enabled resection of malignant tumors. There are no large studies of the best treatment for cardiac tumors, particularly the malignant forms; often, only single case reports are available. With the exception of lymphoma, the treatment of choice is complete resection in combination with chemo-/radiotherapy. Large autopsy series show that the incidence of cardiac tumors is 0.02% (3). Some 75% of the tumors are benign, 25% malignant (3). Metastases are discovered much more frequently than primary neoplasms of the heart, being diagnosed in over 10% of tumor patients at autopsy. Atrial myxoma is the most frequent primary cardiac tumor in adults, while rhabdomyosarcoma predominates in children (3, 4). Our experience over a period of 23 years is summarized in Figure 1.



Figure 1

Distribution of cardiac tumors diagnosed and treated at the Center for Cardiac Surgery of Munster University Hospital between 1989 and 2012

/pdf/cardiac-tumors-diagnosis-and-surgical-treatment-4guxyjqs30.pdf

Cardiac tumors--diagnosis and surgical treatment.

Malignant tumours of the heart: a review of tumour type, diagnosis and therapy.

Primary cardiac sarcomas: a clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival

Rotation thromboelastometry (ROTEM) performed on whole-blood samples provides information on the contribution of fibrinogen and platelets to clot formation. Such measurements are believed superior to classical plasma coagulation measurements as a means of monitoring disturbed haemostasis. On-pump cardiac surgery is associated with high bleeding risk. The study objective was to obtain information on the frequency of abnormal values of ROTEM variables and to assess their value in estimating bleeding risk in such patients. We studied 150 patients undergoing elective on-pump cardiac surgery. We found a significant surgery-induced decrease in haemostatic potential, with more abnormal ROTEM values in intrinsically activated coagulation (up to 50%) than in extrinsically activated coagulation (up to 27%) or the maximum clot firmness in FIBTEM (10%), a test measuring the contribution of fibrinogen. All ROTEM variables tend to normalize within 14-18 h postoperatively. Best positive predictive values and specificity for a postoperative blood loss above 600 ml were found for the clot formation time in extrinsically activated coagulation (71%/94%) and the maximum clot firmness in FIBTEM (73%/95%); these values were superior to the activated partial thromboplastin time or prothrombin time (56%/72% and 43%/5%, respectively). There was no relation between preoperative or early postoperative ROTEM values and intraoperative bleeding. ROTEM recorded a benefit of administration of platelet concentrates or fresh-frozen plasma, particularly when given postoperatively, on haemostasis. In contrast, intraoperative administration of red blood cells impaired haemostasis. ROTEM can provide a more detailed diagnostic basis enabling a focused therapy to cardiac surgery patients with high bleeding risk.

The value of rotation thromboelastometry to monitor disturbed perioperative haemostasis and bleeding risk in patients with cardiopulmonary bypass.

Cardiac tumors prevalence and mortality: A systematic review and meta-analysis.

Background Tumours of the heart are rare. Different histological subtypes are known. The most common tumour entity is benign cardiac myxoma. Malignant heart tumours are less common. Tumours originating in other organs such as the kidney may also affect the heart by tumour progression via the inferior caval vein. A large experience with surgical treatment of different types of heart tumours is presented. Methods Between January 1989 and April 2004, 108 patients with a heart tumour were included in a database. All patients underwent radical surgical resection, except for 2 patients who had malignant lymphoma of the heart. Results Histological findings included 78 myxomas (72.2 %), and 6 other benign cardiac tumours in 5.6 % of the patients. Primary malignant heart tumours were seen in 10 (9.2 %) and renal cell carcinoma with cardiac involvement in 6 (5.6 %) patients. Eight patients presented with tumour metastases inside the heart (7.4 %). Mean overall survival was 12.7 years for myxoma patients and 5.6 years for patients with other benign heart tumours. Patients with primary malignant heart tumours survived 5.5 years on average. Conclusions Heart tumours are rare, but usually life-threatening. Radical surgical resection is the therapy of choice and may offer excellent long-term survival, even in cases with malignant heart tumours.

Neoplastic heart disease -- the Muenster experience with 108 patients.

We report on our experience with six patients with malignant sarcoma of the heart and show that long-term survival is possible after radical resection. At presentation, the 6 patients (3 male, 3 female) were 45.8 +/- 20 years old. Three patients are alive without evidence of metastases since 29.6 +/- 36.8 months, three patients died after 38 +/- 50.2 months due to distant metastases. Precise preoperative localization of the tumor by means of imaging techniques is very important. In some cases, radical surgery requires an ex situ procedure (autotransplantation). If necessary, the right heart can be resected almost completely, and reconstructed in the form of a Fontan-type circulation. A heart transplantation, as suggested by others, is not justified from our point of view, since prognosis is not better and donor organs are too rare. The results of radical resection are promising, but new concepts for treatment--in particular chemotherapy concepts--for these mostly middle-aged or young patients are required.

Radical resection of cardiac sarcoma.

Surgical treatment of cardiac myxomas: 16 years experience of 78 patients

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Neoplastic heart disease -- the Muenster experience with 108 patients.

Radical resection of cardiac sarcoma.

Surgical treatment of cardiac myxomas: 16 years experience of 78 patients