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Asian Cardiovasc Thorac Ann 2000;8:378-380
© 2000 Asia Publishing EXchange Pte Ltd


CASE STUDY

Intracardiac Masses

Kutay Tasdemir, MD, Ö Naci Emirogullari, MD, Hakan Ceyran, MD, Ramazan Asik, MD, Alptekin Yasim, MD

Department of Thoracic and Cardiovascular Surgery
Erciyes University Medical Faculty
Kayseri, Turkey
For reprint information contact: Kutay Tasdemir, MD Tel: 90 352 222 3272 Fax: 90 352 222 3272 email: can_ero{at}hotmail.com Kizilirmak Caddesi, Nergis Apartmani 117/13, Melikgazi, Kayseri 38030, Turkey.

    Abstract
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 Abstract
 Introduction
 Case Reports
 Discussion
 References
 
Between 1991 and 1998, 8 patients (mean age, 29.6 years) underwent surgery for an intracardiac mass. There were 2 hydatid cysts, 1 angiosarcoma, and 5 myxomas. One patient with myxoma underwent a Syme's amputation due to lower extremity ischemia caused by embolization. The patient with angiosarcoma died of cardiopulmonary failure on the 9th postoperative day. The surviving patients experienced no mortality, complications, or recurrence during follow-up of 0.5 to 5 years.


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 Abstract
 Introduction
 Case Reports
 Discussion
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Intracardiac masses include primary and metastatic tumors as well as cysts and thrombi. Primary cardiac tumors are rare, they include myxomas that have a good prognosis and sarcomas with a poor prognosis.1 We describe 8 cases of intracardiac mass treated surgically at this institute.


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 Case Reports
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The 4 male and 4 female patients, mean age 29.6 years (range, 12 to 64 years), were treated between January 1991 and December 1998. Clinical findings are given in Table 1Go. Three patients had peripheral emboli due to myxoma (cases 1 and 5) or ruptured left ventricular hydatid cyst (case 3). The embolization sites were the left lower extremity in case 1, both lower extremities and the right upper extremity in case 5, and both lower extremities in case 3. Patients with hydatid cysts had syncope, dyspnea, and coughing, whereas symptoms in the others were mostly due to mechanical obstruction by the cardiac masses. Physical examinations were either normal or revealed nonspecific cardiac auscultation findings. Definitive diagnosis in all patients was made using two-dimensional transthoracic and transesophageal echocardiography. Chronic myocardial infarction was considered in a case of myxoma with QS formation on electrocardiography (case 1). Angiography showed that the coronary arteries of this patient were normal but ventriculography revealed a left ventricular aneurysm and an apical thrombus due to previous myocardial infarction.


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Table 1. Profile of 8 Patients With Intracardiac Mass
 
Cardiopulmonary bypass was established by elective cannulation of the inferior and superior venae cavae and the ascending aorta, except in a patient with primary angiosarcoma where the right femoral vein was cannulated and sternotomy was performed before cannulation of the ascending aorta and superior vena cava (case 4). Moderate hypothermia was used in all cases. Cardiac arrest was induced by aortic crossclamping and application of cold crystalloid cardioplegia. In the case of primary angiosar-coma (Figure 1Go), following removal of the tumor (15 x 15 cm) invading the right atrium and right atrial wall, the orifices of the superior and inferior venae cavae were reconstructed using a polytetrafluoroethylene patch so that they could drain into the tricuspid orifice. In the 2 cases of right atrial myxoma (Figure 2Go), a right atriotomy was performed (cases 6 and 8), 2 of the 3 cases of left atrial myxoma were treated via a left atriotomy (cases 5 and 7), and a patient with left atrial myxoma and a thrombus in the right atrium underwent a right atriotomy and thrombectomy followed by transseptal excision of the myxoma (case 1). This patient also had thrombectomy and linear plication of an apical aneurysm and thrombus. Of the patients with hydatid cyst, one (case 2) with a cyst (4 x 4 cm) in the left ventricular posterolateral wall with an intramyocardial localization received aspiration and cystotomy. After removal of the germinative membrane, capitonnage was carried out. In the other case (case 3), the hydatid cyst (3.5 x 3.5 cm) on the left side at the base of interventricular septum, was ruptured. The germinative membrane was excised without damaging the inter-ventricular septum. This patient and 2 others (cases 1 and 5) with embolization from myxoma required embolectomy that was performed during the same session. Care was taken to minimize manipulation of the heart prior to placement of the crossclamp.



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Figure 1. Echocardiographic view of a right atrial myxoma (case 6).

 


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Figure 2. Preoperative two-dimensional echocardiogram in long-axis parasternal view, showing a cyst located on the posterolateral left ventricular wall (case 2).

 
The patient with angiosarcoma died of cardiopulmonary failure on the 9th postoperative day. In case 1 with tumor emboli in the upper and both lower and extremities, a Syme amputation of the right lower extremity was necessary because of the delay, despite embolectomy. The other patients with myxoma had no early or late mortality or complications during follow-up of 0.5 to 5 years. No complications or recurrence were observed in the 2 patients with hydatid cyst during follow-up (0.5 to 3 years). The overall mortality rate was 12.5%.


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 Abstract
 Introduction
 Case Reports
 Discussion
 References
 
Primary tumors of the heart have a frequency in postmortem studies of approximately 0.3%, 75% are benign, and 50% are myxomas. Myxomas are seen in all age groups but most often during the third and sixth decades of life.1,2 Most myxomas (75%) arise in the left atrium and 20% are found in the right atrium. It is believed that they originate from the primitive endothelial and subendothelial tissues in the fossa ovalis.3 Three of the myxomas in this series were located in the left atrium and 2 in the right atrium.

Cardiac hydatid cyst is a rare disorder that can lead to fatal complications. Although taenia echinococcus may settle on any organ, they generally involve the liver and the lungs; cardiac involvement accounts for less than 2% of cases.4 The cyst reaches the heart via the portal vessels from the liver or via the pulmonary veins from the lungs. It can also reach the myocardium via the coronary circu-lation.4 The most frequent location is the left ventricle (60%), followed by the atrioventricular sulcus, inter-ventricular septum, left atrium, right atrium, and interatrial septum.4 One of our patients had a cyst on the postero-lateral wall of the right ventricle, it was on the base of the interventricular septum of the left ventricle in the other. There is no specific diagnostic sign of a cardiac hydatid cyst. The patient may be asymptomatic, depending on the location, duration, size, and number of the cysts, but severe symptoms such as anaphylactic shock or congestive heart failure may develop.5 Death may result from cerebral emboli, cystic rupture leading to tamponade, or anaphylactic shock.5 Heart enlargement on tele-cardiography is sometimes the first finding.4 No pathology was observed radiologically in either of our cases.

Primary cardiac malignancies comprise angiosarcomas, fibrosarcomas, rhabdomyosarcomas, and malignant fibrous histiocytomas. Angiosarcomas account for 20% of primary cardiac malignancies and they are 2 to 3 times more frequent in men.1 All angiosarcomas reported to date were located in the right atrium and early diagnosis has been difficult.6 Approximately 75% of patients have right ventricular failure and a pericardial disorder. Other common symptoms are fever, chest pain, hemoptysis and weakness. Our patient was a 22-year-old woman, the tumor had completely invaded her right atrium and ventricle, and she had been admitted with related complaints.

For diagnosis of intracardiac masses, the superiority of transthoracic over transesophageal echocardiography has been debated.7 Our findings were confirmed using each of these methods. Many patients with intracardiac mass have peripheral emboli on admission, as found in 3 cases (37.5%) in this series, but coronary arterial emboli are rare.1 Invasive intervention is time-consuming and not advisable unless needed. We carried out coronary angio-graphy in a case of suspected myocardial infarction. In spite of a left ventricular aneurysm, the coronary arteries were patent and the condition was evaluated as recanal-ization.

As soon as an intracardiac mass is diagnosed, surgical intervention is essential as the clinical picture may deteriorate rapidly to early sudden death. The treatment of choice is surgical resection and the tumor should be resected together with a margin of tissue, to avoid recurrence.1 The deficit is closed primarily (as in our cases of myxoma) or using a pericardial or prosthetic patch (as in our patient with angiosarcoma).1 Those who had hydatid cysts had no defect and they were discharged on 1200 mg of mebendazole daily for 3 months. Following surgery, radiotherapy or epi-doxorubicin as well as chemotherapy in combination with dacarbazine may be used for patients with angiosarcoma.8 Despite all treatment methods, the prognosis of angiosarcoma is poor. All intracardiac masses are potentially fatal and should be completely resected using proper protection techniques. Surgical treatment should be directed towards the elimination of mechanical obstruction and any other complication.


    References
 TOP
 Abstract
 Introduction
 Case Reports
 Discussion
 References
 

  1. Kirklin JW, Barrat-Boyes BG. Cardiac tumor. In: Kirklin JW, Barrat-Boyes BG, editors. Cardiac surgery. New York: Churchill Livingstone, 1993:1635–42.

  2. Bortolotti U, Maraglino G, Rubino M, Santini F, Mazzucco A, Milano A, et al. Surgical excision of intracardiac myxomas: a 20-year follow-up. Ann Thorac Surg 1990; 49:449–53.[Abstract]

  3. Jones DR, Hill RC, Abbot AE Jr, Gustafson RA, Murray GF. Unusual location of an atrial myxoma complicated by a secundum atrial septal defect. Ann Thorac Surg 1993; 55:1252–3.[Abstract]

  4. Ameli M, Moborhan HA, Nouraii SS. Surgical treatment of hydatid cyst of the heart: report of six cases. J Thorac Cardiovasc Surg 1989;98:892–901.[Abstract]

  5. Kabbani SS, Jockadar M, Sundouk A, Nabhani F, Baba B, Shafik AI. Surgical management of cardiac echinococcosis. J Cardiovasc Surg 1992;33:505–10.[Medline]

  6. Jimenez JD, Perez CT, Gonzales AA, Ugarte J, Pajuelo CG, Porras AC, et al. Right coronary artery-right atrium fistula in primary angiosarcoma of the heart. Chest 1992;102:1629–30.[Abstract/Free Full Text]

  7. Samdarshi TE, Mahan EF 3d, Nanda NC, Guthrie FW Jr, Bernstein IJ, Kirklin JW. Transesophageal echo-cardiographic diagnosis of multicentric left ventricular myxomas mimicking a left atrial tumor. J Thorac Cardiovasc Surg 1992;103:471–4.[Abstract]

  8. Potter R, Baumgart P, Greve H, Schnepper E. Primary angiosarcoma of the heart. Thorac Cardiovasc Surg 1989; 37:374–8.[Medline]





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