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Asian Cardiovasc Thorac Ann 2000;8:130-133
© 2000 Asia Publishing EXchange Pte Ltd


ORIGINAL CONTRIBUTION

Stroke and Myxoma

Baran Ugurlu, MD, Öztekin Oto, MD, Hüseyin Okutan, MD, Kürsat Kutluk, MD,1, Erdem Silistreli, MD, Nejat Sariosmanoglu, MD, Eyüp Hazan, MD, Aydanur Kargi, MD,2

Department of Thoracic and Cardiovascular Surgery
1 Department of Neurology
2 Department of Pathology
Dokuz Eylül University Medical School
Izmir, Turkey
For reprint information contact: Öztekin Oto, MD Tel: 90 232 277 2165 Fax: 90 232 277 2165 email: oztekin.oto{at}deu.edu.tr Department of Thoracic and Cardiovascular Surgery, Dokuz Eylül University Medical School, 35340 Izmir, Turkey.

    Abstract
 TOP
 Abstract
 Introduction
 Patients and Methods
 Results
 Discussion
 References
 
Cardiac myxoma was detected by transthoracic echocardiography in 8 patients (aged 11 to 64 years) who were admitted between 1991 and 1999 with stroke or transient ischemic attacks, representing 80% of the total myxoma cases treated in this period. All patients underwent surgery and the myxomas were successfully removed. There were no recurrences during a mean follow-up of 3.1 years. In the presence of unexplained transient ischemic attack, cerebral infarction, or syncope, this relatively rare cardiac lesion should be suspected and investigated by transthoracic echocardiography.


    Introduction
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 Abstract
 Introduction
 Patients and Methods
 Results
 Discussion
 References
 
Primary cardiac tumors are rare, with an incidence of 0.0017% to 0.28% in unselected patients at autopsy.14 Cardiac tumors are usually benign and half of them are myxomas. They occur mainly in the third to sixth decades of life. Myxomas are usually seen sporadically, pre-dominantly in women, and frequently developing in the left atrium.46 Clinical findings are embolism, intracardiac obstruction, and constitutional symptoms. Manifestations of embolism occur in 20% to 45% of these patients.6,7 In most cases, the cerebral arteries are affected.3,8,9 Cardio-embolic infarction is caused by occlusion of an intracranial artery by an embolus associated with a specific cardiac source.6,7 It was recently recognized that cardiac embolism probably accounts for one-quarter to one-third of all ischemic strokes.6,7 This study is reported to draw attention to cardiac myxoma as a possible cause of cerebral ischemic events.


    Patients and Methods
 TOP
 Abstract
 Introduction
 Patients and Methods
 Results
 Discussion
 References
 
Between March 1991 and April 1999, 10 patients were treated for cardiac myxoma, of whom 8 were admitted with symptoms of stroke or transient ischemic attack (TIA). These 8 comprised 1 female (12.5%) and 7 males (87.5%), with ages ranging from 11 to 64 years. Patient characteristics are summarized in Table 1Go. The majority had additional cardiac manifestations including palpi-tations (3), arrhythmias (ventricular arrhythmia and left bundle branch block in 2), and dyspnea (1). An apical systolic murmur was detected in one patient and a diastolic murmur in another. Noncardiac manifestations were stroke (6), TIA (2), syncope (3), and peripheral arterial embolism (1). Three patients had constitutional symptoms such as sweating, arthralgia, fatigue, or fever.


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Table 1. Patient Profile
 
All 8 patients underwent a basic investigation protocol that included cerebral computed tomography scan, chest radiograph, electrocardiogram, and routine laboratory tests. Computed tomography diagnosed embolic cerebral infarctions in 5 patients, chest radiographs were non-specific, and laboratory results were normal. Two-dimensional transthoracic echocardiography was the initial diagnostic tool in all 8 patients (Figure 1Go). Four patients underwent transesophageal echocardiography, 1 had coronary angiography, and cardiac magnetic resonance imaging was carried out in 2, which confirmed an intracardiac mass (Figure 2Go). B-mode carotid Doppler ultrasonography was performed in the 3 patients over 40 years old; the findings were nonspecific. Two-dimensional echocardiography determined the location of the tumor on the posterior wall in 3 patients, on the interatrial septum in 2, on the anterior leaflet of the mitral valve in 2, and on the posterior leaflet of the mitral valve in 1.



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Figure 1. Echocardiogram of patient no. 6. LV = left ventricle, RA = right atrium, RV = right ventricle.

 


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Figure 2. Cardiac magnetic resonance image of patient no. 7.

 
Surgery was performed on an urgent basis. The aorta and the superior and inferior venae cavae were cannulated and myocardial protection was achieved using antegrade cold blood cardioplegia and topical ice slush. Cardio-pulmonary bypass was instituted under moderate hypo-thermia. In all cases, the heart was entered through a lateral left atriotomy incision posterior to the interatrial groove. No cerebral protection methods were used. A patent foramen ovale was detected in one patient and closed primarily. In 3 cases, the myxoma was pedunculated and originated from the atrial septum (Table 1Go). The tumors and pedicles were removed along with their attachments to the atrial septum. In one patient, the myxoma originated from the posterior leaflet of the mitral valve; the myxoma had embolized so only the pedicle was removed. The smallest myxoma was 0.5 x 0.5 x 0.5 cm, the largest measured 6.5 x 3.5 x 2.5 cm (mean, 3 x 2.2 x 2.2 cm). Histopathologic examination of the excised materials confirmed the diagnosis of myxoma in all 8 patients (Figure 3Go).



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Figure 3. Histopathologic examination of excised material from patient no. 2 (Masson trichrome stain, original magnification x200).

 

    Results
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 Abstract
 Introduction
 Patients and Methods
 Results
 Discussion
 References
 
There was no perioperative mortality. The most common complication was onset of new supraventricular arrhyth-mias (atrial fibrillation) in 3 patients (37.5%). Two of these patients returned to sinus rhythm with medical therapy but one required electrical cardioversion. The patients were followed up for 2 months to 7 years (mean, 3.1 years). There were no recurrences of either myxoma or new embolic cerebrovascular events during the early follow-up period. Three patients were enrolled in a phy-siotherapy and rehabilitation program for their neurologic problems but neurological status failed to recover.


    Discussion
 TOP
 Abstract
 Introduction
 Patients and Methods
 Results
 Discussion
 References
 
The most important cardiac disorder associated with embolic stroke is atrial fibrillation. In young patients, 23% to 36% of ischemic strokes or TIAs are presumed to be due to emboli from the heart or in transit through it. Embolic manifestations occur in 20% to 45% of patients with myxoma, sometimes as the first symptom.6,7 Myxoma is the most common type of primary benign cardiac tumor but a relatively rare cause of stroke. Cardiac myxomas are usually pedunculated polypoid friable masses, often located in left atrium.10,11 The most common presenting symptom of myxoma is reported to be congestive heart failure, while neurologic symptoms are observed less frequently (27% to 38%).12,13 TIA and stroke were the presenting symptoms in 80% of patients with myxoma treated in this hospital, probably because constitutional symptoms are given less important consideration in patients in our country.

Since most myxomas are located in the left atrium, systemic embolism is particularly frequent. Cerebral arteries, including the retinal arteries, are affected in the majority of cases.3,8,9 Infarction in the central nervous system due to an embolus originating from a left atrial myxoma is a well-known phenomenon.14 There are also reports of intracranial multiple pseudoaneurysms due to metastasis of myxoma.15,16 The papillary-type myxoma has not only a high risk of preoperative brain infarction but also a high risk of distal metastasis.13 When the retinal arteries are affected, transient or permanent loss of vision may result.3,8,9,11 Unexplained TIA, cerebral infarction, or syncope (with possible features of seizure) are common neurologic manifestations.17 Aneurysm formation and hemorrhage in the late period have also been reported, but the true incidence of these delayed complications is unknown.18

When suspected, a transthoracic echocardiogram can reveal cardiac myxoma in most cases. The initial diagnosis was made with this technique in all of our patients. It has been suggested that transesophageal echocardiography can be a valuable addition to transthoracic echocardio-graphy in the evaluation of potential intracardiac sources of embolism.19 Transesophageal echocardiogram revealed the exact location of the tumor and details of its attachment to cardiac structures in this study. It can be helpful in locating small tumors or their remnants after embolization.

The prognosis after removal of myxoma is excellent and recurrence is uncommon.20 In our series, there were no recurrences during the short follow-up period. Cardiac myxoma is a potentially curable cause of embolism. In the presence of unexplained TIA, cerebral infarction, or syncope, this relatively rare cardiac lesion should be suspected and a carefully performed transthoracic echocardiogram should be added to the investigations.


    References
 TOP
 Abstract
 Introduction
 Patients and Methods
 Results
 Discussion
 References
 

  1. Straus R, Merliss R. Primary tumor of the heart. Arch Pathol 1945;39:74–8.

  2. Heath D. Pathology of cardiac tumors. Am J Cardiol 1968;21:315–27.[Medline]

  3. Wold LE, Lie JT. Cardiac myxomas: a clinicopathologic profile. Am J Pathol 1980;101:219–40.[Medline]

  4. Schanz U, Schneider J. Endokardmyxome: Neue Aspekte zur Histopathogenese. Schweiz Med Wochenschr. 1984;114:850–7.[Medline]

  5. Peters MN, Hall RJ, Cooley DA, Leachman RD, Garcia E. The clinical syndrome of atrial myxoma. JAMA 1974;230:695–701.[Abstract/Free Full Text]

  6. Sirna S, Biller J, Skorton D, Seabold J. Cardiac evaluation of the patient with stroke. Stroke 1990;21:14–23.[Abstract/Free Full Text]

  7. Cerebral Embolism Task Force (1989). Cardiogenic brain embolism: the second report of the Cerebral Embolism Task Force. Arch Neurol 1989;46:727–43.[Abstract/Free Full Text]

  8. Sybers HD, Boake WC. Coronary and retinal embolism from left atrial myxoma. Arch Pathol 1971;91:179–82.[Medline]

  9. Wrisley D, Rosenberg J, Giambartolomei A, Levy I, Turiello C, Antonini T. Left ventricular myxoma discovered incidentally by echocardiography. Am Heart J 1991; 121:1554–5.[Medline]

  10. Blondeau P. Primary cardiac tumors. French studies of 533 cases. Thorac Cardiovasc Surg 1990;38(Suppl 2): 192–5.

  11. Reynen K. Cardiac myxomas. New Engl J Med 1995; 24:1610–7.

  12. Sellke FW, Lemmer JH Jr, Vandenberg BF, Ehrenhaft JL. Surgical treatment of cardiac myxomas: long-term results. Ann Thorac Surg 1990;50:557–61.[Abstract/Free Full Text]

  13. Shimono T, Makino S, Kanamori Y, Kinoshita T, Yada I. Left atrial myxomas. Using gross anatomic tumor types to determine clinical features and coronary angiographic findings. Chest 1995;107:674–9.[Abstract/Free Full Text]

  14. 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/Free Full Text]

  15. Burton C, Johnston J. Multiple cerebral aneurysms and cardiac myxoma. N Engl J Med 1970;282:35–6.

  16. Farah MG. Familial cardiac myxoma: a study of relatives of patients with myxoma. Chest 1994;105:65–8.[Abstract/Free Full Text]

  17. Yufe R, Karpati G, Carpenter S. Cardiac myxoma: a diagnostic challenge for the neurologist. Neurology 1976;26:1060–5.[Abstract/Free Full Text]

  18. Roeltgen DP, Weimer GR, Patterson LF. Delayed neurologic complications of left atrial myxoma. Neurology 1981;31:8–13.[Abstract/Free Full Text]

  19. Lee RJ, Bartzokis T, Yeoh TK, Grogin HR, Choi D, Schnittger I. Enhanced detection of intracardiac sources of cerebral emboli by transesophageal echocardiography. Stroke 1991;22:734–9.[Abstract/Free Full Text]

  20. Reichman H, Romberg HR, Hofmann E, Becker T, Mertnes HG. Neurological long-term follow-up in left atrial myxoma: are late complications frequent or rare? J Neurol 1992;239:170–4.[Medline]





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