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Ann Thorac Surg 1997;63:697-700
© 1997 The Society of Thoracic Surgeons

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Original Article: Cardiovascular

Cardiac Myxoma: 40 Years' Experience in 63 Patients

Department of Thoracic Surgery, Karolinska Hospital, Stockholm, Sweden

Accepted for publication October 8, 1996.

	Abstract

Top Footnotes Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 
Background. Cardiac myxomas were resected in 63 patients, including the first successful procedure in the world.

Methods. Patient data and data obtained during follow-up of the survivors were reviewed.

Results. Preoperatively, valve obstruction occurred in 56% and emboli in 32% of the patients. The myxomas were located in the left atrium in 88%, right atrium in 10%, and both atria in 1 patient (1.6%). One patient died early, and 5 died late. The 20-year survival rate was 85%. At a median of 13 years (range, 1 month to 42 years) after resection, 26% of the survivors reported having various cardiopulmonary symptoms, 5% had had thromboembolic events, and 69% were asymptomatic. After 2 years, there was a recurrence in a young woman who had had a multifocal myxoma at the time of the primary operation. No other evidence of recurrence was found at autopsy or during repeat echocardiography in the remaining patients during an observation period of 787 patient-years.

Conclusions. Surgical resection of a single myxoma is a safe and effective treatment, with a low risk of recurrence. After uncomplicated resection, the frequency with which postoperative echocardiography is performed should be limited.

	Introduction

Top Footnotes Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 
See also page 700a.

Cardiac myxoma is a rare benign tumor with an estimated incidence of 0.5 per million population per year [1]. Approximately 75% to 80% of myxomas are located in the left atrium, 10% to 20% are in the right atrium, and 5% to 10% are in both atria or either ventricle [1–4]. The gelatinous, lobulated tumor arises from a short stalk in the atrial septum in 50% of patients, but myxomas may have multiple foci and can involve valvular tissue [1–5]. Because the symptoms are atypical and vary greatly, the diagnosis was elusive until the advent of echocardiography [6]. The first successful removal of a cardiac myxoma was performed in Stockholm by Clarence Crafoord on July 16, 1954 [7, 8]. That patient was the second in the world to survive cardiopulmonary bypass, and she is still alive.

Our review of the entire experience with cardiac myxomas in Stockholm since 1954 was undertaken to assess the long-term prognosis for such patients after resection.

	Patients and Methods

Top Footnotes Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 
From 1954 to June 1996, 63 patients with a cardiac myxoma were operated on in Stockholm. Seventeen (27%) were male and 46 (73%) were female. The mean age of patients at the time of operation was 51 years (range, 15 to 76 years). The most frequently observed symptoms were associated with mitral obstruction (Table 1). The obstruction caused congestive heart failure in 9 patients (14%), and 26 (42%) reported dyspnea. Nine patients (14%) suffered a stroke, and 5 (8%) had transient neurologic symptoms. A 20-year-old woman had acute bilateral femoral artery emboli, and another patient had an embolus at the aortic bifurcation. In both cases, histologic examination of the removed emboli revealed myxoma. In addition, constitutional symptoms and signs of a generalized illness were reported by many patients. Eleven patients (17%) had a high sedimentation rate, and anemia was present in 1. None of the patients had a family history of myxoma.

Surgical Technique
Surgical excision of the myxoma was performed as soon as possible after the diagnosis, using cardiopulmonary bypass and moderate hypothermia (32° to 34°C). A right thoracotomy was used in 1 patient and a left in 5. A median sternotomy was performed in the remaining patients. Both caval veins were cannulated and taped, and the heart was not manipulated until the aorta had been cross-clamped. The surgical approach was uniatrial in 56 patients (88%); both atria were opened in 4 patients (6%). The notes on the operation were not available in 3 patients (6%), who were among the first to undergo the procedure. To prevent embolization, a spoon was sometimes used to support and expose the tumor until it was excised. The pedicle of the tumor along with an adjacent cuff of endocardium was included in the tissue removed. The surgical technique was, of course, not uniform during the years, but the tumor was always resected, together with a few millimeters of surrounding endocardium. The atrial septum was partially excised in 11 patients (17%). Fifty-six myxomas (88%) were located in the left atrium, and six (10%) were in the right atrium. One patient had myxomas in both atria. The diameter of the tumors ranged from 1 to 10 cm. The diagnosis of a myxoma was confirmed by pathologic examination.

Analyses of Data
Follow-up of the survivors was done by means of questionnaires and telephone calls. Causes of death were ascertained from the National Cause of Death Register. Survival rates were calculated using the Kaplan-Meier method [11].

	Results

Top Footnotes Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 
Two of the 3 patients in whom the correct diagnosis was not made until the time of operation died. Intractable ventricular fibrillation developed in 1 patient, a 59-year-old man, who died in the operating room. The initial closed procedure was successfully switched to cardiopulmonary bypass in the second patient, a 47-year-old woman, and it was possible to remove the myxoma. However, she suffered massive cerebral and peripheral embolization and died 6 months later. There has been no early death during the past 25 years and no signs of postoperative embolization in patients in whom a correct diagnosis was rendered before the operation.

Two patients who lived abroad were lost to follow-up after the first month. There were four late deaths, not including the patient who died at 6 months (Table 2). One 82-year-old woman, who had a 7 x 5–cm single myxoma located in the left atrium, suffered multiple transient ischemic attacks and died of a stroke 16 years after the operation. She had chronic atrial fibrillation. During her last month of life she was treated with acetylsalicylic acid. The overall survival rate was 85% at 20 years. Only 1 patient was followed for more than 40 years (Fig 1).

View larger version (16K): [in this window] [in a new window]   Fig 1. . Survival after resection of cardiac myxoma in 63 patients. The number of patients at risk and the 95% confidence limits are indicated.

Among those who suffered thromboembolic events, 1 patient had an arterial embolus to the left leg, 1 reported two minor strokes, and a third patient had pulmonary emboli secondary to thrombosis in a leg vein. Atrial fibrillation was present in 2 of the patients. Echocardiography showed no recurrence of the myxoma in any patient.

In 1 patient, a 28-year-old woman, suspected myxomatous tissue was cauterized close to the pulmonary veins during resection of a septal myxoma. Echocardiography showed recurrence of the tumor 2 years after the primary operation, and she underwent an uneventful second operation. Thirty-six (65%) of the 55 long-term survivors were followed up regularly, and echocardiographic findings were consistently normal. The frequency of the examinations varied; 5 patients were evaluated only once, but 1 patient had been seen twice a year since the operation in 1982. The first patient who underwent successful removal of an atrial myxoma is currently 84 years of age. Now, 42 years after the operation, she is still perfectly healthy and shows no signs of a recurrence on echocardiograms. Because one myxoma recurred during a total observation period of 787 patient-years, the linearized rate was 0.13 per 100 patient-years.

	Comment

Top Footnotes Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 
The symptoms of a cardiac myxoma are typically quite variable [1–6, 9, 12, 13]. In many patients the diagnosis was made late when the tumor had embolized or was large enough to obstruct the mitral annulus; this was the case for about half of the patients. The difficulties that existed before the era of echocardiography are further demonstrated by the fact that we and other authors encountered unexpected myxomas during operations being performed presumably for the treatment of mitral stenosis [4, 12].

A biatrial approach, together with removal of adequate margins, has been advocated to exclude the presence of multifocal tumors, and thereby eliminate the risk of recurrence, and to minimize the risk of perioperative embolization [2, 3, 14]. Nevertheless, we customarily used a uniatrial approach and encountered no perioperative embolizations in those patients in whom a correct diagnosis was made before the operation.

The prognosis for patients after surgical resection has been excellent; however, late recurrences have been reported to occur in up to 7% of surgically treated patients from 3 months to 14 years after operation [1–4, 13–16]. In 20 of 23 patients (87%), the recurrence happened within 5 years of the operation. Asymptomatic recurrences have also been observed, particularly in young patients who have a family history of the tumor, in those with multifocal myxomas, and in patients with a tumor somewhere other than in the atrial septum [1–3, 14, 16]. Late recurrence has also been reported after removal of an isolated sporadic myxoma [16]. Our single recurrent myxoma occurred in the patient with diffuse spread of myxomatous tissue close to the pulmonary vein, which was not radically resected. None of our patients had familial myxoma, however, which may explain the low recurrence rate.

Postoperative echocardiography has been recommended to detect recurrent myxoma [6, 14, 15]. However, because of the rarity of a recurrence of a single myxoma resected using the current technique, it may not be necessary to perform routine echocardiography frequently throughout life. Except for patients with multiple, atypical, or familial myxomas, a few transthoracic echocardiographic examinations performed at 5-year intervals should be adequate if there has been no recurrence in the first few years.

	Acknowledgments

Top Footnotes Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 
Our thanks to Dr Dan Lindblom for allowing us to include 1 patient operated on at the Thoracic Surgical Clinic, Huddinge Hospital, Stockholm, Sweden.

	Footnotes

Top Footnotes Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 
Address reprint requests to Dr Ivert, Department of Thoracic Surgery, Karolinska Hospital, 171 76 Stockholm, Sweden.

	References

Top Footnotes Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 

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