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Ann Thorac Surg 1998;66:810-813
© 1998 The Society of Thoracic Surgeons

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Original articles: Cardiovascular

Surgical experience with intracardiac myxomas: long-term follow-up

^a Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India

Accepted for publication April 17, 1998.

Address reprint requests to Dr Bhan, Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110 029, India

	Abstract

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Background. Myxomas are the most common benign intracardiac tumors. This report summarizes our 20-year experience with these tumors.

Methods. Sixty-six patients (25 male) with a median age of 39 years (range, 6 to 70 years) underwent surgical excision of primary or recurrent intracardiac myxomas during the years 1976 to 1996. Symptom duration ranged from 2 to 8 months. There were 55 left atrial myxomas, 10 right atrial myxomas, and 1 biatrial myxoma. Three of the patients were in one family. The surgical approach comprised complete wide excision.

Results. There were two early deaths. Late follow-up is 89% (57/64) complete. There was one late death, which was not due to a cardiac cause. Echocardiography at a mean follow-up of 66.9 months (range, 7 to 241 months) showed no recurrence of sporadic myxomas. However, 2 of the 3 patients with familial myxomas had recurrence.

Conclusions. Surgical excision of atrial myxoma gives excellent short-term and long-term results leading to eventual cure of nonfamilial myxomas. However, familial myxomas retain a strong tendency to recur even 20 years after excision.

	Introduction

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Primary cardiac tumors are uncommon and represent only 5% to 10% of all neoplasms of the heart and pericardium [1]. Approximately 80% of primary cardiac tumors are benign, and of these, more than half are myxomas [1, 2]. With the advent of new diagnostic modalities, especially two-dimensional echocardiography, the diagnosis has been easier and less hazardous. Surgical intervention offers a potential for cure. Early detection and improved anesthesia and surgical techniques have made excision of cardiac myxomas possible with gratifying results. This report summarizes our 20-year clinical experience with intracardiac myxomas at All India Institute of Medical Sciences, New Delhi.

	Material and methods

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Between 1976 and 1996, 66 patients underwent complete wide excision of primary or recurrent intracardiac myxoma. These operations comprised 0.23% of the 28,042 cardiac surgical procedures performed in this center during that period. Twenty-five patients (37.9%) were male and 41 (62.1%), female, and the median age was 39 years (range, 6 to 70 years) (Table 1). There were 55 left atrial myxomas (83.3%), 10 right atrial myxomas (15.2%), and 1 biatrial myxoma (1.5%). Three of the tumors were familial myxomas involving mother, son, and daughter whose cases we [3] have reported previously. The mother had a biatrial myxoma, whereas the son and the daughter had left atrial myxomas. However, none of these 3 patients had associated lesions in the form of multiple pigmented skin lesions, skin myxomas, myxoid fibroadenomas of the breast, or nodular adrenocortical disease as described by Carney and colleagues [4] and others [5].

View larger version (109K): [in this window] [in a new window]   Fig 1. Transthoracic echocardiograms showing (top) two-dimensional and (bottom) M-mode images of one left atrial myxoma. The former shows the tumor (curved arrow) and the latter, the characteristic tumor "plop" (straight arrow).

View larger version (71K): [in this window] [in a new window]   Fig 2. Transthoracic two-dimensional echocardiogram showing left atrial myxoma. The myxoma is attached to the interatrial septum and prolapsing into the left ventricle (LV) through the mitral valve. (AO = aorta; LA = left atrium; RV = right ventricle.)

Operative findings and surgical technique
All patients underwent operation soon after the diagnosis of atrial myxoma was made. Three patients needed an emergency procedure because of the severity of the symptoms.

The tumor was excised under cardiopulmonary bypass using aortic and bicaval cannulation and moderate hypothermia. Myocardial protection was achieved by antegrade blood cardioplegia. Care was taken not to manipulate the tumor before the aorta was cross-clamped. Complete excision of the tumor with a cuff of interatrial septum was the basic principle of excision. All four cardiac chambers were thoroughly explored for additional myxomas. In 1 patient with a left atrial myxoma, such exploration resulted in detection and excision of another myxoma inside the right ventricular cavity attached to the lower part of the interventricular septum. This ventricular myxoma was not detected on preoperative echocardiography. In cases where the left atrial myxoma was attached to the posterior left atrial wall, it was excised with the involved portion of the left atrium, and the defect was repaired directly. The surgically created atrial septal defect was repaired directly in 18 patients (27.3%) and with a Dacron patch (C.R. Bard, Inc., Haverhill, MA) in 48 (72.7%). Copious irrigation of the atria and ventricles with cold saline solution was done to eliminate any loose tumor fragments that might have been dislodged during removal of the tumor.

Seven patients with left atrial myxoma had rough and thickened mitral leaflets, and another 2 patients showed degenerative changes in the mitral valve, but only 5 had evidence of mild mitral regurgitation. Repair or replacement of the mitral valve was not necessary in this series. One patient needed coronary artery grafts to the left anterior descending coronary artery and right coronary artery, and another patient underwent aortic valve replacement as an associated procedure.

Pathologic findings
Forty-six patients (69.7%) had a pedunculated tumor; all the other tumors were sessile. The tumor was attached to the fossa ovalis in 64 patients (97%) and to the posterior left atrial wall and adjacent atrioventricular junction in 2 (3%). All the right atrial myxomas were pedunculated. The single biatrial myxoma was sessile and attached to the fossa ovalis on its right and left atrial aspects. The tumors ranged in size from 1 x 1 x 0.5 cm to 10 x 8 x 7 cm and weighed between 2.5 and 132 g. Microscopic examination confirmed the diagnosis of myxoma in every instance.

Follow-up
All the patients were followed up on an outpatient basis at regular intervals. They underwent clinical examination, roentgenography, electrocardiography, and echocardiography. Transthoracic echocardiography was performed routinely prior to discharge and then subsequently every year. Initially transesophageal echocardiography was carried out in patients who had a poor transthoracic echocardiographic window, but over the last 2 years, it has been done routinely.

	Results

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Sixty-four patients (97%) survived the operation. One patient died of progressive hepatic failure and 1, of a cerebrovascular accident.

Ten-year follow-up of our patients was reported in 1988, at which time there was no recurrence [6]. Complete follow-up is available for 57 of the operative survivors. Seven patients were lost to follow-up. Mean follow-up was 66.9 months (range, 7 to 241 months). There was one late death, which was due to hepatic encephalopathy. Forty-six patients were in New York Heart Association class I and 10 patients, in class II at follow-up. The patient who had associated aortic valve replacement sustained an embolic stroke 4 years after the initial operation and recovered fully with conservative treatment.

Trivial mitral regurgitation was present in 4 patients and mild regurgitation, in 2. Trivial tricuspid regurgitation was seen in 3 patients, 1 of whom had excision of a right atrial myxoma.

None of the sporadic myxomas recurred, but 2 of the 3 patients with familial myxomas had recurrence. The mother, who first had excision of a biatrial myxoma, had a biatrial recurrence 2 years after operation. The left atrial component was sessile, and the right atrial component was pedunculated and attached to the fossa ovalis. After excision of the recurrent biatrial myxoma, the atrial septal defect was repaired with a Dacron patch. The patient is doing well at 46 months’ follow-up. The son was first operated on when he was 10 years of age; a pedunculated left atrial myxoma attached to the fossa ovalis was excised, and the atrial septal defect was repaired directly. Twenty years later, he was seen with recurrent peripheral embolism and was found to have tumor recurrence at the junction of the left pulmonary vein with the left atrium. The patient is doing well 10 months after excision of the recurrent left atrial myxoma. In both patients, the diagnosis was made during routine two-dimensional transthoracic echocardiography and was confirmed by a transesophageal echocardiographic study.

The actuarial survival for the whole group was 94.99% ± 2.85% at 20 years (Kaplan-Meier). The probability of disease-free survival for patients with sporadic myxomas was 92.09% ± 3.90 at 20 years, and for those with familial myxomas, it dropped to 0 at 20 years.

	Comment

Top Abstract Introduction Material and methods Results Comment References

 
Cardiac myxomas are benign intracavitary neoplasms. Their incidence in cardiac surgery is between 0.0013% and 0.005% [7, 8]. The exact incidence in India is not known, but these tumors were the cause of 0.24% of all cardiac operations performed at our institution and constituted 91.7% of all primary cardiac tumors (n = 72) operated on during this period in our institution. This incidence is higher than that in the literature (40%) but agrees with figures reported by Miralles and colleagues [9].

Echocardiography is currently the most important diagnostic modality available for imaging cardiac tumors. It is noninvasive and does not pose the risk of tumor embolization. Echocardiography allows a preoperative diagnosis with a fair degree of accuracy. Two-dimensional echocardiography can quantitate tumor size, shape, attachment, and mobility and can screen other cardiac chambers accurately for additional tumors. It provides better imaging of right heart tumors and may preempt the need of diagnostic cardiac catheterization [10–13]. Transesophageal echocardiography has increased the specificity and sensitivity of the diagnosis, especially in patients who have a poor transthoracic echocardiographic window. In our series, echocardiography was uniformly successful in diagnosing the tumor. Only patients requiring coronary angiography undergo the invasive catheterization procedure.

Although little is known of the time course of myxomas before their symptomatic period, we agree with other investigators [14–16] that they grow fairly rapidly, as evidenced by the high mitral valve gradient without appreciable enlargement of the atrium on chest roentgenography and echocardiography. Also, the duration of symptoms in our patients ranged from only 2 to 8 months. More than half of our patients had class IV symptoms. This advanced symptom staging may be the result of rapid progression of disease as well as late referral.

Surgical excision of cardiac myxomas must be done as soon as possible after the diagnosis is established because of the high risk of valvular obstruction or systemic embolization. Although no recurrences have been reported in patients treated with conservative resection, a wide excision with a large cuff of atrial septum is preferable [9, 14]. Lesser resection is justified only in cases where wide resection and reconstruction may be difficult [11], as when the myxoma is attached to the posterior wall of the left atrium and near the atrioventricular groove. In our experience, the transseptal approach, like a biatrial incision, also allows good exposure, ease of resection, and visualization of all the cardiac chambers for any concomitant tumor. We routinely explore all the cardiac chambers for additional tumor, as it can be missed by preoperative studies. In 1 of our patients we found an additional myxoma in the right ventricular cavity that was not detected preoperatively. Murphy and coworkers [17] also recommended exploration of all four chambers of the heart, though by a biatrial approach.

The potential for recurrence was reported by Gerbode and colleagues [2]. Recurrence can be due to inadequate resection, intraoperative implantation, embolization, or multicentric growth [18–20]. This series has one instance of recurrent familial biatrial myxoma and one instance of recurrent familial left atrial myxoma. Biatrial myxomas are known to recur [21], as are familial myxomas. In both patients, the diagnosis was done by routine transthoracic echocardiography. Confirmatory transesophageal echocardiography was carried out preoperatively. None of the sporadic cardiac myxomas in this series recurred. The high incidence of familial myxoma recurrence in this series (66.7%) agrees with that reported in the literature [22, 23]. We think complete excision of the tumor and its pedicle with a cuff of surrounding normal tissue and copious irrigation of the cardiac chambers to remove tumor debris may help avoid recurrence. In addition, careful handling of the myxoma itself may prevent intracardiac implantation or peripheral embolization of tumor fragments [17, 18].

In conclusion, surgical intervention probably offers a cure for patients with sporadic intracardiac myxomas. Familial myxomas have a strong tendency to recur, even 20 years after excision.

	References

Top Abstract Introduction Material and methods Results Comment References

 

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