Contralateral recurrence of atrial myxoma - case report and review of the literature.

Cardiac myxomas are the most frequent cardiac tumors, and they have the capability for recurrence years after resection, in the same or in different cardiac chambers. Hence, follow-up is important. Contralateral recurrences of myxoma are uncommon. We report a 7 year old boy who had a right atrial myxoma resected, and who had recurrent myxoma in the left atrium. The literature regarding such recurrence is reviewed.

Introduction

Myxomas are the most common primary cardiac tumors, comprising 30-50% of the total in most pathological series. More than 90% per cent of myxomas have been reported to occur sporadically.12 The recurrence rate of excised myxomas is between 5% and 14%, with most recurrences at the original tumor site. Only a few cases have been reported of remote recurrence in excised myxomas. The mean age of patients with sporadic myxoma is above 50 years, and 70 per cent are females. Myxomas are rare in teenagers and are extremely uncommon in preschool children. Approximately 86 per cent of myxomas occur in the left atrium, the usual site of attachment is in the area of the fossa ovalis. Myxomas may recurr in the same or different cardiac chambers.34 We report a 7 year old boy who had a right atrial myxoma resected, and who had recurrent myxoma in the left atrium.

Patient

A 7-year-old boy complaining of dyspnea and fatigue was admitted to the Cardiology Division of our hospital with a previous history of right atrial myxoma. He had been operated on 2 years prior for right atrial myxoma. The myxoma had been resected from the interatrial septum with its pedicle, and the defect had been repaired with a Dacron patch. He had no familial history of cardiac tumors.

On physical examination at arrival, blood pressure was 90/50 mmHg, pulse rate 115/min, and he was tachypnoeic. His liver was palpable at 6 cm below the costal margin, and his jugular venous pressure was slightly elevated. Auscultation revealed a mild systolic murmur suggestive of both mitral and tricuspid insufficiency, and a prominent pulmonary component to the second heart sound was also noted. There was evidence of ascites in the abdomen. The skin was normal.

Electrocardiography showed sinus tachycardia, and right ventricular hypertrophy. Chest X-ray showed minimal cardiac enlargement. Other laboratory tests were within normal limits.

Echocardiographic examination revealed a large left atrial myxoma (approximately 40 by 34 mm). The myxoma arose from the interatrial septum of the left atrium. It was mobile during systole and diastole, almost completely obstructing the mitral orifice (figure 1).

Figure 1

Echocardiographic image of myxoma in parasternal long axis view (LV: left ventricle, RV: right ventricle, AORT: aorta)

There was little remnant on the septal surface of the right atrium (figure 2), and mild enlargement of all cardiac chambers was present. Colour Doppler examination showed mild mitral and tricuspid insufficiency.

Figure 2

Echocardiographic image of myxoma on the interatrial septum (Note the mark on the left side of interatrial septum) (LV: left ventricle, RA: right atrium, RV: right ventricle, + : Remnant on the interatrial septum)

A large myxoma along with its pedicle were totally excised at open-heart surgery (figure 3) through a right atrial approach. The atrial septum was reexcised and reconstructed with a Dacron patch. The dimensions of the excised septum were 40 by 40 mm. There were no further tumors within rest of the cardiac chambers. The operation and subsequent recovery were uncomplicated.

Figure 3

Macroscopic image of myxoma (postoperative)

Histologically, the tumor had the typical appearance of a benign myxoma composed of gelatinous appearing material with few cells and no mitotic activity.

Discussion

Myxomas are very common in the left atrium. They may also occur in the right atrium, and less commonly, in the right or left ventricle. Multiple tumors may occur in the same chamber or in a combination of chambers.2 Myxomas may be familial,4 and if so, they appear to be transmitted in an autosomal dominant manner.12

Patients with recurrent myxoma tend to be younger than patients with sporadic myxoma.1 Recurrence may be due to implantation of tumor cells during the first operative procedure, or regrowth from the original site due to incomplete excision. Recurrence of cardiac myxomas may be in the same chamber , and/or different chambers.

Although most reviews on cardiac myxomas do not report recurrence,5–9 several reviews have commented with regard to recurrence.110–21 Recurrences of myxomas are rare and contralateral recurrences are extremely rare in the literature.

Recurrent myxomas may be familial42223 or non-familial.1524 Takashi,10 Martin,11 Dang,15 Aroca,24 Fagan,25 Fujiwara,18 and Ohshima22 have reported contralateral recurrences of cardiac myxomas. Table 1 and Table 2 summarise these reports. Martin11 and Ohshima22 both reported primary right atrial myxomas that recurred in the left atrium. However, both patients were adults.

Table 1

Sporadic recurrences of myxomas in the literature

Table 2

Series dealing with myxomas in the literature

Ohshima et al22 reported a familial cardiac myxoma with multiple and contralateral recurrence. The report described a familial recurrent cardiac myxoma involving a mother and her daughter. The mother had developed recurrent multiple myxomas at 27 years of age, four years after surgical excision of left atrial myxoma in both left and right atrium, and right ventricle. In an asymptomatic 13 year old daughter a recurrent left atrial myxoma was found three years after excision of a right atrial myxoma. Aroca et al24 reported multiple recurrence of sporadic, non-familial cardiac myxoma. The patient underwent complete resection of myxoma three times within nine years.

Because of the possibility of recurrence, postoperative follow-up of all patients with cardiac myxoma is mandatory.23 Besides transthoracic echocardiography, transoesophageal echocardiography may be used for both diagnosis2125 and follow-up21 of cardiac myxomas. In the last few years, recurrent of myxomas is less frequently reported, and this may be due to improvement of surgical techniques, equipment and also experience.