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© 2004 European Association of Cardio-Thoracic Surgery
Right atrial appendage myxoma following recent coronary artery bypass grafting
a Department of Cardiac Surgery, Iaso General Clinic, Mesogeion 264 Ave, 155 62 Holargos, Athens, Greece
* Corresponding author. Address: Department of Cardiac Surgery, Iaso General Clinic, Makedonias 24 str, 145 61 Kifisia, Athens, Greece. Tel: +30-3-6932-428-428; fax: +30-3-210-6502663 Received July 31, 2003; received in revised form November 17, 2003; accepted November 19, 2003
A 71-year-old male presented with recurrent atrial fibrillation, anaemia and thrombocytopenia. Six months ago he underwent an urgent triple coronary artery bypass-grafting elsewhere. Postoperatively he complained of fatigue and low-grade fever. Echocardiographs and magnetic resonance imaging showed a right atrial appendage mass, which afterwards was resected. Histology confirmed a benign myxoma. Patient's symptoms spontaneously resolved. This report demonstrates the unusual sites that myxoma may occur, with probable serious complications in case of urgent heart operations. This case also emphasizes the need for preoperative echocardiography in open heart operations or in cases with recurrent atrial fibrillation.
Key Words: Atrial myxoma; Recurrent atrial fibrillation; Myxoma-constitutional manifestations
A 71-year-old male suffered from hypertension, insulin-dependent diabetes mellitus and frequent episodes of spontaneously resolved atrial fibrillation (AF). He also reported a recent anaemia (haemoglobin 13 g/dl) and thrombocytopenia (platelets 130 000/mm3), attributed to aspirin taking. Six months ago he underwent an urgent triple coronary artery bypass grafting (CABG) under cardiopulmonary bypass (CPB) in another hospital, due to left main and three-vessel disease. His postoperative course was complicated by left phrenic nerve palsy, left lower lobe (LLL) atelectasis, pleural effusion and refractory to medical therapy AF. The patient had never had echocardiography in the past, not even preoperatively, since physical examination was normal and ejection fraction (EF) was estimated during left catheterisation. After his discharge from hospital the patient complained of several episodes of mild dyspnoea, fatigue and low-grade fever. A transthoracic and afterwards a transesophageal echo (TEE) of the heart (Fig. 1) both revealed a round, mobile, pedunculated mass of the right atrial appendage (RAA). Tricuspid valve and EF were normal. The following magnetic resonance imaging (MRI) study suggested that the mass was unlikely to be a thrombus, since the mass received the intravenously administered contrast material (Fig. 2a). Furthermore, MRI showed atelectatic regions in the LLL. Patient's postoperative course and MRI findings raised the suspicion of an atrial mass complicated with pulmonary embolism. A spiral CT pulmonary angiography was performed and excluded this possibility.
Having a possible diagnosis of a RAA myxoma without embolic complications the patient underwent a second sternotomy. The central venous lines were inserted through the left jugular vein, only for a few centimetres. Bicaval cannulation was performed as distal as possible and after initiation of CPB a right atriotomy was made under induced, non-ischemic normothermic fibrillation. A soft, 2x3 cm, spherical mass was found in the lateral atrial wall, between RAA and superior vena cava (SVC). It was totally resected along with its pedicle and a wide cuff of normal free atrial wall. The postoperative course was uneventful and the patient was discharged without AF, despite the fact that he did not receive any anti-arrhythmic drug. Histopathologic examination confirmed the diagnosis of a typical benign myxoma (Fig. 2b). Six months later the patient has no symptoms and haemoglobin and platelets have gradually increased to 14.5 g/dl and 360 000/mm3, respectively.
Cardiac myxomas are primary intracavitary tumors occurring within any of the cardiac chambers. However, 70–90% of them are located in the left atrium. Moreover, the majority of atrial myxomas arise from the atrial septum. Other sites of origin, such as the anterior and posterior atrial wall or the appendage are rare, with the last location the most rare of all. Myxomas in such unusual locations have been described and they were causing complications, such as SVC obstruction, tamponade or pulmonary embolism [1–4]. What makes this case interesting is that myxoma occurred in the very unusual site of RAA and that this was the place of venous cannulation a few months ago, without any embolic events from tumor fragments. Our patient's medical history supports the preexistence of the myxoma. Preoperative presence of anaemia and thrombocytopenia could be considered as constitutional manifestations of the myxoma, especially because both of them improved postoperatively [4,5]. Moreover, the long-standing AF episodes, before and after CABG, totally disappeared after myxoma resection. Another confusing sign in our case was the low-grade fever after CABG. Differential diagnosis included: post-pericardiotomy syndrome, atelectasis and pleural effusion due to phrenic nerve palsy, systemic manifestation of myxoma, infection of the myxoma and pulmonary embolism. The last two causes have a particular surgical interest. Infection of atrial myxoma is a very rare condition which can cause further septic complications, like endocarditis [6]. Pulmonary embolism from tumor fragments is potentially fatal. If diagnosed preoperatively embolectomy is required for removal of the fragments [3]. This was avoided in our case due to the negative result of pulmonary angiography. However, cross clamping of pulmonary artery has been proposed in order to reduce the risk of intraoperative pulmonary embolism [1]. This case demonstrates also the need for preoperative echocardiography in all CABG patients or in cases with recurrent AF. As an alternative, intraoperative TEE will also reveal the dimensions and site of origin of myxomas, even when they are unsuspected [7]. The point of attachment will also be shown in the majority of myxomas with the use of MRI [8]. Furthermore, MRI combined with intravenous administration of contrast material will differentiate the mass from a thrombus, since the mass receives the contrast material. Percutaneous transvenous biopsy for preoperative diagnosis has been proposed but MRI findings in our patient led us to avoid it [9]. En-block excision of the tumor with a wide cuff of normal tissue is the treatment of choice for all atrial myxomas. The classic median sternotomy approach was preferred in our case because of the recent CABG. However, myxoma resection with video-assisted port-access surgery has been reported, as a less invasive procedure [10]. In conclusion, this report emphasise the importance of echocardiography for patients with recurrent AF or persisting anaemia and thrombocytopenia of unknown origin. Moreover, this case supports the necessity of preoperative echocardiography in candidates for open-heart surgery, since cardiac myxomas may have very unusual location.
ICVTS on-line discussion Author: Dr. Sameh Ibrahim Sersar, Assistant Lecturer of Cardiothoracic Surgery, Mansoura University, Department of Cardiothoracic surgery, Mansoura, 123 Egypt Date: 13-Jan-2004 Message: I do not agree with your title ("Right atrial appendage myxoma following recent coronary artery bypass grafting"). It is not accurately representative for this case. I do think that the myxoma was present before the first operation, but was missed due to underestimation of the risk of the case. Physical examination in a patient with anaemia, thrombocytopenia and spontaneously resolving AF cannot be considered normal. Second, what did they do for the postoperative course that was complicated by left phrenic nerve palsy, left lower lobe (LLL) atelectasis, pleural effusion and refractory to medical therapy AF? In the operation of right atrial myxoma removal, do you routinely snare the SVCand IVC or not? We will also raise the possibility of endocarditis in the differential diagnosis. I do not agree with you that median sternotomy was the best incision. This a redo operation with patent grafts which are, as we know, a contraindication for median sternotomy. It is difficult to agree with you that removal of a right atrial myxoma was a cause for disappearance of AF. doi:10.1016/S1569-9293(03)00275-5
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Abstract
1. Case report
