Surgical Management of Cardiac Myxoma

300 Surgical Management of Cardiac Myxoma Gokhan Ipek, M.D.,∗ Vedat Erentug, M.D.,∗ Nilgun Bozbuga, M.D.,∗ Adil Polat, M.D.,∗ Mustafa Guler, M.D.,∗ Kaan Kirali, M.D.,∗ Önder Peker, M.D.,† Mehmet Balkanay, M.D.,∗ Esat Akinci, M.D.,∗ Mete Alp, M.D.,∗ and Cevat Yakut, M.D.∗ ∗ Koşuyolu Heart and Research Hospital, Istanbul, Turkey; and †Haydarpaşa Numune Hospital, Pathology Clinic, Istanbul, Turkey ABSTRACT Objective: Between 1994 and December 2003, 55 patients were operated for cardiac myxoma in Koşuyolu Heart and Research Hospital in Istanbul. Methods: We retrospectively analyzed our results according to the preoperative characteristics, operative procedures, and postoperative courses. Results: Of 55 patients operated, 36 (65.4%) were female and 19 (34.6%) male. The average age of the patients was 48 ± 15.5 years (range, 12–75). Thirteen patients (23.6%) previously had cerebrovascular accidents. Peripheral arterial emboli had occurred in 11 (20%) patients. The majority of the patients (44.4%) were in NYHA Class II preoperatively. One patient was presented with Carney’s complex. Most frequent location was the left atrium (85.2%). Eight patients had concommitant surgery together with myxoma extirpation. Postoperative courses were uneventful. Three patients had a new onset atrial fibrillation, two had transient conduction disturbances. There were two (3.6%) in-hospital deaths. No recurrences have been noted during the 82.4 ± 40.6 months (a total of 315.75 patient/years) follow-up. Conclusions: Surgical management of cardiac myxoma gives excellent results. In selected cases, a conservative approach may be adequate. Despite the scarcity of the neoplastic properties, careful follow-up is necessary. doi: 10.1111/j.15408191.2005.200415.x (J Card Surg 2005;20:300-304) Cardiac myxomas are rare neoplasms as the other tumors of the heart. Incidences between 0.0017 and 0.35% have been stated in literature.1,2 As an important cause of intracardiac masses, myxoma should be differentiated from a number of disorders such as thrombus, infective endocarditis, other cardiac tumors. In the 9-year period between 1994 and 2003, we operated 54 patients with myxoma 2003 in Koşuyolu Heart and Research Hospital. We revised the preoperative, operative, and postoperative characteristics of the patients with myxoma. Intracardiac masses of different etiologies were excluded. MATERIALS AND METHODS We reviewed our hospital records and operation files for the collection of preoperative and operative data. Patients with a pathologic diagnosis other than myxoma were excluded from the study. Patients were diagnosed as having myxoma in our cardiology department or were sent to our clinic for operation. Transesophageal echocardiographic appearance of a huge myxoma with cystic degeneration is shown in Figure 1. The average age of the patients was 48 ± 15.5 (range, 12–75 years). There were two children in our series being 12 and 13 years of age, respectively. Thirty-six patients were female (65.4%) Address for correspondence: Vedat Erentug, M.D., Ulus Vadi Konutlari, A1/9 84040 Ulus, Besiktas, Istanbul, Turkey. Fax: +90-212-249-40-78; e-mail: [email protected] and the remaining 19 were male (34.6%). Thirteen patients (23.6%) had a cerebrovascular accident before and a peripheral arterial emboli was accounted in 11 (20%) of the cases. The preoperative functional status and the presenting symptoms of the patients are summarized in Table 1. Majority of the patients were in normal sinus rhythm as outlined in Table 1. In the medical history, two of the patients had previous cardiac surgery, one for an atrial septal defect closure and the other for total correction of Fallot Tetralogy which was a component of a Carney’s complex.3 Preoperative laboratory values and heart sizes are listed in Table 2. There was only one patient with a hematocrit value below 30%. Morphological defects of erythrocyte were not detected in clinical work-up. No specific cause could be identified. Patients with a history of a cerebrovascular accident were diagnosed at their hospital stays and were operated after a duration not less than 3 months from the event. Each of the peripheral thromboembolic event in our clinic is routinely analyzed in order not to miss a cardiac cause. Surgical technique Median sternotomy was the standard approach in our operations. Aortic arterial bicaval venous cannulation and intermittent antegrade cardioplegia via a 22 G cannula on the ascending aorta was utilized. Moderate systemic hypothermia was used (median 32◦ C ranged 29–34◦ C). We used intermittent cardioplegia in the majority of the cases. In five patients (9.1%), J CARD SURG 2005;20:300-304 IPEK, ET AL. TREATMENT OF CARDIAC MYXOMA Figure 1. Transesophageal echocardiographic appearance of a huge left atrial myxoma showing cystic degeneration. who had CABG and aortic valve replacement (AVR) concommitantly, we used integrated antegrade and retrograde cardioplegia as our routine clinical procedure. We mostly preferred to use isothermic blood cardioplegia but in 10 patients (18.2%) we used cold crystalloid cardioplegia. The majority of the myxomas were in the left atrium. The distribution is outlined in Table 3. Only one patient had a myxoma in more than one chamber. No left ventricular myxoma was detected in our series. The average diameter of the myxomas was 5.7 ± 5.3 (range, 1–24) cm. Attachment of the left atrial myxomas were as follows: Interatrial septum in 25 patients (53.2%), fossa ovalis in 10 (21.3%), posterior wall in 6 (12.8%), free wall in 4 (8.5%), and the eustachian valve in 2 (4.2%). In the gross appearance of the masses, in 15 (27.3%) patients soft, jelly-like appearing tumor with multiple fronds was noted. In the remaining 40 patients (72.7%), it was a single, firm polypoid mass protruding into the cavity with a smooth surface. A stalk was noted TABLE 1 Presenting Symptoms, Preoperative EKG Findings, and Cardiothoracic Indices of the Patients Symptoms No % Dyspnea Palpitations Syncope No symptoms Stroke Chest pain Peripheral arterial emboli Total Normal sinus rhythm Atrial fibrillation Right bundle branch block Functional Class I II III Chest X-ray CTI < 55% CTI > 55% 16 14 11 4 4 4 2 55 43 6 6 29.1 25.4 20.0 7.3 7.3 7.3 3.6 100 78.2 10.9 10.9 15 25 15 27.3 45.4 27.3 40 15 72.7 27.3 CTI = cardiothoracic index. 301 in 41 (74.5%) cases and the remaining 14 (25.5%) were sessile. Gross appearance of a resected mass is shown in Figure 2. All materials resected intraoperatively were taken into the pathologic examination. Left atriotomy was used in 28 cases (Table 3). However for 22 patients, right atrial myxomas biatrial approach was preferred. The full thickness interatrial septum at the myxoma attachment site was resected in 25 (46.3%) cases. All septal defects created were repaired primarily except for the five patients in whom pericardial patches were used. Nine patients had significant comorbidities requiring surgery. Four had aortocoronary bypass (CABG), one AVR for aortic stenosis, two mitral ring annuloplasties for mitral regurgitation (MR), one mitral valve replacement for mitral stenosis (MS), and one left atrial appendage (LAA) ligation to a patient with a history of thromboembolic and an enlarged LAA. There were four patients with pulmonary hypertension (greater than 30 mmHg). Two patients with MS and three patients with MR were not operated for the mitral valve. In the two patients with MS, stenosis was attributed to the myxoma. MR in the three patients were seen to be insignificant central regurgitation in intraoperative control and left untouched. Pathology Diagnosis of the myxoma was confirmed with pathologic examination in each case. Intraoperatively collected samples were fixed with formalin and routinely processed for light microscopy. Staining was done with hematoxylene-eosin. Special stains (Masson’s trichrome, Van Gieson, and Pas staining) were performed in case of a requirement for diagnosis. Follow-up After discharge, follow-ups were conducted in the outpatient clinic of the hospital cardiovascular surgery department. Follow-ups were done every 3 months for the first year, every 6 months for the following 2 years, and annually for the following years. As a tertiary center, we have patients from all over Turkey, some of the patients were followed in their cities’ hospitals. Finally, telephone interviews were conducted for a surveillance search. RESULTS Constitutional symptoms were present in 7 (12.7%) of the cases and they were malaise in 3, fatigue in 2, fever in 1, and weight loss in 1 patients. The mean duration of the total extracorporeal circulation and the aortic cross-clamp periods were 69 ± 29 (ranged, 35–135) and 44 ± 23 (ranged, 18–102) minutes, respectively. In the pathological examination of the resected atrial septae, no tumoral involvement of the unaffected atrial side was observed. Two postoperative in-hospital deaths have been encountered (3.6%). Both of them had severe pulmonary hypertension preoperatively. Postoperatively, hemodynamic problems were rare. Inotropic support was not necessary for 30 (54.5%) patients but used in 25 (45.5%). The postoperative courses are outlined in Table 4. Three patients had new onset atrial fibrillation. 302 IPEK, ET AL. TREATMENT OF CARDIAC MYXOMA J CARD SURG 2005;20:300-304 TABLE 2 Preoperative Peripheral Blood Test Results and Echocardiography Findings Peripheral blood test Hematocrit (%) Hemoglobin (g/dL) White blood cell count (/µL) Platelet count (/µL) Echocardiography findings Left ventricular end systolic diameter (cm) Left ventricular end diastolic diameter (cm) Left atrium (cm) Normal sinus rhythm was restored with medication in one but the others persisted. Two transient complete atrioventricular blocks have been encountered, one of which was seen in a patient who had a concommitant CABG. The normal sinus rhythm was restored in both during follow-up. Two patients had fever during their course after the intensive care unit stay and responded well to appropriate antibiotic therapy. The patient with low Htc value preoperatively received replacement with concentrated erythrocyte solutions after the operation. She had an uneventful postoperative course. No malignancies have been encountered. DISCUSSION Myxomas are the most common of not only benign but also all types of the tumors of the heart. In the pediatric age group, angiosarcoma4 or purkinje cell tumors4 have been addressed for the most common tumors of the heart in different series. Myxomas were thought to be originating from primitive stromal cells that have a capacity to differentiate along endothelial lines. Studies with neuroendocrine markers using immunohistochemical techniques stress on another origin for myxomas. Identification of these markers suggests the endocardial sensory nerve tissue as the origin of myxomas. Occasional association with cutaneous leimyomatosis and systemic findings also resemble other tumors of neural origin.5 The presence of spindle shaped cells with an ovoid nucleus closely associated with thin Mean ± Standard Deviation Range 38.2 ± 6.1 12.7 ± 2.2 8128 ± 2585 250,900 ± 105,111 21.8–47.3 7.1–15.6 4800–13,200 120,000–427,000 3.50 ± 0.85 5.46 ± 0.38 4.5 ± 0.86 2.7–5.5 6.4–5.5 3.4–6 walled capillaries and these cells are embedded in a matrix of acid mucopolysaccharidoses.4,6 In almost 20% of these tumors, a microscopic focus of calcium may be seen.6 Myxomas are more frequently seen in left atrium.7 Atypical occurrences such as left ventricle,8 mitral,9 or tricuspid10 valves have also been reported. Overall, almost 75% to 80% are seen in left atrium, 18% in right atrium, and the remainder in the ventricles. Biatrial myxomas are increasingly being reported.11 In a study, the possibility of the right atrial myxomas and atrial septal defect has been discussed.12 Our results are also consistent with the literature reviews as the left atrium is the most frequent location and the rarity of ventricular distribution. Clinical picture of a typical patient with myxoma is usually a female patient between fourth and sixth decades of life with a sporadic, isolated left atrial myxoma.11,13 However, familial myxomas are usually seen in younger ages (average of 27 years). They are, although not as frequent as the nonfamilial types, mostly seen in left atrium (about 61%). They have a higher recurrence rate. They are sometimes multicentric.13,14 In our group of patients, we found that the female:male ratio was 2 (36:18). The mean age of the patients was found lower than expected. The single patient with the Carney’s complex have been reported before.4 Patients presenting at a younger age, with multiple tumors, and myxoma located elsewhere than right atrium should be TABLE 3 Distribution of the Myxoma and the Approaches Localization No % Left atrium Right atrium (RA) Right ventricle (RV) RA + RV Total Approach Left atriotomy Biatriotomy Right ventriculotomy Right ventriculotomy + biatriotomy Pulmonary arteriotomy Right atriotomy (transeptal) Total 47 4 3 1 55 85.6 7.2 5.4 1.8 100 28 22 2 1 1 1 55 50.9 40.0 3.7 1.8 1.8 1.8 100 Figure 2. Gross appearance of a resected left atrial myxoma with its stalk. J CARD SURG 2005;20:300-304 IPEK, ET AL. TREATMENT OF CARDIAC MYXOMA TABLE 4 Postoperative Results Drainage (cc) Time to extubation (hours) Intensive care stay (days) Total hospital stay (days) Mean Range 475 ± 308 13 ± 5.1 2.3 ± 1.0 8.1 ± 3.2 100–700 4–20 1–5 2–15 suspected for Carney’s complex and a family screening and close follow-up for recurrences should be undertaken.14 Like many of the large series, dyspnea was the most frequently presenting symptom.7,15 The major complications of the cardiac myxomas are several embolic events. The frequency has been stated before.16 Preoperatively, systemic embolization from the left atrial myxomas are reported with 25% to 50% incidences.17 However, pulmonary embolism is surprisingly as low as less than 10%.18 We have seen cerebrovascular accident in 12 patients (22.2%) and peripheral arterial emboli in 11 (20.4%) of the cases. The longer the patient is on the operation list, the higher will the risk be of a thromboembolic event ensues. Apart from the hemodynamic derangements due to the obstruction of tumor or the embolic events, there are also constitutional symptoms seen in a number of patients with myxoma. Particularly, large left atrial myxomas are apt to produce these symptoms such as fever, weight loss, Raynaud’s phenomenon, myalgia, and arthralgia. An immune reaction to the neoplasm and heart muscle is speculated to be the underlying cause. Further nonspecific symptoms may be related to the seeding of multiple small emboli in muscle and joints, hemorrhage or destruction in tumor.5 The laboratory investigations preoperatively did not give significant results. The only anemic patient had no specific cause but the anemia was not of hemolytic type as would be expected in a patient with myxoma. All patients had been diagnosed with transthoracic echocardiography (TTE) preoperatively. Transthoracic echocardiography has been stated as a method of choice in myxoma.19 The electrocardiographic changes in myxomas are not specific. In our series, normal sinus rhythm was encountered in the great majority (Table 1). Coronary angiography and CABG was performed if indicated. The embolic complications of the cardiac myxomas should alert the surgeon even if the patient has no coronary artery disease. Myxoma originated emboli causing myocardial infarction have been reported.20 The debate for uniatrial and biatrial approach continues. Most authors think that a uniatrial approach, especially for the left atrial myxoma is adequate.21 However, a large series reported from Texas Heart Institute advocates an aggressive approach and that the biatrial approach may help the surgeon to handle the tissues more gently.4 We used left atriotomy in 28 (51.85%) of the cases and biatrial approach in 22 (40.75%) patients mostly according to the surgeon’s preference. Thinking of the benign nature of the myxomas, a more conservative approach may prevail. In the early postoperative period supraventricular tachycardias, episodic junctional arrhythmias and complete atrioventricular blocks may complicate the 303 course. A significant number of the patients may require antiarrhythmic medication and a minority to cardiac pacemakers.22 Our patients revealed a fairly uncomplicated postoperative course with low incidence of arrhythmias. In the follow-up, we have not observed any local or extracardiac recurrences, which others have reported as case reports.23,24 Many of the recurrences are attributed to multifocal disease or inadequate resection at the operation and that seeding is unlikely.25 In an unusual case of metastatic left atrial myxoma, long-term remission has been reported with combined surgical resection, chemotherapy, and irradiation.26 Our analysis shows the favorable outcome of these patients with a more conservative approach. REFERENCES 1. 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