Primary cardiac tumors (PCT) are rare neoplasms that represent less than 0.2% of all tumors (1). Myxoma is a type of PCT that comprises 5%–10% of the cardiac and pericardial lesions. It most commonly affects females between the ages of 40 and 60 (2, 3). Generally, myxoma develops in the left atrium (LA), followed by the right atrium (RA) (4, 5). This tumor usually develops from the atrial septum near the fossa ovalis, but it can rarely develop from the posterior wall, anterior wall, or left atrial appendage (6). Based on the location, this neoplasm may be asymptomatic or can cause embolization or intracardiac obstruction, which results in heart failure, sudden cardiac death, and arrhythmia (4). However, surgical intervention has become the standard modality to manage cardiac myxoma, but intraoperative embolization and recurrence are the major complications (7). Therefore, surgical intervention is challenging and needs extensive care and experience to decrease morbidity and mortality (8, 9). This study aims to report an 11-year experience of a single center in the management of cardiac myxoma.

This study included 80 cases with either primary or recurrent intracardiac myxomas. The mean age of the patients was 46.3 years. Females (67.5%) were predominant over males (32.5%). Shortness of breath was the most common symptom (86.25%). The LA was the most affected site (83.75%), followed by the RA (13.75%) (Table 1). Coronary artery bypass grafting (CABG) was required as the secondary or associated intervention in 19 (23.75%) cases (Table 2). Atrial fibrillation (AF) was found in 6.25% of the cases. The mean time of intensive care unit (ICU) admission and hospital stays were 55 h and 9 days, respectively. Re-exploration (once) was done in 3.75% of the cases caused by bleeding. The recurrence and mortality rates were 11.25% and 3.75%, respectively (Table 3). Out of the recurrent cases, four (44.4%) were familial myxomas. The mortality in our cases was 30 days. One of them died after a concomitant procedure (mitral valve replacement) due to low cardiac output (right ventricular failure), and another one died after primary surgery caused by renal impairment. The third one died after reoperation due to a cerebral vascular accident (CVA). During the average 5 years of follow-up, an echocardiogram was done every three months for patients with a positive family history and every six months for patients with a negative family history.

Myxoma is the most common type of benign cardiac tumor that originates from primitive stromal cells and differentiates along the endothelial lines (3, 10). This tumor usually develops in the LA; however, its occurrence in other sites like the RA, left ventricle, mitral, and tricuspid valves has been reported (10, 11). Females between the ages of 40 and 60 are more frequently affected by myxoma, except for familial myxoma, which is commonly seen in young-aged individuals (3, 10). In the current series, females were the dominant group, and the mean age of the patients was 46.3 years. The LA was found to be the affected site in 83.75% of the cases, followed by the RA (13.75%). There was only one case of biatrial myxoma and one of right ventricular myxoma.

Alongside the hemodynamic abnormalities owing to the embolism or tumor obstruction, several constitutional symptoms may be observed in patients with myxoma, including dyspnea, palpitation, fever, and weight loss. The clinical manifestations of the tumor are determined by its size, location, surface, and mobility (1, 3). Dyspnea (70%) and palpitation (35%) have been reported to be the most familiar symptoms (4, 12). An embolism caused by myxoma may result in myocardial infarction (13). Therefore, symptomatic myxomas should be surgically excised immediately after diagnosis to prevent pulmonary embolism and other complications (1). The incidence of pulmonary embolism and systemic embolization from left atrial myxomas has been reported to be less than 10% and 25%–50%, respectively. Another study reported cerebrovascular accidents (CVA) in 22% of the cases (3, 14). In this study, most of the cases (86.25%) presented with shortness of breath (dyspnea), but a small number of patients (8.8%) had palpitations. CVA was found to be 6.3%, and none of the cases were associated with systemic or pulmonary embolism.

Different techniques have been proposed to diagnose cardiac myxoma. Cardiac catheterization and angiocardiography were originally used to diagnose atrial myxoma, but this traditional method is an invasive procedure that can cause peripheral embolism and may provide false-positive or false-negative results (7). Echocardiography is a noninvasive and accurate procedure to detect intracardiac tumors. This modality can determine the character, location, and mobility of cardiac myxoma, with the capability to consider the presence of biatrial tumors and multiple carcinomas. TTE has a sensitivity of 95% in detecting myxoma (4, 7). In open-heart surgical interventions, ischemic cardiac arrest by cardioplegic solutions and cardiac hypothermia have been encouraged, especially in the excision of atrial myxoma. This application provides a relaxed field for operation and decreases the risk of tumor embolism (7). The recent surgical method in the treatment of atrial myxomas is the standard median sternotomy with CPB, mild hypothermia, and cardioplegic solutions (2, 15). In the present study, TTE was conducted in all patients preoperatively, and coronary angiography was also performed in patients older than 40 years. CPB and mild hypothermia were used, and myocardial protection was achieved by cold St. Thomas and Del-Nido cardioplegia solutions. All surgical operations were conducted through the standard median sternotomy.

Selkane et al. in their case series reported six concomitant mitral valve procedures with three cases (7.5%) of secondary replacement of the mitral valve (16). Furthermore, Kabbani et al. conducted four (16.6%) mitral valve replacements in the resection of 24 myxoma tumors. In a study by Semb et al., tricuspid valve replacement was performed in 18.18% of the cases (17, 18). In this study, CABG was performed in 23.75% of the cases, and mitral valve replacement and tricuspid valve repair were conducted in 17.5% and 6.25% of the cases, respectively.

To reduce the risk of recurrence, the myxoma must be completely removed by resection of the tumor's base and a portion of the surrounding interatrial septum (1). The recurrence rate of atrial myxoma has been reported to be 5% to over 14% (6). Several factors, like family history of myxoma, young age, multifocal tumors, and interleukin-6, play a significant role in the recurrence of myxoma (6, 16). Postoperatively, the recurrence in patients with familial myxoma is 21%–67% (19, 20). In previous studies, the mortality rate due to the excision of myxoma has been quite variable. In a study by Sutton et al., the mortality rate was 2.7%, and 5% mortality has also been reported (2, 21). A high mortality rate of about 25% was reported in a series of 17 cases (22). Symbas et al. recorded a high mortality rate in their study due to embolization and obstruction of intracardiac blood flow in the time interval between diagnosis and operation (23). In the current study, total resection of the tumors was conducted. Irrigation of the atria and ventricles with cold saline was done to eliminate any tumor fragments and decrease the risk of recurrence. However, the recurrence rate in this series was 11.25%, and nearly half of them were familial myxomas; this may be an explanation for this rate of recurrence in this study. The mortality rate was 3.75%, which is a fair outcome in comparison to the previously mentioned studies.

Recurrence and tumor embolism are major risks of surgical intervention for myxoma. Good preparation using TTE as a diagnostic tool and conducting a standard median sternotomy by experienced surgeons to complete resection of the tumors can decrease the rate of recurrence, embolism, and even mortality rate.