Primary cardiac masses are rare and usually benign with myxoma being the most common [
1]. Up to 75% of myxomas are located in the left atrium whereas right-sided myxomas are rare (15 to 20%), with right ventricular (3 to 4%) or pulmonary artery myxomas being extremely rare [
3]. There are several case reports of right ventricular myxoma obstructing the outflow tract in adults and older children, but reports of this pathology during early childhood are scarce; cardiac myxomas in the pediatric age group have relevance in particular to a condition known as familial myxoma syndrome. This syndrome, also known as Carney’s syndrome, consists of a variable complex of mucocutaneous, visceral, and endocrine disorders. Symptoms may be variable and are determined by the tumor location and size. Right ventricular myxomas may cause obstruction of the RVOT and pulmonary main trunk, which may also lead to complications such as syncope, pulmonary embolism, and sudden death. Cardiac myxoma extending into the RVOT is a rare cause of right heart failure. Symptoms and signs include peripheral edema, ascites, and shortness of breath as a result of RVOT obstruction. The gold standard noninvasive diagnostic modality for such tumors is transthoracic or transesophageal echocardiogram. An echocardiogram enables preoperative localization of the tumor, size, shape, mobility as well as the risk of RVOT obstruction and the tumor attachment, and therefore facilitates the selection of the optimal surgical approach. Transesophageal echocardiography accurately identifies other localization of myxomas. Cardiac-gated computed tomography and cardiac-gated magnetic resonance scans offer additional information about the structure and function of cardiac tumors before surgical resection [
4]. The differential diagnosis for an intracavitary cardiac mass includes thrombus, myxoma, lipoma and nonmyxomatous neoplasm, most of which are malignant [
5]. The risk of life-threatening complications indicates the importance of early diagnosis and prompt surgical resection as soon as possible. One of the main concerns during anesthesia is the risk of tumor embolization and pulmonary obstruction during anesthesia induction or at any stage thereafter. Hemodynamic instability, inconvenient manipulation of the tumor and heart must be avoided to prevent these complications. Femoral arterial-venous cannulation can be performed for CPB initiation in the case of complete RVOT obstruction with hemodynamic collapse. Several surgical techniques have been suggested, but in each case it depends on the site of the tumor. Follow up for recurrent myxoma in an uncommon location is recommended.