Cardiac myxoma is a rare disease, with an incidence between 0.0017 and 0.03% in autopsy series.3)
Myxoma can occur in nearly all age groups but occurs frequently between the third and sixth decades of life.5)
Sixty-five percent of cardiac myxoma occurs in women.7)
About 75% of myxomas originate from the left atrium, 18% in the right atrium, and 4% in the ventricle.8)
The exact incidence of myxomas originating from the mitral valve is not clear. In one study, it was reported as 1.5% (1 case among 68 myxoma cases).9)
Myxoma originating from the heart valve was first reported by Jaleski10)
in 1934, and the first premortem diagnosis of mitral valve myxoma was reported by Sandrasagra et al.11)
in 1979. In Korea, only 2 cases were reported since 1994.12)
Clinical manifestations of myxoma are determined by the location, size, mobility, and friability. Clinical manifestations can be divided into three general categories: systemic symptoms, embolism, and intracardiac obstruction. Systemic symptoms such as general weakness, fever, weight loss, arthralgia, and erythematous rash have been observed, and laboratory abnormalities such as anemia, elevations in CRP, ESR, and globulin levels have also been reported in patients with myxoma.6)
Myxoma can cause an embolism by way of the tumor emboli or thromboemboli that are released from or formed on the surface of the tumor. As most myxomas are located in the left atrium, systemic embolism frequently occurs. In most cases, the cerebral arteries are affected, and embolization into the renal, visceral, and coronary arteries has also been reported.8)
Symptoms due to intracardiac obstruction depend on the size, mobility, and location of the tumor. These symptoms include dyspnea, orthopnea, dizziness, syncope, and pulmonary edema. Whether myxoma of the mitral valve causes an embolism more frequently than a myxoma originating from the left atrium is not known.
Echocardiography is the most important and widely available method in the diagnosis of myxoma. Echocardiography can provide information on the location, size, shape, and mobility of a myxoma. When abnormal mass lesions are found on the heart valve, it is important to distinguish tumorous conditions from valvular vegetations. The characteristic narrow stalk is the most important feature of cardiac myxoma, and it is helpful when diagnostic confusion exists. The location of the mass also can help to discriminate between theses 2 conditions. As vegetations typically occur on the low pressure side of a high velocity turbulence jet, vegetations are often found on the atrial aspect of the mitral valve.16)
However, there are no echocardiographic features that can absolutely differentiate myxomas from vegetations. Therefore, clinical settings must be considered when diagnosing the patient.
The treatment of choice for myxoma is surgical removal, and complete excision is the goal. Immediate postoperative mortality ranges from 0% to 3.6%.17)
Arrhythmia is a common postoperative complication, which may require long-term medication.18)
Recurrence develops in 3% of the patients, and the rate is higher in familial cardiac myxomas.19)
It is not known whether replacement of mitral valve reduces the recurrence of mitral valve myxoma.