- Clinical science
Cardiac myxomas are the most common type of primary tumor of the heart. They are usually benign and arise from primary connective tissue. Most cardiac myxomas arise sporadically; however, 10% are hereditary (following an autosomal dominant pattern). Even though they may develop in any chamber of the heart, most (∼ 75 %) cardiac myxomas arise in the left atrium, usually from the interatrial septum, while the rest occur in the right atrium (ventricular myxomas are rare). Clinical features are primarily caused by obstruction of the blood flow through the heart and include dyspnea on exertion, palpitations, syncope, weight loss, or even sudden death. Rarely, life-threatening conditions (e.g., stroke) may result from an embolization from the myxoma. Typical examination findings include abnormal heart sounds, such as a rumbling diastolic murmur over the apex or a characteristic “tumor plop.” The diagnosis is not easily established clinically because of the nonspecific nature of symptoms. Echocardiography is the diagnostic procedure of choice. Surgical resection of the tumor is the curative treatment of choice. The prognosis is usually favorable, but tumors can recur after inadequate resection.
- Most common primary cardiac neoplasm (usually benign)
- Most common location: left atrium (∼ 75% of all cases)
- Sex: ♀ > ♂ (3:1)
- Peak incidence: 40–60 years
Epidemiological data refers to the US, unless otherwise specified.
- The exact etiology is unknown.
- Most cases are sporadic.
- Familial inheritance:
- ∼ 10% of all cases
- Autosomal dominant inheritance
Clinical features caused directly by the tumor
Symptoms caused by obstruction
- Dyspnea on exertion, paroxysmal nocturnal dyspnea, and/or orthopnea
- Dizziness or syncope
- Auscultatory findings
- Valve damage may result in mitral regurgitation.
- Further ;
- Symptoms due to embolization
- Symptoms caused by obstruction
Because of the nonspecific nature of the cardiac symptoms, the diagnosis is often only established much later in the course of the disease.
- Echocardiography: diagnostic test of choice
- Other tests: CT and MRI scans help better visualize the intracardiac mass.
- Microscopic appearance:
- Scattered mesenchymal cells within mucoid, gelatinous material
- Produce VEGF
- Macroscopic appearance: often pedunculated, gelatinous consistency
- The only definitive treatment of cardiac myxoma is surgical resection.
- Medical intervention may be required for the treatment of associated conditions like arrhythmias, heart failure, or embolism.