From the Guidelines
Cardiac myxoma is a rare condition, occurring in approximately 0.02% of people, according to a large autopsy series, with the most common primary cardiac tumors being myxoma and fibroelastoma 1. The incidence of cardiac myxoma can be understood by considering the rarity of primary cardiac tumors, which occur in 0.02% of people, as reported in a large autopsy series 1.
- Key points about cardiac myxoma include:
- It is the most common primary cardiac tumor, accounting for a significant proportion of all primary heart tumors.
- Cardiac myxomas most frequently develop in adults, with a slight female predominance.
- About 75% of cardiac myxomas are located in the left atrium, typically attached to the interatrial septum near the fossa ovalis.
- The remaining cases occur in the right atrium and rarely in the ventricles.
- The mechanism of stroke in patients with left-sided cardiac tumors, such as myxoma, is embolic and can be either embolization of thrombus that has formed on the tumor or embolization of the tumor or piece of the tumor 1. Based on the most recent and highest quality study, the incidence of cardiac myxoma is best estimated as part of the 0.02% of primary cardiac tumors, with myxoma being the most common type 1. Considering the information provided, the focus should be on the most recent and highest quality study, which is from 2021, indicating that primary cardiac tumors, including myxoma, are rare, occurring in 0.02% of people 1.
- Important considerations for cardiac myxoma include:
- Surgical resection is the standard treatment, with excellent outcomes and low recurrence rates for sporadic cases.
- Due to their rarity, cardiac myxomas are often discovered incidentally during cardiac imaging or when investigating symptoms like dyspnea, embolism, or constitutional symptoms such as fever and weight loss.
- Approximately 7% of cardiac myxomas are familial, often as part of Carney complex, an autosomal dominant condition characterized by multiple myxomas, endocrine abnormalities, and skin pigmentation.
From the Research
Incidence of Cardiac Myxoma
- The incidence of cardiac myxoma is approximately 0.5-1 case per 1 million people per year 2
- Cardiac myxomas account for 50%-80% of primary cardiac tumors 3
- The yearly incidence of cardiac myxoma is 0.5-1 case per million individuals 4
- Cardiac myxomas have an approximate incidence of up to 0.2% in some autopsy series 5
Demographics and Clinical Presentation
- The mean age of patients with cardiac myxoma is 59.4 ± 13.5 years, with a higher prevalence of women 2
- The most common preoperative symptoms are arterial embolism and dyspnea, and 35.6% of patients are asymptomatic 2
- Patients with cardiac myxoma can exhibit various manifestations, ranging from influenza-like symptoms, heart failure, and stroke, to sudden death 5
Diagnosis and Management
- Echocardiogram is the first-line imaging technique for diagnosing cardiac myxoma, but it is fallible and may overlook uncommonly situated myxomas 5
- Transoesophageal echocardiogram shows excellent precision with a 90% to 96% accuracy in diagnosing cardiac myxoma 4
- Surgical excision is the mainstay of treatment for cardiac myxoma, and it yields very good short and long-term outcomes with a low recurrence rate 2