Left Atrial Myxoma: Diagnostic Work-Up and Management
Immediate Management Priority
Surgical excision should be performed urgently once a left atrial myxoma is diagnosed, as this is the only definitive treatment and prevents life-threatening complications including sudden death, stroke, and recurrent syncope. 1, 2, 3
Diagnostic Work-Up
Initial Imaging
Transthoracic echocardiography (TTE) is the first-line diagnostic modality and can establish the diagnosis in most cases, providing information about tumor location, attachment site (typically the interatrial septum), size, mobility, and surface characteristics 1, 2, 3
Transesophageal echocardiography (TEE) offers enhanced sensitivity for detailed preoperative evaluation, particularly for smaller tumors and surgical planning, and should be performed before surgery 2, 3, 4
Cardiac MRI or CT provides additional characterization for surgical planning, particularly to assess tumor extent and relationship to surrounding structures 1, 2, 3
Key Diagnostic Features to Document
- Tumor attachment site (usually interatrial septum in left atrium) 1, 2
- Surface morphology: Villous or papillary surfaces indicate higher embolic risk (25-40% embolism rate), while smooth surfaces carry lower risk 1, 2, 5
- Tumor size and mobility: Larger tumors correlate with obstructive symptoms and cardiac chamber enlargement 5
- Presence of hemorrhage within tumor: Can cause rapid expansion and acute mitral valve obstruction 4
Additional Preoperative Evaluation
Coronary angiography should be performed in older patients (typically >40 years) or those with coronary artery disease risk factors, as concomitant coronary artery bypass may be needed 6
Do NOT perform cardiac catheterization through the affected chamber, as this risks tumor fragmentation and systemic embolization 6, 5
Surgical Management
Indications and Timing
All symptomatic left atrial myxomas require urgent surgical resection (Class I recommendation) 1, 2, 3
Surgery should be performed as soon as possible after diagnosis due to the 25% overall embolism rate and risk of sudden death from acute mitral valve obstruction 1, 2, 6
Even asymptomatic myxomas warrant prompt surgical excision given the unpredictable risk of catastrophic complications 6, 7
Surgical Technique
Complete tumor excision with an adequate margin of atrial septum (wide excision of attachment site) is essential to prevent recurrence 1, 6, 7
Biatrial approach is preferred as it allows inspection of all four cardiac chambers, limits tumor manipulation (reducing embolization risk), and facilitates complete excision 6
Median sternotomy with cardiopulmonary bypass is the standard surgical approach 7
Surgical Outcomes
- Operative mortality is low (approximately 1-4% in experienced centers) 6
- Recurrence rate is 1-5% for sporadic cases and higher (up to 20%) for familial cases 1, 5
- Long-term prognosis is excellent with most patients remaining asymptomatic after complete resection 6, 7, 5
Critical Management Pitfalls to Avoid
What NOT to Do
Do NOT use anticoagulation or antiplatelet therapy alone as definitive treatment—these do not address the mechanical obstruction and do not adequately prevent embolic complications 1, 2
Do NOT perform pericardiocentesis if pericardial effusion is present, as this may worsen hemodynamics 2
Do NOT delay surgery for medical optimization in symptomatic patients, as the risk of sudden death or stroke is substantial 1, 2, 6
Do NOT manipulate the tumor excessively during surgery, as fragmentation increases embolization risk 6
Post-Operative Follow-Up
Histopathologic confirmation is mandatory to exclude other cardiac tumors and confirm the diagnosis 1
Long-term surveillance with periodic echocardiography is required to monitor for recurrence, particularly in younger patients and those with familial myxoma syndrome 1, 6, 5
Follow-up echocardiography should be performed at regular intervals (typically annually or biannually), as late recurrence can occur years after initial resection 6, 5
Clinical Context and Risk Stratification
Embolic Risk Factors
- Male sex is associated with higher embolic risk compared to females 5
- Villous or friable tumor surface significantly increases embolic potential 2, 5
- Larger tumor size correlates with obstructive symptoms rather than embolic events 5
Presentation Patterns
- Obstructive symptoms (67% of cases): Heart failure, dyspnea, syncope from intermittent mitral valve obstruction 2, 5
- Embolic events (29% of cases): Stroke is the most common, with cerebral embolism being the presenting symptom in up to 50% of embolic cases 1, 2, 5
- Constitutional symptoms (34% of cases): Fever, weight loss, elevated inflammatory markers from interleukin-6 secretion 5