Left Atrial Myxoma and Acute Ischemic Stroke
In a middle-aged adult presenting with acute ischemic stroke without traditional atherosclerotic risk factors, left atrial myxoma is a critical cardioembolic source that requires urgent transesophageal echocardiography for diagnosis and emergent surgical excision to prevent recurrent stroke and sudden death. 1
Epidemiology and Embolic Risk
Left atrial myxoma is the most common primary cardiac tumor, accounting for approximately 50% of all benign cardiac neoplasms, with an autopsy incidence of 0.02%. 1, 2 These tumors carry a substantial embolic burden:
- Overall embolism rate is 25%, with embolic stroke being the presenting symptom in up to 50% of cases. 1, 3
- Myxomas with villous or papillary surface architecture have markedly higher embolic potential compared to smooth-surface tumors. 1, 3
- The mechanism of stroke involves either embolization of thrombus formed on the tumor surface or direct embolization of tumor fragments. 1, 3
Pathophysiology of Stroke
The embolic mechanism operates through two pathways: 1
- Thrombus formation on the friable tumor surface, which then embolizes to cerebral circulation
- Direct tumor fragment embolization from the myxoma itself, particularly in villous morphology variants
Most myxomas arise from the interatrial septum at the fossa ovalis region in the left atrium. 1, 2
Diagnostic Algorithm
Step 1: Recognize the Clinical Pattern
In a patient presenting with acute ischemic stroke who lacks conventional atherosclerotic risk factors (hypertension, diabetes, hyperlipidemia, smoking), immediately consider cardioembolic sources. 4, 5
Key clinical clues suggesting myxoma:
- Middle-aged patient (typically 40s-50s) without vascular risk factors 4, 5
- Multiple cerebral infarcts or "silent" infarcts on imaging 5
- History of intermittent palpitations, chest pain, or syncope 5
- Constitutional symptoms (fever, weight loss) may be present 1
Step 2: Obtain Transesophageal Echocardiography
Transesophageal echocardiography (TEE) is the first-line cardiac imaging modality for younger adults with unexplained cerebrovascular events because it has higher sensitivity than transthoracic imaging for detecting cardiac tumors. 1, 3
- TEE should be performed urgently in the cryptogenic stroke workup. 1
- Transthoracic echocardiography has lower sensitivity and may miss smaller tumors. 3
- Cardiac MRI or CT provides additional characterization for surgical planning once myxoma is identified. 1, 2
Step 3: Assess Tumor Morphology
Villous features on imaging indicate higher embolic risk and reinforce the need for prompt surgery. 1, 3
The morphologic appearance directly correlates with embolic potential—friable, papillary surfaces generate thrombus and shed fragments more readily than smooth surfaces. 1
Treatment Recommendations
Surgical Excision: The Only Definitive Treatment
Urgent surgical resection of left-sided cardiac myxoma is advised to prevent recurrent stroke, sudden death, and cardiac obstruction (Class IIa recommendation, Level of Evidence C-LD). 1, 3
All symptomatic left atrial myxomas require surgical resection. 1, 2 The surgical approach must include:
- Complete tumor removal with adequate margin of atrial septum to prevent recurrence (occurs in 1-5% of sporadic cases) 1
- Histopathologic confirmation is mandatory 1
- Surgery should be performed urgently, not electively 2, 3
Critical Management Pitfalls
Anticoagulation or antiplatelet therapy alone is inadequate and does not prevent embolic complications or relieve mechanical obstruction—it should not be used as definitive therapy. 1, 2, 3
This is a common error: while anticoagulation may seem intuitive for preventing emboli, it:
- Does not address the mechanical obstruction caused by the tumor 2
- May increase bleeding risk without reducing embolic events 2
- Cannot prevent tumor fragment embolization 1
Do not delay surgery for anticoagulation. The priority is tumor removal, not medical management. 2, 3
Thrombolytic Therapy Considerations
For patients presenting within the thrombolytic window, intravenous alteplase can be administered if standard eligibility criteria are met. 6, 7 However:
- Carefully monitor for hemorrhagic transformation, as this may delay necessary cardiac surgery 6
- The extent of anticoagulation therapy post-thrombolysis requires close surveillance 6
- Thrombolysis does not replace the need for urgent surgical excision 6, 7
Special Considerations
For patients with right-sided cardiac tumors, evaluate for patent foramen ovale (PFO) because paradoxical embolism of tumor fragments or venous thrombus can occur. 1, 3
Post-Operative Management
- Long-term follow-up with periodic echocardiography to monitor for recurrence 1
- Recurrence rates are 1-5% in sporadic cases but higher in familial syndromes 1
- Histopathology will show myxoma cells (lepidic cells) with abundant myxoid matrix, vascular channels, and often hemorrhage and hemosiderin deposition 1, 2
Clinical Bottom Line
The diagnostic pathway is straightforward: cryptogenic stroke in a middle-aged patient → urgent TEE → identify myxoma → assess morphology → emergent surgical excision. 1, 3 Do not waste time with anticoagulation trials or delayed surgical planning—the 25% embolism rate and risk of sudden death mandate urgent intervention. 1, 3