What is the best course of action for a patient with a small atrial septal defect (ASD) and a history of systemic lupus erythematosus (SLE) and deep vein thrombosis (DVT), currently maintained on Xarelto (rivaroxaban), with normal ejection fraction (EF) and no symptoms, regarding the potential closure of the ASD and management of anticoagulation therapy?

Management of Small ASD with SLE and DVT History on Anticoagulation

This patient requires TEE with bubble study to quantify the shunt (Qp:Qs ratio) and assess for right ventricular volume overload, which will determine whether ASD closure is indicated; the anticoagulation with Xarelto should continue lifelong given the history of DVT and SLE-associated hypercoagulability, independent of the ASD management decision. 1

Immediate Diagnostic Workup

TEE with Bubble Study - Critical Next Step

• TEE is essential to accurately quantify shunt severity (Qp:Qs ratio) and assess RV volume overload, which are the primary determinants for closure indication, not shunt size alone 1
• TEE will definitively characterize the defect morphology, measure stretched diameter, assess rim adequacy (≥5mm in most locations), and exclude additional defects or anomalous pulmonary venous drainage 1
• Transthoracic echocardiography commonly misses or underestimates ASDs in adults, particularly sinus venosus defects, making TEE mandatory for surgical planning 1

Cardiac Catheterization Considerations

• Not required before closure if TEE demonstrates clear RV volume overload without pulmonary hypertension 1
• However, given the absence of reported Qp:Qs ratio and no documented RV enlargement in the current echo, catheterization may be needed if TEE findings are equivocal 1

Cardiac Event Monitor for Atrial Arrhythmias

• Appropriate to rule out paroxysmal atrial fibrillation, which would provide independent indication for anticoagulation and potentially influence closure timing 1
• Atrial arrhythmias become increasingly common with age in ASD patients and may warrant concomitant ablation procedures 1

ASD Closure Decision Algorithm

Class I Indication for Closure (Must Close)

• Closure is indicated if TEE demonstrates RV volume overload (RV enlargement), regardless of symptoms 1, 2
• The current echo report states "small ASD" but does not document RV size or Qp:Qs ratio—these are the critical missing data points 1

Class IIa Indication for Closure (Should Consider)

• Closure is reasonable if paradoxical embolism is suspected, though other embolic sources must be excluded first 1
• Given the DVT history and SLE-associated hypercoagulability, paradoxical embolism is less likely to be the primary mechanism, but cannot be entirely dismissed 3

Do Not Close

• Closure is contraindicated if severe irreversible pulmonary hypertension with no left-to-right shunt exists 1, 2
• Current echo shows no secondary pulmonary hypertension, making this scenario unlikely 1

Critical Timing Consideration

• If closure is indicated, it should be performed promptly rather than delayed, as outcome is best with repair before age 25 years 1, 4
• Delaying closure based on absence of symptoms is a critical pitfall, as nearly 25% of patients with unoperated ASDs die before age 27 4, 2
• Even after age 40, patients benefit from closure regarding morbidity (exercise capacity, right heart failure prevention), though arrhythmia frequency is not affected 1, 4

Closure Method Selection (If Indicated)

Device Closure - Preferred Approach

• Percutaneous device closure is the method of choice for secundum ASDs when anatomically suitable (stretched diameter <38mm, adequate rim ≥5mm except toward aorta) 1, 2
• Approximately 80% of secundum ASDs are amenable to device closure 1
• Device closure has lower morbidity and shorter hospital stay compared to surgery, with similar success rates and mortality 1

Surgical Closure - Required For

• Sinus venosus, coronary sinus, or primum ASDs (not amenable to device closure) 1
• Secundum ASDs with inadequate anatomy for device placement 1, 2
• When concomitant tricuspid valve repair is needed 1

Anticoagulation Management Strategy

Lifelong Anticoagulation Indicated

• Continue Xarelto indefinitely based on DVT history and SLE-associated hypercoagulability, independent of ASD status 3
• The hematology consultation is appropriate to confirm lifelong anticoagulation indication and evaluate for inherited thrombophilic mutations (Factor V Leiden, prothrombin gene, MTHFR), which are relevant in SLE patients with thrombosis and positive family history 3

Anticoagulation and ASD Closure

• If device closure is performed, antiplatelet therapy (aspirin 100mg daily minimum) is required for at least 6 months post-procedure 1
• The patient's existing anticoagulation with Xarelto provides superior protection against device-related thrombosis compared to antiplatelet therapy alone 1
• Continue Xarelto through and after device closure; do not substitute with antiplatelet therapy alone 1

If Atrial Fibrillation is Detected

• Cardioversion after appropriate anticoagulation should be attempted to restore sinus rhythm 1
• If sinus rhythm cannot be maintained, rate control and continued anticoagulation are required 1
• The patient is already anticoagulated, which is appropriate management 1

Hypercoagulability Workup

Comprehensive Thrombophilia Panel

• Proceed with the planned hypercoagulability studies as outlined in the treatment plan 3
• Test for antiphospholipid antibodies (lupus anticoagulant, anticardiolipin, anti-β2-glycoprotein I), which are common in SLE and strongly associated with thrombosis 3
• Evaluate for inherited thrombophilias (Factor V Leiden, prothrombin G20210A, protein C/S deficiency, antithrombin deficiency, MTHFR) given positive family history 3

Follow-Up Monitoring

If ASD is Not Closed (No RV Enlargement)

• Repeat echocardiography every 2-3 years to assess RV size, function, and pulmonary artery pressure 1, 5
• Monitor for symptoms including arrhythmias and paradoxical embolic events 1, 5
• Reassess if new hypertension, coronary disease, or valvular disease develops, as these increase left-to-right shunting 1

If ASD is Closed

• Annual clinical follow-up is recommended if closure is performed as an adult, monitoring for persistent or new pulmonary hypertension and atrial arrhythmias 1
• Immediate echocardiography is required if postpericardiotomy syndrome symptoms develop (fever, fatigue, chest pain, vomiting) to assess for tamponade 1, 2

Critical Pitfalls to Avoid

• Do not dismiss the ASD as clinically insignificant based solely on "small" size descriptor without quantifying RV volume overload and Qp:Qs ratio 1, 4
• Do not delay closure if RV enlargement is present, even in asymptomatic patients, as symptoms lag behind objective dysfunction 4
• Do not discontinue anticoagulation after ASD closure; this patient requires lifelong anticoagulation for DVT/SLE history 3
• Do not assume the ASD is the indication for Xarelto; verify the primary indication through hematology consultation and cardiac monitoring 1, 3