What is the recommended follow-up care for adults after cardiac surgery for Atrial Septal Defect (ASD) or Ventricular Septal Defect (VSD) or both?

Adult Follow-Up After Cardiac Surgery for ASD or VSD or Both

Adults who underwent ASD or VSD repair require lifelong annual follow-up at a specialized adult congenital heart disease (ACHD) center if the repair was performed in adulthood, or if complications such as pulmonary arterial hypertension (PAH), arrhythmias, ventricular dysfunction, or residual lesions persist. 1

Immediate Postoperative Surveillance (First Weeks to Months)

Early Warning Signs Requiring Urgent Evaluation

• Fever, fatigue, vomiting, chest pain, or abdominal pain in the first weeks after surgery may indicate postpericardiotomy syndrome with cardiac tamponade and require immediate echocardiographic evaluation. 1, 2
• Pericardial effusions can develop up to several weeks after surgical repair, necessitating clinical examination and echocardiography before hospital discharge and at early postoperative visits. 1, 2
• Device erosion (for percutaneous ASD closure) may present with chest pain or syncope and warrants urgent evaluation. 1, 2

Initial Follow-Up Schedule for Device Closure (ASD)

• Echocardiography at 24 hours, 1 month, 6 months, and 1 year post-procedure 1, 2
• Clinical assessment at each visit for symptoms of arrhythmia, chest pain, or embolic events 2
• Evaluation for device migration, erosion, or other complications at 3 months to 1 year and periodically thereafter 1, 2

Long-Term Follow-Up Strategy

ASD Repair Follow-Up Intervals

For adults with ASD repaired in adulthood:

• Annual clinical follow-up is mandatory if any of the following persist or develop: 1
  • Pulmonary arterial hypertension 1, 2
  • Atrial arrhythmias (occur in 15-25% of adult patients post-closure) 1, 3
  • Right or left ventricular dysfunction 1, 2
  • Coexisting valvular or other cardiac lesions 1, 2

For uncomplicated cases (ASD repaired in childhood with no residual abnormalities):

• Follow-up every 2-3 years at an ACHD center 1
• Patients with ASD closed in childhood are generally free of late complications 1

VSD Repair Follow-Up Intervals

For adults with residual complications after VSD closure:

• Annual follow-up at an ACHD regional center is required if residual heart failure, shunts, PAH, aortic regurgitation (AR), or right/left ventricular outflow tract obstruction persist. 1
• Follow-up every 1-2 years for device closure of VSD, depending on location and other factors 1

For uncomplicated VSD repair:

• Follow-up every 3-5 years at an ACHD regional center if there is a small residual VSD and no other lesions 1
• Adults with no residual VSD, no associated lesions, and normal pulmonary artery pressure do not require continued follow-up at a regional ACHD center except on referral 1

Critical caveat: Right ventricular function and exercise capacity remain impaired even years after successful VSD repair (average 9.9 years post-repair), emphasizing the need for continued follow-up into adulthood. 4

Specific Monitoring Components at Each Visit

Clinical Assessment

• Symptoms of arrhythmia, chest pain, embolic events, or heart failure 1, 2
• Exercise tolerance and functional capacity 4
• Signs of device complications (for percutaneous closures) 2, 5

Electrocardiography

• Screen for conduction abnormalities, including late-onset complete heart block (can occur up to 15 years after AVSD repair) 1
• Monitor for new-onset or recurrent atrial arrhythmias 1, 3
• ECG at every visit for patients with residual complications 1

Echocardiographic Surveillance

• Device position and stability (for percutaneous closures) 2, 6
• Residual shunting and quantification of shunt magnitude 1, 2, 6
• Pulmonary artery pressure and right ventricular size/function 1, 2, 4
• Left ventricular function and volume assessment 1
• Valvular function, particularly aortic and tricuspid valves 7, 8
• Pericardial effusion or thrombus formation 2, 6

Advanced Imaging When Indicated

• Cardiac MRI is appropriate for quantification of ventricular volumes and shunt magnitude when clinical status changes or new symptoms develop. 6
• CT is superior for evaluating metallic closure devices and is appropriate for assessment of device position, integrity, and pulmonary venous anatomy. 6
• Transesophageal echocardiography provides superior visualization of device position, atrial septum, and can identify left atrial appendage thrombus. 6

Management of Specific Late Complications

Atrial Arrhythmias (Most Common Late Complication)

• Occur in 15-25% of adult patients after ASD closure, particularly those repaired after age 40 3
• Catheter ablation is the preferred treatment for cavotricuspid isthmus-dependent atrial flutter, which is common and highly amenable to ablation. 3
• Refer to experienced electrophysiology centers with three-dimensional mapping capabilities 3
• Implement rate control and anticoagulation for atrial fibrillation 3

Residual Shunting

• Conservative management with annual surveillance for small residual shunt (<5mm, no RV volume overload) 6
• Re-intervention (percutaneous or surgical) indicated for moderate-to-large residual shunt with RV volume overload, Qp:Qs ratio >1.5 with symptoms, or progressive functional limitation 6

Aortic Regurgitation (Common in VSD Patients)

• AR is a common indication for VSD closure in adults (49.3% in one series) 8
• Preoperative moderate or greater AR is associated with need for reoperation. 8
• Concomitant aortic valve repair at time of VSD closure is a significant risk factor for late AR progression. 8
• VSD with aortic valve deformity or AR should be treated aggressively before irreversible damage occurs 8

Device-Specific Complications (ASD Device Closure)

• Device thrombosis and cardiac erosion are the most severe late complications 5
• Ensure antiplatelet therapy compliance (aspirin minimum 100mg daily for at least 6 months) 6
• Assess for device thrombus on all follow-up echocardiograms 6
• Nickel allergy, cardiac conduction abnormalities, valvular damage, and device endocarditis are other potential delayed complications 5

Endocarditis Prophylaxis

• Endocarditis prophylaxis is indicated only for the first 6 months after ASD or VSD closure. 1, 2
• After 6 months, prophylaxis is not indicated for isolated ASD or VSD unless there are other high-risk features (prosthetic valve, previous endocarditis, unrepaired cyanotic CHD, or completely repaired CHD with prosthetic materials within 6 months) 1

Special Considerations

Pregnancy Counseling

• Pregnancy is absolutely contraindicated in patients with ASD or VSD and severe PAH (Eisenmenger syndrome) due to excessive maternal and fetal mortality. 1
• Women with small defects, no PAH, and no associated lesions have no increased cardiovascular risk for pregnancy 1
• Women with large shunts and PAH may experience arrhythmias, ventricular dysfunction, and progression of PAH during pregnancy 1

Exercise Recommendations

• Patients with small defects and no PAH have normal exercise capacity and require no limitation of physical activity 1
• Exercise is often self-limited in patients with large left-to-right shunts due to decreased cardiopulmonary function 1

Critical Pitfalls to Avoid

• Do not assume patients repaired in childhood are free of complications—right ventricular dysfunction and reduced exercise capacity persist even years after successful repair. 4
• Do not underestimate the risk of late-onset complete heart block, which can occur up to 15 years after repair, particularly in AVSD patients. 1
• Do not assume closure devices are MRI contraindications—they are MRI-conditional and imaging can be safely performed. 6
• Do not delay intervention for VSD with aortic valve involvement—irreversible aortic cusp damage can occur from long-standing shunt flow exposure. 8
• Maintain high clinical suspicion for device erosion in patients with new chest pain or syncope after percutaneous ASD closure—this requires urgent evaluation. 2, 6, 3