What is the pre and post operative management for an adult patient with an atrial septal defect (ASD) with left to right shunt, severe tricuspid regurgitation (TR), and atrial fibrillation?

Management of Adult Atrial Septal Defect with Left-to-Right Shunt, Severe TR, and Atrial Fibrillation

For adults with ASD, left-to-right shunt, severe TR, and atrial fibrillation, surgical closure with concomitant tricuspid valve repair and Maze procedure is the recommended approach to reduce mortality and improve quality of life. 1

Pre-operative Management

Diagnostic Evaluation

• Imaging assessment:

  • Echocardiography (TTE/TEE) to evaluate:
    • ASD size, location, and type (secundum, primum, sinus venosus, or coronary sinus)
    • Severity of TR and tricuspid valve anatomy
    • Right heart chamber enlargement
    • Pulmonary venous connections 1
  • CMR or CCT to evaluate pulmonary venous connections if not adequately visualized by echocardiography 1

• Hemodynamic assessment:

  • Measure shunt magnitude (Qp:Qs ratio)
  • Assess pulmonary artery pressure and pulmonary vascular resistance
  • Cardiac catheterization if pulmonary hypertension is suspected 2

• Rhythm assessment:

  • 12-lead ECG to document atrial fibrillation
  • Consider 24-hour Holter monitoring to assess burden of atrial fibrillation

Medical Management Before Surgery

• Rate control for atrial fibrillation:

  • Beta-blockers or calcium channel blockers to control ventricular rate
  • Consider digoxin if heart failure symptoms are present

• Anticoagulation:

  • Anticoagulation therapy for atrial fibrillation to prevent thromboembolism
  • Continue until surgery with appropriate perioperative bridging 1

• Heart failure management if present:

  • Diuretics for volume overload
  • ACE inhibitors/ARBs if left ventricular dysfunction is present

Surgical Approach

Indications for Surgery

• ASD with right atrial and RV enlargement (Class I) 1
• Presence of significant TR requiring repair (Class IIa) 1
• Chronic atrial fibrillation requiring intervention (Class IIb) 1

Recommended Procedure

1. ASD closure:

   • Direct suture closure or patch closure depending on defect size
   • Surgical approach required for primum, sinus venosus, or coronary sinus defects 1

2. Tricuspid valve repair:

   • Indicated for severe TR 1
   • Techniques include annuloplasty ring placement or Kay's procedure
   • Repair is preferred over replacement when feasible 1

3. Concomitant Maze procedure:

   • Recommended for patients with chronic atrial fibrillation 1
   • Reduces recurrence of atrial arrhythmias post-operatively 1

Post-operative Management

Immediate Post-operative Care

• Monitor for complications:

  • Early postoperative symptoms of fever, fatigue, chest pain may indicate postpericardiotomy syndrome with tamponade 1
  • Immediate echocardiography if these symptoms develop

• Rhythm management:

  • Monitor for recurrence of atrial fibrillation
  • Continue antiarrhythmic medications as needed
  • Anticoagulation may be required if atrial fibrillation persists 1

• Hemodynamic monitoring:

  • Assess for residual shunting
  • Monitor right ventricular function
  • Evaluate tricuspid valve function 1

Long-term Follow-up

• Clinical assessment at 3 months, 6 months, and then annually 1

• Echocardiographic follow-up:

  • At 3-6 months post-procedure
  • Annually thereafter if abnormalities persist 1
  • Evaluate for:
    • Residual shunting
    • Tricuspid valve function
    • Right ventricular size and function
    • Pulmonary artery pressure 1

• Rhythm monitoring:

  • ECG at each follow-up visit
  • Consider Holter monitoring if symptoms suggest arrhythmia recurrence 1

Special Considerations

Residual TR After ASD Closure

Research shows that TR often improves after ASD closure alone, with 55% of patients experiencing at least 1 grade reduction in TR severity 3. However, patients with the following factors are at higher risk for residual TR and should be considered for concomitant tricuspid valve repair:

• Age ≥60 years
• Right atrial end-diastolic area ≥10cm²/m²
• Right ventricular systolic pressure ≥44 mmHg
• Reduced tricuspid annular plane systolic excursion ≤2.3 cm 3

Pulmonary Hypertension Considerations

• ASD closure is contraindicated in patients with severe irreversible pulmonary arterial hypertension (PAH) and right-to-left shunting 1
• For patients with moderate PAH (pulmonary pressure 50-66% of systemic), careful evaluation is required before proceeding with closure 1, 2

Outcomes

Patients with moderate/severe TR at 6-month follow-up have higher rates of adverse events, including cardiovascular death and heart failure hospitalization 3. Therefore, addressing TR at the time of ASD closure is critical for improving long-term outcomes.

Pitfalls and Caveats

• Do not close ASD in patients with severe irreversible PAH and right-to-left shunting, as this can lead to right heart failure and increased mortality 1
• Do not underestimate the importance of the Maze procedure in patients with chronic atrial fibrillation, as it significantly reduces arrhythmia recurrence 1
• Avoid overlooking tricuspid valve assessment before ASD closure, as severe TR may not improve with ASD closure alone in patients with significant valve remodeling 4
• Monitor for device erosion after transcatheter closure, which may present with chest pain or syncope and requires urgent evaluation 1