What is the recommended treatment for a coronary artery fistula to the pulmonary artery?

Management of Coronary Artery Fistula to Pulmonary Artery

Transcatheter occlusion is the recommended first-line treatment for coronary artery fistulas to the pulmonary artery, particularly for symptomatic patients and moderate to large asymptomatic fistulas. 1, 2

Clinical Significance and Presentation

• Coronary artery fistulas (CAFs) represent the second most common coronary artery anomaly with an incidence of 0.1% to 0.2% of catheterized patients 2
• Most commonly arise from either the right coronary artery or left anterior descending artery 2
• Patients may present with:
  • Continuous murmur (highly suggestive of CAF) 2
  • Dyspnea or exertional symptoms 2, 3
  • Myocardial ischemia due to coronary steal phenomenon 4
  • Arrhythmias including paroxysmal atrial fibrillation 2
  • Heart failure symptoms in severe cases 2, 3

Diagnostic Evaluation

• Echocardiography is the initial imaging modality but may have limitations 2
• CT angiography or MRI is recommended for detailed anatomical evaluation 2
• Cardiac catheterization with coronary angiography is necessary for precise delineation of coronary anatomy and assessment of hemodynamics 2, 3

Treatment Indications

• Class I indications (strongly recommended):

  • Symptomatic coronary artery fistulae require transcatheter occlusion 1
  • Transcatheter occlusion is indicated for patients with significant cyanosis or documented history of systemic embolic events 1

• Class IIa indications (reasonable to perform):

  • Transcatheter occlusion is reasonable for moderate or large coronary artery fistulae without clinical symptoms 1, 2
  • Large CAFs should be closed regardless of symptomatology 2

• Class III indications (not recommended):

  • Transcatheter device occlusion is not indicated for clinically insignificant coronary arteriovenous fistulae (e.g., normal-sized cardiac chambers) 1

Treatment Options

1. Transcatheter Closure (Preferred First-Line Approach)

• Preferred for suitable anatomy due to less invasiveness 2
• Can be performed with coils or other occluding devices 2, 5
• Advantages include shorter hospital stay and avoidance of thoracotomy and cardiopulmonary bypass 4
• Potential complications include:
  • Device embolization requiring retrieval 5
  • Myocardial infarction 5
  • Fistula recanalization 5

2. Surgical Closure

• Indicated when:
  • Transcatheter closure is not feasible or has failed 2
  • Complex anatomy is present 6
  • Concomitant cardiac surgery is needed 6, 4
  • Large aneurysmal dilatation is present 7
• Should be performed by surgeons with training and expertise in congenital heart disease 2

Follow-up Recommendations

• Patients with small, asymptomatic CAFs should have clinical follow-up with echocardiography every 3 to 5 years 2
• Patients with treated CAFs require intermediate and long-term follow-up due to potential residual abnormalities 2
• Monitoring for recurrence, residual shunts, or development of coronary artery stenosis is essential 4

Special Considerations

• Careful assessment for potential coronary compression is necessary before intervention 1
• Risk of rupture increases with age and fistula size 2
• Even after successful closure, patients may have residual coronary abnormalities requiring ongoing surveillance 2, 4