What is the management of left ventricular fistulas?

Management of Left Ventricular Fistulas

Left ventricular fistulas should be surgically closed if they are moderate to large in size, cause symptoms, or are associated with myocardial ischemia, arrhythmias, ventricular dysfunction, or endarteritis. 1

Diagnostic Evaluation

• Transthoracic echocardiography is the first-line imaging modality to identify and characterize LV fistulas
• Transcatheter delineation of the fistula course and access to distal drainage should be performed in all patients with audible continuous murmur and suspected LV fistula 1
• Cardiac MRI or CT angiography may provide additional anatomical details about the fistula course and drainage site

Management Algorithm

Asymptomatic Small LV Fistulas

• Clinical follow-up with echocardiography every 3-5 years to monitor for:
  • Development of symptoms
  • Arrhythmias
  • Progression in size
  • Chamber enlargement that might alter management 1
• No intervention is required if the patient remains asymptomatic and the fistula remains small

Indications for Intervention

Intervention is indicated for:

1. Moderate to large LV fistulas, even if asymptomatic 1
2. Small to moderate fistulas with:
   • Documented myocardial ischemia
   • Arrhythmias
   • Ventricular dysfunction or enlargement
   • Evidence of endarteritis 1
3. Symptoms related to the fistula (chest pain, dyspnea, heart failure)

Intervention Options

Surgical Approach

• Surgical closure is recommended when:
  • The fistula is well-defined with clear surgical access 1
  • The patient requires concomitant cardiac surgery
  • The fistula has complex anatomy not amenable to transcatheter closure
  • There is associated aneurysm formation requiring repair
• Surgery should be performed by surgeons with training and expertise in congenital heart disease 1

Transcatheter Approach

• Transcatheter closure should be considered when:
  • The fistula anatomy is favorable for device closure
  • The patient is at high risk for surgical intervention
  • There are no associated cardiac lesions requiring surgery
• Should only be performed in centers with expertise in such procedures 1

Post-Intervention Management

• Regular follow-up with clinical evaluation and echocardiography is essential
• Patients may still have large, patulous epicardial conduits even after repair
• Intermediate and long-term follow-up is mandatory to monitor for:
  • Residual shunts
  • Recurrence of the fistula
  • Aneurysm formation
  • Thrombosis or embolization 1

Special Considerations

• In cases of LV fistulas with associated ascending aortic and root aneurysm, surgical strategy may need to be adapted for cardioplegia administration 2
• Anticoagulation therapy may be necessary for at least 6 months after surgical repair to prevent thrombosis, particularly with larger fistulas 3
• Patients with traumatic aorto-ventricular fistulas require prompt surgical intervention to prevent cardiac decompensation 4

Complications to Monitor

• Residual shunts
• Thrombosis of the involved coronary artery
• Myocardial infarction
• Recurrence of the fistula
• Aneurysm formation at the site of previous fistula

By following this management approach, patients with left ventricular fistulas can be appropriately treated to prevent serious complications including heart failure, myocardial ischemia, and infective endocarditis, thereby improving long-term outcomes.