What is the treatment for a fibroma in the right ventricle (RV)?

Treatment of Right Ventricular Fibroma

Surgical resection is the recommended treatment for right ventricular fibromas, even when extensive, as complete excision provides excellent early and late outcomes with low mortality and morbidity. 1

Diagnostic Evaluation

Before treatment planning, comprehensive evaluation is essential:

• Cardiac imaging:

  • Echocardiography (transthoracic and transesophageal) to determine size, location, and relationship to cardiac structures 2
  • Cardiac MRI is particularly useful for:
    • Tissue characterization (iso-signal on T1, low-signal on T2)
    • Assessment of enhancement patterns (homogeneous hyperenhancement on late gadolinium enhancement)
    • Precise anatomical delineation of the tumor 3
  • CMR can accurately determine RV dimensions and systolic function 2

• Functional assessment:

  • Evaluate for arrhythmias (ventricular tachycardia is common) 4
  • Assess for outflow tract obstruction
  • Determine impact on RV function

Treatment Algorithm

1. Surgical Management (Primary Treatment)

• Complete surgical resection is the treatment of choice for RV fibromas 1, 3

  • Even extensive tumors can be completely resected with excellent outcomes
  • Early intervention is preferred to prevent life-threatening arrhythmias and sudden death 3
  • Mortality risk is higher in children under 2 years of age 5

• Surgical planning considerations:

  • Complex operations may require:
    • Pericardial or synthetic patches
    • Valve repair/replacement if involved
    • Coronary artery grafting if coronary vessels are affected 5
  • For tumors extending into critical locations, subtotal excision can still provide excellent late survival 1

2. Management of Specific Complications

• For arrhythmias:

  • Pre-operative electrophysiological study may be warranted 2
  • Complete resection typically resolves ventricular arrhythmias 4
  • Antiarrhythmic medications may be needed temporarily

• For RV dysfunction:

  • Optimize preload (target CVP 8-12 mmHg)
  • Maintain cardiac index >2.0 L/min/m²
  • Consider inotropic support if needed 6

3. Alternative Approaches

• Heart transplantation has been suggested by some, but is rarely necessary as most fibromas can be successfully resected 1
• Staged tumor resection may be considered for very large tumors 4

Follow-Up Protocol

• Regular echocardiographic assessment to monitor RV function
• Holter monitoring to detect arrhythmias
• CMR imaging every 2-3 years to assess for recurrence 2
• Clinical follow-up to monitor for symptoms of heart failure or arrhythmias

Outcomes and Prognosis

• Complete resection results in excellent early and late outcomes 1
• No recurrence of tumor has been reported after complete resection 1
• Subtotal resection may be appropriate in some cases with excellent survival 1
• Resolution of arrhythmias is common after successful tumor removal 4

Important Considerations and Pitfalls

• Cardiac fibromas are pathologically benign but can have unfavorable prognosis due to risk of lethal arrhythmias if left untreated 3
• Distinguishing fibromas from other cardiac tumors preoperatively is crucial for appropriate treatment planning
• Delay in surgical intervention may lead to increased risk of sudden death 3
• Younger patients (especially <2 years) have higher surgical risk 5
• Tumors involving critical structures may require more complex surgical approaches

By following this approach, most patients with RV fibromas can achieve excellent outcomes with complete resolution of symptoms and low risk of recurrence.