What is the treatment for Ventricular Septal Defect (VSD) with Eisenmenger syndrome?

Treatment of VSD with Eisenmenger Syndrome

Bosentan is the first-line therapy for patients with Eisenmenger syndrome associated with VSD, with PDE-5 inhibitors as reasonable alternatives or combination therapy when monotherapy is insufficient. 1, 2

Diagnosis Confirmation

Before initiating treatment, accurate diagnosis is essential:

• Cardiac catheterization is mandatory to confirm Eisenmenger physiology and exclude other causes of right-to-left shunting 1
• Rule out alternative contributors to pulmonary hypertension:
  • Thromboembolic disease
  • RVOT obstruction
  • Abnormalities of subpulmonary chamber compliance
  • Vascular streaming 1

Pharmacological Management

First-Line Therapy

• Bosentan (endothelin receptor antagonist):
  • Starting dose: 62.5 mg twice daily for 4 weeks
  • Maintenance dose: 125 mg twice daily if well tolerated 2
  • Strongest evidence from randomized controlled trials (BREATHE-5) 1, 2
  • Benefits: Reduced pulmonary vascular resistance, decreased mean pulmonary arterial pressure, increased exercise capacity 2
  • Monitor: Liver function tests monthly (hepatotoxicity risk 5-14%), hemoglobin (anemia risk) 2

Alternative/Add-on Therapy

• PDE-5 inhibitors (sildenafil, tadalafil):
  • Reasonable alternative when bosentan is contraindicated 1, 2
  • Can be used in combination with bosentan when monotherapy is insufficient 1
  • Benefits: Improved functional capacity, enhanced hemodynamics, better ventilatory efficiency 2

Combination Therapy

• Consider adding PDE-5 inhibitor to bosentan if inadequate symptomatic improvement with monotherapy 1, 2
• Combination therapy has shown improvements in:
  • Systemic arterial oxygen saturation
  • 6-minute walk distance
  • Hemodynamic parameters 1, 2

"Treat and Repair" Strategy

For VSD patients with severe pulmonary hypertension who have not yet developed irreversible Eisenmenger physiology:

• Consider pulmonary vasodilator therapy (typically bosentan) before attempting surgical repair 3
• This approach may be viable if:
  • Pulmonary vascular resistance decreases significantly with therapy
  • Patient demonstrates improved hemodynamics 3
• Monitor pulmonary artery pressure and PVR with serial catheterizations to determine if repair becomes feasible 3
• Surgery should NOT be performed if PA systolic pressure remains >2/3 systemic or PVR >2/3 systemic despite therapy 1

Contraindications and Precautions

• Avoid calcium channel blockers in Eisenmenger syndrome 2
• Bosentan is teratogenic - effective contraception is essential 2
• Surgical closure of VSD is contraindicated in patients with:
  • PA systolic pressure >2/3 systemic
  • Pulmonary vascular resistance >2/3 systemic
  • Net right-to-left shunt that does not improve with pulmonary vasodilator therapy 1

Monitoring and Follow-up

• Every 3-6 months:
  • WHO functional class assessment
  • 6-minute walk test
  • Pulse oximetry
  • BNP levels 2
• Every 12 months:
  • Comprehensive echocardiography 2
  • Consider repeat cardiac catheterization if clinical deterioration occurs

Supportive Care

• Supplemental oxygen if systemic arterial oxygen saturation improves with administration
• Avoid circumstances that increase risk:
  • High altitude
  • Pregnancy
  • Extreme heat/humidity
  • Nephrotoxin exposure
  • Extreme exertion
  • Large shifts in intravascular volume 1

Prognosis and Complications

Patients with Eisenmenger syndrome are at risk for multiple complications:

• Stroke and brain abscess
• Hemoptysis and pulmonary hemorrhage
• Thrombosis and paradoxical embolism
• Heart failure and arrhythmias
• Sudden cardiac death 1, 4

The 25-year survival rate for patients with VSD and Eisenmenger syndrome is approximately 42%, significantly lower than those with small restrictive VSDs (96%) 1.