What is the management approach for an adult patient with a ventricular septal defect (VSD)?

Management of Ventricular Septal Defect in Adults

Adults with VSD and hemodynamically significant left-to-right shunt (Qp:Qs ≥1.5:1) with left ventricular volume overload should undergo VSD closure when pulmonary artery systolic pressure is less than 50% of systemic pressure and pulmonary vascular resistance is less than one-third systemic resistance. 1

Indications for VSD Closure

Class I Recommendations (Definitive Indications)

• Hemodynamically significant shunt: VSD closure is mandatory when Qp:Qs ≥1.5:1 with evidence of left ventricular volume overload, provided PA systolic pressure <50% systemic and pulmonary vascular resistance <1/3 systemic 1, 2

• Symptomatic heart failure: Closure is indicated for patients with heart failure symptoms attributable to left-to-right shunting without severe pulmonary vascular disease 2

• History of infective endocarditis: VSD closure is recommended for patients with prior IE caused by the VSD 1, 2

Class IIa Recommendations (Reasonable to Perform)

• Progressive aortic regurgitation: Surgical closure of perimembranous or supracristal VSD is reasonable when worsening AR develops from VSD-associated aortic valve prolapse, regardless of hemodynamic significance 1, 3, 2
  • This is particularly critical as 6% of patients develop progressive AR, and aortic cusp damage may become irreversible with prolonged exposure to shunt flow 4
  • Aortic regurgitation and aortic cusp prolapse were the most common indications for closure in adult series (56.6% and 49.3% respectively) 4

Class IIb Recommendations (May Be Considered)

• Borderline pulmonary hypertension: VSD closure may be considered when Qp:Qs ≥1.5:1 with PA systolic pressure 50% or more of systemic and/or pulmonary vascular resistance greater than one-third systemic 1

  • This represents a gray zone requiring careful hemodynamic assessment via cardiac catheterization 3

• Post-endocarditis: Surgical closure may be reasonable after IE caused by VSD if not otherwise contraindicated 1

Absolute Contraindications to VSD Closure (Class III: Harm)

VSD closure must not be performed in the following scenarios:

• Eisenmenger syndrome: PA systolic pressure >2/3 systemic, pulmonary vascular resistance >2/3 systemic, or net right-to-left shunt 1, 2
• Exercise-induced desaturation indicating severe pulmonary vascular disease 3, 2

These patients have irreversible pulmonary vascular disease and closure would increase left-to-right shunting with high mortality risk 1

Diagnostic Evaluation Algorithm

Initial Assessment

• Echocardiography is the primary diagnostic modality and must assess: 3
  • VSD location, number, and size
  • Presence and extent of aneurysmal tissue (particularly for perimembranous VSDs)
  • Aortic valve morphology and degree of prolapse
  • Severity of aortic regurgitation
  • Left ventricular volume overload and systolic/diastolic function
  • Pulmonary artery pressure estimation

When to Perform Cardiac Catheterization

• Mandatory when pulmonary hypertension is suspected to assess operability and determine if closure is feasible 3, 2
• When noninvasive data are inconclusive regarding hemodynamics 3
• Critical for distinguishing flow-mediated (reversible) from resistance-mediated (irreversible) pulmonary hypertension 2

Surgical Approach and Technical Considerations

Preferred Closure Method

• Surgical closure is the gold standard for perimembranous, supracristal, and inlet VSDs 5
• Patch closure is superior to suture closure: Residual VSDs occurred in 6 of 8 patients with suture closure versus significantly fewer with patch closure 6
• Right atrial approach with patch closure is typically used 2

Role of Catheter-Based Closure

• Muscular VSDs: Catheter closure with Amplatzer Muscular VSD Occluder is effective and appropriate 7, 5
• Perimembranous VSDs: Percutaneous closure with Amplatzer Membranous VSD Occluder is not recommended due to significant risk of complete heart block 5

Intraoperative Considerations

• Transesophageal echocardiography use is associated with:

  • Fewer residual VSDs 6
  • Less moderate or greater tricuspid valve regurgitation 6
  • Less aortic regurgitation 6

• Evaluate for associated muscular VSDs as they may only manifest after closure of the dominant defect 2

Special Scenarios

Closed (Aneurysmal) Perimembranous VSD

Surgical closure is recommended even in asymptomatic patients to prevent life-threatening complications including: 3

• Aortic valve prolapse with progressive AR
• Infective endocarditis (6-fold increased risk, 13.7% incidence)
• Aneurysm rupture
• Right ventricular outflow tract obstruction

Post-Myocardial Infarction VSD

• Immediate IABP insertion with early surgical repair (within 3-9 days) achieves 89% survival versus 92% first-year mortality without surgery 2
• Transcatheter closure is increasingly viable with 32% mortality in recent series, comparable to surgical mortality of 30-50% 8
• Brief delay for patient stabilization is appropriate, but untreated patients risk rapid deterioration 8

Medical Management

For Patients Not Candidates for Closure

• ACE inhibitors for chronic heart failure symptoms and aortic regurgitation 2
• Diuretics (furosemide) for volume management and pulmonary congestion 7, 2
• Nitrates for symptom relief in patients without hypotension 7, 2

Conservative Management

• Small restrictive VSDs (Qp:Qs <1.5:1) with normal pulmonary pressures should be managed conservatively with surveillance 7, 2
• These patients have 96% 25-year survival rate 7, 2

Long-Term Follow-Up Protocol

Frequency Based on Clinical Status

• Annual follow-up required for: 7, 2

  • Residual heart failure
  • Residual shunts
  • Pulmonary arterial hypertension
  • Aortic or tricuspid regurgitation
  • RV or LV outflow tract obstruction

• Every 3-5 years if: Complete closure with no residual shunt or complications 2

Surveillance Echocardiography Must Assess

• Development of aortic or tricuspid regurgitation 7, 2
• Degree of residual shunt 2
• Left ventricular function and volume overload 7
• Pulmonary artery pressure 7
• Development of double-chambered right ventricle 7, 2
• Development of discrete subaortic stenosis 7, 2

Critical Pitfalls to Avoid

• Misdiagnosis: Small VSDs may be mistaken for innocent murmurs; vigilant monitoring for complications such as aortic valve prolapse, double-chambered RV, or subaortic stenosis development is essential 2

• Delayed intervention for AR: Aortic cusp damage may become irreversible with prolonged shunt exposure; VSD with aortic valve deformity or AR should be treated aggressively before disease progression occurs 4

• Concomitant aortic valve repair: This was the only significant risk factor for late AR progression in multivariable analysis, suggesting that isolated VSD closure is preferable when the aortic valve is not severely damaged 4

• Failure to recognize spontaneous closure potential: This is particularly relevant for muscular VSDs 7

Surgical Outcomes

• Early mortality: 0% in contemporary series 6
• Late mortality: 5% with mean follow-up of 10.3 years 6
• Patient survival does not differ from expected survival in reference population (p=0.75) 6
• Morbidity: High-grade AV block requiring pacemaker occurs in <3% 6
• Late reoperation rate: 2.6% due to AR, infective endocarditis, or residual VSD 4