What is the primary treatment for a patient with a Ventricular Septal Defect (VSD)?

Management of Ventricular Septal Defects

Surgical closure is the primary treatment for patients with ventricular septal defects (VSDs) who have evidence of left ventricular volume overload and hemodynamically significant shunts (Qp:Qs ≥1.5:1), provided pulmonary artery systolic pressure is less than 50% systemic and pulmonary vascular resistance is less than one third systemic. 1

Indications for VSD Closure

Class I Indications (Strong Recommendation)

• VSDs with evidence of left ventricular volume overload and hemodynamically significant shunts (Qp:Qs ≥1.5:1) 1
• VSDs with history of infective endocarditis 1
• VSDs with net left-to-right shunting at Qp:Qs greater than 1.5 with pulmonary artery pressure less than two-thirds of systemic pressure 1

Class IIa Indications (Reasonable)

• Perimembranous or supracristal VSDs with worsening aortic regurgitation 1
• VSDs with net left-to-right shunting at Qp:Qs greater than 1.5 in the presence of LV systolic or diastolic failure 1

Class IIb Indications (May Consider)

• VSDs with history of infective endocarditis if not otherwise contraindicated 1
• VSDs with net left-to-right shunt (Qp:Qs ≥1.5:1) when pulmonary artery systolic pressure is 50% or more of systemic and/or pulmonary vascular resistance is greater than one-third systemic 1

Contraindications (Class III: Harm)

• VSDs with severe pulmonary arterial hypertension with pulmonary artery systolic pressure greater than two-thirds systemic 1
• VSDs with pulmonary vascular resistance greater than two-thirds systemic 1
• VSDs with net right-to-left shunt (Eisenmenger syndrome) 1

Treatment Approach Based on VSD Type

Perimembranous VSDs (80% of cases)

• Primary treatment: Surgical closure with patch material (Dacron, polytetrafluoroethylene) 1, 2
• Transcatheter device closure is not recommended due to risk of complete heart block (1-5%) 3

Supracristal VSDs (13% of cases)

• Primary treatment: Surgical closure, especially when associated with aortic valve prolapse 2, 4

Muscular VSDs (4% of cases)

• Primary treatment options:
  • Percutaneous device closure (Amplatzer Muscular VSD Occluder) for older children and adults 5, 4
  • Hybrid approach for infants with large muscular VSDs, especially those located apically or anteriorly 3
  • Surgical closure for multiple or complex muscular VSDs 2

Inlet VSDs (3% of cases)

• Primary treatment: Surgical closure 2

Surgical Outcomes and Expectations

Modern surgical repair of isolated VSDs has excellent outcomes:

• Mortality rate: 0.5% 2
• Risk of complete heart block: <1% 2
• Need for reoperation: <3% 2
• Long-term asymptomatic rate: 99.5% 2

Follow-up Recommendations

• Patients with small residual VSDs and no other lesions: Follow-up every 3-5 years at an adult congenital heart disease (ACHD) center 1
• Patients with VSD closure and residual heart failure, shunts, pulmonary arterial hypertension, aortic regurgitation, or outflow tract obstruction: Annual follow-up at an ACHD center 1
• Patients with device closure: Follow-up every 1-2 years depending on VSD location 1

Pitfalls and Caveats

• Timing is critical: Delaying closure of large VSDs can lead to irreversible pulmonary vascular obstructive disease 5, 4
• Eisenmenger syndrome: Never close VSDs once Eisenmenger physiology develops as this can precipitate right heart failure and increase mortality 6
• Careful assessment: Patients with loud murmur of a known small VSD may develop double-chambered right ventricle or subaortic stenosis with little change in murmur 1
• Aortic valve assessment: Patients with small VSDs and aortic valve prolapse may develop progressive aortic regurgitation 1
• Pulmonary pressure evaluation: Accurate assessment of pulmonary artery pressure is essential before deciding on closure 1

Special Considerations

• Pregnancy: Contraindicated in patients with VSD and Eisenmenger syndrome due to excessive maternal and fetal mortality 1
• Physical activity: No restrictions for patients after VSD closure or with small VSDs without pulmonary hypertension; patients with pulmonary hypertension should limit themselves to low-intensity activities 1
• Infective endocarditis prophylaxis: Recommended only for high-risk patients 1

By following these evidence-based guidelines, the management of VSDs can achieve excellent outcomes with minimal morbidity and mortality while preventing the development of pulmonary vascular obstructive disease and cardiac dysfunction.