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

Management of Ventricular Septal Defects (VSD)

Surgical closure is the primary treatment for patients with VSDs who have left ventricular volume overload and hemodynamically significant shunts (Qp:Qs ≥1.5:1), provided that 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 (Strongly Recommended)

• 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 Qp:Qs of 2.0 or more and clinical evidence of LV volume overload 1

Class IIa Indications (Reasonable)

• VSDs with net left-to-right shunting (Qp:Qs >1.5:1) with pulmonary artery pressure less than two-thirds systemic and pulmonary vascular resistance less than two-thirds systemic 1
• VSDs with net left-to-right shunting (Qp:Qs >1.5:1) in the presence of LV systolic or diastolic failure 1
• Perimembranous or supracristal VSDs with worsening aortic regurgitation 1

Class IIb Indications (May Be Considered)

• 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

Class III (Harm) Contraindications

• VSDs with severe pulmonary arterial hypertension with PA systolic pressure greater than two-thirds systemic, pulmonary vascular resistance greater than two-thirds systemic, and/or net right-to-left shunt 1

Treatment Approach Based on VSD Type

Perimembranous VSDs (80% of cases)

• Surgical closure is the preferred treatment 2, 3
• Risk of complete heart block with percutaneous device closure (1-5%) makes surgery the gold standard 4

Supracristal VSDs (13% of cases)

• Surgical closure is recommended, especially when aortic valve prolapse is present 2, 3

Muscular VSDs (4% of cases)

• Multiple options available:
  • Percutaneous device closure (Amplatzer Muscular VSD Occluder is FDA-approved) 5, 3
  • Hybrid approach for large muscular VSDs in small infants 5, 4
  • Surgical closure for large defects 2

Inlet VSDs (3% of cases)

• Surgical closure is typically recommended 2

Surgical Considerations

• Surgery should be performed by surgeons with training and expertise in congenital heart disease 1
• Patch closure is typically performed, usually with synthetic material (e.g., Dacron, polytetrafluoroethylene) 1
• Careful intraoperative inspection with TEE is indicated to rule out associated VSDs 1
• Excellent outcomes can be achieved regardless of patient age when surgical intervention is required 6

Outcomes and Complications

• Mortality is extremely low (0.5% operative mortality) 2
• Risk of complete heart block requiring permanent pacemaker is minimal with surgical repair (<1%) 2
• Reoperation for residual VSD is rare 2
• Most patients (>99%) remain asymptomatic from a cardiac standpoint at follow-up 2

Follow-up Recommendations

• Adults with VSD with residual heart failure, shunts, pulmonary arterial hypertension, aortic regurgitation, or outflow tract obstruction should be seen at least annually at an adult congenital heart disease regional center 1
• Adults with small residual VSD and no other lesions should be seen every 3-5 years 1
• Adults with device closure should be followed every 1-2 years depending on VSD location and other factors 1

Special Considerations

• Timely intervention is crucial to prevent pulmonary vascular obstructive disease 5, 3
• Pregnancy is contraindicated in patients with VSD and severe pulmonary arterial hypertension (Eisenmenger syndrome) due to excessive maternal and fetal mortality 1
• Exercise restrictions are not required for patients after VSD closure or with small VSDs without pulmonary hypertension, significant arrhythmias, or LV dysfunction 1

The management of VSDs has evolved significantly, with excellent outcomes now achievable through surgical repair. While newer technologies like device closure continue to develop, surgical closure remains the gold standard for most VSD types, particularly perimembranous VSDs, due to its safety, efficacy, and low complication rates.