What is the recommended management for a 1-year-old child with a large muscular Ventricular Septal Defect (VSD) and tubular Patent Ductus Arteriosus (PDA) with left to right shunt?

Management of a 1-Year-Old Child with Large Muscular VSD and Tubular PDA with Left-to-Right Shunt

Surgical repair is recommended for this 1-year-old child with a large muscular VSD and tubular PDA with left-to-right shunt, as this is the optimal age for intervention to prevent irreversible pulmonary vascular disease. 1

Assessment of Pulmonary Vascular Resistance

The first step in management is to determine the pulmonary vascular resistance (PVR) through cardiac catheterization:

• Measure PVR index (PVRI) to determine operability 1
• Assess for reversibility with acute vasoreactivity testing (AVT) 1

Decision Algorithm Based on PVRI:

1. If PVRI <6 Wood units·m² or PVR/SVR <0.3 at baseline:

   • Proceed with surgical repair 1

2. If PVRI ≥6 Wood units·m² or PVR/SVR ≥0.3:

   • Perform AVT to assess reversibility
   • If AVT shows reversibility (absolute PVRI <6 WU·m² and PVR/SVR <0.3), surgical repair is beneficial 1
   • If minimal response to AVT, implement PAH-targeted therapy for 4-6 months, then repeat catheterization 1

Surgical Approach

For this 1-year-old with both large muscular VSD and tubular PDA:

• Complete surgical repair of both defects in a single procedure is recommended

• For the muscular VSD:

  • Surgical closure through right ventriculotomy or transatrial approach
  • Consider device closure for difficult-to-access muscular VSDs 1

• For the tubular PDA:

  • Surgical ligation or division during the same procedure 2

Perioperative Management

• Optimize cardiac function with diuretics if heart failure symptoms are present
• Monitor for pulmonary hypertensive crises in the perioperative period 1
• Implement strategies to avoid hypoxia, which can trigger pulmonary hypertensive crises 1

Post-Operative Follow-Up

After successful repair:

• Regular follow-up during the first 2 years post-repair 1

• Assess for:

  • Residual shunts
  • Right ventricular size and function
  • Tricuspid regurgitation
  • Pulmonary artery pressure 1

• If repair is performed before age 2 years without complications:

  • Long-term prognosis is excellent 1
  • Risk of developing late pulmonary vascular disease is minimal 3

Timing Considerations

The timing of intervention is critical in this case:

• At 1 year of age, this child is at the optimal time for intervention 4
• Delaying repair beyond 1-2 years increases the risk of irreversible pulmonary vascular changes 1, 2
• Early repair (before 2 years) has been shown to prevent elevated PVR-to-SVR ratio in long-term follow-up 1

Potential Complications to Monitor

• Residual shunts
• Arrhythmias
• Right ventricular outflow tract obstruction
• Development of discrete subaortic stenosis (rare) 1
• Endocarditis risk (requires prophylaxis) 1

Prognosis

With timely surgical intervention at this age:

• Excellent survival rates
• Prevention of pulmonary vascular disease
• Normal quality of life can be expected 3

Caution

If significant pulmonary hypertension is already present, careful assessment is crucial before proceeding with complete closure, as some patients may benefit from a staged approach or fenestrated closure to allow decompression if pulmonary pressures remain elevated postoperatively 1, 2.