Ventricular Septal Defect (VSD)

Presentation

• Often asymptomatic at birth due to high pulmonary vascular resistance (PVR)
• As PVR drops (weeks 2-6), left-to-right shunt increases causing symptoms
• Harsh holosystolic/pansystolic murmur at the lower left sternal border
• Signs of heart failure: diaphoresis with feeds, tachypnea, poor weight gain

Workup

• Echocardiogram: confirms size, location, and hemodynamic significance of VSD
• Chest X-ray: cardiomegaly and increased pulmonary vascular markings in large defects
• ECG: Biventricular hypertrophy or LA/LV enlargement

Management

• Nutrition: Fortified high-calorie formula/breast milk (24-27 kcal/oz)
• Medical therapy: Diuretics (furosemide) for volume overload, ACE inhibitors (enalapril) for afterload reduction
• Monitoring: Regular weight checks and growth charting
• Surgical closure indicated if medically refractory heart failure or failure to thrive

Extended Pharmacology & Critical Care

• Alprostadil (PGE1) continuous infusion: Start at 0.05 to 0.1 mcg/kg/min; monitor strictly for apnea, hyperthermia, and extreme vasodilation.
• Milrinone loading dose 50 mcg/kg over 15 mins (only if stable blood pressure), followed by 0.33 to 0.75 mcg/kg/min infusion.
• Strict I&O monitoring; maintain urine output > 1-2 mL/kg/hr using indwelling catheter if necessary.
• Maintain hematocrit > 35-40% via PRBC administration to optimize oxygen delivery in cyanotic lesions.
• Nursing: Q1 hour blood pressure monitoring (arterial line preferred), monitor for feeding intolerance indicating gut hypoperfusion.

Standardized Discharge & Follow-Up Criteria

• Adequate weight gain explicitly > 15-30g/day over a continuous 3-5 day period.
• Maintenance of temperature in an open crib with standard clothing for 48 hours.
• Successful completion of the 90-120 minute Infant Car Seat Challenge.
• Caregiver successfully completed CPR training, rooming-in, and independent medication administration.
• Referrals placed for High-Risk Infant Follow-Up Clinic, Early Intervention (PT/OT), and appropriate sub-specialists.

Diagnostic & Management Algorithm

• VSD Stepwise Care: [START] Holosystolic murmur -> [ACTION] Echo staging -> [EVALUATE] Heart failure symptoms (tachypnea, poor feeding)? -> [YES] Diuretics + High calorie feeds -> [GOAL] Allow time for spontaneous closure if small/moderate

References

1. Penny DJ, Vick GW 3rd. Ventricular septal defect. Lancet.