Coarctation of the Aorta

Presentation

• Pulse discrepancy (bounding brachial pulses, weak/absent femoral pulses)
• Differential cyanosis (if PDA is open right-to-left)
• Upper/Lower extremity blood pressure gradient (> 20 mmHg)
• Heart failure/shock in critical closure of ductus arteriosus

Workup

• Four-extremity blood pressures
• Pre and post-ductal saturations
• Echocardiogram (diagnostic, visualizes the arch and assesses LV function)
• Assess for systemic hypoperfusion (lactate, creatinine, liver enzymes)

Management

• Initiate Prostaglandin E1 (PGE1) infusion immediately for severe/ductal-dependent coarctation
• Inotropic support (dopamine/milrinone) if LV dysfunction or shock present
• Consult cardiology and cardiothoracic surgery for urgent surgical repair (resection and end-to-end anastomosis)
• Avoid over-oxygenation to prevent premature ductal closure before PGE1 takes effect

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

• Coarctation of Aorta Algorithm: [START] Weak femoral pulses + differential cyanosis -> [ACTION] Initiate PGE1 -> [EVALUATE] Echo confirms CoA -> [GOAL] Surgical repair

References

1. Feltes TF, Bacha E, Beekman RH 3rd, et al. Indications for cardiac catheterization and intervention in pediatric cardiac disease. Circulation.