A neonate presents with shock-like symptoms, severe metabolic acidosis, and absent femoral pulses on day 2 of life as the ductus arteriosus starts to close. Chest X-ray shows cardiomegaly and pulmonary edema. Echocardiography reveals a hypoplastic left ventricle, severely hypoplastic aortic root, and critical coarctation. Which of the following is the most appropriate initial medical management?
- A Digoxin and diuretics
- B Prostaglandin E1 infusion to reopen the ductus arteriosus ✓
- C Immediate surgical repair via Norwood procedure
- D Inotropic support with dopamine only
Explanation
This presentation is consistent with hypoplastic left heart syndrome (HLHS), a ductal-dependent lesion where systemic perfusion depends entirely on right-to-left flow through the patent ductus arteriosus. Initial management is Prostaglandin E1 infusion to re-establish ductal patency and restore systemic blood flow, stabilizing the neonate before definitive surgical palliation. The surgical palliation is the staged Norwood procedure (Stage I), but this cannot be attempted in an unstable, acidotic neonate. Digoxin and diuretics alone are insufficient to restore systemic perfusion. Dopamine inotropes may be added but PGE1 is the primary intervention.
Reference: Ghai Essential Pediatrics, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.