A neonate presents on day 5 of life with sudden cardiovascular collapse, absent femoral pulses, and severe metabolic acidosis. Echocardiography shows hypoplastic ascending aorta, mitral stenosis, and aortic valve stenosis. Which of the following interventions is CRITICAL to prevent immediate death in this condition?

• A Prostaglandin E1 infusion to maintain ductal patency for systemic perfusion ✓
• B Immediate surgical closure of PDA to reduce pulmonary overflow
• C Intravenous furosemide to reduce pulmonary edema from left-to-right ductal shunting
• D Intravenous milrinone to improve left ventricular systolic function

Explanation

Hypoplastic left heart syndrome (HLHS) is a ductal-dependent systemic circulation lesion. The hypoplastic left heart cannot support systemic output; systemic perfusion depends entirely on right-to-left flow through the ductus arteriosus. As the ductus closes (day 2–5 of life), systemic perfusion collapses. Prostaglandin E1 (alprostadil) infusion is life-saving to maintain ductal patency. Closing the PDA would be catastrophic. Furosemide and milrinone do not address the structural lesion. Definitive management is staged surgical palliation (Norwood procedure) or transplantation.

Reference: Ghai Essential Pediatrics, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.